Tailored strategy to match anatomy and physiology with intervention can improve outcomes of symptomatic neonates with Ebstein anomaly.

Objective: Neonatal presentation of Ebstein anomaly (EA) represents the most severe form of this condition. Despite significant advances, operative mortality remains high and management decisions represent a formidable challenge. We used a strategy aimed to match anatomy and physiology with type and time of intervention to increase survival. Methods: We performed a review of all patients with fetal or neonatal diagnosis of EA managed at a single center between 2007 and 2020. Results: Among 18 patients with EA, 8 underwent neonatal intervention. The most common indication included cyanosis and heart failure (8/8), end organ dysfunction (6/8), and maldistribution of cardiac output (6/8). Only 2/8 had antegrade pulmonary blood flow. Associated conditions included pulmonary regurgitation in 4/8, atrial tachyarrhythmia in 4, and a ventricular septal defect in 3. Three patients underwent initial stabilization with main pulmonary artery occlusion including bilateral pulmonary artery banding in 2. Five patients underwent biventricular repair with conversion to right ventricle exclusion in 2 cases. Three others underwent the Starnes procedure as initially planned. The median age at surgery was 10 days (range, 1-30) and median weight 2.6 kg (range, 1.9-4.0). The median duration of mechanical ventilation and intensive care unit stay were 9 days (range, 5-34) and 30 days (range, 15-100), respectively. Operative mortality was 1/8. At a median follow-up of 130 months (range, 5-146), there were no late deaths, and all survivors remain in functional class I and free of valvular reintervention. Conclusions: Symptomatic neonates with EA can be effectively managed with good outcomes. Preoperative stabilization and choice of management pathway on the basis of anatomy and physiology can help reduce morbidity and mortality.

Hybrid palliation for critical systemic outflow obstruction: neither rapid stage 1 Norwood nor comprehensive stage 2 mitigate consequences of early risk factors.

OBJECTIVE: Hybrid palliation with branch pulmonary artery banding (bPAB) has become increasingly common in the early management of patients with critical left ventricular outflow obstruction. Optimal subsequent surgical palliation remains undefined. METHODS: We retrospectively reviewed patients undergoing initial bPAB for single ventricle physiology with systemic outflow obstruction (2001-2013, n = 37). Patients were stratified by subsequent surgical palliation: stage 1 Norwood (St1N, n = 14), comprehensive stage 2 (CompSt2, n = 11), and none (n = 12). RESULTS: bPAB was performed at a median of 4 days and 2.7-kg, post-bPAB mortality was increased in patients with aortic atresia (odds ratio [OR] = 3.8, 95% confidence interval [CI] = 0.9-15.8) or birth weight <2 kg (OR = 13.8, 95% CI = 1.4-136.4). Palliation strategy did not affect transplant-free survival through second-stage palliation (St1N: 71.4%, CompSt2: 72.7%, P = .9). Among CompSt2 patients, there was a trend toward poorer survival with aortic atresia (0% vs 80%, P = .09); birth weight <2.5 kg was associated with decreased survival (0% vs 89.0%, P = .01). A trend toward lower survival with low birth weight was evident among St1N patients (<2 kg, OR = 0.1, 95% CI = 0.01-1.9, P = .09). CompSt2 mortality occurred on postoperative days 0 and 1. Mortality following St1N occurred at a median of 38.5 days (range = 23.5-104.5). Among survivors of stage 2 palliation, Fontan completion was performed in the same number of patients in each group (St1N: 6/8, 75%, CompSt2: 6/8, 75%). CONCLUSIONS: Both St1N and CompSt2 are viable options for subsequent palliation following initial hybrid procedure. Transplant-free survival and eventual Fontan candidacy are similar between groups. Delaying surgical palliation with the CompSt2 did not mitigate the impact of early risk factors such as low birth weight and aortic atresia.

Cone reconstruction and ventricular septal defect closure for neonatal Ebstein's anomaly.

Neonatal presentation of Ebstein's anomaly is associated with significant morbidity and mortality. We illustrate the technical aspects of the cone procedure and the closure of a ventricular septal defect, to achieve biventricular repair in a neonate. This includes the assessment of the leaftlet apparatus, detachment, division of chordae, annular plication, leaflet rotation and reinsertion in the neoannulus.

Bailout after failed biventricular management of critical aortic stenosis: another application of the hybrid approach.

The decision between a single-ventricle and biventricular strategy can be particularly difficult in patients with critical left ventricular outflow obstruction who have marginal left ventricular size or function. Overzealous pursuit of a biventricular strategy in borderline cases can lead to death or require conversion to a single-ventricle physiology at increased risk. We describe novel use of the hybrid approach as a bailout option for a patient with critical aortic stenosis who, despite balloon valvuloplasty, exhibited persistent severe left ventricular dysfunction. This approach provides a simple and effective pathway to treat patients with a failed biventricular circulation, at a considerably lower risk, while keeping all therapeutic options open.