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Unusual case of an 18-year-old heart transplant recipient with endocardial fibroelastosis.

Abstract
An 18-year-old female Japanese patient who suffered from heart failure and severe pulmonary hypertension was referred to our clinic. The etiology of her cardiomyopathy was unclear. Inhaled prostacyclin therapy resulted in an improvement of pulmonary arterial pressure and allowed us to avoid lung transplantation. Heart transplantation resulted in a complete remission of her respiratory function. Autopsies of the explanted heart revealed massive endomyocardial fibroelastosis. We concluded that endomyocardial fibroelastosis has to be considered a cause of heart failure in young adults with unclear cardiomyopathy.
AuthorsU Fuchs, A Zittermann, U Schulz, G Tenderich, U Raute-Kreinsen, K Minami, R Körfer
JournalTransplantation proceedings (Transplant Proc) Vol. 38 Issue 5 Pg. 1511-3 (Jun 2006) ISSN: 0041-1345 [Print] United States
PMID16797345 (Publication Type: Case Reports, Journal Article)
Topics
  • Adolescent
  • Blood Pressure
  • Cardiomyopathies (surgery)
  • Endocardial Fibroelastosis (pathology, surgery)
  • Female
  • Heart Transplantation
  • Humans
  • Lung Transplantation
  • Myocardium (pathology)
  • Vascular Resistance

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