CLINICAL-EXPERIENCE WITH HEART-TRANSPLANTATION IN INFANTS

Objective: Orthotopic heart transplantation has become an accepted the rapeutic concept for adult patients with endstage heart disease. In ne wborns and infants this procedure is still a matter of discussion beca use of unknown long-term results and the lack of donor organs. Methods : Since March 1988 we have performed 40 orthotopic heart transplantati on in 39 infants who were from 1 to 280 days of age. Indications for t ransplantation included hypoplastic left-heart syndrome (n = 28), dila tive cardiomyopathy (n = 4), endocardial fibroelastosis (n = 4) and ot her complex structural anomalies (n = 3), The mean waiting period for transplantation was 53 days. A donor-recipient weight ratio up to 4.0 was accepted. Profound hypothermic circulatory arrest was used for gra ft implantation in all those patients who required extensive aortic ar ch reconstruction (71%). The initial immunomodulation was based on Cyc losporine, Azathioprine and Prednisolone, Patients who underwent trans plantation during the first 6 weeks of life received a chronic single- drug therapy with Cyclosporine after 1 year. Results: There were six p eri-operative deaths caused by drug-resistant right-heart failure in t hree cases, humoral rejection (n = 1), CMV infection (n = 1) and multi organ failure (n = 1), One infant died late, due to rejection. The ac tuarial survival rate for the entire group is now 82%. There is a rema rkable influence of increasing experience. Whereas six of 15 infants w ho had heart transplantation between 1988 and 1993 died early post-ope ratively (survival rate: 60%), only one lace death occurred among 24 r ecipients in the period from 1994 to April 1997 (survival rate: 96%). Episodes of rejection occurred once or several times in about half of the patients in this series (48%). All surviving children are living a t home in excellent condition. Conclusions: Heart transplantation duri ng early infancy is a rational and durable therapy for heart diseases with irreversible myocardial failure or severe structural anomalies. T he intermediate-term results have been encouraging in many centers, bu t more data must be accumulated to determine the sequelae of chronic i mmunosuppression, The lack of donor organs remains one of the major pr oblems in pediatric heart transplantation. (C) 1998 Elsevier Science B .V. All rights reserved.