Surgical management of hypoplastic left heart syndrome

The treatment of infants with hypoplastic left heart syndrome has been challenging and controversial.

To assess the operative management and intermediate-term outcome, we retrospectively analyzed our surgical experience with 50 newborns with hypoplastic left heart syndrome operated on between January 1989 and June 1995.

Surgical palliation with a first-stage Norwood operation was offered to 28 patients. The remaining 22 infants were initially listed for heart transplantation, and 15 underwent the operation. Ten of the 15 recipients are alive, and all are in New York Heart Association class I. Seven infants underwent a Norwood procedure after being on the list for transplantation for 12 to 42 days. A total of 34 patients underwent Norwood procedures with one operation aborted because of inoperable anatomy. Two infants who survived the first-stage Norwood operation underwent subsequent heart transplantation and are currently doing well. The 1-year mortality rate for heart transplantation was 18% (3/17) versus 50% (17/34) for the Norwood procedure. Risk factors for early mortality after a Norwood procedure include longer circulatory arrest time (> 50 minutes), preoperative acidosis (pH < 7.20), larger systemic-pulmonary artery shunt (> or = 4 mm), diminutive ascending aorta (< or = 2.0 mm), and anatomic subtype of aortic and mitral atresia. The 1-year survival rate for the Norwood procedure improved from 36% for the patients operated on during 1989 through 1992 to 75% during 1993 to mid-1995 (p = 0.005). Of the 17 survivors of a first-stage Norwood operation, 10 have undergone the second stage (bidirectional Glenn procedure), and 7 have completed a Fontan procedure. Heart transplantation results have also improved, with no deaths since 1992.

Both the Norwood procedure and heart transplantation have encouraging early to intermediate results in infants with hypoplastic left heart syndrome. Hypoplastic left heart syndrome should be managed selectively on the basis of cardiac morphology, donor availability, and family wishes. Development of a flexible program involving the use of both procedures may aid in the successful management of infants with hypoplastic left heart syndrome.