Familial idiopathic dilated cardiomyopathy (IDC).

A three-generation family is presented in which several relatives died from or are affected by idiopathic dilated cardiomyopathy (IDC). The transmission pattern is autosomal dominant. Although familial instances of IDC have been reported the proportion of familial cases tends to be underestimated. Moreover, different transmission patterns have been associated with IDC. This report adds further evidence to the fact that an autosomal dominant form of IDC clearly exists and that early screening of asymptomatic first degree relatives is mandatory for accurate genetic counselling of patients and their family members.

Non-invasive evaluation of cardiac function in professional cyclists.

Cardiac dimensions and left ventricular function were investigated at rest with non-invasive methods in 14 professional road race cyclists and in 11 age-matched sedentary control subjects. The electrocardiographic findings were in agreement with previous studies in endurance athletes and the vectocardiographic data showed anterior displacement of the electrical forces. Echocardiographic dimensions at end-diastole showed higher values in the cyclists for left ventricular internal diameter, left ventricular posterior wall thickness, and interventricular septal thickness. Derived values for left ventricular volume and left ventricular mass were also much larger in the cyclists and there was excellent agreement between total heart volume measured with radiology and total measured by echocardiography. There was a significant correlation between maximal oxygen consumption and end-diastolic left ventricular diameter.

Evaluation of the antiarrhythmic efficacy of mexiletine in patients with chronic ventricular arrhythmias.

For the evaluation of arrhythmias and the assessment of antiarrhythmic therapy prolonged periods of recording, as provided by the Holter system, are needed. The 12 lead electrocardiogram and rhythm strips are unsuitable for these purposes. However an exercise test may provide useful additional information. In two trials mexiletine was compared with other antiarrhythmic drugs (atenolol, disopyramide, lorcainide) and placebo. In both trials mexiletine was shown to have a good antiarrhythmic efficacy on chronic ventricular premature beats. The high incidence of side-effects was probably due to the high dosage used.

Design of a study to evaluate drug therapy of serious ventricular rhythm disturbances after an acute myocardial infarction.

A study was designed to investigate whether long-term use of aprindine can prevent sudden death from primary ventricular fibrillation. Patients with a proven recent myocardial infarction and malignant ventricular arrhythmias occurring late after the acute episode were asked to participate in a 1-yr, double-blind, randomized, placebo-controlled trial to suppress the rhythm disturbances observed on an ambulatory electrocardiogram. Particular care was taken to monitor drug adherence. Arrhythmia detection by ambulatory electrocardiography was used to assess drug efficacy; side-effects establish the maximum tollerated dose for each individual patient. Aprindine was therefore used under optimal circumstances. An interactive computer system served as a data base and provided the investigators and the monitoring committee with all the information required for a proper evaluation of the progress of the study.