Abstract 11978: Arrhythmias in Isolated Noncompaction Cardiomyopathy Data From the German Noncompaction Registry (ALKK)
Objective: Isolated noncompaction cardiomyopathy (NCCM) is considered a primary genetic cardiomyopathy associated with arrhythmias, heart failure and thromboembolic events. Nevertheless, the occurrence, management and prognosis of arrhythmias in respect to the left ventricular (LV) function of patients (pts) with NCCM is not clear yet. To further investigate this question, the data of the German NCCM registry initiated by the “Arbeitsgemeinschaft Leitende Kardiologische Krankenhausärzte (ALKK)” was analyzed.
Method: As of May 15th 2011, the German NCCM registry consisted of 193 pts with NCCM (130 male, age 18 to 87 yrs, mean 54.1 yrs) with a mean follow up of 29 mths. Clinical events, symptoms and therapeutic interventions were documented every 6 months and correlated with the echocardiographic ejection fraction (EF).
Results: 51 out of the 193 pts presented with arrhythmias and were subsequently diagnosed with NCCM. In 32 pts arrhythmias were found incidentally. 159 pts presented with sinus rhythm, 34 with atrial fibrillation (AF). AF occurred in 18 % of the pts with EF < 35% and in 10% of the pts with EF > 35%. 10 pts required pacemaker implantation (impl). WPW syndrome was observed in 1.5%, AVNRT in 1% and typical atrial flutter in 1,5% of the pts and resulted in ablation therapy. In 2 pts a loop recorder was implanted. 5 sudden cardiac deaths (SD) occurred in pts with EF < 35%. 2 aborted SD occurred as initial symptom in asymptomatic pts with only mild reduced LV function. In 33 pts ICD impl was performed, 22 of them with EF < 35%. 15 of these 22 pts received ICD impl for primary prevention; in 4 of these pts adequate shock therapy was documented during follow up. In 11 pts with EF > 35% ICD impl was performed, 8 for primary prevention; in none of the pts adequate shock therapy was documented.
Conclusion: NCCM is associated with frequent ventricular and supraventricular arrhythmias. Life threatening ventricular arrhythmias mainly occurred in pts with severely reduced LV function, whereas supraventicular arrhythmias were observed in pts with normal to moderately reduced LV function as well. ICD therapy reduced SD in pts with NCCM and severe LV dysfunction. The high concomitant incidence of supraventricular arrhythmias needs to be considered in the adjustment of ICD systems.
- © 2011 by American Heart Association, Inc.