Abstract
Cardiac disease is second only to pulmonary complications as the leading cause of death for patients with progressive neuromuscular disease. The extent of cardiomyopathy in patients with myopathic disease correlates poorly with the extent of skeletal or respiratory muscle weakness. Until recently, little was done to evaluate and treat this problem. However, resting gated radionuclide ventriculography (RNV or MUGA) is a reliable method for determining left ventricular function and wall motion and is more reliable than M-mode echocardiography for the measurement of left ventricular ejection fraction (LVEF) in these patients. The reliable determination of ventricular function permits closer monitoring of cardiomyopathic progression and the results of treatment. We report preliminary success in using captopril, an angiotensin converting enzyme (ACE) inhibitor, and hydralazine, a cardiac afterload reducing agent, in improving LVEFs and, therefore, cardiac function for some patients with myopathic cardiomyopathy. Until specific therapy is available, treatment must be guided by patient response to the therapeutic modalities used for cardiac patients in general.