Academic Appointments

Administrative Appointments

  • Program Director-Master of Science in PA Studies program, Stanford Medicine (2017 - Present)
  • Associate Dean for PA Education, Stanford Medicine (2017 - Present)
  • Program Director, Adult Congenital Heart Program at Stanford, Lucile Packard Children's Hospital and Stanford Hospital and Clinics (2012 - Present)


All Publications

  • Substantial Cardiovascular Morbidity in Adults With Lower-Complexity Congenital Heart Disease CIRCULATION Saha, P., Potiny, P., Rigdon, J., Morello, M., Tcheandjieu, C., Romfh, A., Fernandes, S. M., McElhinney, D. B., Bernstein, D., Lui, G. K., Shaw, G. M., Ingelsson, E., Priest, J. R. 2019; 139 (16): 1889–99
  • Perceived Health Mediates Effects of Physical Activity on Quality of Life in Patients With a Fontan Circulation. The American journal of cardiology Holbein, C. E., Veldtman, G. R., Moons, P., Kovacs, A. H., Luyckx, K., Apers, S., Chidambarathanu, S., Soufi, A., Eriksen, K., Jackson, J. L., Enomoto, J., Fernandes, S. M., Johansson, B., Alday, L., Dellborg, M., Berghammer, M., Menahem, S., Caruana, M., Kutty, S., Mackie, A. S., Thomet, C., Budts, W., White, K., Sluman, M. A., Callus, E., Cook, S. C., Khairy, P., Cedars, A., APPROACH-IS consortium and the International Society for Adult Congenital Heart Disease (ISACHD) 2019


    Patients with a Fontan circulation are at risk of a sedentary lifestyle. Given the direct relationship between physical activity and health, promotion of physical activity has the potential to improve outcomes, including quality of life (QOL). This study aimed to describe self-reported physical activity levels in adult Fontan patients and examine associations between physical activity, perceived health status and QOL. The sample consisted of 177 Fontan patients (Mage = 27.5 ± 7.6 years, 52% male) who reported their physical activity, perceived health status, and QOL as part of the cross-sectional Assessment of Patterns of Patient-Reported Outcomes in Adults with Congenital Heart disease - International Study. Descriptive statistics and univariate analyses of variance with planned contrasts were computed to describe physical activity characteristics. Mediation analyses tested whether perceived health status variables mediated the association between physical activity and QOL. Forty-six percent of patients were sedentary while only 40% met international physical activity guidelines. Higher physical activity was associated with younger age, lower NYHA class, higher perceived general health, and greater QOL. Patients who commuted by walking and engaged in sports reported better perceived health and QOL. Mediation analyses revealed that perceived general health but not NYHA functional class mediated the association between physical activity and QOL (alphabeta = 0.22, 95% confidence interval = 0.04 to 0.49). In conclusion, Fontan patients likely benefit from regular physical activity, having both higher perceived general health and functional capacity; greater perceived health status may contribute to enhanced QOL. In conclusion, these data support the pivotal role of regular physical activity for Fontan patients.

    View details for PubMedID 31030969

  • Geographical variation and predictors of physical activity level in adults with congenital heart disease. International journal of cardiology. Heart & vasculature Larsson, L., Johansson, B., Sandberg, C., Apers, S., Kovacs, A. H., Luyckx, K., Thomet, C., Budts, W., Enomoto, J., Sluman, M. A., Wang, J., Jackson, J. L., Khairy, P., Cook, S. C., Alday, L., Eriksen, K., Dellborg, M., Berghammer, M., Rempel, G., Menahem, S., Caruana, M., Tomlin, M., Soufi, A., Fernandes, S. M., White, K., Callus, E., Kutty, S., Moons, P., APPROACH-IS Consortium, International Society for Adult Congenital Heart Disease (ISACHD) 2019; 22: 20–25


    Background: Physical activity is important to maintain and promote health. This is of particular interest in patients with congenital heart disease (CHD) where acquired heart disease should be prevented. The World Health Organization (WHO) recommends a minimum of 2.5 h/week of physical activity exceeding 3 metabolic equivalents (METS) to achieve positive health effects. It is unknown whether physical activity levels (PAL) in adult CHD patients differ by country of origin.Methods: 3896 adults with CHD recruited from 15 countries over 5 continents completed self-reported instruments, including the Health Behaviour Scale (HBS-CHD), within the APPROACH-IS project. For each patient, we calculated whether WHO recommendations were achieved or not. Associated factors were investigated using Generalized Linear Mixed Models.Results: On average, 31% reached the WHO recommendations but with a great variation between geographical areas (India: 10%-Norway: 53%). Predictors for physical activity level in line with the WHO recommendations, with country of residence as random effect, were male sex (OR 1.78, 95%CI 1.52-2.08), NYHA-class I (OR 3.10, 95%CI 1.71-5.62) and less complex disease (OR 1.46, 95%CI 1.16-1.83). In contrast, older age (OR 0.97, 95%CI 0.96-0.98), lower educational level (OR 0.41, 95%CI 0.26-0.64) and being unemployed (OR 0.57, 95%CI 0.42-0.77) were negatively associated with reaching WHO recommendations.Conclusions: A significant proportion of patients with CHD did not reach the WHO physical activity recommendations. There was a large variation in physical activity level by country of origin. Based on identified predictors, vulnerable patients may be identified and offered specific behavioral interventions.

    View details for PubMedID 30511012

  • Substantial Cardiovascular Morbidity in Adults with Lower-Complexity Congenital Heart Disease. Circulation Saha, P., Potiny, P., Rigdon, J., Morello, M., Tcheandjieu, C., Romfh, A., Fernandes, S. M., McElhinney, D. B., Bernstein, D., Lui, G. K., Shaw, G. M., Ingelsson, E., Priest, J. R. 2019


    BACKGROUND: Although lower-complexity cardiac malformations constitute the majority of adult congenital heart disease (ACHD), the long-term risks of adverse cardiovascular events and relationship with conventional risk factors in this population are poorly understood. We aimed to quantify the risk of adverse cardiovascular events associated with lower-complexity ACHD that is unmeasured by conventional risk factors.METHODS: A multi-tiered classification algorithm was used to select individuals with lower-complexity ACHD and individuals without ACHD for comparison amongst >500,000 British adults in the UK Biobank (UKB). ACHD diagnoses were sub-classified as "isolated aortic valve (AoV)" and "non-complex" defects. Time-to-event analyses were conducted for primary endpoints of fatal or non-fatal acute coronary syndrome (ACS), ischemic stroke, heart failure (HF), and atrial fibrillation, and a secondary combined endpoint for major adverse cardiovascular event (MACE). Maximum follow-up time for the study period was 22 years using retrospectively and prospectively collected data from the UKB.RESULTS: We identified 2,006 individuals with lower-complexity ACHD and 497,983 unexposed individuals in the UKB (median [IQR] age at enrollment 58 [51,63]). Of the ACHD-exposed group, 59% were male; 51% were current or former smokers; 30% were obese; 69%, 41%, and 7% were diagnosed or treated for hypertension, hyperlipidemia, and diabetes respectively. After adjustment for 12 measured cardiovascular risk factors, ACHD remained strongly associated with the primary endpoints, with hazard ratios (HR) ranging from 2.0 (95% confidence interval [CI] 1.5-2.8, p<0.001) for ACS to 13.0 (95% CI 9.4-18.1, p<0.001) for HF. ACHD-exposed individuals with ≤2 cardiovascular risk factors had a 29% age-adjusted incidence rate of MACE in contrast to 13% in non-ACHD individuals with ≥5 risk factors.CONCLUSIONS: Individuals with lower-complexity ACHD had higher burden of adverse cardiovascular events relative to the general population that was unaccounted for by conventional cardiovascular risk factors. These findings highlight the need for closer surveillance of patients with mild to moderate ACHD and further investigation into management and mechanisms of cardiovascular risk unique to this growing population of high-risk adults.

    View details for PubMedID 30813762

  • Education as important predictor for successful employment in adults with congenital heart disease worldwide. Congenital heart disease Sluman, M. A., Apers, S., Sluiter, J. K., Nieuwenhuijsen, K., Moons, P., Luyckx, K., Kovacs, A. H., Thomet, C., Budts, W., Enomoto, J., Yang, H., Jackson, J. L., Khairy, P., Cook, S. C., Subramanyan, R., Alday, L., Eriksen, K., Dellborg, M., Berghammer, M., Mattsson, E., Mackie, A. S., Menahem, S., Caruana, M., Gosney, K., Soufi, A., Fernandes, S. M., White, K. S., Callus, E., Kutty, S., Bouma, B. J., Mulder, B. J., APPROACH-IS consortium, t. I. 2019


    BACKGROUND: Conflicting results have been reported regarding employment status and work ability in adults with congenital heart disease (CHD). Since this is an important determinant for quality of life, we assessed this in a large international adult CHD cohort.METHODS: Data from 4028 adults with CHD (53% women) from 15 different countries were collected by a uniform survey in the cross-sectional APPROACH International Study. Predictors for employment and work limitations were studied using general linear mixed models.RESULTS: Median age was 32years (IQR 25-42) and 94% of patients had at least a high school degree. Overall employment rate was 69%, but varied substantially among countries. Higher education (OR 1.99-3.69) and having a partner (OR 1.72) were associated with more employment; female sex (OR 0.66, worse NYHA functional class (OR 0.67-0.13), and a history of congestive heart failure (OR 0.74) were associated with less employment. Limitations at work were reported in 34% and were associated with female sex (OR 1.36), increasing age (OR 1.03 per year), more severe CHD (OR 1.31-2.10), and a history of congestive heart failure (OR 1.57) or mental disorders (OR 2.26). Only a university degree was associated with fewer limitations at work (OR 0.62).CONCLUSIONS: There are genuine differences in the impact of CHD on employment status in different countries. Although the majority of adult CHD patients are employed, limitations at work are common. Education appears to be the main predictor for successful employment and should therefore be encouraged in patients with CHD.

    View details for PubMedID 30714326

  • Extended cardiac ambulatory rhythm monitoring in adults with congenital heart disease: Arrhythmia detection and impact of extended monitoring. Congenital heart disease Schultz, K. E., Lui, G. K., McElhinney, D. B., Long, J., Balasubramanian, V., Sakarovitch, C., Fernandes, S. M., Dubin, A. M., Rogers, I. S., Romfh, A. W., Motonaga, K. S., Viswanathan, M. N., Ceresnak, S. R. 2019


    BACKGROUND: Arrhythmias are a leading cause of death in adults with congenital heart disease (ACHD). While 24-48-hour monitors are often used to assess arrhythmia burden, extended continuous ambulatory rhythm monitors (ECAM) can record 2 weeks of data. The utility of this device and the arrhythmia burden identified beyond 48-hour monitoring have not been evaluated in the ACHD population. Additionally, the impact of ECAM has not been studied to determine management recommendations.OBJECTIVE: To address the preliminary question, we hypothesized that clinically significant arrhythmias would be detected on ECAM beyond 48hours and this would lead to clinical management changes.METHODS: A single center retrospective cohort study of ACHD patients undergoing ECAM from June 2013 to May 2016 was performed. The number and type of arrhythmias detected within and beyond the first 48hours of monitoring were compared using Kaplan-Meier curves and Cox proportional hazard models.RESULTS: Three hundred fourteen patients had monitors performed [median age 31 (IQR 25-41) years, 61% female). Significant arrhythmias were identified in 156 patients (50%), of which 46% were noted within 48 hours. A management change based on an arrhythmia was made in 49 patients (16%).CONCLUSIONS: ECAM detects more clinically significant arrhythmias than standard 48-hour monitoring in ACHD patients. Management changes, including medication changes, further testing or imaging, and procedures, were made based on results of ECAM. Recommendations and guidelines have been made based on arrhythmias on 48-hour monitoring; the predictive ability and clinical consequence of arrhythmias found on ECAM are not yet known.

    View details for PubMedID 30604934

  • Religion and spirituality as predictors of patient-reported outcomes in adults with congenital heart disease around the globe INTERNATIONAL JOURNAL OF CARDIOLOGY Moons, P., Luyckx, K., Dezutter, J., Kovacs, A. H., Thomet, C., Budts, W., Enomoto, J., Sluman, M. A., Yang, H., Jackson, J. L., Khairy, P., Subramanyan, R., Alday, L., Eriksen, K., Dellborg, M., Berghammer, M., Johansson, B., Mackie, A. S., Menahem, S., Caruana, M., Veldtman, G., Soufi, A., Fernandes, S. M., White, K., Callus, E., Kutty, S., Apers, S., APPROACH-IS Consortium, ISACHD 2019; 274: 93–99


    Religion and spirituality can be resources for internal strength and resilience, and may assist with managing life's challenges. Prior studies have been undertaken primarily in countries with high proportions of religious/spiritual people. We investigated (i) whether being religious/spiritual is an independent predictor of patient-reported outcomes (PROs) in a large international sample of adults with congenital heart disease, (ii) whether the individual level of importance of religion/spirituality is an independent predictor for PROs, and (iii) if these relationships are moderated by the degree to which the respective countries are religious or secular.APPROACH-IS was a cross-sectional study, in which 4028 patients from 15 countries were enrolled. Patients completed questionnaires to measure perceived health status; psychological functioning; health behaviors; and quality of life. Religion/spirituality was measured using three questions: Do you consider yourself religious or spiritual?; How important is religion, spirituality, or faith in your life?; and If religious, to what religion do you belong?. The country level of religiosity/secularity was appraised using data from the Gallup Poll 2005-2009. General linear mixed models, adjusting for patient characteristics and country differences were applied. Overall, 49.2% of patients considered themselves to be religious/spiritual. Being religious/spiritual and considering religion/spirituality as important in one's life was positively associated with quality of life, satisfaction with life and health behaviors. However, among patients living in more secular countries, religion/spirituality was negatively associated with physical and mental health.Religiosity/spirituality is an independent predictor for some PROs, but has differential impact across countries.

