Heart transplants and muscular dystrophies: reticence shattered

A review of the literature on the 275 heart transplants performed for muscular dystrophies up to July 2023 shows that :

  • these operations involved 116 patients with Becker’s myopathy (BMD) and 102 with laminopathy, but also 17 patients with limb-girdle muscular dystrophy (LGMD), 12 with Steinert’s myotonic dystrophy (DM1), 11 with Duchenne muscular dystrophy (DMD), 7 with desminopathy and 3 women with dystrophinopathies,
  • the rate of transplants has accelerated over the decades, with only 2 heart transplants described in BMD before 1990, 8 in BMD and 3 in Emery-Dreifuss muscular dystrophy (EDMD) between 1990 and 2000, then 25 in BMD and 22 in laminopathies between 2000 and 2010, reaching 81 in BMD and 73 in laminopathies in the following decade, up to 2023,
  • the operative and post-operative course is no more complicated than in other heart transplant patients,
  • anti-rejection immunosuppressive treatment must be finely adjusted to avoid causing secondary myopathy and/or rhabdomyolysis with cyclosporine,
  • rehabilitation before and after the operation must be reinforced to minimise the risk of infection or respiratory failure.

The author concludes that it is time to overcome the last remaining reservations about heart transplantation in cases of slowly progressive muscular dystrophy, linked to a presumed shortened lifespan, especially as this procedure is likely to improve the functional and vital prognosis.

 

Is Cardiac Transplantation Still a Contraindication in Patients with Muscular Dystrophy-Related End-Stage Dilated Cardiomyopathy? A Systematic Review. Politano L. Int J Mol Sci. 2024 May 13;25(10):5289.