Impact of functional status in patients with muscular dystrophy-associated cardiomyopathy on survival after heart transplantation.

Abstract

Background: Heart transplant (HTx) in dystrophy patients has been shown to have a similar survival to cardiomyopathy from other causes, but postoperative rehabilitation remains an issue. This study aimed to review and analyze the reports in the literature to determine whether pre- and post-transplant functional status along with wheelchair dependence in dystrophy patients can influence post-HTx outcomes.

Research design and methods: Relevant databases were queried for all case reports and case series regarding HTx in patients with dystrophy-associated cardiomyopathy published in the literature. Clinical data were extracted and tabulated. Patient survival was stratified according to preoperative and postoperative functional status, and Kaplan-Meier survival analysis was performed.

Results: We identified 22 studies yielding 36 patients with muscular dystrophy who underwent HTx. At baseline, there were three patients who were wheelchair dependent, and 22 patients had a diminished functional status preoperatively. Overall survival did not differ significantly between patients with normal baseline functional status and those with diminished functional status, either before or after transplantation.

Conclusions: Although no significant survival difference was found, diminished functional status, both pre- and post-transplant, appear to be associated with worse survival, highlighting its importance in transplant decision-making.