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J Thorac Cardiovasc Surg 2004;128:693-702
© 2004 The American Association for Thoracic Surgery

Surgery for congenital heart disease

Evidence of pulmonary vascular disease after heart transplantation for Fontan circulation failure

^a Department of Surgery, University of Colorado Health Sciences Center and the Children's Hospital, Denver, Colo
^b Department of Pediatrics, University of Colorado Health Sciences Center and the Children's Hospital, Denver, Colo

Read at the Eighty-third Annual Meeting of The American Association for Thoracic Surgery, Boston, Mass, May 4-7, 2003.

Received for publication May 1, 2003; revisions received June 30, 2004; accepted for publication July 14, 2004.

^* Address for reprints: Max B. Mitchell, MD, The Children's Hospital, 1056 E 19th Ave, B200, Denver, CO 80218 (E-mail: mitchell.max{at}tchden.org).

OBJECTIVES: Elevated pulmonary vascular resistance may contribute to late Fontan circulation failure but is difficult to assess in such patients. Our aims were to assess outcomes of patients with failed Fontan circulation after heart transplantation and to determine whether elevated pulmonary vascular resistance might have contributed to the failure.

METHODS: Fifteen patients (14 Fontan circulations, 1 Kawashima circulation) underwent transplantation. The most common indication was ventricular dysfunction (mean ventricular end-diastolic pressure 12.5 mm Hg). Patients with early failures (n = 4) required transplantation less than 1 year after the Fontan operation. Those with late failures (n = 11) underwent transplantation at least 1 year after the Fontan operation. Mean age at transplantation was 11.6 years. Mean Fontan-transplantation interval was 7.4 years. Mean pulmonary arterial pressure, transpulmonary gradient, and pulmonary vascular resistance before and after transplantation were assessed. Paired ttests of variable differences were used to compare variables. Survival was estimated by the Kaplan-Meier method.

RESULTS: In-hospital mortality was 7%. There were 2 late events (1 death, 1 retransplantation) related to compliance or rejection issues. Graft survivals were 93%, 82%, and 82% at 3, 5, and 7 years, respectively. Posttransplantation pulmonary vascular resistance was elevated (>2.0 Wood units · m²) in 11 of 14 survivors past initial hospitalization (mean 3.3 ± 1.7 Wood units · m²). Only patients with early Fontan failures (3 of 4) had normal posttransplantation pulmonary vascular resistance. In paired comparisons, posttransplantation transpulmonary gradient was increased by a mean of 6.8 mm Hg (P< .0001) relative to pretransplantation value.

CONCLUSIONS: Outcomes after heart transplantation for failed Fontan circulation were good. Mild-to-moderate pulmonary vascular disease was evident after heart transplantation for late failure. Elevated pulmonary vascular resistance is a likely contributor to Fontan circulation failure.

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