Heart transplantation in patients with muscular dystrophy associated with end-stage cardiomyopathy

J Heart Lung Transplant. Sep-Oct 1993;12(5):804-7.

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.

MeSH terms

  • Adult
  • Azathioprine / therapeutic use
  • Blood Pressure / physiology
  • Cardiac Output / physiology
  • Cardiomyopathies / physiopathology
  • Cardiomyopathies / surgery*
  • Child
  • Cyclosporine / therapeutic use
  • Female
  • Follow-Up Studies
  • Graft Rejection / pathology
  • Graft Rejection / prevention & control
  • Heart Transplantation* / pathology
  • Heart Transplantation* / physiology
  • Heart Transplantation* / rehabilitation
  • Humans
  • Male
  • Methylprednisolone / therapeutic use
  • Middle Aged
  • Muscular Dystrophies / physiopathology
  • Muscular Dystrophies / surgery*
  • Pulmonary Artery / physiology
  • Stroke Volume / physiology
  • Ventricular Function, Left / physiology
  • Ventricular Pressure / physiology

Substances

  • Cyclosporine
  • Azathioprine
  • Methylprednisolone