Outcomes of one-lung fontan operation: A retrospective multicenter study in Japan

Background: The Fontan operation for patients with one available lung is an extremely challenging situation. However, few reports are available on this procedure. The purpose of this study was to describe outcomes of one-lung Fontan operation. Methods: A retrospective multicenter study was performed. Twelve of 1,142 patients whose data were recorded here underwent one-lung Fontan operation between September 1989 and October 2009. Preoperative, operative, and postoperative data were reviewed. Results: Median age at operation was 3.5 years (range, 1.0 to 22.8), the preoperative mean pulmonary pressure was 11.5 ± 3.3 mm Hg (range, 7.0 to 18.0), the ventricular ejection fraction was 58% ± 13% (range, 39 to 76), and end-diastolic ventricular pressure was 7.5 ± 3.5 mm Hg (range, 1.0 to 12.0). The available lung was right in 9 patients and left in 3 patients. Eleven patients underwent a two-staged Fontan completion. Extracardiac conduit total cavopulmonary connection, intraatrial extracardiac conduit total cavopulmonary connection, and atriopulmonary connection were performed in 10 patients, 1 patient, and 1 patient, respectively. The estimated actuarial survival was 83% at 1year, 73% at 5 years, and 73% at 10 years. Impaired ventricular function was found to be a significant risk factor for mortality by univariate analysis (43.0% ± 9.5% versus 64.0% ± 9.5%, p < 0.01), but not by multivariate analysis. Conclusions: One-lung Fontan operation can be performed with an acceptable midterm to long-term mortality rate in patients without impaired ventricular function. Thus, absence of one lung itself is not a contraindication to the Fontan operation.