Hypoplastic left heart syndrome. Outcome after initial reconstruction and before modified Fontan procedure

The outcome and clinical course before modified Fontan procedure were reviewed for 200 patients with hypoplastic left heart syndrome who underwent initial reconstructive surgery between August 1985 and March 1989. The median age at the time of initial reconstruction was 6 days (range, 1 day to 7.2 months). In 28 patients, a right modified Blalock-Taussig shunt was used; in 172 patients, a central shunt was placed. Additional procedures (n = 41) performed in 38 patients (median age, 5 months; range, 6 days to 17.5 months) were revision of systemic-to-pulmonary shunt (n = 15), arch reconstruction (n = 8), balloon angioplasty of arch obstruction (n = 7), atrial septectomy (n = 4), pulmonary artery angioplasty (n = 2), tricuspid valve annuloplasty or replacement (n = 4), and modified Glenn shunt (n = 1). There was no significant difference in the frequency of additional procedures performed more than 30 postoperative days in the survivors compared with the nonsurvivors. Actuarial survival rates were 0.66 (1 month), 0.48 (12 months), and 0.44 (18 months). Seventy percent of all deaths occurred during the initial admission, with 32% resulting from acute cardiovascular collapse during the first postoperative day. There was no statistical difference in actuarial survival when assessed by the type of shunt used or by anatomical subtype or when the influence of additional interventions was considered. Substantial improvement in outcome may be possible if immediate perioperative mortality can be reduced. We speculate that some of the intermediate mortality (30 days to 1 year) may be related to the effects of chronic exposure of the right ventricle to volume overload at systemic pressure.