Long-Term Outcomes of Childhood Left Ventricular Noncompaction Cardiomyopathy: Results From a National Population-Based Study

• Published in: Circulation (New York, N.Y.) Vol. 138; no. 4; pp. 367 - 376
• Main Authors: Shi, William Y., Moreno-Betancur, Margarita, Nugent, Alan W., Cheung, Michael, Colan, Steven, Turner, Christian, Sholler, Gary F., Robertson, Terry, Justo, Robert, Bullock, Andrew, King, Ingrid, Davis, Andrew M., Daubeney, Piers E.F., Weintraub, Robert G., D’Orsogna, L., Bullock, A., Ramsey, J., Kothari, D., Robinson, T., Richardson, M., Wheaton, G., Radford, D., Whight, C., Justo, R., Ward, C., Sholler, G., Hawker, R., Cooper, S., Lau, K., Sherwood, M., Jones, O., Warner, G., Wilkinson, J.L., Goh, T., Edis, B., Penny, D., Lane, G., Menahem, S., Fong, L., Semsarian, C., Gailbraith, A., Jeremy, R., Fryda, R., Robinson, P., Lee, L.
• Format: Journal Article
• Language: English
• Published: United States by the American College of Cardiology Foundation and the American Heart Association, Inc 24.07.2018
• Subjects: Australia - epidemiology; Cardiomyopathy, Dilated - diagnostic imaging; Cardiomyopathy, Dilated - mortality; Cardiomyopathy, Dilated - physiopathology; Cardiomyopathy, Dilated - therapy; Child; Child, Preschool; Disease Progression; Female; Heart Failure - diagnostic imaging; Heart Failure - mortality; Heart Failure - physiopathology; Heart Failure - therapy; Heart Transplantation; Humans; Incidence; Infant; Isolated Noncompaction of the Ventricular Myocardium - diagnostic imaging; Isolated Noncompaction of the Ventricular Myocardium - mortality; Isolated Noncompaction of the Ventricular Myocardium - physiopathology; Isolated Noncompaction of the Ventricular Myocardium - therapy; Longitudinal Studies; Male; Phenotype; Prognosis; Risk Assessment; Risk Factors; Time Factors; Ventricular Function, Left; Australia; long-term Follow-up; cardiomyopathy; left ventricular noncompaction; survival
• ISSN: 0009-7322; 1524-4539; 1524-4539
• DOI: 10.1161/CIRCULATIONAHA.117.032262

BACKGROUND:Long-term outcomes for childhood left ventricular noncompaction (LVNC) are uncertain. We examined late outcomes for children with LVNC enrolled in a national population-based study. METHODS:The National Australian Childhood Cardiomyopathy Study includes all children in Australia with primary cardiomyopathy diagnosed before 10 years of age between 1987 and 1996. Outcomes for subjects with LVNC with a dilated phenotype (LVNC-D) were compared with outcomes for those with dilated cardiomyopathy. Propensity-score analysis was used for risk factor adjustment. RESULTS:There were 29 subjects with LVNC (9.2% of all cardiomyopathy subjects), with a mean annual incidence of newly diagnosed cases of 0.11 per 100 000 at-risk individuals. Congestive heart failure was the initial symptom in 24 of 29 subjects (83%), and 27 (93%) had LVNC-D. The median age at diagnosis was 0.3 (interquartile interval, 0.08–1.3) years. The median duration of follow-up was 6.8 (interquartile interval, 0.7–24.0) years for all subjects and 24.7 (interquartile interval, 23.3 – 27.7) years for surviving subjects. Freedom from death or transplantation was 48% (95% confidence interval [CI], 30–65) at 10 years after diagnosis and 45% (95% CI, 27–63) at 15 years. In competing-risk analysis, 21% of subjects with LVNC were alive with normal left ventricular systolic function, and 31% were alive with abnormal function at 15 years. Propensity-score matching between subjects with LVNC-D and those with dilated cardiomyopathy suggested a lower freedom from death/transplantation at 15 years after diagnosis in the subjects with LVNC-D (LVNC-D, 46% [95% CI, 26–66] versus dilated cardiomyopathy, 70% [95% CI, 42–97]; P=0.08). Using propensity-score inverse probability of treatment–weighted Cox regression, we found evidence that LVNC-D was associated with a greater risk of death or transplantation (hazard ratio, 2.3; 95% CI, 1.4–3.8; P=0.0012). CONCLUSIONS:Symptomatic children with LVNC usually present in early infancy with a predominant dilated phenotype. Long-term outcomes are worse than for matched children with dilated cardiomyopathy.