Published Online
on January 29, 2007

A more recent version of this article appeared on February 13, 2007

This Article Full Text (PDF) Data Supplement All Versions of this Article: 115/6/773    most recent CIRCULATIONAHA.106.621185v1 Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Request Permissions Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Colan, S. D. Articles by Towbin, J. A. Search for Related Content PubMed PubMed Citation Articles by Colan, S. D. Articles by Towbin, J. A. Pubmed/NCBI databases Medline Plus Health Information Cardiomyopathy Related Collections Epidemiology Congestive Myocardial cardiomyopathy disease Other etiology Pediatric and congenital heart disease, including cardiovascular surgery

Submitted on February 17, 2006
Accepted on December 11, 2006

Epidemiology and Cause-Specific Outcome of Hypertrophic Cardiomyopathy in Children. Findings From the Pediatric Cardiomyopathy Registry

Steven D. Colan MD^*, Steven E. Lipshultz MD, April M. Lowe MS, Lynn A. Sleeper ScD, Jane Messere RN, Gerald F. Cox MD, PhD, Paul R. Lurie MD, E. John Orav PhD, and Jeffrey A. Towbin MD

From the Children’s Hospital, Harvard Medical School, Boston, Mass (S.D.C., J.M., G.F.C.); Holtz Children’s Hospital, University of Miami, Miami, Fla (S.E.L.); New England Research Institutes, Watertown, Mass (A.M.L., L.A.S.); Genzyme Corporation, Cambridge, Mass (G.F.C.); Albany Medical College, Albany, NY (P.R.L.); Brigham and Women’s Hospital, Harvard Medical School, Boston, Mass (E.J.O.); and Texas Children’s Hospital, Baylor College of Medicine, Houston (J.A.T.).

^* To whom correspondence should be addressed. E-mail: colan{at}alum.mit.edu.

Background--Current information on the epidemiology and outcomes of hypertrophic cardiomyopathy (HCM) in children is limited by disease diversity and small case series.

Methods and Results--The Pediatric Cardiomyopathy Registry has collected prospective and retrospective data on children diagnosed with HCM since 1990. We identified the various causes of HCM in childhood and determined the relationship between outcomes, cause, and age at presentation. Of 855 patients <18 years of age with HCM, 8.7% (n=74) had inborn errors of metabolism, 9.0% (n=77) had malformation syndromes, 7.5% (n=64) had neuromuscular disorders, and 74.2% (n=634) had idiopathic HCM. Children with HCM associated with inborn errors of metabolism and malformation syndromes have significantly worse survival than the other 2 groups. Patients with idiopathic HCM diagnosed before 1 year of age (n=227) had worse survival from the time of diagnosis than those diagnosed after 1 year of age (n=407). Patients with idiopathic HCM who survived to at least 1 year of age, however, had an annual mortality rate of 1% that was similar regardless of whether they were diagnosed before or after 1 year of age.

Conclusions--In children, HCM is a diverse disorder with outcomes that depend largely on cause and age. Patients presenting before 1 year of age have the broadest spectrum of causes and the poorest outcome. In those children with idiopathic HCM who survive beyond age 1, however, survival is independent of age at diagnosis, with an annual mortality rate (1%) that is much lower than previously reported in children and is not different from has been found in population-based studies in adults.

Key words: cardiomyopathy • death, sudden • heart diseases • heart failure • hypertrophy • pediatrics • survival

This article has been cited by other articles:

				A. E. Epstein, J. P. DiMarco, K. A. Ellenbogen, N.A. M. Estes III, R. A. Freedman, L. S. Gettes, A. M. Gillinov, G. Gregoratos, S. C. Hammill, D. L. Hayes, et al. ACC/AHA/HRS 2008 Guidelines for Device-Based Therapy of Cardiac Rhythm Abnormalities: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the ACC/AHA/NASPE 2002 Guideline Update for Implantation of Cardiac Pacemakers and Antiarrhythmia Devices) Developed in Collaboration With the American Association for Thoracic Surgery and Society of Thoracic Surgeons J. Am. Coll. Cardiol., May 27, 2008; 51(21): e1 - e62. [Full Text] [PDF]

				A. E. Epstein, J. P. DiMarco, K. A. Ellenbogen, N.A. M. Estes III, R. A. Freedman, L. S. Gettes, A. M. Gillinov, G. Gregoratos, S. C. Hammill, D. L. Hayes, et al. ACC/AHA/HRS 2008 Guidelines for Device-Based Therapy of Cardiac Rhythm Abnormalities: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the ACC/AHA/NASPE 2002 Guideline Update for Implantation of Cardiac Pacemakers and Antiarrhythmia Devices): Developed in Collaboration With the American Association for Thoracic Surgery and Society of Thoracic Surgeons Circulation, May 27, 2008; 117(21): e350 - e408. [Full Text] [PDF]

				I. Ostman-Smith, G. Wettrell, B. Keeton, D. Holmgren, U. Ergander, S. Gould, C. Bowker, and M. Verdicchio Age- and gender-specific mortality rates in childhood hypertrophic cardiomyopathy Eur. Heart J., May 1, 2008; 29(9): 1160 - 1167. [Abstract] [Full Text] [PDF]

				H. Morita, H. L. Rehm, A. Menesses, B. McDonough, A. E. Roberts, R. Kucherlapati, J. A. Towbin, J.G. Seidman, and C. E. Seidman Shared Genetic Causes of Cardiac Hypertrophy in Children and Adults N. Engl. J. Med., May 1, 2008; 358(18): 1899 - 1908. [Abstract] [Full Text] [PDF]

				J. Ganame, R. H. Pignatelli, B. W. Eidem, P. Claus, J. D'hooge, C. J. McMahon, G. Buyse, J. A. Towbin, N. A. Ayres, and L. Mertens Myocardial deformation abnormalities in paediatric hypertrophic cardiomyopathy: are all aetiologies identical? Eur J Echocardiogr, April 27, 2008; (2008) jen150v1. [Abstract] [Full Text] [PDF]

				S. A. Webber New-Onset Heart Failure in Children in the Absence of Structural Congenital Heart Disease Circulation, January 1, 2008; 117(1): 11 - 12. [Full Text] [PDF]

				T. P. Graham Jr The Year in Congenital Heart Disease J. Am. Coll. Cardiol., July 24, 2007; 50(4): 368 - 377. [Full Text] [PDF]