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Successful Heart Transplantation From a Donor With Ullrich Congenital Muscular Dystrophy
Author(s) -
Plonka C.,
Wearden P. D.,
Morell V. O.,
Miller S. A.,
Webber S. A.,
Feingold B.
Publication year - 2013
Publication title -
american journal of transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.89
H-Index - 188
eISSN - 1600-6143
pISSN - 1600-6135
DOI - 10.1111/ajt.12246
Subject(s) - medicine , heart transplantation , transplantation , heart disease , inotrope , intensive care medicine , economic shortage , organ transplantation , muscular dystrophy , cardiology , surgery , linguistics , philosophy , government (linguistics)
Heart transplantation is the most effective therapy for children with end‐stage heart disease; however, its use is limited by the number of donor organs available. This shortage may be further compounded by concerns about organ quality, leading to refusal of potential donor organ offers. We report on the successful transplantation and 5‐year follow‐up of a heart from a donor with Ullrich congenital muscular dystrophy (UCMD). The candidate was critically ill at the time of the transplant and the donor organ was declined repeatedly on the match run list due to concerns about organ quality, despite having normal cardiac function by echocardiography on minimal inotropic support. We believe the diagnosis of “muscular dystrophy” in the donor combined with a lack of understanding about the specifics of the diagnosis of UCMD enabled our candidate to receive a primary offer for this organ. We are unaware of any previous reports of the use of a heart from a donor with UCMD for orthotopic heart transplantation in adults or children.