Stephens, Elizabeth H.

Abstract Background Transcatheter closure of patent ductus arteriosus (PDA) in small, premature infants is increasingly performed in the contemporary practice. Data regarding optimal timing of PDA closure are scarce. We therefore decided to compare morbidity and mortality of early and late device closure in preterm infants. Methods Hospitalizations of 166,583 newborn infants diagnosed with PDA were identified from the Kids’ Inpatient Database (KID) in 2009, 2012, 2016, and 2019. Of these, 299 preterm infants (gestational age (GA) ≤32 weeks, birth weight <1500 grams) underwent percutaneous PDA closure. Characteristics and peri-procedural outcomes were compared between early (≤ 30 days) (n=120) and late PDA closure (>30 days) (n=179). Results Mean age (±standard deviation) for early PDA closure was 18.6 days ± 7 and 67.3 days ± 36.5 in late PDA closure. Co-morbidities including respiratory distress syndrome/bronchopulmonary dysplasia, heart failure and pulmonary hypertension were comparable between the two groups. There was no difference in mortality or referral for surgical ligation between early and late closure. Post-procedural vascular complications were low, but higher in patients with early PDA closure (3.3% vs 0%; p=0.025). Length of hospital stay was longer in late PDA closure group (121.1 days ± 51.7 vs 81.5 days ± 48.8; p=<0.01). Conclusion Preterm or very low birth weight infants may benefit from early percutaneous PDA closure as it is associated with a shorter hospital LOS. There was no difference in in-hospital mortality or major periprocedural complications between early and late PDA closure, except vascular complications that were higher with early PDA closure.

Background Quadricuspid aortic valve (QAV) is an exceedingly rare congenital heart defect (CHD) which has not been well-defined in a pediatric population. Methods The Mayo Clinic echocardiography database was retrospectively analyzed to identify patients ≤18 years diagnosed with QAV from January 1990 to December 2023. Patients with truncus arteriosus were excluded. All images were independently reviewed to define morphology of the QAV by using the Hurwitz and Roberts classification. Results Fourteen patients with QAV were identified with a median age at time of diagnosis being 10.5 years (interquartile range [IQR] 6-14 years). Male-to-female ratio was 3:1. Associated CHDs were present in 50% (n = 7) patients. The most common morphological subtypes of QAV were Type D in 43% (n = 6) and Type B in 29% (n = 4). Aortic regurgitation was the most frequently associated valvular abnormality affecting 86% (n = 12) cases, with greater than moderate regurgitation in only two patients. Aortic valve stenosis was observed in 14% (n = 2) patients. Ascending aortic dilatation was present in 21% (3/14) of the cohort, but only 14% (1/7) of isolated QAV patients. At a mean follow up of 11 ± 6.6 years and a median follow-up age of 22 years (IQR 14-27 years), survival was 100% with no primary interventions on the aortic valve or aorta. However, four patients required surgical interventions for associated CHDs. Conclusion Among children with QAV, almost half of the patients had additional CHD. Aortic regurgitation was the predominant hemodynamic abnormality. Long-term survival was excellent with minimal progression during childhood and adolescence.

Background Pulmonary atresia with ventricular septal defect without major aortopulmonary collateral arteries (MAPCAs) is an uncommon form of congenital heart disease. As more patients with congenital heart disease live to adulthood, the objective of this article was to review the long-term results of this specific population. Methods A review of the PubMed database was performed using pertinent key words (pulmonary atresia, tetralogy of Fallot, conduit, right ventricle-pulmonary artery) concentrating on studies from 1990-present and published in English. Most studies of pulmonary atresia-ventricular septal defect patients included those with and without MAPCAs. Analysis included examination of the entire cohort, consideration of the proportion of patients with MAPCAs, and any subgroup analysis of the patients without MAPCAs. Results Survival is approximately 80% at ten years and is improved with complete repair and larger pulmonary arteries. Some studies have found genetic syndromes and extracardiac anomalies to impact survival, while others have not. Incomplete repair has been shown to be associated with worse survival. Independent of initial management strategy, patients with pulmonary atresia and ventricular septal defects without MAPCAs require repeat intervention on the right ventricular outflow tract. Hypoplastic pulmonary arteries have been shown to be a risk factor for reintervention, and decreased conduit durability has been shown with younger age at implantation of conduit. Conclusions Long-term outcomes have improved for patients with pulmonary atresia-ventricular septal defect without MAPCAs, with complete repair and adequate pulmonary arteries favorable for survival. Long-term outcomes include reinterventions, both catheter-based and surgical, predominantly on the right ventricular outflow tract.

Ebstein anomaly is a myopathy of the right ventricle characterized by failure of tricuspid valve delamination with accompanying tricuspid regurgitation. Its rarity, considerable anatomic variability, and frequent absence of symptoms can make the timing of surgery challenging. Contemporary tricuspid repair techniques can be performed with low mortality and bidirectional cavopulmonary anastomosis reduces the risk of operation when right ventricular function is poor. Here, we present a patient who presented late for surgery, failed high-risk conventional surgery and required heart transplantation.