    View details for PubMedID 30077534

  • Physical Activity-Related Drivers of Perceived Health Status in Adults With Congenital Heart Disease AMERICAN JOURNAL OF CARDIOLOGY Ko, J., White, K. S., Kovacs, A. H., Tecson, K. M., Apers, S., Luyckx, K., Thomet, C., Budts, W., Enomoto, J., Sluman, M. A., Wang, J., Jackson, J. L., Khairy, P., Cook, S. C., Subramanyan, R., Alday, L., Eriksen, K., Dellborg, M., Berghammer, M., Johansson, B., Mackie, A. S., Menahem, S., Caruana, M., Veldtman, G., Soufi, A., Fernandes, S. M., Callus, E., Kutty, S., Gandhi, A., Moons, P., Cedars, A. M., APPROACH-IS Consortium, ISACHD 2018; 122 (8): 1437–42


    Data on the differential impact of physical activity on perceived health status (PHS) in a large adult congenital heart disease (ACHD) patient population are lacking. We conducted a cross-sectional assessment of 4,028 ACHD patients recruited from 24 ACHD-specialized centers in 15 countries across 5 continents to examine the association between physical activity and PHS in a large international cohort of ACHD patients. A linear analog scale of the EuroQol-5D 3 level version and the 12-item Short Form Health Survey-version 2 were used to assess self-reported health status and the Health-Behavior Scale-Congenital Heart Disease was used as a subjective measurement of physical activity type, participation, and level. Correlation analyses and Wilcoxon Rank Sum tests examined bivariate relations between sample characteristics and PHS scores. Then, multivariable models were constructed to understand the impact of physical activity on PHS. Only 30% of our sample achieved recommended physical activity levels. Physically active patients reported better PHS than sedentary patients; however, the amount of physical activity was not associated with PHS. Further statistical analyses demonstrated that specifically sport participation regardless of physical activity level was a predictor of PHS. In conclusion, the majority of ACHD patients across the world are physically inactive. Sport participation appears to be the primary physical activity-related driver of PHS. By promoting sport-related exercise ACHD specialists thus may improve PHS in ACHD patients.

    View details for PubMedID 30139525

  • A multinational observational investigation of illness perceptions and quality of life among patients with a Fontan circulation CONGENITAL HEART DISEASE Holbein, C. E., Fogleman, N. D., Hommel, K., Apers, S., Rassart, J., Moons, P., Luyckx, K., Sluman, M. A., Enomoto, J., Johansson, B., Yang, H., Dellborg, M., Subramanyan, R., Jackson, J. L., Budts, W., Kovacs, A. H., Morrison, S., Tomlin, M., Gosney, K., Soufi, A., Eriksen, K., Thomet, C., Berghammer, M., Alday, L., Callus, E., Fernandes, S. M., Caruana, M., Menahem, S., Cook, S. C., Rempel, G. R., White, K., Khairy, P., Kutty, S., Veldtman, G., APPROACH-IS Consortium, Int Soc Adult Congenital Heart Dis 2018; 13 (3): 392–400


    First, to compare QOL and illness perceptions between patients with a Fontan circulation and patients with anatomically simple defects (ie, atrial septal defects [ASD] or ventricular septal defects [VSD]). Second, to explore illness perceptions as a mediator of the association between congenital heart disease (CHD) diagnosis and QOL.Cross-sectional observational study.Twenty-four cardiology centers from 15 countries across five continents.Four hundred thirty-five adult patients with congenital heart disease (177 Fontan and 258 ASD/VSD) ages 18-83 years.QOL and illness perceptions were assessed by the Satisfaction With Life Scale and the Brief Illness Perceptions Questionnaire, respectively.Patients with a Fontan circulation reported lower QOL (Wald Z = -3.59, p = <.001) and more negative perceptions of their CHD (Wald Z = -7.66, p < .001) compared with patients with ASD/VSD. After controlling for demographics, anxiety, depressive symptoms, and New York Heart Association functional class, path analyses revealed a significant mediation model, αβ = 0.15, p = .002, 95% CI = 0.06-0.25, such that CHD diagnosis was indirectly related to QOL through illness perceptions.The Fontan sample's more negative perceptions of CHD were likely a reflection of life with a more complex defect. Illness perceptions appear to account for unique differences in QOL between groups of varying CHD complexity. Psychosocial screening and interventions may be important treatment components for patients with CHD, particularly those with Fontan circulations.

    View details for PubMedID 29457362

    View details for PubMedCentralID PMC5993574

  • Patient-reported outcomes in adults with congenital heart disease: Inter-country variation, standard of living and healthcare system factors INTERNATIONAL JOURNAL OF CARDIOLOGY Moons, P., Kovacs, A. H., Luyckx, K., Thomet, C., Budts, W., Enomoto, J., Sluman, M. A., Yang, H., Jackson, J. L., Khairy, P., Cook, S. C., Subramanyan, R., Alday, L., Eriksen, K., Dellborg, M., Berghammer, M., Johansson, B., Mackie, A. S., Menahem, S., Caruana, M., Veldtman, G., Soufi, A., Fernandes, S. M., White, K., Callus, E., Kutty, S., Van Bulck, L., Apers, S., APPROACH-IS Consortium Int Soc, Adult Congenital Heart Dis 2018; 251: 34–41
  • Regional variation in quality of life in patients with a Fontan circulation: A multinational perspective AMERICAN HEART JOURNAL Fogleman, N. D., Apers, S., Moons, P., Morrison, S., Wittekind, S. G., Tomlin, M., Gosney, K., Sluman, M. A., Johansson, B., Enomoto, J., Dellborg, M., Lu, C., Subramanyan, R., Luyckx, K., Budts, W., Jackson, J., Kovacs, A., Soufi, A., Eriksen, K., Thomet, C., Berghammer, M., Callus, E., Fernandes, S. M., Caruana, M., Cook, S. C., Mackie, A. S., White, K. S., Khairy, P., Kutty, S., Veldtman, G., APPROACH-IS Consortium 2017; 193: 55–62


    Impaired quality of life (QOL) is associated with congenital heart disease (CHD) and country of residence; however, few studies have compared QOL in patients with differing complexities of CHD across regional populations. The current study examined regional variation in QOL outcomes in a large multinational sample of patients with a Fontan relative to patients with atrial septal defects (ASDs) and ventricular septal defects (VSDs).From the Assessment of Patterns of Patient-Reported Outcomes in Adults with Congenital Heart disease-International Study (APPROACH-IS), 405 patients (163 Fontan and 242 ASD/VSD) across Asia, Europe, and North America provided consent for access to their medical records and completed a survey evaluating QOL (0 to 100 linear analog scale). Primary CHD diagnosis, disease complexity, surgical history, and documented history of mood and anxiety disorders were recorded. Differences in QOL, medical complications, and mood and anxiety disorders between Fontan and ASD/VSD patients, and across geographic regions, were examined using analysis of covariance. Hierarchical regression analyses were conducted to identify variables associated with the QOL ratings.Patients with a Fontan reported significantly lower QOL, and greater medical complications and mood and anxiety disorders relative to patients with ASD/VSD. Inpatient cardiac admissions, mood disorders, and anxiety disorders were associated with lower QOL among patients with a Fontan, and mood disorders were associated with lower QOL among patients with ASD/VSD. Regional differences for QOL were not observed in patients with a Fontan; however, significant differences were identified in patients with ASD/VSD.Regional variation of QOL is commonplace in adults with CHD; however, it appears affected by greater disease burden. Among patients with a Fontan, regional variation of QOL is lost. Specific attempts to screen for QOL and mood and anxiety disorders among CHD patients may improve the care of patients with the greatest disease burden.

    View details for DOI 10.1016/j.ahj.2017.07.019

    View details for Web of Science ID 000415058800007

    View details for PubMedID 29129255

  • Illness perceptions in adult congenital heart disease: A multi-center international study INTERNATIONAL JOURNAL OF CARDIOLOGY Rassart, J., Apers, S., Kovacs, A. H., Moons, P., Thomet, C., Budts, W., Enomoto, J., Sluman, M. A., Wang, J., Jackson, J. L., Khairy, P., Cook, S. C., Subramanyan, R., Alday, L., Eriksen, K., Dellborg, M., Berghammer, M., Johansson, B., Rempel, G. R., Menahem, S., Caruana, M., Veldtman, G., Soufi, A., Fernandes, S. M., White, K. S., Callus, E., Kutty, S., Luyckx, K., APPROACH-IS Consortium, ISACHD 2017; 244: 130–38


    Illness perceptions are cognitive frameworks that patients construct to make sense of their illness. Although the importance of these perceptions has been demonstrated in other chronic illness populations, few studies have focused on the illness perceptions of adults with congenital heart disease (CHD). This study examined (1) inter-country variation in illness perceptions, (2) associations between patient characteristics and illness perceptions, and (3) associations between illness perceptions and patient-reported outcomes.Our sample, taken from APPROACH-IS, consisted of 3258 adults with CHD from 15 different countries. Patients completed questionnaires on illness perceptions and patient-reported outcomes (i.e., quality of life, perceived health status, and symptoms of depression and anxiety). Patient characteristics included sex, age, marital status, educational level, employment status, CHD complexity, functional class, and ethnicity. Linear mixed models were applied.The inter-country variation in illness perceptions was generally small, yet patients from different countries differed in the extent to which they perceived their illness as chronic and worried about their illness. Patient characteristics that were linked to illness perceptions were sex, age, employment status, CHD complexity, functional class, and ethnicity. Higher scores on consequences, identity, and emotional representation, as well as lower scores on illness coherence and personal and treatment control, were associated with poorer patient-reported outcomes.This study emphasizes that, in order to gain a deeper understanding of patients' functioning, health-care providers should focus not only on objective indicators of illness severity such as the complexity of the heart defect, but also on subjective illness experiences.

    View details for PubMedID 28669511

  • Increasing Prevalence of Atrial Fibrillation and Permanent Atrial Arrhythmias in Congenital Heart Disease JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY Labombarda, F., Hamilton, R., Shohoudi, A., Aboulhosn, J., Broberg, C. S., Chaix, M. A., Cohen, S., Cook, S., Dore, A., Fernandes, S. M., Fournier, A., Kay, J., Macle, L., Mondesert, B., Mongeon, F., Opotowsky, A. R., Proietti, A., Rivard, L., Ting, J., Thibault, B., Zaidi, A., Khairy, P., AARCC 2017; 70 (7): 857–65


    Atrial arrhythmias are the most common complication encountered in the growing and aging population with congenital heart disease.This study sought to assess the types and patterns of atrial arrhythmias, associated factors, and age-related trends.A multicenter cohort study enrolled 482 patients with congenital heart disease and atrial arrhythmias, age 32.0 ± 18.0 years, 45.2% female, from 12 North American centers. Qualifying arrhythmias were classified by a blinded adjudicating committee.The most common presenting arrhythmia was intra-atrial re-entrant tachycardia (IART) (61.6%), followed by atrial fibrillation (28.8%), and focal atrial tachycardia (9.5%). The proportion of arrhythmias due to IART increased with congenital heart disease complexity from 47.2% to 62.1% to 67.0% in patients with simple, moderate, and complex defects, respectively (p = 0.0013). Atrial fibrillation increased with age to surpass IART as the most common arrhythmia in those ≥50 years of age (51.2% vs. 44.2%; p < 0.0001). Older age (odds ratio [OR]: 1.024 per year; 95% confidence interval [CI]: 1.010 to 1.039; p = 0.001) and hypertension (OR: 2.00; 95% CI: 1.08 to 3.71; p = 0.029) were independently associated with atrial fibrillation. During a mean follow-up of 11.3 ± 9.4 years, the predominant arrhythmia pattern was paroxysmal in 62.3%, persistent in 28.2%, and permanent in 9.5%. Permanent atrial arrhythmias increased with age from 3.1% to 22.6% in patients <20 years to ≥50 years, respectively (p < 0.0001).IART is the most common presenting atrial arrhythmia in patients with congenital heart disease, with a predominantly paroxysmal pattern. However, atrial fibrillation increases in prevalence and atrial arrhythmias progressively become permanent as the population ages.

    View details for PubMedID 28797355

  • Balancing the Training of Future Cardiologists With the Provision of Team-Based Care. JAMA cardiology Fernandes, S. M., Larsen, R. L., Chan, G. K. 2017

    View details for DOI 10.1001/jamacardio.2017.0555

    View details for PubMedID 28467549

  • Contraceptive Practices of Women With Complex Congenital Heart Disease. American journal of cardiology Miner, P. D., Canobbio, M. M., Pearson, D. D., Schlater, M., Balon, Y., Junge, K. J., Bhatt, A., Barber, D., Nickolaus, M. J., Kovacs, A. H., Moons, P., Shaw, K., Fernandes, S. M. 2017; 119 (6): 911-915


    Understanding the contraceptive practices of women with complex congenital heart disease (CHD) and providing them individualized contraception counseling may prevent adverse events and unplanned high-risk pregnancies. Given this, we sought to examine the contraceptive practices in women with CHD, describe adverse events associated with contraceptive use, and describe the provision of contraception counseling. Women >18 years were recruited from 2011 to 2014 from 9 adult CHD (ACHD) centers throughout North America. Subjects completed a 48-item questionnaire regarding contraceptive use and perceptions of contraception counseling, and a medical record review was performed. Of 505 subjects, median age was 33 (interquartile range 26 to 44) and 81% had CHD of moderate or great complexity. The majority (86%, 435 of 505) of the cohort had used contraception. The types included barrier methods (87%), oral contraception (OC) 84%, intrauterine device (18%), Depo-Provera (15%), vaginal ring (7%), patch (6%), hormonal implant (2%), Plan B (19%), and sterilization (16%). Overall OC use was not significantly different by CHD complexity. Women with CHD of great complexity were more likely to report a thrombotic event while taking OC than those with less complex CHD (9% vs 1%, p = 0.003). Contraception counseling by the ACHD team was noted by 43% of subjects. Unplanned pregnancy was reported by 25% with no statistical difference by CHD complexity. In conclusion, contraceptive practices of women with complex CHD are highly variable, and the prevalence of blood clots while taking OC is not insignificant while provision of contraception counseling by ACHD providers appears lacking.

