Abstract 2826: Ventricular Tachy-arrhythmias and Sudden Cardiac Death as a First Sign of Noncompaction Cardiomyopathy
Purpose. Noncompaction cardiomyopathy (NCCM) is a rare, recently classified primary cardiomyopathy. Initial presentation includes congestive heart failure, arrhythmias and thrombo-embolic events. Ventricular arrhythmias are frequently recognized, including sustained ventricular tachycardia’s (VT) and fibrillation (VF), resulting in hemodynamic collapse, sudden cardiac arrest or death (SCD). The aim of this study was to determine the clinical profile of NCCM patients in whom the presenting sign was malignant ventricular arrhythmia, including SCD.
Methods. We used the prospective data from our NCCM cohort registry from 2005 to present (n=67 pts, mean age: 40±14 years, range [16 –73]). The patients are divided in SCD group (n=9 (13%) with initial presentation of sustained VT or VF and non-SCD group 2 (n=58) with initial presentation of heart failure or other reasons.
Results. Median age at first presentation in SCD pts (3 pts with VT and 6 with VF; 33 % male)) was 33 years, range 16 –53 years. Family history was positive in three patients, with one of SCD at the age of 22 years. In the SCD pts median QRS duration of was 93 ms, range 72 to 198 ms, versus 106 ms, range 72–208 (p=ns); and their the QTc was mean 417±39 ms vs 424±46 ms (p=ns). The SCD patients had obviously (n= 6 (67%) frequent (one of more) premature ventricular complexes (PVC’s) at their resting ECG compared to the non-SCD patients (n=5 (9%), p=<0.0001). Echocardiographically, the SCD patients had only mild LV dilatation (LVEDD: median 56 mm, range 45– 68 mm) and dysfunction (fractional shortening (FS): median 28 %, range 16 to 39 %) compared to non SCD patients (LVEDD: median 60 mm, range 41–93 mm, FS: median 18 %, range 6 – 43 %; p= 0.007). All the SCD patients were treated with an ICD and beta-blockers/ACE -inhibitors. During a median follow-up of 20 months range 7– 66, three patients presented with appropriate ICD therapy (median 6 months, range 1–16) and two with inappropriate ICD therapy after a median follow-up of 4 months, range 2–23.
Conclusions. Malignant ventricular arrhythmias including SCD as first presentation are frequently encountered in NCCM. The majorities of them were young, female and frequent PVC’s, urging the need of further research for appropriate risk stratification in NCCM patients.