William Y Shi1,2,3,4, Margarita Moreno-Betancur3,4, Alan W Nugent5, Michael Cheung6, Steven Colan7, Christian Turner8, Gary F Sholler9, Terry Robertson10, Robert Justo10, Andrew Bullock11, Ingrid King3, Andrew M Davis6,3,4, Piers E F Daubeney12,13, Robert G Weintraub6,3,4. 1. Department of Cardiac Surgery (W.Y.S.). 2. Melbourne, Australia (W.Y.S.). 3. Murdoch Children's Research Institute, Melbourne, Australia (W.Y.S., M.M.-B., I.K., A.M.D., R.G.W.). 4. University of Melbourne, Australia (W.Y.S., M.M.-B., A.M.D., R.G.W.). 5. Department of Pediatrics, University of Texas Southwestern, Dallas (A.W.N.). 6. Department of Cardiology, Royal Children's Hospital (M.C., A.M.D., R.G.W.). 7. Department of Cardiology, Boston Children's Hospital, MA (S.C.). 8. Department of Cardiology, Children's Hospital at Westmead, Sydney, Australia (C.T.). 9. Department of Cardiology, Women's and Children's Hospital, Adelaide, Australia (G.F.S.). 10. Department of Cardiology, Mater Children's Hospital, Brisbane, Australia (T.R., R.J.). 11. Department of Cardiology, Princess Margaret Hospital, Perth, Australia (A.B.). 12. Department of Paediatric Cardiology, Royal Brompton Hospital, London, United Kingdom (P.E.F.D.). 13. National Heart and Lung Institute, Imperial College, London, United Kingdom (P.E.F.D.).
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.
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.
Entities:
Keywords:
cardiomyopathy; left ventricular noncompaction; long-term Follow-up; survival
Authors: Sylvia Krupickova; Suzan Hatipoglu; Giovanni DiSalvo; Inga Voges; Daniel Redfearn; Sandrine Foldvari; Christian Eichhorn; Sian Chivers; Filippo Puricelli; Grazia Delle-Donne; Courtney Barth; Dudley J Pennell; Sanjay K Prasad; Piers E F Daubeney Journal: J Cardiovasc Magn Reson Date: 2021-07-08 Impact factor: 5.364