Cardiac Transplantation in a Patient With Muscular Dystrophy and Cardiomyopathy

Cardiac involvement in Becker muscular dystrophy

“…Ultrastructural examinations showed foci of disorganization of myofibrils and Z bands, foci of severe clumping of intercalated discs and severe pericellular fibrosis. The sarcoplasmic reticulum and mitochondria showed dilation and disorganization (57). In another study of explanted hearts (59), utrophin appeared to be highly upregulated.…”

“…One examination of an explanted heart showed patchy staining of dystrophin (57). Ultrastructural examinations showed foci of disorganization of myofibrils and Z bands, foci of severe clumping of intercalated discs and severe pericellular fibrosis.…”

Evolution of cardiac abnormalities in Becker muscular dystrophy over a 13-year period

“…Over the past few years cardiac transplantation has been carried out in a number of patients with an end-stage congestive heart failure and relatively mild muscle weakness [8,13,34,39,41]. Supportive measures and vasodilator therapy should be standard treatment in all BMD patients with congestive heart failure.…”

“…Despite the high proportion of DCM in BMD, symptomatic patients are not common. The occasional patient with severe DCM necessitating cardiac transplantation or with a lethal outcome has been described [8,13,34,39,41,50].…”

Subclinical Becker's Muscular Dystrophy Presenting with Severe Heart Failure

“…Due to the severity of the heart failure, the patient applied to become a registered candidate for cardiac transplantation, since skeletal muscle disorder was subclinical (10). Unfortunately, before the registration, the patient died of cardiogenic shock and consciousness disturbance with sudden onset.…”