Objectives: We sought to determine anatomic features associated with evidence of myocardial ischemia and sudden cardiac events (arrest or death) for patients with anomalous aortic origin of a coronary artery.Methods: We enrolled 560 patients, less than or equal to 30 years, at diagnosis from 40 institutions. Ischemia was defined as the presence of exertional syncope, a sudden cardiac event (arrest/death), or abnormal investigation results. Data on detailed anatomic features were abstracted from echocardiography, computed tomography, magnetic resonance imaging, operative, and/or surgeon-completed reports.Results: There were 236 patients with negative ischemia test results, and 49 with evidence of ischemia (including 18 who presented with a sudden cardiac event); 275 asymptomatic patients who had not undergone provocative ischemia testing were excluded from primary analyses. Patients with ischemia (vs without), were more likely to have left anomalous coronary arteries (28/49 vs 46/236; P<.0001). Of patients with ischemia (vs without), those with anomalous left coronary arteries were more likely to have an intramural coronary artery course, or a high or slit-like coronary artery orifice. Of patients with ischemia (vs without), those with anomalous right coronary arteries were more likely to have a longer intramural course. Among patients with ischemia, the occurrence of sudden cardiac events was not shown to have any associated anatomic features.Conclusions: Anatomic features including coronary artery involved, intramural course and length, and orifice anomalies were associated with evidence of myocardial ischemia for patients with anomalous aortic origin of a coronary artery. These features might importantly inform risk stratification and decisions regarding surgical management.

No abstract

No abstract

Background. Improved survival for patients with dextro-transposition of the great arteries (d-TGA) has led to an increased focus on functional health status (FHS). We assessed late survival and patient-perceived FHS for repaired TGA patients.Methods. From 1985-1990, 830 neonates admitted to 24 Congenital Heart Surgeons' Society (CHSS) institutions with d-TGA underwent repair, including 516 arterial switch, 110 Mustard, 175 Senning, and 29 Rastelli operations. Median follow-up was 24.0 years (range, 0-32.7 years). We performed multiphase parametric hazard analysis for death after repair. Patients completed Pediatric Quality of Life Inventory (PedsQL) Core Scales and Cardiac Module Adult Forms. Patient and operative factors and CHSS General Questionnaire responses were analyzed for association with FHS using multiple linear regression.Results.

No abstract

No abstract

Objective: Arch obstruction after the Norwood procedure is common and contributes to mortality. We determined the prevalence, associated factors, and practice variability of arch reintervention and assessed whether arch reintervention is associated with mortality.Methods: From 2005 to 2017, 593 neonates in the Congenital Heart Surgeons' Society Critical Left Heart Obstruction cohort underwent a Norwood procedure. Median follow-up was 3.7 years. Multivariable parametric models, including a modulated renewal analysis, were performed.Results: Of the 593 neonates, 146 (25%) underwent 218 reinterventions for arch obstruction after the Norwood procedure: catheter-based (n ¼ 168) or surgical (n ¼ 50) at a median age of 4.3 months (quartile 1-quartile 3, 2.6-5.7). Interdigitation of the distal aortic anastomosis was protective against arch reintervention. Development of ! moderate tricuspid valve regurgitation and right ventricular dysfunction at any point was associated with arch reintervention. Nonsignificant variables for arch reintervention included shunt type and preoperative aortic measurements. Surgical arch reintervention was protective against arch reintervention, but transcatheter reintervention was associated with increased reintervention. Arch reintervention was not associated with increased mortality. There was wide institutional variation in incidence of arch reintervention (range, 0-40 reinterventions per 100 years patient follow-up) and in preintervention gradient (range, 0-64 mm Hg).Conclusions: Interdigitation of the distal aortic anastomosis during the Norwood procedure decreased the risk of arch reintervention. Surgical arch reintervention is n: 0 3 6 9 1 2 Months after Norwood Freedom from Arch Reintervention (%)

No abstract