Hypoplastic Left Heart Syndrome With Right Ventricle Compression.

Abstract

A male neonate presented with the aortic atresia-mitral stenosis (AA-MS) variant of hypoplastic left heart syndrome (HLHS) wherein the suprasystemic left ventricular (LV) pressure and large left ventricle shifted the intraventricular septum. A compressed right ventricle made it difficult to maintain stroke volume. To avoid high right ventricular (RV) volume load and output, we used a 2-stage Norwood-Glenn (NW-G) procedure. Bilateral pulmonary artery banding (bPAB) was performed on day of life (DOL) 4. On DOL 58, we performed percutaneous balloon dilation of the bPAB portion. At age 3 months, the NW-G procedure was performed without peripheral pulmonary hypoplasia. The patient is currently doing well.