| Survival implications: hypertrophic cardiomyopathy in Noonan syndrome. | |
| | |
MedLine Citation:
|
PMID: 21269411 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
|
OBJECTIVES: To understand relationships and survival implications between structural heart disease and hypertrophic cardiomyopathy in Noonan syndrome (Noonan syndrome-HCM), we reviewed the clinical course of 138 children with Noonan syndrome diagnosed with cardiovascular abnormalities and compared survival with the 30 children with Noonan syndrome-HCM with 120 contemporaneous children with nonsyndromic HCM. METHODS: Study cohorts represent consecutive cases diagnosed at our institution 1966 through 2006. Outcomes were modeled using multiphase parametric techniques followed by multivariable regression with bagging. RESULTS: Cardiac abnormalities in Noonan syndrome: Cardiac abnormalities in the 138 Noonan syndrome children included pulmonary valve dysplasia (52%), hypertrophic cardiomyopathy (22%), atrial septal defect (20%), ventricular septal defect (10%), mitral valve dysplasia (6%), coarctation (3%), and Fallot's tetralogy (2%). Need for surgery was high but not different from children with structural defects coexisting with HCM. Overall, late survival in children with Noonan syndrome and cardiac defects was good (91 ± 3% at 15 years), although significantly worse for those with Noonan syndrome-HCM (P < .01). Noonan syndrome-HCM vs. nonsyndromic HCM: In the 30 children with Noonan syndrome-HCM, structural cardiac malformations coexisted in 18 (57%). The incidence of structural cardiac malformations in nonsyndromic HCM was instead 3/120 (2.5%, P < .001). Risk-adjusted late survival was significantly worse for Noonan syndrome-HCM than for nonsyndromic HCM (P= .02). CONCLUSIONS: Noonan syndrome-HCM frequently coexists with structural cardiac malformations, whereas nonsyndromic HCM does not; their natural histories may therefore be different. Late survival is significantly worse for Noonan syndrome-HCM than nonsyndromic HCM. |
| | |
Authors:
|
Edward J Hickey; Rohit Mehta; Maryam Elmi; Kentaro Asoh; Brian W McCrindle; William G Williams; Cedric Manlhiot; Lee Benson |
Publication Detail:
|
Type: Comparative Study; Journal Article |
Journal Detail:
|
Title: Congenital heart disease Volume: 6 ISSN: 1747-0803 ISO Abbreviation: Congenit Heart Dis Publication Date: 2011 Jan-Feb |
Date Detail:
|
Created Date: 2011-01-28 Completed Date: 2011-05-09 Revised Date: 2011-09-07 |
Medline Journal Info:
|
Nlm Unique ID: 101256510 Medline TA: Congenit Heart Dis Country: United States |
Other Details:
|
Languages: eng Pagination: 41-7 Citation Subset: IM |
Copyright Information:
|
© 2011 Copyright the Authors. Congenital Heart Disease © 2011 Wiley Periodicals, Inc. |
Affiliation:
|
Department of Pediatrics, University of Toronto School of Medicine, Toronto, Canada. |
Export Citation:
|
APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
|
Adolescent Cardiomyopathy, Hypertrophic / mortality*, surgery Chi-Square Distribution Child Child, Preschool Heart Defects, Congenital / mortality*, surgery Humans Incidence Infant Kaplan-Meier Estimate Logistic Models Noonan Syndrome / mortality*, surgery Ontario Prognosis Risk Assessment Risk Factors Survival Rate Time Factors |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
Previous Document: Congenital portosystemic venous connections and other abdominal venous abnormalities in patients wit...
Next Document: Diagnosis and repair of anomalous right coronary artery from pulmonary artery in a neonate.