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Heart Transplantation in Muscular Dystrophy Patients: Is it a Viable Option?
Dennis Wells1, Raheel Rizwan2, John L Jefferies3,2
1From the Department of Cardiothoracic Surgery (D.W.), University of Cincinnati College of Medicine, OH.
Circulation. Heart Failure
|March 29, 2020
Summary
Heart transplantation (HTx) outcomes for muscular dystrophy (MD) patients show similar survival rates compared to other cardiomyopathy patients. This suggests HTx is an effective treatment for select MD patients with end-stage heart failure.
Area of Science:
- Cardiology
- Transplantation Medicine
- Genetics
Background:
- Cardiomyopathy is a frequent and serious complication in muscular dystrophy (MD) patients, often leading to advanced heart failure and early mortality.
- Despite the severity, there's a notable reluctance to consider heart transplantation (HTx) for these individuals.
- This study investigates the outcomes of HTx in patients diagnosed with MD within the United States.
Purpose of the Study:
- To evaluate the outcomes of heart transplantation (HTx) in patients with muscular dystrophy (MD).
- To compare the post-transplant survival rates of MD patients with those of other cardiomyopathy patients.
- To assess the efficacy of HTx as a treatment for end-stage heart failure in select MD patients.
Main Methods:
- Data from the United Network for Organ Sharing database (October 1987–March 2016) were analyzed.
- A cohort of 81 MD patients undergoing HTx was compared with a control group of 41,317 other cardiomyopathy patients.
- Propensity score matching was employed to create a comparable cohort of 162 patients, controlling for age, gender, transplant year, renal function, and inotropic support.
Main Results:
- Pre-transplant characteristics were similar between MD and matched cardiomyopathy cohorts, with exceptions in ventricular assist device use, ventilator support, and donor race mismatch.
- Median waitlist time did not differ significantly between the groups.
- Post-transplant survival for the MD cohort was comparable to the matched cardiomyopathy cohort (P=0.18) and superior to the unmatched cardiomyopathy cohort (P=0.004).
Conclusions:
- Patients with muscular dystrophy who undergo heart transplantation experience long-term survival rates similar to recipients with other forms of cardiomyopathy.
- Heart transplantation is a viable and effective treatment option for carefully selected muscular dystrophy patients suffering from end-stage heart failure.
- No significant difference in post-transplant survival was observed between Becker MD and non-Becker MD subtypes.
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