Mortality and Sudden Death in Pediatric Left Ventricular Noncompaction in a Tertiary Referral Center
Background—Left ventricular noncompaction (LVNC) is a cardiomyopathy characterized by excessive trabeculation of the left ventricle, progressive myocardial dysfunction, and early mortality. LVNC has a heterogeneous clinical presentation including arrhythmia and sudden cardiac death.
Methods and Results—We retrospectively reviewed all children diagnosed with LVNC at Texas Children's Hospital (TCH) from January 1990 to January 2009. Patients with congenital cardiac lesions were excluded. Two hundred forty two children were diagnosed with isolated LVNC over the study period. Thirty-one (12.8%) died and 13 (5.4%) were transplanted. One-hundred fifty (62%) presented with or developed cardiac dysfunction. The presence of cardiac dysfunction was strongly associated with mortality (HR 11; p<0.001). Electrocardiographic abnormalities were present in 87%, with ventricular hypertrophy and repolarization abnormalities occurring most commonly. Repolarization abnormalities were associated with increased mortality (HR 2.1; p=0.02). Eighty (33.1%) had an arrhythmia, and those with arrhythmias had increased mortality (HR 2.8; p=0.002). Forty-two (17.4%) had ventricular tachycardia (VT) with 5 presenting with resuscitated sudden cardiac death. In total, there were 15 cases of sudden cardiac death in the cohort (6.2%). Nearly all patients with sudden death (14/15) had abnormal cardiac dimensions or cardiac dysfunction. No patient with normal cardiac dimensions and function without preceding arrhythmias died.
Conclusions—LVNC has a high mortality rate and is strongly associated with arrhythmias in children. Preceding cardiac dysfunction or ventricular arrhythmias are associated with increased mortality. Children with normal cardiac dimensions and normal function are at low risk for sudden death.
- Received March 18, 2013.
- Accepted April 22, 2013.