Modified Fontan Procedure in Patients Less Than 4 Years of Age

Pearl, Jeffrey M. MD
Laks, Hillel MD
Drinkwater, Davis C. MD
Capouya, Eli R. MD
George, Barbara L. MD
Williams, Roberta G. MD

Division of Cardiothoracic Surgery, Department of Surgery (J.M.P., H.L., D.C.D., E.R.C.), and the Division of Pediatric Cardiology (B.L.G., R.G.W.), University of California, Los Angeles Medical School, Los Angeles.

Address for reprints: Hillel Laks, MD, Division of Cardiothoracic Surgery, UCLA Medical Center CHS 62-182, 10833 LeConte Avenue, Los Angeles, CA 90024.

Circulation 86(5):p II-105, November 1992.

Background

Initial experience with the modified Fontan procedure in patients <4 years of age has met with variable success, with early reports showing a high mortality. More recently, improved results with the modified Fontan procedure in younger patients have been achieved. Important advantages of an early Fontan include protection of the pulmonary vascular bed and preservation of ventricular function by reduction in ventricular volume overload and chronic hypoxemia.

Methods and Results

From 1982 through May 1991, 90 patients <4 years of age underwent a modified Fontan procedure. The average age was 29 months (range, 7–48 months), and 30 patients were <2 years of age. Diagnoses included tricuspid atresia in 36, single ventricle in 45, pulmonary atresia intact septum in seven, and hypoplastic left heart syndrome in two patients. An adjustable ASD was placed in 21. Early mortality was 8.9% (eight of 90), which is only slightly higher than our overall Fontan early mortality of 7.4% in 242 patients. The early mortality in the 30 patients <2 years of age was 6.7% (two of 30). In a mean follow-up of 38 months (range, 7–89 months), there have been three late deaths (3.7%), and one patient has undergone cardiac transplantation.

Conclusions

Early Fontan should be undertaken in patients >6 months old who present with increasing cyanosis provided good hemodynamics are present. A modified Fontan procedure can be performed safely with good clinical results in patients <4 years old.