Self-Assessment: NM Complications of Cancer Care | Cardiac & Pulmonary Manifestations of NMDs-Dr. Silvestri Presentation

Dr. Silvestri Presentation

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Dr. Nicholas J. Silvestri's presentation on "Cardiac Dysfunction in Neuromuscular Disorders" covers the types, evaluations, and treatments of cardiac conditions associated with various neuromuscular disorders, with a focus on muscular dystrophies such as Duchenne Muscular Dystrophy (DMD) and Becker Muscular Dystrophy (BMD).<br /><br /><strong>Key Points:</strong><br /><br />1. <strong>Cardiac Involvement in Muscular Dystrophies (MD):</strong><br />   - Duchenne Muscular Dystrophy (DMD): Characterized by the absence of dystrophin, resulting in significant cardiac issues like cardiomyopathy and conduction abnormalities typically presenting by age 10. Annual or biannual ECG and echocardiograms are recommended. ACE inhibitors and ARBs are commonly used treatments.<br />   - Becker Muscular Dystrophy (BMD): Similar to DMD but with reduced dystrophin levels, resulting in a more protracted disease course with prominent cardiac issues. Evaluation and treatment closely mirror those for DMD.<br />  <br />2. <strong>Female Carriers and X-linked Cardiomyopathy:</strong><br />   - Female carriers of dystrophin mutations can develop cardiac dysfunction. Regular cardiac assessments every five years are recommended.<br />   - X-linked dilated cardiomyopathy is rare but severe, often leading to rapid progression of heart failure in teenage males.<br /><br />3. <strong>Type 1 Myotonic Dystrophy (DM1):</strong><br />   - Autosomal dominant disorder with significant cardiac conduction system involvement. Annual ECGs are necessary to monitor for conduction abnormalities, with synchronous permanent pacing advised as needed.<br /><br />4. <strong>Emery-Dreifuss Muscular Dystrophy (EDMD):</strong><br />   - Cardiac issues include significant conduction abnormalities and rare left ventricular dysfunction. Regular ECG and arrhythmia monitoring are essential. ICD placement is often necessary.<br /><br />5. <strong>Limb Girdle Muscular Dystrophies (LGMD):</strong><br />   - Diverse group of disorders where cardiac disease, including cardiomyopathy and conduction defects, vary. Regular cardiac evaluations are critical.<br /><br />6. <strong>Other Neuromuscular Disorders:</strong><br />   - Inflammatory myopathies, myasthenia gravis, polyneuropathies (e.g., Guillain-Barré syndrome), and motor neuron disorders (like ALS) may also show cardiac dysfunctions, ranging from mild arrhythmias to severe autonomic disturbances.<br /><br /><strong>Highlights:</strong><br />- Cardiac MRI is emerging as a crucial diagnostic tool for assessing cardiac involvement.<br />- Coordination between neurologists and cardiologists is essential to improve patient outcomes in neuromuscular disorders with cardiac involvement.

Keywords

Cardiac Dysfunction

Neuromuscular Disorders

Muscular Dystrophies

Duchenne Muscular Dystrophy

Becker Muscular Dystrophy

Cardiac Evaluation

Cardiac Treatment

Cardiac MRI

Conduction Abnormalities

Cardiomyopathy