The Natural and Unnatural History of Ventricular Septal Defects Presenting in Infancy: An Echocardiography-Based Review.
Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography
Younger age remains a risk factor for prolonged length of stay after bidirectional cavopulmonary anastomosis.
Cardiology in the young
BACKGROUND: Ventricular septal defect (VSD), the most common congenital heart defect, accounts for 40% of heart malformations. Despite this prevalence, there remains no consensus on the utility of echocardiography to guide modern-era treatment. In this study, we evaluated patients with isolated VSDs to test the hypothesis that echocardiographic evidence of left ventricular (LV) volume overload and type of VSD are associated with surgical intervention and to identify useful echocardiographic indicators for management of VSDs in infants and children.METHODS: We reviewed 350 patients with VSDs diagnosed during the first year of life. Echocardiographic measurements were made at the time of diagnosis and at the endpoint. The VSD area was calculated using inner edge to inner edge dimensions obtained from two planes and indexed to body surface area. Aortic annulus dimension, left atrium to aortic root ratio, LV end-diastolic diameter, left atrial volume, VSD velocity-time integral, ejection fraction, and pulmonary to systemic blood flow ratio (Qp:Qs) were measured using conventional methods.RESULTS: One hundred seventy-seven muscular (50.5%) and 162 perimembranous (46%) VSDs accounted for the vast majority of defects. Only seven (4%) muscular defects required surgical closure, while 76 (47%) perimembranous defects required surgery. Indexed VSD area, VSD to aortic valve ratio, indexed left atrium volume, LV end-diastolic diameter, VSD velocity-time integral, and Qp:Qs at diagnosis were significantly different between the surgical and nonsurgical groups. Ventricular septal defect area > 50mm2/m2 at initial diagnosis was independently associated with risk for surgery (P=.0055).CONCLUSIONS: Indexed VSD area is an echocardiographic variable that can be easily measured at diagnosis and can provide insight into the likelihood of requiring surgical intervention regardless of the type and location of the defect.
View details for DOI 10.1016/j.echo.2020.01.013
View details for PubMedID 32249125
Diagnostic errors in paediatric cardiac intensive care
CARDIOLOGY IN THE YOUNG
2018; 28 (5): 675–82
OBJECTIVE: This study sets out to determine the influence of age at the time of surgery as a risk factor for post-operative length of stay after bidirectional cavopulmonary anastomosis.METHODS: All patients undergoing a Glenn procedure between January 2010 and July 2015 were included in this retrospective cohort study. Demographic data were examined. Standard descriptive statistics was used. A univariable analysis was conducted using the appropriate test based on data distribution. A propensity score for balancing the group difference was included in the multi-variable analysis, which was then completed using predictors from the univariable analysis that achieved significance of p<0.1.RESULTS: Over the study period, 50 patients met the inclusion criteria. Patients were separated into two cohorts of ⩾4 months (28 patients) and <4 months (22 patients). Other than height and weight, the two cohorts were indistinguishable in their pre-operative saturation, medications, catheterisation haemodynamics, atrioventricular valve regurgitation, and ventricular function. After adjusting group differences, younger age was associated with longer post-operative length of hospitalisation - adjusted mean 15 (±2.53) versus 8 (±2.15) days (p=0.03). In a multi-variable regression analysis, in addition to ventricular dysfunction (beta coefficient=8.8, p=0.05), Glenn procedures performed before 4 months were independently associated with longer length of stay (beta coefficient=-6.9, p=0.03).CONCLUSION: We found that Glenn procedures performed after 4 months of age had shorter post-operative length of stay when compared to a younger cohort. These findings suggest that balancing timing of surgery to decrease the inter-stage period should take into consideration differences in post-operative recovery with earlier operations.
View details for PubMedID 30698131
Pulmonary reinterventions after complete unifocalization and repair in infants and young children with tetralogy of Fallot with major aortopulmonary collaterals
JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY
2018; 155 (4): 1696–1707
IntroductionDiagnostic errors cause significant patient harm and increase costs. Data characterising such errors in the paediatric cardiac intensive care population are limited. We sought to understand the perceived frequency and types of diagnostic errors in the paediatric cardiac ICU.Paediatric cardiac ICU practitioners including attending and trainee physicians, nurse practitioners, physician assistants, and registered nurses at three North American tertiary cardiac centres were surveyed between October 2014 and January 2015.The response rate was 46% (N=200). Most respondents (81%) perceived that diagnostic errors harm patients more than five times per year. More than half (65%) reported that errors permanently harm patients, and up to 18% perceived that diagnostic errors contributed to death or severe permanent harm more than five times per year. Medication side effects and psychiatric conditions were thought to be most commonly misdiagnosed. Physician groups also ranked pulmonary overcirculation and viral illness to be commonly misdiagnosed as bacterial illness. Inadequate care coordination, data assessment, and high clinician workload were cited as contributory factors. Delayed diagnostic studies and interventions related to the severity of the patient's condition were thought to be the most commonly reported process breakdowns. All surveyed groups ranked improving teamwork and feedback pathways as strategies to explore for preventing future diagnostic errors.Paediatric cardiac intensive care practitioners perceive that diagnostic errors causing permanent harm are common and associated more with systematic and process breakdowns than with cognitive limitations.