    View details for DOI 10.1016/j.amjcard.2016.11.047

    View details for PubMedID 28087052

  • Adult congenital heart disease nurse coordination: Essential skills and role in optimizing team-based care a position statement from the International Society for Adult Congenital Heart Disease (ISACHD) INTERNATIONAL JOURNAL OF CARDIOLOGY Sillman, C., Morin, J., Thomet, C., Barber, D., Mizuno, Y., Yang, H., Malpas, T., Flocco, S. F., Finlay, C., Chen, C., Balon, Y., Fernandes, S. M. 2017; 229: 125-131


    Founded in 1992, the International Society for Adult Congenital Heart Disease (ISACHD) is the leading global organization of professionals dedicated to pursuing excellence in the care of adults with congenital heart disease (CHD) worldwide. Among ISACHD's objectives is to "promote a holistic team-based approach to the care of the adult with CHD that is comprehensive, patient-centered, and interdisciplinary" ( This emphasis on team-based care reflects the fact that adults with CHD constitute a heterogeneous population with a wide spectrum of disease complexity, frequent association with other organ involvement, and varied co-morbidities and psychosocial issues.Recognizing the vital role of the adult CHD (ACHD) nurse coordinator (ACHD-NC) in optimizing team-based care, ISACHD established a task force to elucidate and provide guidance on the roles and responsibilities of the ACHD-NC. Acknowledging that nursing roles can vary widely from region to region based on factors such as credentials, scopes of practice, regulations, and local culture and tradition, an international panel was assembled with experts from North America, Europe, East Asia, and Oceania. The writing committee was tasked with reviewing key aspects of the ACHD-NC's role in team-based ACHD care.The resulting ISACHD position statement addresses the ACHD-NC's role and skills required in organizing, coordinating, and facilitating the care of adults with CHD, holistic assessment of the ACHD patient, patient education and counseling, and support for self-care management and self-advocacy.

    View details for DOI 10.1016/j.ijcard.2016.10.051

    View details for Web of Science ID 000394695800076

    View details for PubMedID 28340978

  • Transition and transfer of childhood cancer survivors to adult care: A national survey of pediatric oncologists. Pediatric blood & cancer Kenney, L. B., Melvin, P., Fishman, L. N., O'Sullivan-Oliveira, J., Sawicki, G. S., Ziniel, S., Diller, L., Fernandes, S. M. 2017; 64 (2): 346-352


    Pediatric oncologists are responsible for ensuring that adolescent and young adult (AYA) childhood cancer survivors have the knowledge and skills necessary to manage their follow-up care in adult healthcare systems.To describe transition practices and barriers to transfer, we electronically surveyed U.S. Children's Oncology Group members: 507/1449 responded (35%) and 347/507 (68%) met eligibility criteria.Of 347 respondents, 50% are male, median years in practice 10 (range 5-22), 37% practice in freestanding children's hospitals. Almost all care for survivors up to age 21 years (96%), 42% care for survivors over age 25 years, and only 16% over age 30 years. While 66% of oncologists reported providing transition education to their patients, very few (8%) reported using standardized transition assessments. The most frequent barriers to transfer were perceived attachment to provider (91%), lack of adult providers with cancer survivor expertise (86%), patient's cognitive delay (81%), or unstable social situation (80%). Oncologists who continue to care for patients older than 25 years are more likely to perceive parents' attachment to provider (P = 0.037) and patients' social situation as barriers to transfer (P = 0.044).  Four themes emerged from a content analysis of 75 respondents to the open-ended question inviting comments on transition/transfer practices: desire for flexible transfer criteria; providers as barriers; provider lack of transition knowledge, skills, and resources; and desire for collaboration.Although most pediatric oncologists reported transferring AYA cancer survivors to adult care and providing some transition education, they endorse deficits in transition skills, emotional readiness, and institutional resources.

    View details for DOI 10.1002/pbc.26156

    View details for PubMedID 27463688

  • Risk Estimates for Atherosclerotic Cardiovascular Disease in Adults With Congenital Heart Disease AMERICAN JOURNAL OF CARDIOLOGY Lui, G. K., Rogers, I. S., Ding, V. Y., Hedlin, H. K., MacMillen, K., Maron, D. J., Sillman, C., Romfh, A., Dade, T. C., Haeffele, C., Grady, S. R., McElhinney, D. B., Murphy, D. J., Fernandes, S. M. 2017; 119 (1): 112-118


    The adult with congenital heart disease (CHD) is at risk of developing atherosclerotic cardiovascular disease (ASCVD). We performed a cross-sectional study to describe established ASCVD risk factors and estimate 10-year and lifetime risk of ASCVD in adults over age 18 with CHD of moderate or great complexity using 3 validated risk assessment tools-the Framingham Study Cardiovascular Disease Risk Assessment, the Reynolds Risk Score, and the ASCVD Risk Estimator. We obtained extensive clinical and survey data on 178 enrolled patients, with average age 37.1 ± 12.6 years, 51% men. At least 1 modifiable ASCVD risk factor was present in 70%; the 2 most common were overweight/obesity (53%) and systemic hypertension (24%). Laboratory data were available in 103 of the 178 patients. Abnormal levels of glycated hemoglobin, high-sensitivity C-reactive protein, and high-density lipoprotein were each found in around 30% of patients. The 10-year ASCVD predicted risk using all 3 tools was relatively low (i.e., at least 90% of patients <10% risk), yet the median estimated lifetime risk was 36%. In conclusion, ASCVD risk factors are prevalent in adults with CHD. The risk estimation tools suggest that this population is particularly vulnerable to ASCVD with aging and should undergo guideline-based screening and management of modifiable risk factors.

    View details for DOI 10.1016/j.amjcard.2016.09.023

    View details for PubMedID 28247847

  • Thromboprophylaxis for atrial arrhythmias in congenital heart disease: A multicenter study. International journal of cardiology Khairy, P., Aboulhosn, J., Broberg, C. S., Cohen, S., Cook, S., Dore, A., Fernandes, S. M., Fournier, A., Kay, J., Levesque, S., Macle, L., Marcotte, F., Mondésert, B., Mongeon, F., Opotowsky, A. R., Proietti, A., Rivard, L., Ting, J., Thibault, B., Zaidi, A., Hamilton, R. 2016; 223: 729-735


    There is a paucity of data to guide decisions regarding thromboprophylaxis for atrial arrhythmias in congenital heart disease.A retrospective multicenter cohort study enrolled patients with documented sustained atrial arrhythmias and congenital heart disease from 12 North American centers to quantify thromboembolic and bleeding rates associated with antiplatelet and anticoagulation therapy, and explore associated factors. A blinded committee adjudicated all qualifying arrhythmias and outcomes.A total of 482 patients, 45.2% female, age 32.0±18.0years, were followed for 11.3±9.4years since the qualifying arrhythmia. Antiplatelet therapy was administered to 37.8%, anticoagulation to 54.4%, and neither to 7.9%. Congenital heart disease complexity was simple, moderate, and severe in 18.5%, 34.4%, and 47.1%, respectively. Freedom from thromboembolic events was 84.7±2.7% at 15years, with no difference between anticoagulation versus antiplatelet therapy (P=0.97). Congenital heart disease complexity was independently associated with thromboembolic events, with rates of 0.00%, 0.93%, and 1.95%/year in those with simple, moderate, and severe forms (P<0.001). CHADS2 and CHA2DS2-VASc scores were not predictive of thromboembolic risk. Annualized bleeding rates with antiplatelet and anticoagulation therapy were 0.66% and 1.82% (P=0.039). In multivariable analyses, anticoagulation [hazard ratio (HR) 4.76, 95% CI (1.05-21.58), P=0.043] and HAS-BLED score [HR 3.15, 95% CI (1.02, 9.78), P=0.047] were independently associated with major bleeds.Current management of atrial arrhythmias in congenital heart disease is associated with a modest rate of thromboembolic events, which is predicted by disease complexity but not CHADS2/CHA2DS2-VASc scores. HAS-BLED score is applicable to the congenital population in predicting major bleeds.

    View details for DOI 10.1016/j.ijcard.2016.08.223

    View details for PubMedID 27573597

  • Transitions in Pediatric Gastroenterology: Results of a National Provider Survey. Journal of pediatric gastroenterology and nutrition Bensen, R., McKenzie, R. B., Fernandes, S. M., Fishman, L. N. 2016; 63 (5): 488-493


    Transition and transfer to adult-oriented health care is an important yet challenging task for adolescents and young adults with chronic medical conditions. Transition practices vary widely, but a paucity of data makes determination of best practices difficult. We describe North American pediatric gastroenterologists' preferences and current transition practice patterns and explore whether experience affects providers' perspectives.An on-line survey was distributed via email to members of the North American Society of Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN). Participation was voluntary and answers were anonymous. Quantitative and qualitative analysis was performed.Almost three quarters of the 175 respondents describe providing transition or self-care management education, but only 23% use structured readiness assessments. Most respondents (88%) report having age cut-offs above which they no longer accept new referrals, with the most common age being 18 years (57%). One third report the ability to provide age-appropriate care to patients over age 21 years. Only 6% indicate that their practice or institution should provide care for individuals over age 25 years. Many (63%) indicate that their practice or institution has a policy regarding age of transfer, but most (79%) are flexible. Provider preferences for triggers to transfer to adult care diverge widely between age, milestones and comorbidities. Overall, parent (81%) and patient (74%) attachment to pediatric healthcare providers are cited as the most common barriers to transition.Preferences and practices surrounding transition preparation and transfer to adult care vary widely, reflecting continued uncertainty regarding optimal transition strategies.

    View details for PubMedID 27027904

  • Provision of Transition Education and Referral Patterns from Pediatric Cardiology to Adult Cardiac Care. Pediatric cardiology Harbison, A. L., Grady, S., Chi, K., Fernandes, S. M. 2016; 37 (2): 232-238


    ACC/AHA guidelines recommend a structured preparation for and transfer to adult-oriented cardiac care for adult survivors of pediatric onset heart disease (POHD). Given this, we sought to describe the transition and transfer practices for a cohort of young adults with POHD and to determine factors associated with successful transfer to adult-oriented cardiac care. We performed a single-center, retrospective chart review on patients ≥18 years of age, with POHD likely to require lifelong cardiac care, who were seen in outpatient pediatric cardiology (PC) between 2008 and 2011. Successful transfer was defined as the subsequent attendance at adult cardiology (AC) within 2 years of PC visit. We identified 118 patients who met study criteria. Mean age 22.4 ± 2.0 years, 59 % male, 64 % white and 40 % Hispanic. Mean transition education topics noted was 3.3 ± 1.8 out of 20 and covered the underlying cardiac disease (89 %), follow-up and current medications (56 %) and exercise limitations (34 %). Recommendations for follow-up were AC (57 %) and PC (33 %). Of those told to transfer to AC, 79 % successfully transferred. Characteristics of successful transfer included: prior cardiac surgery (p = 0.008), cardiac medication use (p = 0.006) and frequency of follow-up ≤1 year (p = 0.037). One-quarter of all subjects did not follow-up within at least 2 years. Despite published guidelines, transition education appears lacking and the approach to transfer to adult cardiac care is not consistent. Given the increased risk of morbidity and mortality in this patient population, standardization of transition education and transfer processes appear warranted.

    View details for DOI 10.1007/s00246-015-1267-5

    View details for PubMedID 26385471

  • 2015 ACC/AHA/HRS Advanced Training Statement on Clinical Cardiac Electrophysiology (A Revision of the ACC/AHA 2006 Update of the Clinical Competence Statement on Invasive Electrophysiology Studies, Catheter Ablation, and Cardioversion) CIRCULATION-ARRHYTHMIA AND ELECTROPHYSIOLOGY Zipes, D. P., Calkins, H., Daubert, J. P., Ellenbogen, K. A., Field, M. E., Fisher, J. D., Fogel, R. I., Frankel, D. S., Gupta, A., Indik, J. H., Kusumoto, F. M., Lindsay, B. D., Marine, J. E., Mehta, L. S., Mendes, L. A., Miller, J. M., Munger, T. M., Sauer, W. H., Shen, W., Stevenson, W. G., Su, W. W., Tracy, C. M., Tsiperfal, A., Williams, E. S., Halperin, J. L., Arrighi, J. A., Awtry, E. H., Bates, E. R., Brush, J. E., Costa, S., Daniels, L., Desai, A., Drachman, D. E., Fernandes, S., Freeman, R., Ijioma, N., Khan, S. S., Kuvin, J. T., Marine, J. E., McPherson, J. A., Mendes, L. A., Sivaram, C. A., Spicer, R. L., Wang, A., Weitz, H. H. 2015; 8 (6): 1522-1551

    View details for DOI 10.1161/HAE.0000000000000014

    View details for Web of Science ID 000366604600034

    View details for PubMedID 26386016

  • Patient-centered medical home for patients with complex congenital heart disease. Current opinion in pediatrics Fernandes, S. M., Sanders, L. M. 2015; 27 (5): 581-586


    Originally conceived by pediatricians as a model for the care of children with special healthcare needs, the 'patient-centered medical home' (PCMH) has been identified by the Affordable Care Act as a model for all future outpatient care delivery. Although few studies have demonstrated its efficacy in improving care for children with congenital heart disease (CHD), access to a PCMH is likely to improve CHD-patient outcomes, including global function and quality of life, while reducing healthcare costs.To date, most patients with complex CHD have their care anchored in cardiology subspecialty-care clinics, which lack many of the attributes of a PCMH. Given that many of these patients have noncardiac morbidities, including neurocognitive impairment and multiorgan dysfunction, we believe such patients will benefit from a PCMH model of care based on primary care.The PCMH based on primary care for patients with complex CHD could result in improved clinical outcomes, improved patient satisfaction and quality of life as well as decreased healthcare costs. Policy and practice reform are required to increase CHD-patient access to a PCMH, based on primary-care settings.

