Cardiomyopathy in muscular dystrophies

Cardiomyopathy in muscular dystrophies
Important muscular dystrophies due to mutations in structural cytoskeletal dystrophin gene are known as dystrophinopathies. They include Duchenne muscular dystrophy, Becker muscular dystrophy and X-linked dilated cardiomyopathy. Primary presentation of most dystrophinopathies is skeletal muscle weakness. Duchenne muscular dystrophy is caused by mutations leading to absence of functional dystrophin. Becker muscular dystrophy is due to mutations resulting in reduced amounts of shortened dystrophin protein.
Cardiac muscle being a striated muscle, is affected in many types of muscular dystrophies. Cardiomyopathy would contribute significantly to the morbidity and mortality of dystrophinopathies. Those with dystrophinopathies would benefit from annual cardiovascular imaging studies and cardiovascular therapies if needed [1].
Important causes of mortality in dystrophinopathies with cardiomyopathy are heart failure, heart block and ventricular arrhythmias. Early detection and initiation of heart failure treatment may delay progression of cardiomyopathy in muscular dystrophy. Important ECG finding in cardiomyopathy due to muscular dystrophy is a dominant R wave in lead V1. Cardiac magnetic resonance (CMR) imaging can show late gadolinium enhancement typically in the epicardial lateral wall [2].
The effect of early angiotensin-converting enzyme (ACE) inhibitor therapy in patients with normal left ventricular function on the progression of myocard...

Source: Cardiophile MD - August 30, 2022 Category: Cardiology Authors: Johnson Francis Tags: General Cardiology Source Type: blogs