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PEDIATRICS Vol. 100 No. 4 October 1997, pp. 695-699

Cardiac Transplantation for Kawasaki Disease

Received Dec 6, 1996; accepted Mar 20, 1997.

Paul A. Checchia*, Elfriede PahlDagger , Robert E. Shaddyparallel , and Stanford T. Shulman§

From the Divisions of * Pediatric Critical Care Medicine, Dagger  Pediatric Cardiology, and § Pediatric Infectious Diseases, Children's Memorial Hospital, Northwestern University Medical School, Chicago, Illinois; and the parallel  Division of Pediatric Cardiology, Primary Children's Medical Center, University of Utah, Salt Lake City, Utah.

Objective.  Severe cardiac sequelae from Kawasaki disease include coronary ischemia and have been treated with a variety of coronary artery bypass procedures. There is only one published report of a child who underwent cardiac transplantation for severe Kawasaki disease-related cardiac complications. The purpose of this study was to gather the worldwide experience with cardiac transplantation for Kawasaki disease.

Methods.  Data were obtained from the United Network for Organ Sharing Registry, the European transplant experience, and a phone survey of many Kawasaki disease investigators. Diagnostic and surgical reports as well as clinical records were reviewed.

Results.  We identified 13 Kawasaki disease patients who underwent cardiac transplantation and obtained data on 10. In these 10 patients, the timing of transplantation was within 6 months after diagnosis of Kawasaki disease (4 patients), 1 to 5 years after diagnosis (3 patients), and 9 to 12 years after diagnosis (3 patients). Indications for transplantation included severe myocardial dysfunction, severe ventricular arrhythmias including cardiac arrest, and severe distal multivessel occlusive coronary artery disease. Nine of the 10 patients remain alive and healthy, with up to 6 years' posttransplant follow-up. One patient died 10 months posttransplant after severe refractory rejection. In addition, 1 patient required retransplantation at 4 years for severe rejection.

Conclusions.  Cardiac transplantation for severe ischemic heart disease as a sequela of Kawasaki disease is feasible and can benefit the small subgroup of patients who are not candidates for revascularization because of distal coronary stenosis or aneurysms and/or those with severe irreversible myocardial dysfunction.

Key words: Kawasaki disease, cardiac transplant.




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