AbstractBackgroundRoutine defibrillation threshold testing (DFT) of transvenous implantable defibrillators (ICDs) has largely been in decline. In patients with non‐transvenous ICDs that utilize subcutaneous and pleural ICD leads, serial DFT testing can detect a significant number of failures. Data about the utility of follow‐up defibrillation safety margin testing (DSM) testing in pediatric patients and young adults with an epicardial ICD are lacking.MethodsPatients aged < 25 years old who underwent epicardial ICD placement at Mayo Clinic from 2014 to 2023 with at least one follow‐up DSM test were included. The patients were divided into a “routine” (R) and “clinically indicated” (CI) group based on the index of clinical concern. Inadequate DSM was defined as unsuccessful defibrillation at an output of less than 10 J below the maximum output of the device. The purpose of this study was to assess the utility of follow‐up DSM testing.ResultsAn epicardial ICD system was placed in 122 patients. A total of 26 patients met inclusion criteria and underwent a total of 47 DSM follow up tests. Inadequate DSM occurred in 1/33 (3%) in the R group and 2/14 (14%) DSM tests in the CI group. The median follow‐up period was 54 and 36 months for the R and CI group, respectively.ConclusionsOur data suggest that epicardial ICDs are reliable and routine follow‐up DSM testing may not be necessary for all patients. DSM testing should be performed in individuals with epicardial ICD systems when there is clinical concern about lead or coil performance.

Background Little is known about outcomes following heart failure (HF) hospitalization among adults with congenital heart disease (CHD) in the United States. We aim to compare the outcomes of HF versus non‐HF hospitalizations in adults with CHD. Methods and Results Using a national deidentified administrative claims data set, patients with adult congenital heart disease (ACHD) hospitalized with and without HF (ACHDHF+, ACHDHF−) were characterized to determine the predictors of 90‐day and 1‐year mortality and quantify the risk of mortality, major adverse cardiac and cerebrovascular events, and health resource use. Cox proportional hazard regression was used to compare ACHDHF+ versus ACHDHF− for risk of events and health resource use. Of 26 454 unique ACHD admissions between January 1, 2010 and December 31, 2020, 5826 (22%) were ACHDHF+ and 20 628 (78%) were ACHDHF−. The ACHD HF+ hospitalizations increased from 6.6% to 14.0% ( P <0.0001). Over a mean follow‐up period of 2.23 ± 2.19 years, patients with ACHDHF+ had a higher risk of mortality (hazard ratio [HR], 1.86 [95% CI, 1.67–2.07], P <0.001), major adverse cardiac and cerebrovascular events (HR, 1.73 [95% CI, 1.63–1.83], P <0.001) and health resource use including rehospitalization (HR, 1.09 [95% CI, 1.05–1.14], P <0.001) and increased postacute care service use (HR, 1.56 [95% CI, 1.32–1.85], P <0.001). Cardiology clinic visits within 30 days of hospital admission were associated with lower 90‐day and 1‐year all‐cause mortality (odds ratio [OR], 0.62 [95% CI, 0.49–0.78], P <0.001; OR, 0.69 [95% CI, 0.58–0.83], P <0.001, respectively). Conclusions HF hospitalization is associated with increased risk of mortality and morbidity with high health resource use in patients with ACHD. Recent cardiology clinic attendance appears to mitigate these risks.

Background: National data about the outcomes of children undergoing mechanical mitral valve replacement (m-MVR) are scarce. Methods: A retrospective review of hospitalizations from the Kids’ Inpatient Database was performed for patients ≤18 years of age in the United States. A total of 500 patients underwent m-MVR in 2009, 2012, 2016, and 2019. Patients with single ventricle physiology were excluded (n = 13). These patients were categorized into three groups according to age: neonates (<1 month, n = 20), infants (1-12 months, n = 76 patients), and children (1-18 years, n = 404). Outcomes were compared between the three groups. Results: The proportion of m-MVR involving children undergoing MV procedures (repair and replacement) has increased from 17.3% in 2009 to 30.8% in 2019 (Ptrend < .01). History of cardiac surgery was present in 256 patients (51.2%). Concomitant procedures were performed in 119 patients (23.8%). Intra- or postoperative extracorporeal membrane oxygenation was required in 19 patients (3.8%). The overall in-hospital mortality was 4.8% and was significantly higher in neonates and infants compared with older children (10% vs 11.8% vs 3.2%, P = .003). The length of hospital stay was longer in the neonatal group (median, 57 days, interquartile range, [24.8-90] vs 29.5 days [15.5-61] vs 10 days [7-18], P < .01). Nonhome discharges were more common in neonates and infants (40% vs 36.8% vs 13.1%, P < .01). Conclusion: Mechanical mitral valve replacement is increasingly performed over time with acceptable in-hospital morbidity and mortality, especially in older children and adolescents. Neonates and infants are associated with worse hospital survival, prolonged hospitalization, and significant rates of nonhome discharges.