Cardiovascular complications of neuromuscular disorders

Abstract

In the past decade, advances in molecular genetics have shown that many familial neuromuscular and cardiovascular diseases share a common pathophysiology. They are caused by inherited mutations in the cellular cytoskeleton of cardiac and skeletal muscle cells. The clinical manifestation of cardiac disease in neuromuscular disorders is common and their management should include both periodic cardiac assessment and appropriate symptomatic and definitive therapy. Dilated cardiomyopathy is a common complication of neuromuscular diseases. Cardiac function may decline progressively as part of the natural history of the disease, but current medical therapy, including angiotensin-converting enzyme inhibitors, beta-blockers, and diuretics, can be used to alleviate symptoms of left ventricular dysfunction. Conduction disturbances may be an important cause of mortality, especially in patients with Emery Dreifuss muscular dystrophy, Kearns-Sayre syndrome, and myotonic dystrophy, and thus pacemaker implantation can be life-saving. Rhythm disturbances, such as atrial fibrillation and ventricular tachyarrhythmias, have been reported in patients with neuromuscular diseases. Treatment is based on preventing sudden death and embolic phenomena and cardioverting or controlling atrial fibrillation. In these patients, problems may arise with anticoagulation and antiarrhythmic therapy due to the inherent locomotor instability associated with the disease, and the presence of concomitant atrioventricular disease. Although uncommon, hypertrophic cardiomyopathy may be a feature of some neuromuscular disorders. Patients should undergo regular risk stratification for sudden cardiac death and symptoms such as heart failure can be treated with medical therapy.

Prophylactic Implantable Cardioverter Defibrillator Placement in a Sporadic Desmin Related Myopathy and Cardiomyopathy

“…Ventricular arrhythmias are a well‐recognized problem in myopathies like myotonic dystrophy or X‐linked muscular dystrophy 7 . Implantation of an ICD in these situations has been reported 8 .…”

“…6 Ventricular arrhythmias are a well-recognized problem in myopathies like myotonic dystrophy or X-linked muscular dystrophy. 7 Implantation of an ICD in these situations has been reported. 8 However the desmin related myopathy has up until now not frequently been recognized as a cause of ventricular arrhythmias.…”

Progression of Conduction System Disease in a Paced Patient With Kearns‐Sayre Syndrome

“…On the other hand, ventricular dysfunction has been reported in neuromuscular diseases, and cardiac function may decline progressively as a part of natural history. 10 In spite of the continuous right ventricular pacing, the myocardial function has remained within normal limits echocardiographically in our case at the end of this long follow-up period.…”

Echocardiographic assessment of left ventricular morphology and function in patients with Emery–Dreifuss muscular dystrophy

“…Although, it is well documented that patients with EDMD develop supraventricular arrhythmias and atrioventricular conduction abnormalities [4,14,15], data on left ventricular morphology and function in this group are limited. We performed an echocardiographic study to address this problem.…”