    View details for DOI 10.1097/MOP.0000000000000258

    View details for PubMedID 26348668

  • Implementation of the American College of Cardiology/American Heart Association 2008 Guidelines for the Management of Adults With Congenital Heart Disease. American journal of cardiology Goossens, E., Fernandes, S. M., Landzberg, M. J., Moons, P. 2015; 116 (3): 452-457


    Although different guidelines on adult congenital heart disease (ACHD) care advocate for lifetime cardiac follow-up, a critical appraisal of the guideline implementation is lacking. We investigated the implementation of the American College of Cardiology/American Heart Association 2008 guidelines for ACHD follow-up by investigating the type of health care professional, care setting, and frequency of outpatient visits in young adults with CHD. Furthermore, correlates for care in line with the recommendations or untraceability were investigated. A cross-sectional observational study was conducted, including 306 patients with CHD who had a documented outpatient visit at pediatric cardiology before age 18 years. In all, 210 patients (68.6%) were in cardiac follow-up; 20 (6.5%) withdrew from follow-up and 76 (24.9%) were untraceable. Overall, 198 patients were followed up in tertiary care, 1/4 (n = 52) of which were seen at a formalized ACHD care program and 3/4 (n = 146) remained at pediatric cardiology. Of those followed in formalized ACHD and pediatric cardiology care, the recommended frequency was implemented in 94.2% and 89%, respectively (p = 0.412). No predictors for the implementation of the guidelines were identified. Risk factors for becoming untraceable were none or lower number of heart surgeries, health insurance issues, and nonwhite ethnicity. In conclusion, a significant number of adults continue to be cared for by pediatric cardiologists, indicating that transfer to adult-oriented care was not standard practice. Frequency of follow-up for most patients was in line with the ACC/AHA 2008 guidelines. A considerable proportion of young adults were untraceable in the system, which makes them vulnerable for discontinuation of care.

    View details for DOI 10.1016/j.amjcard.2015.04.041

    View details for PubMedID 26092269

  • Variation in Use of Pediatric Cardiology Subspecialty Care A Total Population Study in California, 1983 to 2011 JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY Chamberlain, L. J., Fernandes, S. M., Saynina, O., Grady, S., Sanders, L., Staves, K., Wise, P. H. 2015; 66 (1): 37-44


    American Academy of Pediatrics guidelines emphasize regionalized systems of care for pediatric chronic illness. There remains a paucity of information on the status of regionalized systems of care for pediatric congenital heart disease (CHD).This study evaluated variations in use of pediatric cardiology specialty care centers (PCSCC) for pediatric patients with CHD in California between 1983 and 2011.We performed a retrospective, total population analysis of pediatric CHD patients using the California Office of Statewide Health Planning and Development unmasked database. PCSCCs were identified by California's Title V program.There were 164,310 discharges meeting inclusion criterion. Discharges from PCSCCs grew from 58% to 88% between 1983 and 2011. Regionalized care was highest for surgical (96%) versus nonsurgical (71%) admissions. Admissions with a public payer increased from 42% (1983) to 61% (2011). Total bed days nearly doubled, and median length of stay increased from 2 to 3 days (nonspecialty care) and from 4 to 5 days (specialty care). There was a decrease in the pediatric CHD in-hospital death rate from 5.1 to 2.3 per 100,000 between 1983 and 2011, and a shift toward a larger percent of deaths occurring in the newborn period.California's inpatient regionalized specialty care of pediatric CHD has increased substantially since 1983, especially for surgical CHD discharges. The death rate has decreased, the number of bed days has increased, and a large proportion of these discharges now have public payers. Health care reform efforts must consider these shifts while protecting advances in regionalization of pediatric CHD care.

    View details for DOI 10.1016/j.jacc.2015.04.053

    View details for PubMedID 26139056

  • Trends in utilization of specialty care centers in california for adults with congenital heart disease. American journal of cardiology Fernandes, S. M., Chamberlain, L. J., Grady, S., Saynina, O., Opotowsky, A. R., Sanders, L., Wise, P. H. 2015; 115 (9): 1298-1304


    The American College of Cardiology and American Heart Association guidelines recommend that management of adult congenital heart disease (ACHD) be coordinated by specialty ACHD centers and that ACHD surgery for patients with moderate or complex congenital heart disease (CHD) be performed by surgeons with expertise and training in CHD. Given this, the aim of this study was to determine the proportion of ACHD surgery performed at specialty ACHD centers and to identify factors associated with ACHD surgery being performed outside of specialty centers. This retrospective population analysis used California's Office of Statewide Health Planning and Development's discharge database to analyze ACHD cardiac surgery (in patients 21 to 65 years of age) in California from 2000 to 2011. Designation as a "specialty ACHD center" was defined on the basis of a national ACHD directory. A total of 4,611 ACHD procedures were identified. The proportion of procedures in patients with moderate and complex CHD delivered at specialty centers increased from 46% to 71% from 2000 to 2011. In multivariate analysis among those discharges for ACHD surgery in patients with moderate or complex CHD, performance of surgery outside a specialty center was more likely to be associated with patients who were older, Hispanic, insured by health maintenance organizations, and living farther from a specialty center. In conclusion, although the proportion of ACHD surgery for moderate or complex CHD being performed at specialty ACHD centers has been increasing, 1 in 4 patients undergo surgery at nonspecialty centers. Increased awareness of ACHD care guidelines and of the patient characteristics associated with differential access to ACHD centers may help improve the delivery of appropriate care for all adults with CHD.

    View details for DOI 10.1016/j.amjcard.2015.02.013

    View details for PubMedID 25765587

  • Assessment of Patterns of Patient-Reported Outcomes in Adults with Congenital Heart disease - International Study (APPROACH-IS): Rationale, design, and methods INTERNATIONAL JOURNAL OF CARDIOLOGY Apers, S., Kovacs, A. H., Luyckx, K., Alday, L., Berghammer, M., Budts, W., Callus, E., Caruana, M., Chidambarathanu, S., Cook, S. C., Dellborg, M., Enomoto, J., Eriksen, K., Fernandes, S. M., Jackson, J. L., Johansson, B., Khairy, P., Kutty, S., Menahem, S., Rempel, G., Sluman, M. A., Soufi, A., Thomet, C., Veldtman, G., Wang, J., White, K., Moons, P. 2015; 179: 334-342


    Data on patient-reported outcomes (PROs) in adults with congenital heart disease (CHD) are inconsistent and vary across the world. Better understanding of PROs and their differences across cultural and geographic barriers can best be accomplished via international studies using uniform research methods. The APPROACH-IS consortium (Assessment of Patterns of Patient-Reported Outcomes in Adults with Congenital Heart disease - International Study) was created for this purpose and investigates PROs in adults with CHD worldwide. This paper outlines the project rationale, design, and methods.APPROACH-IS is a cross-sectional study. The goal is to recruit 3500-4000 adults with CHD from 15 countries in five major regions of the world (Asia, Australia, Europe, North and South America). Self-report questionnaires are administered to capture information on PRO domains: (i) perceived health status (12-item Short-form Health Survey & EuroQOL-5D); (ii) psychological functioning (Hospital Anxiety and Depression Scale); (iii) health behaviors (Health-Behavior Scale-Congenital Heart Disease); and (iv) quality of life (Linear Analog Scale & Satisfaction With Life Scale). Additionally, potential explanatory variables are assessed: (i) socio-demographic variables; (ii) medical history (chart review); (iii) sense of coherence (Orientation to Life Questionnaire); and (iv) illness perceptions (Brief Illness Perception Questionnaire). Descriptive analyses and multilevel models will examine differences in PROs and investigate potential explanatory variables.APPROACH-IS represents a global effort to increase research understanding and capacity in the field of CHD, and will have major implications for patient care. Results will generate valuable information for developing interventions to optimize patients' health and NCT02150603.

    View details for DOI 10.1016/j.ijcard.2014.11.084

    View details for Web of Science ID 000346089400093

    View details for PubMedID 25464481

  • Management of cardiovascular risk factors in adults with congenital heart disease. Journal of the American Heart Association Lui, G. K., Fernandes, S., McElhinney, D. B. 2014; 3 (6)

    View details for DOI 10.1161/JAHA.114.001076

    View details for PubMedID 25359401

  • TRANSITIONING CHILDHOOD CANCER SURVIVORS TO ADULT CARE: A SURVEY OF PEDIATRIC ONCOLOGISTS Kenney, L. B., Melvin, P., Fishman, L., O'Sullivan-Oliveira, J., Sawicki, G. S., Ziniel, S., Diller, L., Fernandes, S. M. WILEY-BLACKWELL. 2014: S198
  • Management of Cardiovascular Risk Factors in Adults With Congenital Heart Disease JOURNAL OF THE AMERICAN HEART ASSOCIATION Lui, G. K., Fernandes, S., McElhinney, D. B. 2014; 3 (6)
  • Provider awareness alone does not improve transition readiness skills in adolescent patients with inflammatory bowel disease. Journal of pediatric gastroenterology and nutrition Fishman, L. N., Ziniel, S. I., Adrichem, M. E., Fernandes, S. M., Arnold, J. 2014; 59 (2): 221-224


    Adolescent patients with chronic health conditions must gradually assume responsibility for their health. Self-management skills are needed for a successful transfer from adolescent to adult health care, but the development of these skills could be resource intensive. Pediatric providers are already instrumental in teaching patients about their health and may improve these skills. The aim of the study was to evaluate whether informal education of pediatric providers regarding transition improves inflammatory bowel disease (IBD) patient self-management skills.Consecutive patients with IBD older than 10 years who presented to the outpatient setting were administered a survey regarding self-management behaviors in 2008 and 2011. During this time, several conferences on transition were presented to the providers.In 2008, 294 patients completed the survey (82%) compared with 121 patients (89%) in 2011. The patient groups were comparable with respect to sex (boys 50% vs 42%), mean age (16.7 vs 16.2 years), and type of IBD (Crohn 68% vs 66%). The 13- to 15-year-olds reported calling in refills (11%, 8%, respectively), scheduling clinic appointment (0, 1%), preparing questions (13%, 5%), and taking the main role in talking during clinic visits (15%, 24%). The 16- to 18-year-olds reported calling in refills (13%, 27%), scheduling clinic appointments (9%, 6%), preparing questions (9%, 16%), and taking the main role in talking in clinic visits (36%, 45%). Responsibility for behaviors gradually increases with age, but did not differ significantly between 2008 and 2011.Increasing awareness around transition readiness for pediatric providers had an insignificant effect on the self-management skills of patients with IBD. A more formal or structured approach is likely required to improve transition skills in adolescent patients.

    View details for DOI 10.1097/MPG.0000000000000405

    View details for PubMedID 24762453

  • Transitioning childhood cancer survivors to adult care: A survey of pediatric oncologists. Kenney, L., Melvin, P., Fishman, L., O'Sullivan-Oliveira, J., Sawicki, G. S., Ziniel, S., Diller, L., Fernandes, S. M. AMER SOC CLINICAL ONCOLOGY. 2014
  • Aortic Valve Dysfunction and Aortic Dilation in Adults with Coarctation of the Aorta CONGENITAL HEART DISEASE Clair, M., Fernandes, S. M., Khairy, P., Graham, D. A., Krieger, E. V., Opotowsky, A. R., Singh, M. N., Colan, S. D., Meijboom, E. J., Landzberg, M. J. 2014; 9 (3): 235-243


    To determine the prevalence of aortic valve dysfunction, aortic dilation, and aortic valve and ascending aortic intervention in adults with coarctation of the aorta (CoA).Aortic valve dysfunction and aortic dilation are rare among children and adolescents with CoA. With longer follow-up, adults may be more likely to have progressive disease.We retrospectively reviewed all adults with CoA, repaired or unrepaired, seen at our center between 2004 and 2010.Two hundred sixteen adults (56.0% male) with CoA were identified. Median age at last evaluation was 28.3 (range 18.0 to 75.3) years. Bicuspid aortic valve (BAV) was present in 65.7%. At last follow-up, 3.2% had moderate or severe aortic stenosis, and 3.7% had moderate or severe aortic regurgitation. Dilation of the aortic root or ascending aorta was present in 28.0% and 41.6% of patients, respectively. Moderate or severe aortic root or ascending aortic dilation (z-score > 4) was present in 8.2% and 13.7%, respectively. Patients with BAV were more likely to have moderate or severe ascending aortic dilation compared with those without BAV (19.5% vs. 0%; P < 0.001). Age was associated with ascending aortic dilation (P = 0.04). At most recent follow-up, 5.6% had undergone aortic valve intervention, and 3.2% had aortic root or ascending aortic replacement.In adults with CoA, significant aortic valve dysfunction and interventions during early adulthood were uncommon. However, aortic dilation was prevalent, especially of the ascending aorta, in patients with BAV.

    View details for DOI 10.1111/chd.12109

    View details for Web of Science ID 000337721700016

    View details for PubMedID 23764014

  • Transition and transfer of adolescents and young adults with pediatric onset chronic disease: The patient and parent perspective. Journal of pediatric rehabilitation medicine Fernandes, S. M., O'Sullivan-Oliveira, J., Landzberg, M. J., Khairy, P., Melvin, P., Sawicki, G. S., Ziniel, S., Kenney, L. B., Garvey, K. C., Sobota, A., O'Brien, R., Nigrovic, P. A., Sharma, N., Fishman, L. N. 2014; 7 (1): 43-51


    To determine patients' and parents' perceptions regarding the delivery of transition education and perceived barriers to transfer to adult oriented care.A self-report survey was administered to a convenience sample of patients (16-25 years old) with various childhood onset chronic diseases. A similar survey was administered to their parents/guardians.A total of 155 patients and 104 parents participated in the study. The mean age of patients was 18.8 ± 2.3 years; 57% were female. Although most patients and parents reported receiving information and training about their medical condition, significant gaps in other aspects of transition education were identified. These included stated deficiencies in education regarding unprotected intercourse, health of future offspring, birth control, pregnancy, illicit drug use, and future career or vocation counseling. Commonly cited barriers to transfer were emotional attachments and lack of adult medicine specialty providers; however, the majority anticipated being ready to transfer to adult oriented care by age 25 years.There are significant gaps in the delivery of transition education as perceived by patients and their parents. Standardization of transition education may help ensure that patients acquire the knowledge and skills for health care self-management in adulthood and successful transfer to adult oriented care.

