Single Ventricle Reconstruction Trial

Hypoplastic left heart syndrome, which occurs in 1 per 5000 live births, refers to a family of cardiac defects that are characterized by hypoplasia of the structures making up the left side of the circulation, including the mitral and aortic valves, the left atrium and left ventricle, and the aorta. Infants with this syndrome typically undergo a 3-stage surgical palliation. Without this surgery, the defect is lethal. Since the report of the first Norwood procedure1 for hypoplastic left heart syndrome by Dr William Norwood in 1981, results from treatment of hypoplastic left heart syndrome have improved considerably. In a large single-center report, operative mortality associated with the first-stage procedure was reduced from 59% to 19% between 1990 and 2001.2 Nonetheless, the complete palliative sequence entails 3 surgeries and has considerable interstage mortality, so the overall survival for patients with hypoplastic left heart syndrome remains poor.Article see p 2013In 2003, a major modification of the classic Norwood stage 1 procedure was introduced, substituting a shunt from the right ventricle to the pulmonary artery (RVPA) for the traditional modified Blalock-Taussig shunt (MBT) that carries blood from the brachiocephalic artery to the pulmonary artery.3 There are several theoretical advantages to the RVPA shunt, stemming from the absence of diastolic blood flow from the systemic circulation to the pulmonary circulation. These include increased coronary flow, enhanced postoperative stability, and reduced interstage mortality. …