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Heart transplantation in patients with muscular dystrophy associated with end-stage cardiomyopathy
1Department of Cardiovascular and Thoracic Surgery, German Heart Institute, Berlin.
Insights
Heart transplantation is a viable option for muscular dystrophy patients with cardiomyopathy, showing good recovery and graft function. This study found no increased perioperative risk or rapid graft failure in these unique recipients.
Area of Science:
- Cardiology
- Neurology
- Transplantation Medicine
Background:
- Patients with muscular dystrophy and cardiomyopathy traditionally face reluctance for heart transplantation due to perceived risks.
- Concerns include higher perioperative mortality and potential rapid onset of cardiomyopathy in the transplanted heart.
Purpose of the Study:
- To evaluate the outcomes of heart transplantation in patients with muscular dystrophy and end-stage cardiomyopathy.
- To assess perioperative risks, graft function, and long-term survival in this patient cohort.
Main Methods:
- A retrospective analysis of six heart transplant recipients with muscular dystrophy and cardiomyopathy.
- Patients received standard triple-drug immunosuppression (azathioprine, cyclosporine, steroids).
- Postoperative follow-up included clinical assessment, rehabilitation, and annual recatheterization studies.
Main Results:
- All six patients experienced an uneventful postoperative course with no prolonged intubation.
- Patients achieved physical rehabilitation, with a mean follow-up of 40 months.
- Annual recatheterization showed normal left ventricular ejection fraction; no coronary artery disease or progression of muscular dystrophy was observed.
Conclusions:
- Heart transplantation can be safely performed in select patients with muscular dystrophy and cardiomyopathy.
- These patients demonstrate good graft function and physical rehabilitation post-transplant.
- Long-term outcomes appear favorable, challenging previous concerns about increased risk.
Abstract:
Until now, patients with muscular dystrophy and concomitant cardiomyopathy have been accepted for heart transplantation only reluctantly, because of a higher perioperative risk caused by physical disability and a suspected rapid onset of cardiomyopathy of the transplanted heart. Of a total of 582 heart transplant recipients, six patients had muscular dystrophy associated with end-stage cardiomyopathy. In three patients, preoperative neurologic diagnostic investigation revealed type Duchenne muscular dystrophy (three male); one female patient had Emery-Dreifuss; one male patient had Becker-Kiener dystrophy, and the specific classification could not be established in one male patient. Mean age was 25 years, ranging from 9 to 45 years. The postoperative follow-up ranges from 10 months to 7 years, with a mean of 40 months. All patients received triple-drug immunosuppression, consisting of azathioprine, cyclosporine, and steroids. All patients had an uneventful postoperative course. Postoperative time of intubation was not prolonged in these patients compared to other patients. All patients are physically rehabilitated. One patient died suddenly 27 months after operation. Annual recatheterization studies showed normal left ventricular ejection fraction (59.5 +/- 13.4% SD). Signs of coronary artery disease could not be observed. No progression of preexisting muscular dystrophy could be diagnosed, until now.