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Gordon Cohen
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Marc R. de Leval
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Right arrow Transplantation - heart

J Thorac Cardiovasc Surg 2004;126:1781-1787
© 2004 The American Association for Thoracic Surgery

Surgery for congenital heart disease

Improved early outcome for end-stage dilated cardiomyopathy in children

Anne-Marie McMahon, MRCPCHa, Carin van Doorn, FRCS (C/Th)a,*, Michael Burch, FRCPa, Pauline Whitmore, RSCNa, Sophie Neligan, RSCNa, Philip Rees, FRCPa, Rosemary Radley-Smith, FRCPa, Allan Goldman, FRCPCHa, Katherine Brown, MRCPa, Gordon Cohen, MDa, Victor Tsang, FRCSa, Martin Elliott, MD, FRCSa, Marc R. de Leval, MD, FRCSa

a Great Ormond Street Hospital for Children National Health Service Trust, London, United Kingdom

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

Received for publication May 2, 2003; revisions received July 16, 2003; accepted for publication July 31, 2003.

* Address for reprints: Carin van Doorn, FRCS (C/Th), Cardiothoracic Unit, Cardiac Wing, Level 2, Great Ormond Street Hospital for Children NHS Trust, Great Ormond Street, London WC2A 3JH, United Kingdom
vandoc{at}gosh.nhs.uk

OBJECTIVE: To review the impact of management changes on the early outcomes of end-stage dilated cardiomyopathy in children.

METHODS: We conducted a retrospective study of all consecutive children with end-stage dilated cardiomyopathy who received hospital treatment since 1992. Over the past 3 years the following management changes were made: (1) more aggressive use of mechanical cardiac assistance; (2) high priority listing for transplantation; and (3) ABO incompatible transplants for infants. Outcomes for 46 patients admitted between 1992 and 1999 (group I) were compared with 53 patients between 2000 and March 2003 (group II).

RESULTS: In group I, 12 (26%) patients received mechanical support with recovery in 3 and transplantation in 5 (1 died). In group II, 19 (36%) patients received extracorporeal membrane oxygenation, with recovery in 5 and transplantation in 12 (all survived). The use of mechanical assistance was associated with high morbidity related to bleeding, end-organ failure, and long-term mechanical ventilation. Five patients in group II received ABO incompatible transplants and all survived. There have been no episodes of rejection or need for increased immunosuppressive therapy. Hospital mortality has been significantly reduced (group I, 37% vs group II, 11%; P < .05).

CONCLUSIONS: Recent refinements in the management of end-stage dilated cardiomyopathy in children have significantly reduced early mortality. Identification of markers of early myocardial recovery and development of mechanical devices for longer term and more physiologic support are essential to achieve further improvements in outcome.






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