LIMB-GIRDLE MUSCULAR-DYSTROPHY - A NONINVASIVE CARDIAC EVALUATION

The heart was evaluated noninvasively in 20 strictly selected patients with limb girdle muscular dystrophy (LGMD) without cardiopulmonary symptoms. In 80% of the patients (16/ 20) abnormalities were detected of: cardiac auscultation in 45% (9/20); creatine kinase isoenzyme levels in 40% (8/20); electrocardiogram (ECG) in 50% (10/20); ambulatory 24-hour ECG in 24% (4/17), and echocardiogram in 25% (5/20). Chest radiographs showed normal cardiac shadows and lung fields. In this cross-sectional study there was no correlation between the presence of cardiac abnormalities and patient age, disease duration or degree of weakness. There was no relation between the findings detected by the different techniques. Whether the cardiac abnormalities are related to dystrophic affection of the heart requires pathological study. It is also unclear if these 'benign' abnormalities are indicators of future, potentially dangerous, cardiac disease in LGMD, and whether close follow-up is necessary or beneficial.