    View details for DOI 10.3233/PRM-140269

    View details for PubMedID 24919937

  • Heart Failure Caused by Congenital Left-Sided Lesions HEART FAILURE CLINICS Krieger, E. V., Fernandes, S. M. 2014; 10 (1): 155-?


    There are diverse mechanisms by which congenital left-sided cardiac lesions can precipitate heart failure. Left heart outflow obstruction can impose abnormal pressure load on the left ventricle, inducing adverse remodeling, hypertrophy, and diastolic and systolic dysfunction. Abnormalities in left ventricular inflow can increase pulmonary venous pressure and predisposing to pulmonary edema. In addition, inborn abnormalities in left ventricular myocardial structure and function can impair both systolic and diastolic function and manifest as heart failure later in life. In this article, the different mechanisms, outcomes, and treatments of heart failure in patients with congenital left-sided lesions are discussed.

    View details for DOI 10.1016/j.hfc.2013.09.015

    View details for Web of Science ID 000329086600014

    View details for PubMedID 24275301

  • Effect of Aspirin and warfarin therapy on thromboembolic events in patients with univentricular hearts and Fontan palliation INTERNATIONAL JOURNAL OF CARDIOLOGY Potter, B. J., Leong-Sit, P., Fernandes, S. M., Feifer, A., Mayer, J. E., Triedman, J. K., Walsh, E. P., Landzberg, M. J., Khairy, P. 2013; 168 (4): 3940-3943


    Patients with univentricular hearts and Fontan palliation are at risk for thromboembolic complications. While aspirin and warfarin therapies are currently the mainstay of prophylaxis, controversy exists as to the optimal prevention strategy.A cohort study was conducted on the New England registry of patients born in 1985 or earlier with Fontan surgery at Boston Children's Hospital, in order to assess and compare the effect of prophylactic aspirin and warfarin on incident thromboembolic events.A total of 210 qualifying patients (49% male) underwent Fontan surgery at a median age of 8.5 years: 48.6% had a right atrium to pulmonary artery anastomosis, 11% a right atrium to right ventricle conduit, 38.6% a lateral tunnel, and 1.9% an extracardiac conduit. No thromboembolic prophylaxis was prescribed to 50.0%, whereas 24.3% received aspirin, and 25.7% warfarin. In multivariate analyses, lack of aspirin or warfarin was associated with a significantly higher thromboembolic event rate when compared to therapy with either [hazard ratio 8.5, 95% confidence interval (3.6-19.9), P < 0.001], with no difference between the two treatment strategies (P = 0.768). Twenty-year freedom from thromboemboli was 86% versus 52% in patients with and without thromboprophylaxis, respectively. Other factors independently associated with thromboemboli were a low post-operative cardiac index [hazard ratio 2.6, 95% confidence interval (1.2, 5.9)] and atrial fibrillation or flutter [hazard ratio 3.1, 95% confidence interval (1.2, 8.0)].Prophylaxis with either aspirin or warfarin was associated with a significantly lower rate of incident thromboembolic events following Fontan palliation, with no difference between the two therapies.

    View details for DOI 10.1016/j.ijcard.2013.06.058

    View details for Web of Science ID 000326219600128

    View details for PubMedID 23870650

  • Effect of inhaled iloprost on the exercise function of Fontan patients: A demonstration of concept INTERNATIONAL JOURNAL OF CARDIOLOGY Rhodes, J., Ubeda-Tikkanen, A., Clair, M., Fernandes, S. M., Graham, D. A., Milliren, C. E., Daly, K. P., Mullen, M. P., Landzberg, M. J. 2013; 168 (3): 2435-2440


    Exercise capacity following Fontan surgery is often depressed. An inability to reduce pulmonary vascular resistance appropriately during exercise may contribute to this phenomenon. The aim of this study was to determine whether administration of iloprost, a selective pulmonary vasodilator, would improve exercise function after Fontan procedure.Double-blind, randomized, placebo controlled, crossover trial. Patients performed two cardiopulmonary exercise tests (CPX) separated by <1 month. A single nebulizer treatment (iloprost or placebo) was administered before each CPX.18 patients aged 12-49 (median 17) years were recruited. Mild throat discomfort developed in 10/18 patients during iloprost administration; all but 1 were able to complete treatment. No symptoms developed during placebo treatments (p<0.001). Two additional patients did not complete CPX: one with atrial flutter; another with developmental issues that precluded adequate CPX. In the 15 remaining subjects oxygen pulse (a surrogate for forward stroke volume) at peak exercise was higher following iloprost (median increase 1.2 ml/beat; p<0.001). Peak VO2 also rose (median increase 1.3 ml/kg/min; p<0.04). Nine patients had peak VO2 <30 ml/kg/min; each of these patients had higher peak VO2 following iloprost. Only 3/6 patients with peak VO2 >30 ml/kg/min had higher peak VO2 following iloprost (p<0.04).Iloprost improves the peak oxygen pulse and peak VO2 of patients with Fontan physiology and appears to be particularly beneficial among patients with impaired exercise function. Treatment is associated with minor side effects. These findings support the concept of pulmonary vasodilator therapy in Fontan patients with limited functional capacity.

    View details for DOI 10.1016/j.ijcard.2013.03.014

    View details for Web of Science ID 000326184400115

    View details for PubMedID 23545150

  • MELD-XI score and cardiac mortality or transplantation in patients after Fontan surgery HEART Assenza, G. E., Graham, D. A., Landzberg, M. J., Valente, A. M., Singh, M. N., Bashir, A., Fernandes, S., Mortele, K. J., Ukomadu, C., Volpe, M., Wu, F. 2013; 99 (7): 491-496


    The Fontan operation is a staged palliation for complex congenital heart disease and single ventricle physiology. Perioperative survivors of the Fontan operation experience long-term cardiac complications, including death. Liver and renal dysfunction are reported in these patients and have a direct effect on morbidity and mortality. This study aims to investigate whether the Model for End-stage Liver Disease eXcluding INR score (function of creatinine and total bilirubin, MELD-XI) predicts risk for cardiac mortality or transplantation in patients with Fontan circulation.Retrospective, single-centre study. Time of first evaluation was the time of the earliest available MELD-XI score measurement, and follow-up was terminated by a cardiac event or by the last clinical evaluation.Patients surviving after Fontan surgery and evaluated at Boston Children's Hospital between 1993 and 2008.Composite endpoint of sudden death, death from congestive heart failure or cardiac transplantation.The MELD-XI score was calculated as MELD-XI=11.76(loge creatinine)+5.112(loge total bilirubin)+9.44. Ninety-six patients were included (52 male, median age 26 years). After a mean follow-up period of 5.7 years, 18 patients (19%) experienced the composite end point. Baseline MELD-XI score was independently and directly related to the incidence of the composite endpoint (HR for high MELD-XI score group of 7.76, 95% CI 2.05 to 29.33, p=0.008).Fontan patients with a higher MELD-XI score have shorter freedom from sudden cardiac death, death from congestive heart failure and cardiac transplantation.

    View details for DOI 10.1136/heartjnl-2012-303347

    View details for Web of Science ID 000315920100011

    View details for PubMedID 23406689

  • Adult Congenital Heart Disease Incidence and Consultation: A Survey of General Adult Cardiologists JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY Fernandes, S. M., Pearson, D. D., Rzeszut, A., Mitchell, S. J., Landzberg, M. J., Martin, G. R. 2013; 61 (12): 1303-1304

    View details for DOI 10.1016/j.jacc.2013.01.005

    View details for Web of Science ID 000316751100014

    View details for PubMedID 23395071

  • CARDIOVASCULAR SURGERY FOR ADULT CONGENITAL HEART DISEASE: VARIATION IN USE OF SPECIALTY-CARE CENTERS 62nd Annual Scientific Session of the American-College-of-Cardiology Fernandes, S. M., Chamberlain, L., Grady, S., Mayo, J., Optowsky, A., Sanders, L. M., Wise, P. H. ELSEVIER SCIENCE INC. 2013: E506–E506
  • Cardiovascular Outcomes After the Arterial Switch Operation for D-Transposition of the Great Arteries CIRCULATION Khairy, P., Clair, M., Fernandes, S. M., Blume, E. D., Powell, A. J., Newburger, J. W., Landzberg, M. J., Mayer, J. E. 2013; 127 (3): 331-339


    Data regarding long-term outcomes after the arterial switch operation for D-transposition of the great arteries are scarce.A single-institution retrospective cohort study was conducted to assess cardiovascular outcomes after an arterial switch operation between 1983 and 1999. Patients without follow-up visits within 3 years were contacted and secondary sources of information obtained. Overall, 400 patients, 154 (38.3%) with a ventricular septal defect, 238 (59.5%) with an intact septum, and 9 (2.3%) with a Taussig-Bing anomaly, were followed for a median of 18.7 years. In perioperative survivors, overall and arrhythmia-free survival rates at 25 years were 96.7±1.8% and 96.6±0.1%, respectively. Late mortality was predominantly a result of sudden deaths and myocardial infarction. At 25 years, 75.5±2.5% remained free from surgical or catheter-based reintervention. Freedom from an adverse cardiovascular event was 92.9±1.9% at 25 years. Independent predictors were a single right coronary artery (hazard ratio, 4.58; 95% confidence interval, 1.32-15.90), P=0.0166) and postoperative heart failure (hazard ratio, 6.93; 95% confidence interval, 1.57-30.62; P=0.0107). At last follow-up, the left ventricular ejection fraction was 60.3±8.9%, 97.3% had class I symptoms, and 5.2% obstructive coronary artery disease. Peak oxygen uptake was 35.1±7.6 mL/kg/min (86.1±15.1% predicted), with a chronotropic index <80% in 34.2%. At least moderate neoaortic and pulmonary regurgitation were present in 3.4% and 6.6%, respectively, and more than mild neoaortic and pulmonary stenosis in 3.2% and 10.3%.Long-term and arrhythmia-free survival is excellent after arterial switch operation. Although sequelae include chronotropic incompetence and neoaortic, pulmonary, and coronary artery complications, most patients maintain normal systolic function and exercise capacity.

    View details for DOI 10.1161/CIRCULATIONAHA.12.135046

    View details for Web of Science ID 000313741300013

    View details for PubMedID 23239839

  • Aortic Root Dilatation in Adults with Surgically Repaired Tetralogy of Fallot A Multicenter Cross-Sectional Study CIRCULATION Mongeon, F., Gurvitz, M. Z., Broberg, C. S., Aboulhosn, J., Opotowsky, A. R., Kay, J. D., Valente, A. M., Earing, M. G., Lui, G. K., Fernandes, S. M., Gersony, D. R., Cook, S. C., Ting, J. G., Nickolaus, M. J., Landzberg, M. J., Khairy, P. 2013; 127 (2): 172-?


    Although aortic root pathology has been described in patients with tetralogy of Fallot, the scope of the problem remains poorly defined. We sought to determine the prevalence and predictors of aortic root dilatation in adults with repaired tetralogy of Fallot.A multicenter cross-sectional study was conducted with standardized reassessment of echocardiographic parameters in 474 adults (≥18 years) with surgically repaired tetralogy of Fallot or pulmonary atresia with ventricular septal defect. The aortic root was measured in a parasternal long-axis view, in diastole, at the level of the sinus of Valsalva. Prevalence and predictors of an absolute diameter ≥40 mm and of an observed-to-expected ratio >1.5 were assessed. The aortic root dimension was ≥40 mm in 28.9% (95% confidence interval, 26.9%-30.9%). In multivariate analyses, the only independently associated factor was male sex (odds ratio, 4.48; 95% confidence interval, 1.55-12.89; P=0.006). The prevalence of an observed-to-expected aortic root dimension ratio >1.5 was 6.6% (95% confidence interval, 5.3%-7.9%). It was associated with pulmonary atresia and moderate or severe aortic regurgitation in univariate analyses, but no independent predictor was identified. The side of the aortic arch was not associated with aortic root dimension. The prevalence of moderate or severe aortic regurgitation was 3.5% (95% confidence interval, 2.7%-4.2%).Although nearly one third of adults with repaired tetralogy of Fallot have an aortic root diameter ≥40 mm, the prevalence of a dilated aortic root, when defined by an indexed ratio of observed-to-expected values, is low. Similarly, moderate or severe aortic regurgitation is uncommon.

    View details for DOI 10.1161/CIRCULATIONAHA.112.129585

    View details for Web of Science ID 000313637200013

    View details for PubMedID 23224208

  • Referral Patterns and Perceived Barriers to Adult Congenital Heart Disease Care Results of a Survey of U.S. Pediatric Cardiologists JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY Fernandes, S. M., Khairy, P., Fishman, L., Melvin, P., O'Sullivan-Oliveira, J., Sawicki, G. S., Ziniel, S., Breitinger, P., Williams, R., Takahashi, M., Landzberg, M. J. 2012; 60 (23): 2411-2418


    This study sought to elucidate referral patterns and barriers to adult congenital heart disease (ACHD) care, as perceived by pediatric cardiologists (PCs).Management guidelines recommend that care of adults with moderate/complex congenital heart disease be guided by clinicians trained in ACHD.A cross-sectional survey was distributed to randomly selected U.S. PCs.Overall response rate was 48% (291 of 610); 88% (257 of 291) of respondents met inclusion criteria (outpatient care to patients >11 years of age). Participants were in practice for 18.2 ± 10.7 years; 70% were male, and 72% were affiliated with an academic institution; 79% stated that they provide care to adults (>18 years). The most commonly perceived patient characteristic prompting referral to ACHD care was adult comorbidities (83%). The most perceived barrier to ACHD care was emotional attachment of parents and patients to the PC (87% and 86%, respectively). Clinician attachment to the patient/family was indicated as a barrier by 70% of PCs and was more commonly identified by responders with an academic institutional affiliation (p = 0.001). A lack of qualified ACHD care providers was noted by 76% of PCs. Those affiliated with an academic institution were less likely to identify this barrier to ACHD care (p = 0.002).Most PC respondents in the United States provide care to ACHD patients. Common triggers that prompt referral and perceived barriers to ACHD care were identified. These findings might assist ACHD programs in developing strategies to identify and retain patients, improve collaborative care, and address emotional needs during the transition and transfer process.

