A 40-Year-Old Man With Tricuspid Atresia, Status Post-Fontan, With Severe COVID-19 Pneumonia and Pneumothorax.
JACC. Case reports
We report a case of COVID-19 in an adult single-ventricle patient post-Fontan-to our knowledge, the first report in this population documenting the use of the latest management recommendations for this novel disease. Additionally, this patient had significant pre-existing ventricular dysfunction, valvular disease, and comorbidities including HIV. (Level of Difficulty: Advanced.).
View details for DOI 10.1016/j.jaccas.2020.10.032
View details for PubMedID 33558861
Comparison of combined heart?liver vs heart-only transplantation in pediatric and young adult Fontan recipients.
The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation
BACKGROUND: Indications for a heart?liver transplantation (HLT) for Fontan recipients are not well defined. We compared listing characteristics, post-operative complications, and post-transplant outcomes of Fontan recipients who underwent HLT with those of patients who underwent heart-only transplantation (HT). We hypothesized that patients who underwent HLT have increased post-operative complications but superior survival outcomes compared with patients who underwent HT.METHODS: We performed a retrospective review of Fontan recipients who underwent HLT or HT at a single institution. Characteristics at the time of listing, including the extent of liver disease determined by laboratory, imaging, and biopsy data, were compared. Post-operative complications were assessed, and the Kaplan?Meier survival method was used to compare post-transplant survival. Univariate regression analyses were performed to identify the risk factors for increased mortality and morbidity among patients who underwent HT.RESULTS: A total of 47 patients (9 for HLT, 38 for HT) were included. Patients who underwent HLT were older, were more likely to be on dual inotrope therapy, and had evidence of worse liver disease. Whereas ischemic time was longer for the group who underwent HLT, post-operative complications were similar. Over a median post-transplant follow-up of 17 (interquartile range: 5-52) months, overall mortality for the cohort was 17%; only 1 patient who underwent HLT died (11%) vs 7 patients who underwent HT (18%) (p?=?0.64). Among patients who underwent HT, cirrhosis on pre-transplant imaging was associated with worse outcomes.CONCLUSIONS: Despite greater inotrope need and more severe liver disease at the time of listing, Fontan recipients undergoing HLT have post-transplant outcomes comparable with those of patients undergoing HT. HLT may offer a survival benefit for Fontan recipients with liver disease.
View details for DOI 10.1016/j.healun.2020.12.008
View details for PubMedID 33485775
Does liver biopsy accurately measure fibrosis in Fontan associated liver disease? A comparison of liver biopsy pre-combined heart and liver transplant and liver explant post-transplant.
The accuracy of liver biopsy to stage fibrosis due to Fontan associated liver disease (FALD) remains unclear. We compared results of biopsy pre-combined heart and liver transplantation (CHLT) to results of whole liver explant. Liver biopsy and explants from 15 Fontan patients (ages 16 - 49, median 28 years) were retrospectively reviewed. Staging was as follows: stage 0: no fibrosis, stage 1: pericellular fibrosis, stage 2: bridging fibrosis, stage 3: regenerative nodules. There is no stage 4. Clinical characteristics including Model of End-stage Liver Disease eXcluding INR and Varices, Ascites, Splenomegaly, and Thrombocytopenia (VAST) scores were collected, and descriptive statistics and Mann-Whitney U tests used to analyze data. All patients had biopsies with at least bridging fibrosis, and all had nodularity on explant; transjugular biopsy never overestimated fibrosis. Explant showed higher grade fibrosis (Stage 3) than pre-CHLT biopsy (Stage 2) in 6 of 15 patients and equal grade of fibrosis (Stage 3) in 9 of 15 patients. Though clinical characteristics varied significantly, VAST score was ? 2 in all but two patients. Transjugular liver biopsy does not overestimate and can underestimate fibrosis in Fontan patients undergoing CHLT, likely due to the patchy nature of fibrosis in FALD.
View details for DOI 10.1111/ctr.14120
View details for PubMedID 33053213
- Short-term outcomes of en bloc combined heart and liver transplantation in the failing Fontan CLINICAL TRANSPLANTATION 2019; 33 (6)
Short-Term Outcomes of en bloc Combined Heart and Liver Transplantation in the Failing Fontan.
