Thorac Cardiovasc Surg 2005; 53: S146-S148
DOI: 10.1055/s-2004-830455
Original

© Georg Thieme Verlag KG Stuttgart · New York

Cardiac Transplantation in Neonatal Marfan Syndrome - A Life-Saving Approach

T. Krasemann1 , S. Kotthoff1 , H.-G. Kehl1 , V. Debus1 , T. D. T. Tjan2 , C. Schmid2 , J. Vogt1 , H. H. Scheld2
  • 1Department of Pediatric Cardiology, University Children's Hospital Münster, Münster, Germany
  • 2Department of Thoracic and Cardiovascular Surgery, University Hospital Münster, Münster, Germany
Further Information

Publication History

Received September 30, 2004

Publication Date:
09 February 2005 (online)

Abstract

Marfan syndrome is a connective tissue disease with typical clinical signs and cardiac involvement. Its appearance in the neonatal period has a bad prognosis due to incompetence of all cardiac valves with subsequent congestive heart failure. Conservative management usually fails, the children die during their first year of life. We report on a girl with neonatal Marfan syndrome who suffered from regurgitance of all cardiac valves, enlarged ventricles, and dilated great arteries. She was NYHA class IV. At the age of six months she underwent heart transplantation. To prevent aneurysm formation and dissection of the great vessels, the whole aortic arch and pulmonary trunk were replaced as well.

References

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MD Thomas Krasemann

University Children's Hospital Münster, Department of Pediatric Cardiology

Albert-Schweitzer-Straße 33

48149 Münster

Germany

Phone: + 492518347773

Fax: + 49 25 18 34 95 93

Email: kraseman@uni-muenster.de