Infants with an unbalanced atrioventricular septal defect (AVSD) frequently present with comorbidities that may have an impact on their medical course and outcome. This study aimed to assess outcomes and explore possible prognostic indicators for patients undergoing surgical palliation for an unbalanced AVSD. The medical records of all infants presenting to the authors' institution with an unbalanced AVSD over a 5-year period were retrospectively reviewed for assessment of outcomes and comorbidities. The study group consisted of 44 patients with an overall survival rate of 51% for the entire follow-up period. The majority of these patients (88%) underwent single-ventricle palliation, with an 83% rate of survival to initial hospital discharge and an overall long-term survival rate of 50%. The midterm outcome was significantly worse than that for a cohort of hypoplastic left heart syndrome patients undergoing single-ventricle palliation during the same period (P = 0.03). In addition, 30% of the patients required either repair or replacement of their systemic atrioventricular valve at initial palliation or during subsequent follow-up evaluation. Of the patients with an unbalanced AVSD, 75% had associated congenital anomalies. In conclusion, infants with an unbalanced AVSD are a high-risk population with diminished midterm survival compared with palliated patients who have more classic forms of hypoplastic heart syndromes. This may be due to the higher incidence of both severe atrioventricular valve regurgitation and important associated congenital anomalies.

Symptomatic neonates with Ebstein anomaly requiring no intervention or shunting alone have good long-term survival. For patients needing intervention on the tricuspid valve, overall survival is lower. For these patients, right ventricular exclusion may be superior to tricuspid valve repair.

In most cases, careful evaluation by fetal echocardiography can determine essential anatomic details in fetuses with DORV with enough accuracy to allow for accurate counseling regarding the type of surgery needed. Survival is better than previously reported but is poor in patients with extracardiac or chromosomal abnormalities.

Thrombus formation in the native aortic root is a rare but potentially life-threatening complication in patients with hypoplastic left heart syndrome. The native aortic root in these patients serves as a conduit for the retrograde filling of the coronary arteries. Thrombus in this location could result in myocardial ischemia. We present three cases of native aortic root thrombosis at our institution with clinical presentations ranging from asymptomatic to sudden cardiac arrest. Our experience with these patients argues for careful and consistent evaluation of the native aortic root regardless of clinical symptoms.