Barriers to heart transplantation in MELAS syndrome remain numerous

MELAS syndrome is associated with mitochondrial encephalomyopathy, elevated lactic acid and pseudo strokes. Impairment of cardiac and renal function is also frequently reported. Italian researchers retrospectively studied the cardiac status of 23 MELAS patients diagnosed in 1998 and 2021 :

  • all had been referred to cardiology for evaluation of first hypertrophic and then dilated cardiomyopathy.
  • half of them were candidates for heart transplantation, but none of them have been able to benefit from it to date.
  • the rapid evolution of this disease and the existence of comorbidities, particularly renal, remain major obstacles to this indication.

The treatment of cardiomyopathy in the MELAS syndrome therefore remains purely palliative and poorly codified.

 

Impediments to Heart Transplantation in Adults With MELASMT-TL1:m.3243A>G Cardiomyopathy. Di Toro A, Urtis M, Narula N et al. J Am Coll Cardiol. 2022 Oct 11;80(15):1431-1443.