Prognosis of the hypoplastic left heart syndrome

Experience with 164 patients with the hypoplastic left heart syndrome seen at C.S. Mott Children's Hospital from 1990 through August, 1995 was reviewed to describe the prognosis of this condition in contemporary patients. Norwood palliation was performed in 158 patients with 120 perioperative (30 days) survivors (76%, CL(70) 72-79%). Nonwood survival was significantly (P = 0.0002) better in the 114 patients considered at standard risk: typical hypoplastic left heart syndrome operated at < 1 month of age without severe pulmonary venous obstruction or significant congenital non-cardiac conditions (84%, CL(70) 80-87%). Second stage palliation (hemi-Fontan or bidirectional Glenn shunt) was performed in 89 patients, with 86 survivors (97%, CL(70) 94-98%). Fontan operations were performed in 47 patients, with 38 survivors (81%, CL(70), 74-86%). Actuarial survival for the 164 patient cohort was 78 +/- 3% at 1 month, 63 +/- 5% at 1 year, and 58 +/- 4% from 3 to 5 years of age, with 85 +/- 4%, 80 +/- 5%, and 73 +/- 5% survival in the standard risk group. Significant or potentially significant morbid conditions were reported in 25 of the 120 operative survivors (21%, CL(70) 17-25%), including neurologic conditions in 6%, cardiovascular conditions in 10%, and respiratory conditions in 11%. Prognosis of the hypoplastic left heart syndrome can be improved substantially by a program of surgical palliation. Comprehensive assessment of neurodevelopmental and cardiopulmonary functional status in long-term survivors will be necessary to better define contemporary prognosis in the hypoplastic left heart syndrome.