View details for PubMedID 29409553
Programmatic Approach to Management of Tetralogy of Fallot With Major Aortopulmonary Collateral Arteries A 15-Year Experience With 458 Patients
2017; 10 (4)
Our institutional approach to tetralogy of Fallot (TOF) with major aortopulmonary collaterals (MAPCAs) emphasizes unifocalization and augmentation of the reconstructed pulmonary arterial (PA) circulation and complete intracardiac repair in infancy, usually in a single procedure. This approach yields a high rate of complete repair with excellent survival and low right ventricular (RV) pressure. However, little is known about remodeling of the unifocalized and reconstructed pulmonary circulation or about reinterventions on the reconstructed PAs or the RV outflow tract conduit.We reviewed patients who underwent complete repair of TOF with MAPCAs at our center at <2 years of age, either as a single-stage procedure or after previous procedures. Outcomes included freedom from conduit or PA intervention after repair, which were assessed by Cox regression and Kaplan-Meier analysis.The study cohort included 272 patients. There were 6 early deaths and a median of follow-up of 3.6 years after complete repair. Reinterventions on the pulmonary circulation were performed in 134 patients, including conduit interventions in 101 patients, branch PA interventions in 101, and closure of residual MAPCAs in 9. The first conduit reintervention consisted of surgical conduit replacement in 77 patients, transcatheter pulmonary valve replacement with a Melody valve in 14, and angioplasty or bare metal stenting in 10. Surgical PA reinterventions were performed in 46 patients and transcatheter reinterventions in 75 (both in 20). Most PA reinterventions involved a single lung, and most transcatheter reinterventions a single vessel. Freedom from conduit replacement or transcatheter pulmonary valve replacement was 70 ± 3% at 5 years and was shorter in patients with smaller initial conduit size. Freedom from any PA reintervention was 64 ± 3% at 5 years, with the greatest rate during the first year. On multivariable analysis, factors associated with longer freedom from any PA reintervention included lower postrepair RV:aortic pressure ratio and larger original conduit size.We were able to obtain follow-up data for the majority of patients, which demonstrated freedom from PA reintervention for two thirds of patients. The time course of and risk factors for conduit reintervention in this cohort appeared similar to previously reported findings in patients who received RV-PA conduits in early childhood for other anomalies. Relative to the severity of baseline pulmonary vascular anatomy in TOF with MAPCAs, reinterventions on the reconstructed PAs were uncommon after repair according to our approach, and major reinterventions were rare. Nevertheless, PA reinterventions are an important aspect of the overall management strategy.
View details for PubMedID 29352588
Acute Kidney Injury in Patients Undergoing the Extracardiac Fontan Operation With and Without the Use of Cardiopulmonary Bypass
PEDIATRIC CRITICAL CARE MEDICINE
2017; 18 (1): 34-43
Tetralogy of Fallot with major aortopulmonary collateral arteries is a complex and heterogeneous condition. Our institutional approach to this lesion emphasizes early complete repair with the incorporation of all lung segments and extensive lobar and segmental pulmonary artery reconstruction.We reviewed all patients who underwent surgical intervention for tetralogy of Fallot and major aortopulmonary collateral arteries at Lucile Packard Children's Hospital Stanford (LPCHS) since November 2001. A total of 458 patients underwent surgery, 291 (64%) of whom underwent their initial procedure at LPCHS. Patients were followed for a median of 2.7 years (mean 4.3 years) after the first LPCHS surgery, with an estimated survival of 85% at 5 years after first surgical intervention. Factors associated with worse survival included first LPCHS surgery type other than complete repair and Alagille syndrome. Of the overall cohort, 402 patients achieved complete unifocalization and repair, either as a single-stage procedure (n=186), after initial palliation at our center (n=74), or after surgery elsewhere followed by repair/revision at LPCHS (n=142). The median right ventricle:aortic pressure ratio after repair was 0.35. Estimated survival after repair was 92.5% at 10 years and was shorter in patients with chromosomal anomalies, older age, a greater number of collaterals unifocalized, and higher postrepair right ventricle pressure.Using an approach that emphasizes early complete unifocalization and repair with incorporation of all pulmonary vascular supply, we have achieved excellent results in patients with both native and previously operated tetralogy of Fallot and major aortopulmonary collateral arteries.