Patients with failing Fontan physiology and liver cirrhosis are being considered for combined heart and liver transplantation. We performed a retrospective review of our experience with en bloc combined heart and liver transplantation in Fontan patients > 10 years old from 2006-18 per Institutional Review Board approval. Six females and 3 males (median age 20.7, range 14.2-41.3 years) underwent en bloc combined heart and liver transplantation. Indications for heart transplant included ventricular dysfunction, atrioventricular valve regurgitation, arrhythmia and/or lymphatic abnormalities. Indication for liver transplant included portal hypertension and cirrhosis. Median Fontan/single ventricular end diastolic pressure was 18/12 mm Hg, respectively. Median Model for End-Stage Liver Disease excluding International Normalized Ratio score was 10 (7-26), eight patients had a Varices, Ascites, Splenomegaly, Thrombocytopenia score of>2, and all patients had cirrhosis. Median cardiopulmonary bypass and donor ischemic times were 262 (178-307) and 287 (227-396) minutes, respectively. Median intensive care and hospital stay were 19 (5-96) and 29 (13-197) days, respectively. Survival was 100% and rejection was 0% at 30 days and 1 year post-transplant. En bloc combined heart and liver transplantation is an acceptable treatment in the failing Fontan patient with liver cirrhosis. This article is protected by copyright. All rights reserved.
View details for PubMedID 30891780
Heart failure with reduced and preserved ejection fraction in adult congenital heart disease.
Heart failure reviews
Heart failure with reduced ejection fraction (HFrEF) is common in patients with adult congenital heart disease. Many of the most common congenital defects have a high prevalence of HFrEF, including left-sided obstructive lesions (aortic stenosis, coarctation of the aorta, Shone complex), tetralogy of Fallot, Ebstein anomaly, lesions in which there is a systemic right ventricle, and lesions palliated with a Fontan circulation. However, heart failure with preserved ejection fraction (HFpEF) is also prevalent in all these lesions. Comprehensive evaluation includes physical exam, biomarkers, echocardiography and advanced imaging, exercise stress testing, and, in some cases, invasive hemodynamics. Guideline-directed medical therapy for HFrEF can be applied to left-sided lesions and may be considered on an individual basis for systemic right ventricle and single-ventricle patients. Medical therapy is limited for HFpEF. However, in both HFrEF and HFpEF, ventricular dyssynchrony and arrhythmias play an important role, and medications for rhythm control, ablation, and cardiac resynchronization therapy should be considered. Finally, aggressive management of cardiovascular risk factors and comorbidities, including, but not limited to, hypertension, obesity, diabetes, dyslipidemia, and obstructive sleep apnea, cannot be overemphasized.
View details for DOI 10.1007/s10741-019-09904-z
View details for PubMedID 31873841
Repair of Untreated Older Patients with Tetralogy of Fallot with Major Aortopulmonary Collaterals.
The Annals of thoracic surgery
BACKGROUND: Our programmatic approach to tetralogy of Fallot with major aortopulmonary collaterals emphasizes single-stage unifocalization with complete intracardiac repair during infancy. Little is known about suitability for complete repair in patients beyond infancy. We sought to analyze outcomes of our approach in older patients with previously untreated tetralogy of Fallot with major aortopulmonary collaterals.METHODS: Any patient with this lesion not treated prior to 2 years of age referred to our center from 2002-17 met inclusion criteria.RESULTS: Of 33 patients, 32 were out-of-state (64% international) referrals, and 33% (n=11) were >9 years old, had polycythemia, and/or at least 1 high pressure collateral (>25 mm Hg). Complete repair was achieved in 94% (n=31) of patients, 82% (n=27) in one stage and 12% (n=4) after unifocalization to a central shunt. The median right ventricular to aortic pressure ratio was 0.31 after surgery and 0.37 at follow-up. At a median of 4.8 years after repair, 9 patients (19%) underwent reintervention, including 5 conduit and 7 branch pulmonary artery interventions. Three patients also underwent aortic valve replacement.CONCLUSIONS: In this selected cohort of older patients with previously unoperated tetralogy of Fallot with major aortopulmonary collaterals, outcomes were comparable to infants undergoing treatment according to our approach. These findings support the notion that patients who are either born in low-resource settings or present to healthcare providers beyond infancy should be considered candidates and evaluated for complete repair.
