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

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Abstract
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. 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. 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. 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.

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