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Hypertrophic Cardiomyopathy Registry: The rationale and design of an international, observational study of hypertrophic cardiomyopathy

The American heart journal, 2015-08, Vol.170 (2), p.223-230 [Peer Reviewed Journal]

Elsevier Inc. ;2015 Elsevier Inc. ;Copyright © 2015 Elsevier Inc. All rights reserved. ;Copyright Elsevier Limited Aug 2015 ;ISSN: 0002-8703 ;EISSN: 1097-6744 ;DOI: 10.1016/j.ahj.2015.05.013 ;PMID: 26299218 ;CODEN: AHJOA2

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Title:
Hypertrophic Cardiomyopathy Registry: The rationale and design of an international, observational study of hypertrophic cardiomyopathy
Author: Kramer, Christopher M., MD ; Appelbaum, Evan, MD ; Desai, Milind Y., MD ; Desvigne-Nickens, Patrice, MD ; DiMarco, John P., MD, PhD ; Friedrich, Matthias G., MD ; Geller, Nancy, PhD ; Heckler, Sarahfaye, BA ; Ho, Carolyn Y., MD ; Jerosch-Herold, Michael, PhD ; Ivey, Elizabeth A., AS ; Keleti, Julianna, PhD ; Kim, Dong-Yun, PhD ; Kolm, Paul, PhD ; Kwong, Raymond Y., MD ; Maron, Martin S., MD ; Schulz-Menger, Jeanette, MD ; Piechnik, Stefan, PhD ; Watkins, Hugh, MD ; Weintraub, William S., MD ; Wu, Pan, PhD ; Neubauer, Stefan, MD
Subjects: Adolescent ; Adult ; Aged ; Biomarkers - blood ; Cardiology ; Cardiomyopathy, Hypertrophic - diagnosis ; Cardiomyopathy, Hypertrophic - epidemiology ; Cardiomyopathy, Hypertrophic - physiopathology ; Cardiovascular ; Cardiovascular disease ; Echocardiography, Doppler - methods ; Female ; Follow-Up Studies ; Genetic Testing - methods ; Global Health ; Heart attacks ; Heart Ventricles - diagnostic imaging ; Heart Ventricles - pathology ; Heart Ventricles - physiopathology ; Humans ; Magnetic Resonance Imaging, Cine - methods ; Male ; Middle Aged ; Morbidity - trends ; Mutation ; Prospective Studies ; Registries ; Severity of Illness Index ; Stroke Volume ; Time Factors ; Young Adult
Is Part Of: The American heart journal, 2015-08, Vol.170 (2), p.223-230
Description: Hypertrophic cardiomyopathy (HCM) is the most common monogenic heart disease with a frequency as high as 1 in 200. In many cases, HCM is caused by mutations in genes encoding the different components of the sarcomere apparatus. Hypertrophic cardiomyopathy is characterized by unexplained left ventricular hypertrophy, myofibrillar disarray, and myocardial fibrosis. The phenotypic expression is quite variable. Although most patients with HCM are asymptomatic, serious consequences are experienced in a subset of affected individuals who present initially with sudden cardiac death or progress to refractory heart failure. The Hypertrophic Cardiomyopathy Registry study is a National Heart, Lung, and Blood Institute–sponsored 2,750-patient, 44-site, international registry and natural history study designed to address limitations in extant evidence to improve prognostication in HCM (NCT01915615). In addition to the collection of standard demographic, clinical, and echocardiographic variables, patients will undergo state-of-the-art cardiac magnetic resonance for assessment of left ventricular mass and volumes as well as replacement scarring and interstitial fibrosis. In addition, genetic and biomarker analyses will be performed. The Hypertrophic Cardiomyopathy Registry has the potential to change the paradigm of risk stratification in HCM, using novel markers to identify those at higher risk.
Publisher: United States: Elsevier Inc
Language: English
Identifier: ISSN: 0002-8703
EISSN: 1097-6744
DOI: 10.1016/j.ahj.2015.05.013
PMID: 26299218
CODEN: AHJOA2
Source: AUTh Library subscriptions: ProQuest Central
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Hypertrophic Cardiomyopathy Registry: The rationale and design of an international, observational study of hypertrophic cardiomyopathy

Elsevier Inc. ;2015 Elsevier Inc. ;Copyright © 2015 Elsevier Inc. All rights reserved. ;Copyright Elsevier Limited Aug 2015 ;ISSN: 0002-8703 ;EISSN: 1097-6744 ;DOI: 10.1016/j.ahj.2015.05.013 ;PMID: 26299218 ;CODEN: AHJOA2

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