Results of staged reconstruction for hypoplasia of the left heart: an experience of 12 years from one institution.

BACKGROUND

We reviewed our 12-year experience with staged reconstruction for hypoplasia of the left heart, examining the results of each surgical step and the impact of the year of the Norwood operation on survival. We compared survival of patients with hypoplasia of the left heart subsequent to completion of the Fontan circulation to survival of patients with a dominant left ventricle undergoing a Fontan procedure.

PATIENTS: Between 1989 and 2001, we performed a first stage procedure in 89 patients. Their median age was 9 days, with a range from 2 to 140 days, and the median weight was 3.4 kg, with a range from 2.4 to 5.4 kg.

RESULTS

Survival at 1, 4, and 10 years was 55%, 49%, and 49%, respectively. We experienced 23 early deaths (26%), and 12 deaths between the stages of the Norwood cascade. Of our patients, 42 underwent the second stage, and 30 the third stage. Prior to the first stage, symptoms of necrotising enterocolitis, and of obstructed pulmonary venous return, influenced survival significantly. The latter was eliminated as risk factor when surgery was performed within the first week of life. During the later part of our experience, survival at the first stage operation improved significantly, with survival at 3 years increasing from 42% to 75% for the patients at standard-risk (p = 0.017), and from 17% to 42% for those deemed to be at high-risk (p = 0.1). No deaths occurred in 23 patients older than 3 years of age, all of whom had proceeded through the third stage. After completion of the Fontan circulation, the survival of the patients with hypoplasia of the left heart at 4 years was comparable to the survival of patients undergoing the Fontan procedure with a dominant left ventricle (88% versus 90%, p = 0.8).

CONCLUSIONS

Early and intermediate survival has improved significantly over the period of 12 years. Late death has been uncommon, and none of our patients are listed for cardiac transplantation.