Author + information
- William Shi,
- Piers Daubeney,
- Alan Nugent,
- Gary Sholler,
- Robert Justo,
- Jim Ramsay,
- Terry Robertson,
- Margarita Betancur,
- Andrew Davis,
- Steven Colan,
- Ingrid King and
- Robert Weintraub
Introduction: Long-term outcomes for childhood left ventricular non-compaction (LVNC) are uncertain. We examined this question as part of a national population-based study.
Methods: The National Australian Childhood Cardiomyopathy Study includes all children in Australia diagnosed with primary cardiomyopathy presenting <10 years of age, diagnosed between 1987 and 1996. Patients with LVNC and a dilated phenotype (LVNC-D) were compared to those with isolated dilated cardiomyopathy (DCM, n=175). Propensity-score analysis was used for risk factor adjustment.
Results: There were 29 newly diagnosed cases of LVNC corresponding to a mean annual incidence of newly diagnosed cases of 0.11 per 100,000 at-risk persons. Of the 29 cases, 27 had dilated phenotype (LVNC-D). Median age at diagnosis was 0.3 (interquartile range 0.08 – 1.3) years of age. The median (interquartile range) duration of follow-up for all subjects was 6.8 (0.7-14.1) years. Freedom from death or transplantation was 48% (30 – 65%) at 10 years and 38% (20-56%) at 20 years. Unadjusted survival between LVNC-D and DCM patients was similar at 20 years (LVNC-D: 38% (20-56%) vs. DCM: 42% (21-63%), p=0.53). Propensity-score matching between LVNC-D and DCM patients yielded 24 matched pairs, whereby LVNC-D patients experienced poorer freedom from death/transplantation at 15 years (LVNC-D: 46% (26-66%) vs. DCM: 70% (42-97%), p=0.045). 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 (HR 2.0, 95% CI 1.1-3.7, p=0.02).
Conclusions: This population-based study suggests an ongoing risk of death or transplantation in children with LVNC with a predominant dilated phenotype. Patients with LVNC-D have worse outcomes compared to those with isolated DCM.
Poster Hall, Hall C
Saturday, March 18, 2017, 9:45 a.m.-10:30 a.m.
Session Title: Advances in HCM, PPCM and Other Cardiomyopathies
Abstract Category: 13. Heart Failure and Cardiomyopathies: Clinical
Presentation Number: 1201-285
- 2017 American College of Cardiology Foundation