    View details for DOI 10.1016/j.jacc.2012.09.015

    View details for Web of Science ID 000311952400013

    View details for PubMedID 23141490

  • Bicuspid aortic valve and associated aortic dilation in the young HEART Fernandes, S., Khairy, P., Graham, D. A., Colan, S. D., Galvin, T. C., Sanders, S. P., Singh, M. N., Bhatt, A., Lacro, R. V. 2012; 98 (13): 1014-1019


    The aorta in patients with bicuspid aortic valve (BAV) is larger and grows more rapidly than in patients with tricommissural aortic valve. Young patients with BAV can have significant aortic dilation that places them at risk for morbidity and mortality.The aims of this study were to determine the rate of growth of the aorta in young patients with BAV and to identify predictors of significant dilation and rapid aortic growth.333 patients were randomly selected from an inception cohort of 1192 patients with BAV identified between 1986 and 1999.Median age at the most recent study was 13.5 (0-30) years, 74% were male. Moderate/severe (Z>4) aortic root and ascending aortic dilation was present in 14/333 (5%) and 53/333 (16%) of patients, respectively. In longitudinal follow-up, only a minimal change in aortic Z-score was noted. Predictors of moderate/severe aortic root dilation included moderate/severe aortic regurgitation, absence of moderate/severe aortic stenosis and fusion of the right and left coronary leaflets. Predictors of moderate/severe ascending aortic dilation included moderate/severe aortic regurgitation and absence of aortic coarctation.Moderate/severe dilation of the ascending aorta is common in young patients with BAV, but moderate/severe dilation of the aortic root is less common. The Z-scores for both remained relatively constant over time even in patients with significant dilation, implying that young children with moderate/severe aortic dilation may be at the highest risk for dilation-related complications as adults.

    View details for DOI 10.1136/heartjnl-2012-301773

    View details for Web of Science ID 000305000400011

    View details for PubMedID 22668868

  • Maternal cardiovascular events during childbirth among women with congenital heart disease HEART Opotowsky, A. R., Siddiqi, O. K., D'Souza, B., Webb, G. D., Fernandes, S. M., Landzberg, M. J. 2012; 98 (2): 145-151


    To define the epidemiology of adverse cardiovascular events among women with congenital heart disease (CHD) hospitalised for childbirth in the USA.The 1998-2007 Nationwide Inpatient Sample, an administrative dataset representative of overall US hospital admissions, was used to identify hospitalisations for delivery.Logistic regression was used to estimate ORs for cardiovascular outcomes (arrhythmia, heart failure, cerebrovascular accident, embolism, death or a combined outcome) for women with and without CHD. Covariates included age, number of medical comorbidities, pulmonary hypertension, hospital teaching status, insurance status and method of delivery.Annual deliveries for women with CHD increased 34.9% from 1998 to 2007 compared with an increase of 21.3% in the general population. Women with CHD were more likely to sustain a cardiovascular event (4042/100,000 vs 278/100,000 deliveries, univariate OR 15.1, 95% CI 13.1 to 17.4, multivariable OR 8.4, 95% CI 7.0 to 10.0). Arrhythmia, the most common cardiovascular event, was more frequent among women with CHD (2637/100,000 vs 210/100,000, univariate OR 12.9, 95% CI 10.9 to 15.3, multivariable OR 8.3, 95% CI 6.7 to 10.1). Death occurred in 150/100,000 patients with CHD compared with 8.2/100,000 patients without CHD (multivariable OR 6.7, 95% CI 2.9 to 15.4). Complex CHD was associated with greater odds of having an adverse cardiovascular event than simple CHD (8158/100,000 vs 3166/100,000, multivariable OR 2.0, 95% CI 1.4 to 3.0).Maternal CHD is associated with a markedly increased risk of adverse cardiovascular events and death during admission for delivery.

    View details for DOI 10.1136/heartjnl-2011-300828

    View details for Web of Science ID 000298350700010

    View details for PubMedID 21990383

  • Parental Knowledge Regarding Lifelong Congenital Cardiac Care PEDIATRICS Fernandes, S. M., Verstappen, A., Ackerman, K., Adams, E. E., Barton, C., Breitinger, P., Crumb, S., Dummer, K., Harada, K., Khairy, P., Landzberg, M. J., Linstead-Goldsmith, R., Meadows, A. K., Nieves, J. A., Saidi, A., Takahashi, M., Zhou, J., Ziniel, S., Williams, R. 2011; 128 (6): E1489-E1495


    To assess parental knowledge regarding lifelong congenital cardiac care (LLCCC).National guidelines recommend that nearly 50% of adult survivors with congenital heart disease (CHD) receive LLCCC; the number of adults who receive such care seems far less. Inadequate parental knowledge of LLCCC might contribute to care interruption.In this multicenter study, we administered a questionnaire to parents of children with moderate and complex CHD to assess knowledge of LLCCC.A total of 500 parents participated; the median age of their children was 10 years (range: 2-18 years). Most parents (81%) understood that their child would need LLCCC, but only 44% recognized that their child's cardiology care should be guided by an adult congenital heart specialist in adulthood. More than half (59%) of the parents stated that their current cardiology team had never spoken to them about LLCCC, but 96% wished to learn more. Variables associated with parental LLCCC knowledge included previous discussions regarding LLCCC, underlying cardiac surgical diagnosis, and level of parental education.A substantial number of parents of children with moderate and complex CHD lack knowledge about LLCCC, but almost all of them have a desire to learn more about the care their child will need as an adult.

    View details for DOI 10.1542/peds.2010-3068

    View details for Web of Science ID 000298131400016

    View details for PubMedID 22123874

  • A Case Series of the Anesthetic Management of Parturients with Surgically Repaired Tetralogy of Fallot ANESTHESIA AND ANALGESIA Arendt, K. W., Fernandes, S. M., Khairy, P., Warnes, C. A., Rose, C. H., Landzberg, M. J., Craigo, P. A., Hebl, J. R. 2011; 113 (2): 307-317


    Most case reports of pregnancies after surgical repair of tetralogy of Fallot have focused on cardiovascular and obstetric concerns, with relatively few authors focusing on specific intrapartum and postpartum anesthetic management strategies.The Mayo Clinic Congenital Heart Disease Clinic and the Boston Adult Congenital Heart Disease Service databases were cross-referenced with the Mayo Clinic and the Brigham and Women's Hospital Department of Anesthesiology databases to identify patients with tetralogy of Fallot who delivered at their respective hospital from January 1, 1994, to January 1, 2008. We reviewed each medical record to evaluate parturient care during pregnancy, labor, and delivery with a focus on anesthetic management.During the 14-year study period, a total of 27 deliveries in 20 patients with repaired tetralogy of Fallot were identified. Twenty-one deliveries (78%) among 15 parturients (75%) involved a trial of labor; all parturients received neuraxial analgesia for labor and delivery, including 18 (86%) epidural, 2 (10%) combined spinal-epidural, and 1 (5%) continuous spinal anesthetic after an unintended dural puncture. Of the 21 patients undergoing labor, 3 (14%) received invasive arterial blood pressure monitoring; 5 (24%) received continuous telemetry; 3 (14%) experienced congestive heart failure that required diuresis; 4 (19%) had obstetric or neonatal complications; and 3 (14%) had anesthesia complications. Cesarean delivery was required in 4 patients (19%) because of labor complications. Concurrent cardiovascular, obstetric, and anesthetic complications in 1 patient resulted in neonatal death. Six (22%) parturients underwent elective cesarean delivery; 4 received epidural and 2 received spinal anesthesia; no anesthetic or immediate obstetric complications occurred. Among all parturients, 5 deliveries in 5 separate parturients (19% of deliveries) reported symptoms of congestive heart failure at the time of delivery.Pregnancy outcomes for patients with repaired tetralogy of Fallot were found to be generally favorable. All patients undergoing a trial of labor or cesarean delivery had neuraxial analgesia or anesthesia. Recognition and management of congestive heart failure was necessary in 19% of deliveries.

    View details for DOI 10.1213/ANE.0b013e31821ad83e

    View details for Web of Science ID 000293064500017

    View details for PubMedID 21596882

  • Exercise Testing Identifies Patients at Increased Risk for Morbidity and Mortality Following Fontan Surgery CONGENITAL HEART DISEASE Fernandes, S. M., Alexander, M. E., Graham, D. A., Khairy, P., Clair, M., Rodriguez, E., Pearson, D. D., Landzberg, M. J., Rhodes, J. 2011; 6 (4): 294-303


    The objective of this study was to examine the relationship between exercise test data and mortality in patients who have had the Fontan procedure.The study was designed as a retrospective cohort study.The study was set in a tertiary care center.All study participants were Fontan patients ≥16 years old who had cardiopulmonary exercise tests at our institution between November 2002 and March 2010. The first exercise test with adequate effort during the study period was retained for analysis. We enrolled 146 patients at a median age of 21.5 years (16.0-51.6); 15.8 years (1.2-29.9) after Fontan surgery.The outcome measures were exercise test data (peak oxygen consumption, peak heart rate, etc.); mortality.Peak oxygen consumption averaged 21.2 ± 6.2 mL/kg/min, 57.1 ± 14.1% predicted. Follow-up data were collected 4.0 ± 2.0 years (range 0.3-7.7) after the exercise test. Sixteen patients (11%) died during follow-up; their peak oxygen consumption (16.3 ± 4.0 mL/kg/min) was significantly less than the survivors' (21.8 ± 6.2 mL/kg/min; P < .0001). Recursive partitioning and Cox proportional hazards modeling revealed that the hazard for death for patients with a peak oxygen consumption of <16.6 mL/kg/min was 7.5 (95% confidence interval: 2.6, 21.6; P < .0002) times that of patients with a higher peak oxygen consumption. Similarly, the hazard ratio for patients with peak-exercise heart rates of <122.5 bpm was 10.6 (3.0, 37.1; 0 < 0.0002). Data from exercise tests could also identify patients at increased risk for a combined morbidity/mortality end point.In adults with Fontan surgery, exercise test data can identify patients at increased risk of midterm morbidity and mortality.

    View details for DOI 10.1111/j.1747-0803.2011.00500.x

    View details for Web of Science ID 000292981900003

    View details for PubMedID 21418537

  • Heart Rate Response During Exercise and Pregnancy Outcome in Women With Congenital Heart Disease CIRCULATION Lui, G. K., Silversides, C. K., Khairy, P., Fernandes, S. M., Valente, A. M., Nickolaus, M. J., Earing, M. G., AboulHosn, J. A., Rosenbaum, M. S., Cook, S., Kay, J. D., Jin, Z., Gersony, D. R. 2011; 123 (3): 242-248


    Cardiopulmonary exercise testing is often used to evaluate exercise capacity in adults with congenital heart disease including women who are considering pregnancy. The relationship between cardiopulmonary exercise testing parameters and pregnancy outcome has not been defined.We conducted a multicenter retrospective observational study of women with congenital heart disease who had undergone cardiopulmonary exercise testing within 2 years of pregnancy or during the first trimester. Cardiopulmonary exercise testing variables included peak oxygen consumption and measures of chronotropic response: peak heart rate, percentage of maximum age predicted heart rate, heart rate reserve (peak heart rate-resting heart rate), and chronotropic index [(peak heart rate-resting heart rate)/(220-age-resting heart rate)]. We identified 89 pregnancies in 83 women. There were 4 spontaneous abortions and 1 termination. One or more adverse cardiac events occurred in 18%; congestive heart failure in 14%, and sustained arrhythmia in 7%. Peak heart rate (odds ratio [OR] 0.71; 95% confidence interval [CI] [0.53, 0.94]; P=0.02), percentage of maximum age predicted heart rate (OR 0.93; 95% CI [0.88, 0.98]; P=0.01), and chronotropic index (OR 0.65; 95% CI [0.47, 0.90]; P=0.01) were associated with a cardiac event. Neonatal events occurred in 20%. Peak heart rate (OR 0.75; 95% CI [0.58, 0.98]; P=0.04), percentage of maximum age predicted heart rate (OR 0.94; 95% CI [0.89, 0.99]; P=0.02), heart rate reserve (OR 0.8; 95% CI [0.64, 0.99]; P=0.04), and chronotropic index (OR 0.73; 95% CI [0.54, 0.98]; P=0.04) correlated with a neonatal event. Peak oxygen consumption was not associated with an adverse pregnancy outcome.Abnormal chronotropic response correlates with adverse pregnancy outcomes in women with congenital heart disease and should be considered in refining risk stratification schemes.

    View details for DOI 10.1161/CIRCULATIONAHA.110.953380

    View details for Web of Science ID 000286507800010

    View details for PubMedID 21220738

  • Obstetric outcomes in pregnant women with congenital heart disease INTERNATIONAL JOURNAL OF CARDIOLOGY Ouyang, D. W., Khairy, P., Fernandes, S. M., Landzberg, M. J., Economy, K. E. 2010; 144 (2): 195-199


    Predictors of adverse maternal and neonatal outcomes in pregnant women with congenital heart disease (CHD) have been described, but not for obstetrical outcomes. The primary aim of this study was to determine what risk factors predict sustaining adverse obstetric events in pregnant women with CHD. In addition, a secondary aim was to assess the impact of avoiding Valsalva on obstetrical outcomes, an intervention commonly recommended, but never studied.A retrospective cohort study examined outcomes in women with CHD who delivered between 1998 and 2005. We examined baseline cardiac characteristics in a multivariate logistic regression model to assess which were associated with adverse obstetric events. We also compared outcomes of women who avoided Valsalva versus those who were allowed to Valsalva.The study included 65 women with 112 pregnancies. An adverse obstetric event occurred in 32.6% (n=32) of ongoing pregnancies, the most common being preterm delivery (n=19), post-partum hemorrhage (n=13), and preterm premature rupture of membranes (n=9). There were no independent predictors for sustaining an adverse obstetric event. Women who avoided Valsalva had increased rates of post-partum hemorrhage and 3rd/4th degree lacerations.Although one-third of pregnancies were associated with an adverse obstetric outcome, these events could not be predicted by baseline hemodynamic characteristics. The routine practice of avoiding Valsalva may be associated with high rates of post-partum hemorrhage and 3rd/4th degree lacerations.

