Left ventricular myocardial function in myotonic dystrophy

Ventricular dysfunction in type 1 myotonic dystrophy: Electrical, mechanical, or both?

“…DM1-patients short survival is mostly due to respiratory disease, but arrhythmias are regarded as a possible cause of early unexpected death in up to 29% [1]. Electrocardiographic abnormalities indicating cardiac involvement are common [3][4][5]. Studies have suggested that the extent of conduction disease in DM1 correlates with the severity of muscular dysfunction [6].…”

Early detection of biventricular involvement in myotonic dystrophy by tissue Doppler

“…Cardiac involvement in Steinert's MD is usually characterized by arrhythmias, mainly due to conduction system abnormalities, increased risk of sudden death [2][3][4][5] and, less frequently, by symptoms of heart failure [7,8]. Standard Doppler echocardiography has allowed to establish that LV diastolic dysfunction is an early marker of heart involvement in MD, even when the clinical signs of heart failure are not clinically overt [9][10][11][12].…”

“…Cardiac involvement is usually characterized by conduction system abnormalities with prolonged PR interval, frequent bradyarrhythmias and increased risk of sudden death [2][3][4]. Despite some controversial results [5,6], it is known that patients with myotonic dystrophy may develop a specific cardiomyopathy and symptoms of heart failure [7,8], while only subclinical left ventricular (LV) diastolic dysfunction is detectable in the early stages of disease [9][10][11][12]. In this view, and because of a very variable phenotypic expression of the International Journal of Cardiology 118 (2007) 227 -232 www.elsevier.com/locate/ijcard disease, the detection of early markers of cardiac involvement may be crucial for an appropriate management of patients affected by MD [13].…”

Increased Plasma Concentration of Atrial Natriuretic Hormone in Myotonic Dystrophy

“…Some authors [1,[8][9][10] have found that systolic and diastolic function of the myocardium is normal and that there is no correlation between the degree of cardiac involvement and the stage of the disease. Others [11][12][13] have demonstrated a preclinical cardiac involvement, and abnormalities of the cardiac conduction tissue have been found quite frequently [1,14,15].…”