SURGICAL-MANAGEMENT OF HYPOPLASTIC LEFT-HEART SYNDROME

Background. The treatment of infants with hypoplastic left heart syndr ome has been challenging and controversial. Methods. To assess the ope rative management and intermediate-term outcome, we retrospectively an alyzed our surgical experience with 50 newborns with hypoplastic left heart syndrome operated on between January 1989 and June 1995. Results . Surgical palliation with a first-stage Norwood operation was offered to 28 patients. The remaining 22 infants were initially listed for he art transplantation, and 15 underwent the operation. Ten of the 15 rec ipients are alive, and all ape in New York Heart Association class I. Seven infants underwent a Norwood procedure after being on the list fo r transplantation for 12 to 42 days. A total of 34 patients underwent Norwood procedures with one operation aborted because of inoperable an atomy. Two infants who survived the first-stage Norwood operation unde rwent subsequent heart transplantation and are currently doing well. T he 1-year mortality rate for heart transplantation was 18% (3/17) vers us 50% (17/34) for the Norwood procedure, Risk factors for early morta lity after a Norwood procedure include longer circulatory arrest time (>50 minutes), preoperative acidosis (pH < 7.20), larger systemic-pulm onary artery shunt (greater than or equal to 4 mm), diminutive ascendi ng aorta (less than or equal to 2.0 mm), and anatomic subtype of aorti c and mitral atresia, The 1-year survival rate for the Norwood procedu re 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 o f a first-stage Norwood operation, 10 have undergone the second stage (bidirectional Glenn procedure), and 7 have completed a Fontan procedu re. Heart transplantation results have also improved, with no deaths s ince 1992. Conclusions. Both the Norwood procedure and heart transplan tation have encouraging early to intermediate results in infants with hypoplastic left heart syndrome. Hypoplastic left heart syndrome shoul d be managed selectively on the basis of cardiac morphology, donor ava ilability, and family wishes. Development of a flexible program involv ing the use of both procedures may aid in the successful management of infants with hypoplastic left heart syndrome.