The prevalence of structural cardiac abnormalities in patients with myotonic dystrophy type I

Cardiac Involvement in Patients With Muscular Dystrophies

“…in the absence of severe neuromuscular impairment. Structural heart disease is also frequently observed in DM, with LV dilatation or hypertrophy observed in approximately 20% of patients, and LV systolic dysfunction in 14%61. Clinical heart failure, however, is less common—2% according to that same report.…”

Mechanisms and management of the heart in myotonic dystrophy

“…Patients with DM are also prone to developing structural cardiomyopathy 8 14. When applied, imaging modalities often identify evidence of structural heart disease before symptoms develop.…”

“…When applied, imaging modalities often identify evidence of structural heart disease before symptoms develop. One large registry of over 400 DM patients found some form of structural heart disease in roughly 20% of patients, but only 2% of the patients had overt symptoms of heart failure 14. Radionuclide imaging and echocardiography, and in particular tissue Doppler imaging (TDI), may reveal diastolic dysfunction early in the disease course, and later may show systolic dysfunction in either ventricle.…”

“…Mitral valve prolapse has been identified in 13–40% of patients 6 14. Myocardial myotonia, or impaired relaxation of cardiac muscle, is the cardiac equivalent of what occurs in the skeletal muscle of these patients.…”

Anaesthesiological and Ventilatory Precautions During Cardiac Surgery in Steinert's Disease: A Case Report

“…Moreover the heart may be affected, showing conduction system abnormalities, supraventricular and ventricular arrhythmias, myocardial dysfunction, and ischaemic heart disease 1–3 . Although there may be structural cardiac abnormalities, 1–3,8 there is no known association of congenital heart defects and Steinert's disease and the tetralogy of Fallot in this case is to be regarded as coincidental. Furthermore the endocrine system can also be affected.…”