View details for PubMedID 30550802
Cost-effectiveness of Humanitarian Pediatric Cardiac Surgery Programs in Low- and Middle-Income Countries.
JAMA network open
2018; 1 (7): e184707
Endorsement of global humanitarian interventions is based on either proven cost-effectiveness or perceived public health benefits. The cost-effectiveness and long-term benefits of global humanitarian pediatric cardiac surgery are unknown, and funding for this intervention is insufficient.To determine the cost-effectiveness of the intervention (multiple 2-week-long humanitarian pediatric cardiac surgery program assistance trips to various low- and middle-income countries [LMICs]) and to produce a measure of the long-lasting effects of global humanitarian programs.International, multicenter cost-effectiveness analysis of a cohort of children (aged <16 years) undergoing surgical treatment of congenital heart disease during 2015 in LMICs, including China, Macedonia, Honduras, Iran, Iraq, Libya, Nigeria, Pakistan, Russia, and Ukraine. The study also assessed estimated improvement in the United Nations Human Development Indicators (life expectancy, years of schooling, and gross national income) for each individual survivor, as a proxy for long-term benefits of the intervention.The primary outcome was cost-effectiveness of the intervention. The secondary outcomes were potential gains in life expectancy, years of schooling, and gross national income per capita for each survivor.During 2015, 446 patients (192 [43%] female; mean [SD] age, 3.7 [5.4] years) were served in 10 LMICs at an overall cost of $3?210?873. Of them, 424 were children. The cost-effectiveness of the intervention was $171 per disability-adjusted life-year averted. Each survivor in the cohort (390 of 424) potentially gained 39.9 disability-adjusted life-years averted, 3.5 years of schooling, and $159?533 in gross national income per capita during his or her extended lifetime at purchasing power parity and 3% discounting.Humanitarian pediatric cardiac surgery in LMICs is highly cost-effective. It also leaves behind a lasting humanitarian footprint by potentially improving individual development indices.
View details for PubMedID 30646368
Short-term outcomes following implementation of a dedicated young adult congenital heart disease transition program
CONGENITAL HEART DISEASE
2018; 13 (1): 85?91
Transition from pediatric to adult care is a critical time for patients with congenital heart disease. Lapses in care can lead to poor outcomes, including increased mortality. Formal transition clinics have been implemented to improve success of transferring care from pediatric to adult providers; however, data regarding outcomes remain limited. We sought to evaluate outcomes of transfer within a dedicated transition clinic for young adult patients with congenital heart disease.We performed a retrospective analysis of all 73 patients seen in a dedicated young adult congenital heart disease transition clinic from January 2012 to December 2015 within a single academic institution that delivered pediatric and adult care at separate children's and adult hospitals, respectively.Demographic characteristics including congenital heart disease severity, gender, age, presence of comorbidities, presence of cardiac implantable electronic devices, and type of insurance were correlated to success of transfer. Rate of successful transfer was evaluated, and multivariate analysis was performed to determine which demographic variables were favorably associated with transfer.Thirty-nine percent of patients successfully transferred from pediatric to adult services during the study period. Severe congenital heart disease (OR 4.44, 95% CI 1.25-15.79, P = .02) and presence of a cardiac implantable electronic device (OR 4.93, 95% CI 1.18-20.58, P = .03) correlated with transfer. Trends favoring successful transfer with presence of comorbidities and private insurance were also noted.Despite a dedicated transition clinic, successful transfer rates remained relatively low though comparable to previously published rates. Severity of disease and presence of implantable devices correlated with successful transfer. Other obstacles to transfer remain and require combined efforts from pediatric and adult care systems, insurance carriers, and policy makers to improve transfer outcomes.
View details for DOI 10.1111/chd.12549
View details for Web of Science ID 000424113300012
View details for PubMedID 29152906
- Isolated Double Chambered Right Ventricle in an Adult Imaged With Magnetic Resonance. World journal for pediatric & congenital heart surgery 2016; 7 (6): 758?60