Abstract 18173: Clinical Events during Follow up of Patients with Noncompaction Cardiomyopathy: Data from the German Left Ventricular Noncompaction Registry (ALKK)
Noncompaction cardiomyopathy (NCCM) is considered a genetic cardiomyopathy with a high prevalence of heart failure, arrhythmias, and thromboembolic events. However, the clinical course of patients (pts) with NCCM is not clear yet. We analyzed clinical events during follow up of the pts from the German left ventricular noncompaction registry (ALKK), which was initiated in 2006.
Results: The registry consists of 127 pts with NCCM, 85 men, age 56.9 yrs (18 to 87 yrs). During a mean follow up period of 34.0 mths, the pts were evaluated clinically every 6 months. The functional status, left ventricular (LV) ejection fraction, cardiovascular and other clinical events were documented. The clinical outcome was analyzed in respect to the LV function of the patients. 43 pts showed severe LV dysfunction (EF<35%) at initial presentation. 14 of the 127 pts died (3 sudden cardiac deaths, 2 of them prior to scheduled ICD implantation in case of severely reduced LV function; 6 nonarrhythmic cardiac deaths due to myocardial pump failure; 5 pts with non cardiac deaths, 2 following stroke, 1 following pneumonia, 1 following sepsis, 1 pt at the age of 79 yrs died of unknown cause. All deaths occurred in pts with severely reduced LV function. 1 aborted sudden cardiac death was observed in a pt with only mild LV dysfunction. 4 pts with severe heart failure underwent cardiac resynchronization therapy and 3 pts underwent heart transplantation. In 19 pts ICD implantation was performed, and in 3 pts ablation therapy due to atrial flutter or AV node reentry tachycardia. In 20 pts palpitations due to ventricular ectopic beats or atrial ectopic beats were treated with beta blockers. 4 pts with primary preventive ICD implantation and severely reduced LV function experienced appropriate shock therapy. During the follow up period a deterioration of LV function was observed in 8% of the pts.
Summary: Clinical events of pts with NCCM resulting in heart failure symptoms, arrhythmias, and thrombembolic events mainly occurred in the pts with reduced LV function. Deterioration of the clinical status during a follow up of 34 mths at 6 months intervals was rare suggesting that pts with NCCM should be followed closely to identify pts at risk early.
- © 2010 by American Heart Association, Inc.