    View details for DOI 10.1016/j.ijcard.2009.04.006

    View details for Web of Science ID 000282678300013

    View details for PubMedID 19411123

  • Pregnancy Outcomes in Women With Transposition of the Great Arteries and Arterial Switch Operation AMERICAN JOURNAL OF CARDIOLOGY Tobler, D., Fernandes, S. M., Wald, R. M., Landzberg, M., Salehian, O., Siu, S. C., Colman, J. M., Sermer, M., Silversides, C. K. 2010; 106 (3): 417-420


    There is a growing population of young women of child-bearing age with complete transposition of the great arteries (TGA) who have had an arterial switch operation (ASO). Pregnancy imposes a hemodynamic stress on the heart and, therefore, adverse cardiac events can occur during this period; however, pregnancy outcomes in this population have not been well studied. We sought to describe cardiac outcomes during pregnancy in women with TGA who had undergone an ASO in childhood. Women were identified from 2 large tertiary care hospitals. A retrospective chart review was performed to determine the prevalence of adverse maternal cardiac events during pregnancy. Overall, 74 women of child-bearing age were identified, 9 of whom had 17 pregnancies. There were 4 miscarriages. Six women (67%) had clinically important valve (n = 5) and ventricular (n = 1) lesions before the index pregnancy. Two women developed cardiac complications during pregnancy; 1 woman with impaired left ventricular systolic function had nonsustained ventricular tachycardia and 1 woman with a mechanical systemic atrioventricular valve developed postpartum valve thrombosis. There were no maternal deaths. In conclusion, young women with TGA from this early cohort repaired with ASO are reaching child-bearing age. A significant proportion have residua and/or sequelae that can confer risk for adverse cardiac events in pregnancy. Co-ordinated care between a congenital heart disease specialist and a high-risk obstetrician should be implemented.

    View details for DOI 10.1016/j.amjcard.2010.03.047

    View details for Web of Science ID 000280697700021

    View details for PubMedID 20643256

  • The CALF (Congenital Heart Disease in Adults Lower Extremity Systemic Venous Health in Fontan Patients) Study JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY Valente, A. M., Bhatt, A. B., Cook, S., Earing, M. G., Gersony, D. R., Aboulhosn, J., Opotowsky, A. R., Lui, G., Gurvitz, M., Graham, D., Fernandes, S. M., Khairy, P., Webb, G., Gerhard-Herman, M., Landzberg, M. J. 2010; 56 (2): 144-150


    The objective of this study was to document the prevalence of chronic venous insufficiency (CVI) and its associated factors in adults with Fontan physiology.As the population of adults with complex congenital heart disease and Fontan physiology increases, so does the occurrence of highly morbid and mortal outcomes, including heart failure and thromboembolism. The presence of abnormal peripheral hemodynamic conditions in this population and their potential contribution to adverse outcomes is not well known. The primary objective of this study was to document the prevalence of CVI in adults with Fontan physiology.A total of 159 adults with Fontan physiology from 7 adult congenital heart centers were prospectively assessed for lower extremity CVI, with the assignment of clinical, etiological, anatomical, and pathophysiological classification grades, and compared with age-matched and sex-matched controls. Leg photographs were independently reassessed to confirm interobserver reliability.The prevalence of CVI was significantly greater in the Fontan population (60%; 95% confidence interval [CI]: 52% to 68%) compared with healthy controls (32%; 95% CI: 15% to 54%) (p = 0.008). Strikingly, the prevalence of severe CVI (clinical, etiological, anatomical, and pathophysiological grade > or = 4) was significantly higher in the Fontan group (22%; 95% CI: 16% to 29%) versus the healthy controls (0%; 95% CI: 0% to 14%) (p = 0.005). In a multivariate analysis, several factors were independently associated with severe CVI, including increased numbers of catheterizations with groin venous access, lower extremity itching, and deep venous thrombosis.CVI is common in adult patients with congenital heart disease with Fontan physiology. The contribution of abnormal peripheral hemodynamic conditions to comorbidities, including thromboembolism and heart failure, and interventions to improve peripheral hemodynamic conditions require further exploration.

    View details for DOI 10.1016/j.jacc.2010.02.048

    View details for Web of Science ID 000279313600009

    View details for PubMedID 20620728

  • Pregnancy in patients with tetralogy of fallot: invited commentary. World journal for pediatric & congenital heart surgery Fernandes, S. M., Landzberg, M. J. 2010; 1 (2): 175-176

    View details for DOI 10.1177/2150135110371811

    View details for PubMedID 23804815

  • A preliminary look at duplicate testing associated with lack of electronic health record interoperability for transferred patients JOURNAL OF THE AMERICAN MEDICAL INFORMATICS ASSOCIATION Stewart, B. A., Fernandes, S., Rodriguez-Huertas, E., Landzberg, M. 2010; 17 (3): 341–44


    Duplication of medical testing results in a financial burden to the healthcare system. Authors undertook a retrospective review of duplicate testing on patients receiving coordinated care across two institutions, each with its own electronic medical record system. In order to determine whether duplicate testing occurred and if such testing was clinically indicated, authors analyzed records of 85 patients transferred from one site to the other between January 1, 2006 and December 31, 2007. Duplication of testing (repeat within 12 hours) was found in 32% of the cases examined; 20% of cases had at least one duplicate test not clinically indicated. While previous studies document that inaccessibility of paper records leads to duplicate testing when patients are transferred between care facilities, the current study suggests that incomplete electronic record transfer among incompatible electronic medical record systems can also lead to potentially costly duplicate testing behaviors. The authors believe that interoperable systems with integrated decision support could assist in minimizing duplication of testing at time of patient transfers.

    View details for PubMedID 20442154

  • Pregnant women with congenital heart disease: cardiac, anesthetic and obstetrical implications. Expert review of cardiovascular therapy Fernandes, S. M., Arendt, K. W., Landzberg, M. J., Economy, K. E., Khairy, P. 2010; 8 (3): 439-448


    Remarkable advances in surgical and clinical management have resulted in survival to adulthood in the large majority of patients with congenital heart malformations, even with the most complex disease. Over 1 million adults with congenital heart disease currently live in the USA, approximately half of whom are women of childbearing age. Collectively, congenital malformations are the most common form of heart disease in pregnant women. Indeed, in North America, congenital defects are now the leading cause of maternal morbidity and mortality from heart disease. This article begins with a summary of cardiovascular changes during pregnancy and highlights key features in pre-pregnancy counseling, maternal cardiac and obstetric risk, and neonatal complications. Management issues regarding pregnancy and delivery are elaborated, including anesthesia considerations. While it is beyond the scope of this article to discuss particulars related to all forms of congenital heart disease, selected subtypes are detailed at greater length. In the absence of clinical trial evidence to inform the care of pregnant women with congenital heart disease, this article is inspired by the premise that knowledgeable multidisciplinary assessment and management provides the best opportunity to substantially improve outcomes for mother and baby.

    View details for DOI 10.1586/erc.09.179

    View details for PubMedID 20222821

  • Serial Cardiopulmonary Exercise Testing in Patients with Previous Fontan Surgery PEDIATRIC CARDIOLOGY Fernandes, S. M., McElhinney, D. B., Khairy, P., Graham, D. A., Landzberg, M. J., Rhodes, J. 2010; 31 (2): 175-180


    Patients with previous Fontan surgery have reduced peak oxygen consumption (Vo 2), and data regarding progression of exercise intolerance is limited. The purpose of this study was to assess the evolution of exercise tolerance in patients with previous Fontan surgery. We performed a retrospective cohort study of patients with previous Fontan surgery who underwent cardiopulmonary exercise testing between November 2002 and January 2009. Patients were required to have completed at least two tests, with adequate levels of effort, >or-6 months apart. We identified 78 patients (55% male) who had undergone a total of 215 cardiopulmonary exercise tests. Age at initial study was 19.7 +/- 10.2 years; time interval since previous Fontan surgery 13.3 +/- 5.7 years; and time interval between the initial and the most recent exercise test was 3.0 +/- 1.4 years. Morphologic left ventricle was present in 58 (74.4%) patients. At baseline, peak Vo 2 averaged 24.7 +/- 7.0 ml/kg/min (63.7% +/- 15.8% predicted). At most recent study, peak Vo 2 averaged 23.2 +/- 7.2 ml/kg/min, (60.4% +/- 13.9% predicted). Percent predicted peak Vo 2 declined slowly, with a mean rate of decline of 1.25 +/- 0.36 percentage points/y (p < 0.001). Most of the decline occurred in patients < 18 years of age (mean rate of decline 1.78 +/- 0.46 percentage points/y; p = 0.0004). Thereafter, the rate of decline was less marked (mean rate of decline 0.54 +/- 0.57 percentage points/y; p = not significant). Ventricular morphology, type of Fontan procedure, and cardiac medications were not predictive of progressive exercise intolerance. In conclusion, although the exercise function of previous Fontan patients tends to decline during late adolescence, it appears to stabilize during early adulthood.

    View details for DOI 10.1007/s00246-009-9580-5

    View details for Web of Science ID 000274333900001

    View details for PubMedID 19915891

  • Attitude Toward and Current Practice of Transfer and Transition of Adolescents with Congenital Heart Disease in the United States of America and Europe PEDIATRIC CARDIOLOGY Hilderson, D., Saidi, A. S., van Deyk, K., Verstappen, A., Kovacs, A. H., Fernandes, S. M., Canobbio, M. M., Fleck, D., Meadows, A., Linstead, R., Moons, P. 2009; 30 (6): 786-793


    The objective of this study was to explore the current practice and attitudes of pediatric cardiologists in the United States and Europe on the transfer and transition of children with congenital heart disease (CHD). A survey among pediatric cardiology programs in the United States and Europe was undertaken. Sixty-nine centers completed and returned the 61-item questionnaire that was specifically devised for this survey. Of 69 participating centers, 74% reported that they transfer their patients to adult-focused care. When a center transfers its patients, 80% transfer them to a formalized Adult Congenital Heart Disease Program. The median age of transfer is 18 years. Comorbidities, pregnancy, and patient/family request to leave pediatric cardiology were identified as initiators for transfer. Complexity of the heart defect was relatively less important when deciding whether to transfer patients. Only one-third of the centers that transfer their patients provide a structured preparation for patients and family. Development of a formal transition program is planned at 59% of the centers that transfer patients. In conclusion, timely transfer and a structured transition process of children with CHD are not implemented in all pediatric cardiology programs. Health-care providers working in pediatric cardiology should make their transfer policies explicit and transition programs ought to be developed.

    View details for DOI 10.1007/s00246-009-9442-1

    View details for Web of Science ID 000268328000012

    View details for PubMedID 19365651

  • Sudden Death and Defibrillators in Transposition of the Great Arteries With Intra-atrial Baffles A Multicenter Study CIRCULATION-ARRHYTHMIA AND ELECTROPHYSIOLOGY Khairy, P., Harris, L., Landzberg, M. J., Fernandes, S. M., Barlow, A., Mercier, L., Viswanathan, S., Chetaille, P., Gordon, E., Dore, A., Cecchin, F. 2008; 1 (4): 250-257


    Transposition of the great arteries with intra-atrial baffle repair is among the congenital heart defects at highest risk of sudden death. Little is known about mechanisms of sudden death and the role of implantable cardioverter defibrillators.We conducted a multicenter cohort study in patients with transposition of the great arteries to determine actuarial rates of implantable cardioverter defibrillator shocks, identify risk factors, assess underlying arrhythmias, and characterize complications. Overall, 37 patients (age, 28.0+/-7.6 years; 89.2% male) were enrolled from 7 sites. Implantable cardioverter defibrillators were implanted for primary prevention in 23 (62.1%) patients and secondary prevention in 14 patients (37.8%). Annual rates of appropriate shocks were 0.5% and 6.0% in primary and secondary prevention, respectively (P=0.0366). Independent predictors were a secondary prevention indication (hazard ratio, 18.0; P=0.0341) and lack of beta-blockers (hazard ratio, 16.7; P=0.0301). In patients with appropriate shocks, intracardiac electrograms documented supraventricular tachycardia preceding or coexisting with ventricular tachycardia in 50%. No patient with inducible ventricular tachycardia received an appropriate shock in comparison with 37.5% of noninducible patients (P=0.0429). Inappropriate shocks occurred in 6.6% per year, more so in patients of lesser weight (hazard ratio, 0.91 per kg; P=0.0168). Additionally, 14 patients (37.8%) experienced complications: 5 (13.5%) acute, 1 (2.7%) late generator related, and 12 (32.4%) late lead related.In patients with transposition of the great arteries, high rates of appropriate shocks are noted in secondary but not primary prevention. Supraventricular arrhythmias may be implicated in the etiology of ventricular tachyarrhythmias; beta-blockers seem protective, and inducible ventricular tachycardia does not seem to predict future events. Inappropriate shocks and late lead-related complications are common.

    View details for DOI 10.1161/CIRCEP.108.776120

    View details for Web of Science ID 000266127100004

    View details for PubMedID 19808416

  • Implantable cardioverter-defibrillators in tetralogy of Fallot CIRCULATION Khairy, P., Harris, L., Landzberg, M. J., Viswanathan, S., Barlow, A., Gatzoulis, M. A., Fernandes, S. M., Beauchesne, L., Therrien, J., Chetaille, P., Gordon, E., Muhll, I. V., Cecchin, F. 2008; 117 (3): 363-370


    Tetralogy of Fallot is the most common form of congenital heart disease in implantable cardioverter-defibrillator (ICD) recipients, yet little is known about the value of ICDs in this patient population.We conducted a multicenter cohort study in high-risk patients with Tetralogy of Fallot to determine actuarial rates of ICD discharges, identify risk factors, and characterize ICD-related complications. A total of 121 patients (median age 33.3 years; 59.5% male) were enrolled from 11 sites and followed up for a median of 3.7 years. ICDs were implanted for primary prevention in 68 patients (56.2%) and for secondary prevention in 53 (43.8%), defined by clinical sustained ventricular tachyarrhythmia or resuscitated sudden death. Overall, 37 patients (30.6%) received at least 1 appropriate and effective ICD discharge, with a median ventricular tachyarrhythmia rate of 213 bpm. Annual actuarial rates of appropriate ICD shocks were 7.7% and 9.8% in primary and secondary prevention, respectively (P=0.11). A higher left ventricular end-diastolic pressure (hazard ratio 1.3 per mm Hg, P=0.004) and nonsustained ventricular tachycardia (hazard ratio 3.7, P=0.023) independently predicted appropriate ICD shocks in primary prevention. Inappropriate shocks occurred in 5.8% of patients yearly. Additionally, 36 patients (29.8%) experienced complications, of which 6 (5.0%) were acute, 25 (20.7%) were late lead-related, and 7 (5.8%) were late generator-related complications. Nine patients died during follow-up, which corresponds to an actuarial annual mortality rate of 2.2%, which did not differ between the primary and secondary prevention groups.Patients with tetralogy of Fallot and ICDs for primary and secondary prevention experience high rates of appropriate and effective shocks; however, inappropriate shocks and late lead-related complications are common.

