Abstract 14769: Heart Failure in Noncompaction Cardiomyopathy - Data from the German Noncompaction Registry (ALKK)
Objective: Isolated noncompaction cardiomyopathy (NCCM) is considered a primary genetic cardiomyopathy associated with heart failure (HF), arrhythmias, and thrombembolic events with limited prognosis. To further investigate the occurrence, management and prognosis of HF in this disease we analysed the data of the German NCCM registry (ALKK) in a real life clinical setting of experienced centers.
Methods: By May 31th, the German NCCM registry had enrolled a total of 241 pts with NCCM (163 male, age 18 to 87 yrs, mean age 56.3 yrs) with a mean follow up period of 27 months. The pts were followed in 6 mths intervals for clinical events, symptoms and therapeutic interventions. The data were analysed in respect to LV function (group A: EF >= 50%, n= 78; group B: EF 36-49%, n= 66; group C: EF <= 35%, n= 97), the incidence of persistent or permanent atrial fibrillation (AF) and the incidence of left bundle branch block (LBBB).
Results: 61 % of the pts in the registry showed HF symptoms at the time of initial diagnosis of NCCM, 15 % developed HF symptoms during follow up or deteriorated. 19 % of all pts in the registry showed AF, 14 % of the pts in group A, 23 % of the pts in group B, and 21 % of the pts in group C. LBBB was observed in 23 % of the whole group and in 6 % of group A, in 26 % of group B, and in 35 % of group C. HF medication was applied according to the symptoms and stage of HF: 77 % of all NCCM pts received beta blocker therapy, 54 % in group A, 78 % in group B, and 95 % in group C. ACE inhibitors or ARB were given in 76 % of all pts, 54 % in group A, 81 % in group B, and 91 % in group C. Diuretics were provided in 65 % of all pts, 20 % of group A, 64 % of group B, and 98 % of group C. 7 pts died due to severe HF, 5 due to sudden cardiac death. 3 pts received HTX therapy for end stage HF. These pts belonged to group C. Primary preventive ICD implantations (impl) in cases with EF < 35% was performed in 24 pts, followed by adequate shock therapy in 5. 11 pts of group C with LBBB received CRT impl with improvement of symptoms in 10 pts.
Summary: In a real life clinical setting of the German NCCM registry prognosis was better than previously reported. Pts received pharmacological and device therapy according to the guidelines with proper response. It can be speculated that the close clinical follow up contributed to the better than expected outcome.
- © 2012 by American Heart Association, Inc.