Anomalous origin of the left coronary artery from the right pulmonary artery.

Anomalous origin of the left coronary artery (LCA) from the pulmonary artery (ALCAPA) is a rare congenital anomaly, one which requires a high index of suspicion in order to be diagnosed promptly and managed accurately. ALCAPA from the right branch pulmonary artery (RPA) is a small subset of ALCAPA in general, with only a few reported cases in the world literature. We report two cases of ALCAPA from the RPA: the first case, an infant, presented in severe cardiac failure requiring preoperative extracorporeal membrane oxygenation (ECMO) for stabilization; and the second, a neonate, presented with severe aortic coarctation (CoA). Both patients underwent successful complete surgical repair using a modified surgical technique, and are doing well in midterm follow-up. We describe our operative technique and review the literature of this rare congenital anomaly.

Individualized approach to the surgical treatment of tetralogy of Fallot with pulmonary atresia.

BACKGROUND: Tetralogy of Fallot with pulmonary atresia is a heterogeneous group of defects, characterised by diverse sources of flow of blood to the lungs, which often include multiple systemic-to-pulmonary collateral arteries. Controversy surrounds the optimal method to achieve a biventricular repair with the fewest operations while basing flow to the lungs on the native intrapericardial pulmonary arterial circulation whenever possible. We describe an individualized approach to this group of patients that optimizes these variables. METHODS: Over a consecutive 10-year period, we treated 66 patients presenting with tetralogy of Fallot and pulmonary atresia according to the source of the pulmonary arterial flow. Patients were grouped according to whether the flow of blood to the lungs was derived exclusively from the intrapericardial pulmonary arteries, as seen in 29 patients, exclusively from systemic-to-pulmonary collateral arteries, as in 5 patients, or from both the intrapericardial pulmonary and collateral arteries, as in the remaining 32 patients. We divided the latter group into 9 patients deemed simple, and 23 considered complex, according to whether the pulmonary arterial index was greater than or less than 90 millimetres squared per metre squared, and whether the number of collateral arteries was less than or greater than 2, respectively. RESULTS: We achieved complete biventricular repair in 58 patients (88%), with an overall mortality of 3%. Repair was accomplished in a single stage in all patients without systemic-to-pulmonary collateral arteries, but was staged, with unifocalization, in the patients lacking intrapericardial pulmonary arteries. Complete repair without unifocalization was achieved in all patients with the simple variant of the mixed morphology, and in 56% of patients with the complex variant. The average number of procedures per patient to achieve complete repair was 1, 2.2, 3.8, and 2.6 in patients with exclusively native intrapericardial, simple and mixed, complex and mixed and exclusively collateral pulmonary arterial flow, respectively. CONCLUSIONS: An individualized approach based on the morphology of the pulmonary arterial supply permits achievement of a high rate of complete intracardiac repairs, basing pulmonary arterial flow on the intrapericardial pulmonary arteries in the great majority of cases, and has a low rate of reoperation and mortality.

Right ventricular aneurysm following modified Norwood-Sano operation for hypoplastic left heart syndrome.

Hypoplastic left heart syndrome is a rare congenital heart defect characterized by underdevelopment of left-sided heart structures, including the aortic arch. The contemporary surgical management of this anomaly includes the Norwood procedure and provision of pulmonary blood flow by either a modified Blalock-Taussig shunt (MBTS) or a right ventricle-to-pulmonary artery (RV-PA) conduit, commonly referred to as the Sano shunt. We report on an unusual complication of the Sano shunt, that of a giant right ventricular pseudoaneurysm occurring at the shunt insertion site.