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Outcomes of Restrictive Cardiomyopathy in Childhood and the Influence of Phenotype: A Report From the Pediatric Cardiomyopathy Registry

Circulation (New York, N.Y.), 2012-09, Vol.126 (10), p.1237-1244 [Peer Reviewed Journal]

2015 INIST-CNRS ;ISSN: 0009-7322 ;EISSN: 1524-4539 ;DOI: 10.1161/CIRCULATIONAHA.112.104638 ;PMID: 22843787 ;CODEN: CIRCAZ

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Title:
Outcomes of Restrictive Cardiomyopathy in Childhood and the Influence of Phenotype: A Report From the Pediatric Cardiomyopathy Registry
Author: WEBBER, Steven A ; LIPSHULTZ, Steven E ; KANTOR, Paul F ; LAMOUR, Jacqueline M ; MARGOSSIAN, Renee ; PAHL, Elfriede ; RUSCONI, Paolo G ; TOWBIN, Jeffrey A ; SLEEPER, Lynn A ; MINMIN LU ; WILKINSON, James D ; ADDONIZIO, Linda J ; CANTER, Charles E ; COLAN, Steven D ; EVERITT, Melanie D ; JEFFERIES, John Lynn
Subjects: Arrhythmias, Cardiac - mortality ; Biological and medical sciences ; Blood and lymphatic vessels ; Cardiology. Vascular system ; Cardiomyopathy, Hypertrophic - mortality ; Cardiomyopathy, Hypertrophic - physiopathology ; Cardiomyopathy, Hypertrophic - surgery ; Cardiomyopathy, Restrictive - mortality ; Cardiomyopathy, Restrictive - physiopathology ; Cardiomyopathy, Restrictive - surgery ; Child ; Child, Preschool ; Diseases of the peripheral vessels. Diseases of the vena cava. Miscellaneous ; Female ; Fibrinolytic Agents - therapeutic use ; Follow-Up Studies ; Heart Transplantation - mortality ; Humans ; Infant ; Male ; Medical sciences ; Phenotype ; Proportional Hazards Models ; Prospective Studies ; Registries - statistics & numerical data ; Retrospective Studies ; Risk Factors ; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis ; Stroke - drug therapy ; Stroke - mortality
Is Part Of: Circulation (New York, N.Y.), 2012-09, Vol.126 (10), p.1237-1244
Description: Restrictive cardiomyopathy (RCM) has been associated with poor prognosis in childhood. The goal of the present analysis was to use the Pediatric Cardiomyopathy Registry to analyze outcomes of childhood RCM, with a focus on the impact of phenotype comparing pure RCM with cases that have additional features of hypertrophic cardiomyopathy (HCM). We analyzed the Pediatric Cardiomyopathy Registry database (1990-2008; N=3375) for cases of RCM. Cases were defined as pure when RCM was the only assigned diagnosis. Additional documentation of HCM at any time was used as the criterion for RCM/HCM phenotype. RCM accounted for 4.5% of cases of cardiomyopathy. In 101 (66%), pure RCM was diagnosed; in 51 (34%), there was a mixed phenotype. Age at diagnosis was not different between groups, but 10% of the pure RCM group was diagnosed in infancy versus 24% of the RCM/HCM group. Freedom from death was comparable between groups with 1-, 2-, and 5-year survival of RCM 82%, 80%, and 68% versus RCM/HCM 77%, 74%, and 68%. Transplant-free survival was 48%, 34%, and 22% and 65%, 53%, and 43%, respectively (P=0.011). Independent risk factors at diagnosis for lower transplant-free survival were heart failure (hazard ratio 2.20, P=0.005), lower fractional shortening z score (hazard ratio 1.12 per 1 SD decrease in z score, P=0.014), and higher posterior wall thickness in the RCM/HCM group only (hazard ratio 1.32, P<0.001). Overall, outcomes were worse than for all other forms of cardiomyopathy. Transplant-free survival is poor for RCM in childhood. Survival is independent of phenotype; however, the RCM/HCM phenotype has significantly better transplant-free survival. URL: http://www.clinicaltrials.gov. Unique Identifier: NCT00005391.
Publisher: Hagerstown, MD: Lippincott Williams & Wilkins
Language: English
Identifier: ISSN: 0009-7322
EISSN: 1524-4539
DOI: 10.1161/CIRCULATIONAHA.112.104638
PMID: 22843787
CODEN: CIRCAZ
Source: GFMER Free Medical Journals
MEDLINE

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Outcomes of Restrictive Cardiomyopathy in Childhood and the Influence of Phenotype: A Report From the Pediatric Cardiomyopathy Registry

2015 INIST-CNRS ;ISSN: 0009-7322 ;EISSN: 1524-4539 ;DOI: 10.1161/CIRCULATIONAHA.112.104638 ;PMID: 22843787 ;CODEN: CIRCAZ

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