Document Detail

Clinical features of hypertrophic cardiomyopathy in the young.
MedLine Citation:
PMID:  12018719     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: We analysed the experience with hypertrophic cardiomyopathy in two paediatric centres to establish the differences from older patients. METHODS: Out of 45 young patients seen from 1974 to 1999, we included 38. Criterions for exclusion were secondary forms, or association with severe congenital cardiac disease which could alter the outcome. RESULTS: The patients presented at the age of 5.7 years, and were followed for 7.0 years. The 34 patients referred because of a murmur or cardiomegaly were older than the four with heart failure, presenting at 6.2 as opposed to 2.1 years of age, p = 0.08. Of the patients, 29 (76%) had primary cardiomyopathy, while 9 (24%) had secondary forms associated with Noonan's and LEOPARD syndromes. Familial tendency was ascertained in 7 patients (18%). The septal thickness in mm/m2 at presentation was greater in patients under 2 years than in older children (29 vs 18, p = 0.02). Obstructive hypertrophic cardiomyopathy was found in 17 patients (45%), with six of these having mild associated congenital cardiac defects. Nine had symptomatic arrhythmias. Overall, treatment was medical in 31, with DDD pacing used in 5, and surgery, radiofrequency ablation, and transplantation in one patient each. Total mortality was 24%, at a rate of 4.3% per year. Four patients died in heart failure and 5 had sudden death. Those in failure were significantly younger (p = 0.01). CONCLUSIONS: Hypertrophic cardiomyopathy in the young is characterized by referral for murmur or heart failure; frequent secondary forms; the obstructive variant being as common as the non-obstructive form; a mortality rate similar to that for adults attending tertiary centres; and less frequent familial forms than in older populations.
Eva Bruno; Héctor Maisuls; Ernesto Juaneda; Eduardo Moreyra; Luis E Alday
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Publication Detail:
Type:  Comparative Study; Journal Article    
Journal Detail:
Title:  Cardiology in the young     Volume:  12     ISSN:  1047-9511     ISO Abbreviation:  Cardiol Young     Publication Date:  2002 Mar 
Date Detail:
Created Date:  2002-05-20     Completed Date:  2002-11-20     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  9200019     Medline TA:  Cardiol Young     Country:  England    
Other Details:
Languages:  eng     Pagination:  147-52     Citation Subset:  IM    
Division of Cardiology, Hospital de Niños, Córdoba, Argentina.
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MeSH Terms
Age Distribution
Argentina / epidemiology
Cardiomyopathy, Hypertrophic / diagnosis*,  mortality*,  therapy
Cohort Studies
Death, Sudden, Cardiac
Echocardiography / methods
Heart Catheterization
Retrospective Studies
Risk Factors
Severity of Illness Index
Sex Distribution
Survival Rate

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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