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CONGENITAL HEART DISEASE

Cardiac transplantation after the Fontan or Glenn procedure

K. Anitha Jayakumar, MD^*,*, Linda J. Addonizio, MD, FACC^*, Maryanne R. Kichuk-Chrisant, MD, Mark E. Galantowicz, MD, Jacqueline M. Lamour, MD^*, Jan M. Quaegebeur, MD and Daphne T. Hsu, MD, FACC^*

^* Pediatrics
Surgery, College of Physicians & Surgeons, Columbia University, New York, New York
Children's Hospital of Philadelphia, Philadelphia, Pennsylvania
Columbus Children's Hospital, Columbus, Ohio

Manuscript received May 23, 2003; revised manuscript received July 21, 2004, accepted August 9, 2004.

^* Reprint requests and correspondence: Dr. K. Anitha Jayakumar, Children's Hospital of New York, 2 North, 3959 Broadway, New York, New York 10032 (Email: kaj9{at}columbia.edu).

OBJECTIVES: The purpose of this study was to review the clinical course and outcome of cardiac transplantation after a failed Glenn or Fontan procedure.

BACKGROUND: Late complications of the Glenn or Fontan procedure, including ventricular failure, cyanosis, protein-losing enteropathy, thromboembolism, and dysrhythmias often lead to significant morbidity and mortality. If other therapies are ineffective, cardiac transplantation is the only therapeutic recourse. Transplantation in this unique population presents significant challenges in the operative and perioperative periods.

METHODS: The anatomic diagnoses, previous operations, clinical status, and indications for transplantation were characterized in patients transplanted after a Glenn or Fontan procedure. Outcomes after transplantation, including postoperative complications and mortality, were reviewed. Comparisons were made between survivors and nonsurvivors.

RESULTS: Primary orthotopic cardiac transplantation was performed in 35 patients (mean age 15.7 ± 8.5 years) with a mean follow-up of 54 ± 46 months. A total of 11 patients had undergone a Glenn shunt and 24 patients a Fontan procedure. Indications for transplantation were a combination of causes including ventricular dysfunction, failed Fontan physiology, and/or cyanosis. Ten patients died 2 months after transplantation; nine of the deaths occurred in the Fontan patients. Overall, one-year survival was 71.5%, and five-year survival was 67.5%. Survival was not significantly different between patients transplanted after a Glenn or Fontan procedure and patients transplanted for other etiologies.

CONCLUSIONS: Cardiac transplantation can be performed successfully in patients with end-stage congenital heart disease after a Glenn or Fontan procedure, with outcomes similar to transplantation for end-stage heart failure secondary to other etiologies.

Abbreviations and Acronyms   AVM = arteriovenous malformation   LV = left ventricle/ventricular   NYHA = New York Heart Association   PLE = protein-losing enteropathy   RV = right ventricle/ventricular

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