Single Ventricle Reconstruction Trial: A Work in Progress
Hypoplastic left heart syndrome, which occurs in 1 per 5,000 live births, refers to a family of cardiac defects which are characterized by hypoplasia of the structures comprising 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 procedure for hypoplastic left heart syndrome by Dr. William Norwood in 1981, results from treatment of hypoplastic left heart syndrome have improved considerably.1 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 entails considerable inter-stage mortality as well, so the overall survival for patients with hypoplastic left heart syndrome remains poor.
- Received March 28, 2014.
- Accepted March 31, 2014.