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

Circulation. 2018 Jul 24;138(4):367-376. doi: 10.1161/CIRCULATIONAHA.117.032262.

Abstract

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.

Keywords: cardiomyopathy; left ventricular noncompaction; long-term Follow-up; survival.

Publication types

  • Comparative Study
  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • 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