View details for DOI 10.1161/CIRCINTERVENTIONS.116.004952
View details for PubMedID 28356265
Use of a Checklist and Clinical Decision Support Tool Reduces Laboratory Use and Improves Cost.
2016; 137 (1): 1-8
To describe the prevalence and risk factors for acute kidney injury in patients undergoing the extracardiac Fontan operation with and without cardiopulmonary bypass, and to determine whether acute kidney injury is associated with duration of mechanical ventilation, cardiovascular ICU and hospital postoperative length of stay, and early mortality.Single-center retrospective cohort study.Pediatric cardiovascular ICU, university-affiliated children's hospital.Patients with a preoperative creatinine before undergoing first-time extracardiac Fontan between January 1, 2004, and April 30, 2012.None.Acute kidney injury occurred in 55 of 138 patients (39.9%), including 41 (29.7%) with stage 1, six (4.4%) with stage 2, and eight (5.8%) with stage 3 acute kidney injury. Cardiopulmonary bypass was strongly associated with a higher risk of any acute kidney injury (adjusted odds ratio, 4.8 [95% CI, 1.4-16.0]; p = 0.01) but not stage 2/3 acute kidney injury. Lower renal perfusion pressure on the day of surgery (postoperative day, 0) was associated with a higher risk of stage 2/3 acute kidney injury (adjusted odds ratio, 1.2 [95% CI, 1.0-1.5]; p = 0.03). Higher vasoactive-inotropic score on postoperative day 0 was associated with a higher risk for stage 2/3 acute kidney injury (adjusted odds ratio, 1.9 [95% CI, 1.0-3.4]; p = 0.04). Stage 2/3 acute kidney injury was associated with longer cardiovascular ICU length of stay (mean, 7.3 greater d [95% CI, 3.4-11.3]; p < 0.001) and hospital postoperative length of stay (mean, 6.4 greater d [95% CI, 0.06-12.5]; p = 0.04).Postoperative acute kidney injury in patients undergoing the extracardiac Fontan operation is common and is associated with lower postoperative renal perfusion pressure and higher vasoactive-inotropic score. Cardiopulmonary bypass was strongly associated with any acute kidney injury, although not stage 2/3 acute kidney injury. Stage 2/3 acute kidney injury is a compelling risk factor for longer cardiovascular ICU and hospital postoperative length of stay. Increased attention to and management of renal perfusion pressure may reduce postoperative acute kidney injury and improve outcomes.
View details for DOI 10.1097/PCC.0000000000000984
View details for Web of Science ID 000392249300005
Expanding the Phenotype of Cardiovascular Malformations in Adams-Oliver Syndrome
AMERICAN JOURNAL OF MEDICAL GENETICS PART A
2013; 161A (6): 1386-1389
We hypothesized that a daily rounding checklist and a computerized order entry (CPOE) rule that limited the scheduling of complete blood cell counts and chemistry and coagulation panels to a 24-hour interval would reduce laboratory utilization and associated costs.We performed a retrospective analysis of these initiatives in a pediatric cardiovascular ICU (CVICU) that included all patients with congenital or acquired heart disease admitted to the cardiovascular ICU from September 1, 2008, until April 1, 2011. Our primary outcomes were the number of laboratory orders and cost of laboratory orders. Our secondary outcomes were mortality and CVICU and hospital length of stay.We found a reduction in laboratory utilization frequency in the checklist intervention period and additional reduction in the CPOE intervention period [complete blood count: 31% and 44% (P < .0001); comprehensive chemistry panel: 48% and 72% (P < .0001); coagulation panel: 26% and 55% (P < .0001); point of care blood gas: 43% and 44% (P < .0001)] compared with the preintervention period. Projected yearly cost reduction was $717, 538.8. There was no change in adjusted mortality rate (odds ratio 1.1, 95% confidence interval 0.7-1.9, P = .65). CVICU and total length of stay (days) was similar in the pre- and postintervention periods.Use of a daily checklist and CPOE rule reduced laboratory resource utilization and cost without adversely affecting adjusted mortality or length of stay. CPOE has the potential to hardwire resource management interventions to augment and sustain the daily checklist.
View details for DOI 10.1542/peds.2014-3019
View details for PubMedID 26681782
We describe a newborn with a phenotype consistent with Adams-Oliver syndrome and truncus arteriosus. Although cardiovascular malformations associated with this syndrome have been previously published in the literature, this is the first description of truncus arteriosus in a patient with Adams-Oliver syndrome. We review other reports of Adams-Oliver syndrome previously described with cardiovascular malformations, consider possible genetic and embryologic mechanisms, and emphasize the need for cardiology consultation when a diagnosis of Adams-Oliver syndrome is suspected in the differential diagnosis. © 2013 Wiley Periodicals, Inc.
View details for DOI 10.1002/ajmg.a.35864
View details for Web of Science ID 000320649700021