    View details for DOI 10.1161/CIRCULATIONAHA.107.726372

    View details for Web of Science ID 000252601600014

    View details for PubMedID 18172030

  • Long-term survival, modes of death, and predictors of mortality in patients with Fontan surgery CIRCULATION Khairy, P., Fernandes, S. M., Mayer, J. E., Triedman, J. K., Walsh, E. P., Lock, J. E., Landzberg, M. J. 2008; 117 (1): 85-92


    To better define determinants of mortality in patients with univentricular physiology, a database registry was created of patients born in 1985 or earlier with Fontan surgery who were followed up at Children's Hospital Boston.A total of 261 patients, 121 of whom (46.4%) were women, had a first Fontan surgery at a median age of 7.9 years: right atrium-to-pulmonary artery connection in 135 (51.7%); right atrium to right ventricle in 25 (9.6%); and total cavopulmonary connection in 101 (38.7%). Over a median of 12.2 years, 76 (29.1%) died, 5 (1.9%) had cardiac transplantation, 5 (1.9%) had Fontan revision, and 21 (8.0%) had Fontan conversion. Perioperative mortality decreased steadily over time and accounted for 68.4% of all deaths. In early survivors, actuarial freedom from death or transplantation was 93.7%, 89.9%, 87.3%, and 82.6% at 5, 10, 15, and 20 years, respectively, with no significant difference between right atrium to pulmonary artery versus total cavopulmonary connection. Late deaths were classified as sudden in 7 patients (9.2%), thromboembolic in 6 (7.9%), heart failure-related in 5 (6.7%), sepsis in 2 (2.6%), and other in 4 (5.2%). Most sudden deaths were of presumed arrhythmic origin with no identifiable predictor. Independent risk factors for thromboembolic death were lack of antiplatelet or anticoagulant therapy (hazard ratio [HR], 91.6; P=0.0041) and clinically diagnosed intracardiac thrombus (HR, 22.7; P=0.0002). Independent predictors of heart failure death were protein-losing enteropathy (HR, 7.1; P=0.0043), single morphologically right ventricle (HR, 10.5; P=0.0429), and higher right atrial pressure (HR, 1.3 per 1 mm Hg; P=0.0016).In perioperative survivors of Fontan surgery, gradual attrition occurs predominantly from thromboembolic, heart failure-related, and sudden deaths.

    View details for DOI 10.1161/CIRCULATIONAHA.107.738559

    View details for Web of Science ID 000252094100014

    View details for PubMedID 18071068

  • Bicuspid aortic valve morphology and interventions in the Young JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY Fernandes, S. M., Khairy, P., Sanders, S. P., Colan, S. D. 2007; 49 (22): 2211-2214


    The aim of this study was to determine whether the morphologic subtype of bicuspid aortic valve (BAV) is associated with valve intervention in the young.Analysis of BAV morphology is of prognostic relevance, as the fusion of right- and noncoronary leaflets (R-N) is associated with a greater degree of valve dysfunction compared with other subtypes. However, it is currently unknown whether morphologic differences translate into clinically relevant outcomes such as valve intervention.A nested cohort study was conducted on 310 patients with right- and left-coronary leaflet (R-L) and R-N fusion who were selected randomly from an inception cohort of 1,192 patients with BAV who were identified between 1986 and 1999. Supplementary information on clinical parameters was collected retrospectively from medical charts and databases.Median age at follow-up was 16.1 years (range 5.6 to 34.4 years), and 71% were male. The R-N fusion (n = 108) was strongly predictive of valve intervention when compared with The R-L fusion (n = 202; hazard ratio 4.5, 95% confidence interval [CI] 2.5 to 8.1; p < 0.0001). In a longitudinal analysis of 799 echocardiograms, R-N fusion also was associated with a greater progression of valve dysfunction. This was true for both increasing aortic valve gradient (generalized estimating equations [GEE] risk ratio 27.2, 95% CI 1.2 to 619.6, p = 0.0386) and aortic regurgitation (GEE risk ratio 2.4, 95% CI 1.3 to 4.3, p = 0.0029).The morphology of BAV is predictive of clinically important end points. The R-N fusion is associated with a more rapid progression of aortic stenosis and regurgitation and a shorter time to valve intervention.

    View details for DOI 10.1016/j.jacc.2007.01.090

    View details for Web of Science ID 000246925400010

    View details for PubMedID 17543642

  • Transvenous pacing leads and systemic thromboemboli in patients with intracardiac shunts - A multicenter study CIRCULATION Khairy, P., Landzberg, M. J., Gatzoulis, M. A., Mercier, L., Fernandes, S. M., Cote, J., Lavoie, J., Fournier, A., Guerra, P. G., Frogoudaki, A., Walsh, E. P., Dore, A. 2006; 113 (20): 2391-2397


    The risk of systemic thromboemboli associated with transvenous leads in the presence of an intracardiac shunt is currently unknown.To define this risk, we conducted a multicenter, retrospective cohort study of 202 patients with intracardiac shunts: Sixty-four had transvenous leads (group 1), 56 had epicardial leads (group 2), and 82 had right-to-left shunts but no pacemaker or implantable cardioverter defibrillator leads (group 3). Patient-years were accrued until the occurrence of systemic thromboemboli or study termination. Censoring occurred in the event of complete shunt closure, death, or loss to follow-up. Mean ages for groups 1, 2, and 3 were 33.9+/-18.0, 22.2+/-12.6, and 22.9+/-15.0 years, respectively. Respective oxygen saturations were 91.2+/-9.1%, 88.1+/-8.1%, and 79.7+/-6.7%. During respective median follow-ups of 7.3, 9.3, and 17.0 years, 24 patients had at least 1 systemic thromboembolus: 10 (15.6%), 5 (8.9%), and 9 (11.0%) in groups 1, 2, and 3, respectively. Univariate risk factors were older age (hazard ratio [HR], 1.05; P=0.0001), ongoing phlebotomy (HR, 3.1; P=0.0415), and an transvenous lead (HR, 2.4; P=0.0421). In multivariate, stepwise regression analyses, transvenous leads remained an independent predictor of systemic thromboemboli (HR, 2.6; P=0.0265). In patients with transvenous leads, independent risk factors were older age (HR, 1.05; P=0.0080), atrial fibrillation or flutter (HR, 6.7; P=0.0214), and ongoing phlebotomy (HR, 14.4; P=0.0349). Having had aspirin or warfarin prescribed was not protective. Epicardial leads were, however, associated with higher atrial (P=0.0407) and ventricular (P=0.0270) thresholds and shorter generator longevity (HR, 1.9; P=0.0176).Transvenous leads incur a >2-fold increased risk of systemic thromboemboli in patients with intracardiac shunts.

    View details for DOI 10.1161/CIRCULATIONAHA.106.622076

    View details for Web of Science ID 000237710700008

    View details for PubMedID 16702467

  • Pregnancy outcomes in women with congenital heart disease CIRCULATION Khairy, P., Ouyang, D. W., Fernandes, S. M., Lee-Parritz, A., Economy, K. E., Landzberg, M. J. 2006; 113 (4): 517-524


    Pregnant women with congenital heart disease are at increased risk for cardiac and neonatal complications, yet risk factors for adverse outcomes are not fully defined.Between January 1998 and September 2004, 90 pregnancies at age 27.7+/-6.1 years were followed in 53 women with congenital heart disease. Spontaneous abortions occurred in 11 pregnancies at 10.8+/-3.7 weeks, and 7 underwent elective pregnancy termination. There were no maternal deaths. Primary maternal cardiac events complicated 19.4% of ongoing pregnancies, with pulmonary edema in 16.7% and sustained arrhythmias in 2.8%. Univariate risk factors included prior history of heart failure (odds ratio [OR], 15.5), NYHA functional class > or =2 (OR, 5.4), and decreased subpulmonary ventricular ejection fraction (OR, 7.7). Independent predictors were decreased subpulmonary ventricular ejection fraction and/or severe pulmonary regurgitation (OR, 9.0) and smoking history (OR, 27.2). Adverse neonatal outcomes occurred in 27.8% of ongoing pregnancies and included preterm delivery (20.8%), small for gestational age (8.3%), respiratory distress syndrome (8.3%), intraventricular hemorrhage (1.4%), intrauterine fetal demise (2.8%), and neonatal death (1.4%). A subaortic ventricular outflow tract gradient >30 mm Hg independently predicted an adverse neonatal outcome (OR, 7.5). Cardiac risk assessment was improved by including decreased subpulmonary ventricular systolic function and/or severe pulmonary regurgitation (OR, 10.3) in a previously proposed risk index developed in pregnant women with acquired and congenital heart disease.Maternal cardiac and neonatal complication rates are considerable in pregnant women with congenital heart disease. Patients with impaired subpulmonary ventricular systolic function and/or severe pulmonary regurgitation are at increased risk for adverse cardiac outcomes.

    View details for DOI 10.1161/CIRCULATIONAHA.105.589655

    View details for Web of Science ID 000235031600012

    View details for PubMedID 16449731

  • Morphology of bicuspid aortic valve in children and adolescents JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY Fernandes, S. M., Sanders, S. P., Khairy, P., Jenkins, K. J., Gauvreau, K., Lang, P., Simonds, H., Colan, S. D. 2004; 44 (8): 1648-1651


    The aim of this study was to determine the relationship between aortic valve morphology and valve dysfunction.The morphology of the bicuspid or bicommissural aortic valve (BAV) may predict the severity of valve dysfunction. Therefore, we assessed the relationship between BAV, aortic coarctation, and the degree of valve pathology in children.A retrospective review of 1,135 patients with BAV who were identified between 1986 and 1999 was performed. Patients younger than 18 years of age with BAV that was identifiable via echocardiography were included. The most recent or last study of each patient before intervention or endocarditis was reviewed. Mild stenosis was defined as a valve gradient > or =2 m/s, moderate or greater aortic stenosis as > or =3.5 m/s. Aortic regurgitation was quantified using standard criteria.Median age was 3 years (range, 1 day to 17.9 years), and 67% of the patients were male. Right-coronary and left-coronary leaflet fusion were the most common types of BAV (70%). Aortic stenosis that was moderate or greater was observed most often in patients with right-coronary and non-coronary leaflet fusion (odds ratio 2.4, 95% confidence interval 1.6 to 3.6; p < or = 0.001). Similarly, right-coronary and non-coronary leaflet fusion was more often associated with moderate aortic regurgitation or greater (odds ratio 2.4, 95% confidence interval 1.2 to 4.7; p = 0.01). The majority of patients with aortic coarctation had fusion of the right-coronary and left-coronary leaflets (89%), and aortic coarctation was associated with lesser degrees of valve stenosis or regurgitation.Analysis of BAV morphology is of clinical and prognostic relevance. Fusion of the right-coronary and non-coronary leaflets was associated with more significant valve pathology, whereas fusion of the right-coronary and left-coronary leaflets was associated overwhelmingly with aortic coarctation and less aortic valve pathology.

    View details for DOI 10.1016/j.jacc.2004.05.063

    View details for Web of Science ID 000224573700016

    View details for PubMedID 15489098

  • Usefulness of epoprostenol therapy in the severely ill adolescent/adult with Eisenmenger physiology AMERICAN JOURNAL OF CARDIOLOGY Fernandes, S. M., Newburger, J. W., Lang, P., Pearson, D. D., Feinstein, J. A., Gauvreau, K., Landzberg, M. J. 2003; 91 (5): 632-635
  • Balloon pulmonary angioplasty for treatment of chronic thromboembolic pulmonary hypertension CIRCULATION Feinstein, J. A., Goldhaber, S. Z., Lock, J. E., Ferndandes, S. R., Landzberg, M. J. 2001; 103 (1): 10-13


    Although pulmonary thromboendarterectomy is increasingly successful for the definitive treatment of chronic thromboembolic pulmonary hypertension (CTEPH), not all patients have surgically accessible disease. Others are poor surgical candidates because of comorbid illness. Therefore, for selected patients, we defined and implemented an alternative interventional strategy of balloon pulmonary angioplasty (BPA).Eighteen patients (mean age, 51.8 years; range, 14 to 75 years) with CTEPH underwent BPA; they averaged 2.6 procedures (range, 1 to 5) and 6 dilations (range, 1 to 12). Selection of pulmonary artery segments for dilation required (1) complete occlusion, (2) filling defects, or (3) signs of intravascular webs. After an average of 36 months of follow-up (range, 0.5 to 66 months), the average New York Heart Association class improved from 3.3 to 1.8 (P:<0.001), and 6-minute walking distances increased from 209 to 497 yards (P:<0.0001). Pulmonary artery mean pressures decreased from 43.0+/-12.1 to 33. 7+/-10.2 mm Hg (P:=0.007). Eleven patients developed reperfusion pulmonary edema; 3 required mechanical ventilation.BPA reduces pulmonary artery hypertension in patients with CTEPH and is associated with long-term improvement in New York Heart Association class and 6-minute walking distances. BPA is a promising interventional technique that warrants randomized comparison with medical therapy in CTEPH patients who are not surgical candidates.

    View details for Web of Science ID 000166287300006

    View details for PubMedID 11136677