Luisa Mestroni

Ventricular arrhythmias in dilated cardiomyopathy: Efficacy of amiodarone

Sixty-five patients with dilated cardiomyopathy were studied by means of 24-hour ECG monitoring. Ventricular arrhythmias were present in 62 (95.4%), of whom 52 (80%) showed a complex form (multiform ventricular extrasystoles, pairs, and ventricular tachycardia). Forty-one patients, presenting with complex ventricular arrhythmias, received antiarrhythmic treatment with amiodarone (600 mg/day in the first week, 400 mg/day in the second week, and 200 to 400 mg/day chronically), and were then controlled with periodic 24-hour ambulatory monitoring. A significant reduction in the number of ventricular extrasystoles was seen in over 70% of patients during a 3-year period. There was also a significant decrease in the incidence of complex ventricular arrhythmias (particularly of ventricular tachycardia). Adverse effects were noted in 23 patients, but only four had to stop treatment. During the follow-up period, 19 patients died: 14 of heart failure, four of sudden death, and one of a noncardiac cause; all patients who died suddenly were not treated with amiodarone (p = 0.022). Complex ventricular arrhythmias are frequent in dilated cardiomyopathy and it is suggested that amiodarone is effective in short- and long-term control of these arrhythmias.

Genetic cardiomyopathies - a clinical approach

Genetic Cardiomyopathies. A Clinical Approach - Libros de Medicina - Cardiologia general - 145,55

The Role of Clinical Observation: Red Flag 6 — Left Ventricular Non-Compaction

Left ventricular non-compaction (LVNC) is characterized by a pseudohypertrophic “spongy” left ventricle with deep trabeculations. It is considered to be a rare cardiomyopathy (CMP), with a prevalence of approximately 1:2,000 to 1:7,000 individuals. The condition shows excessive and unusually trabeculated myocardium within the mature heart muscle in the apical and midlateral/inferior portions of the left ventricle, which is usually hypokinetic (Fig. 8.1). LVNC represents a developmental failure of the heart to form the fully compact myocardium during the later stages of cardiac development.

The Role of Clinical Observation: Red Flag 7 — Syndromic and Multi-system Cardiomyopathies

In some patients, the phenotype of a cardiomyopathy (CMP) may be associated with the involvement of multiple organs, and with systemic clinical manifestations. These genetically determined diseases are characterized by complex, unusual and sometimes bizarre clinical pictures. The associations observed are not surprising, considering that in these diseases the defective gene is expressed in different structures or tissues. This is for example the case in the mitochondrial genes where production of abnormal proteins can cause multiform tissue abnormalities and/or dysfunction of different organs.