Two-Ventricle Repair for Hypoplastic Left Heart Syndrome

Abstract

Hypoplastic left heart syndrome encompasses a spectrum of structural cardiac malformations characterized by severe underdevelopment of the structures in the left heart-aorta complex. The severe end of the spectrum consists of aortic atresia and mitral atresia, whereas at the mild end patients have aortic valve and mitral valve hypoplasia without intrinsic valve stenosis, recently described as hypoplastic left heart complex. These patients may be candidates for biventricular repair. Since November 1988, 12 of 13 patients (92%) with hypoplastic left heart complex have undergone two-ventricle repair and one patient the Norwood operation. Median age at surgery was 7 days (5 to 45 days) and median weight was 3.59 kg (2.6 to 4.3 kg). Biventricular repair consisted of pulmonary homograft patch aortoplasty of the aortic arch and ascending aorta and closure of the interatrial and interventricular communications. The left heart was able to support the systemic circulation in 11 of 12 patients undergoing biventricular repair (92%). The early mortality was two patients (15.4%). The mean follow-up was 67 months (range, 11 months to 133 months). There was one late death at 39 months. Reoperations were necessary in four patients for left ventricular outflow tract obstruction and in two patients for recurrent coarctation. We have successfully achieved biventricular repair in most patients with hypoplastic left heart complex, a subset of patients with hypoplastic left heart syndrome. Reoperation, particularly for left ventricular outflow tract obstruction, appears likely. Finally, with increased experience, predictive criteria for the feasibility and optimal outcome of biventricular repair will be more accurately defined.