Cardiac Management in Neuromuscular Diseases

This article addresses the pathophysiology, diagnostic approaches, and therapeutic options in the more common forms of muscular dystrophy, especially those seen in pediatric and young adult populations. The major emphasis is on the dystrophinopathies because their treatment options are templates for those used in various other forms of dystrophy. Most patients with cardiomyopathy are treated with angiotensin-converting enzyme inhibitors or angiotensin receptor blockers, with other agents added as the disease progresses. Destination therapies and transplantation options are mentioned where appropriate. Some dystrophies can have significant conduction abnormalities requiring pacemaker treatment. Others with ventricular tachydysrhythmias may necessitate internal cardiac defibrillator placement.