Publication: Hypertrophic Cardiomyopathy: From Genes to Bedside
Issued Date
2014
Resource Type
Language
eng
ISSN
0125-3611 (Print)
2651-0561 (Online)
2651-0561 (Online)
Rights
Mahidol University
Rights Holder(s)
Department of Medicine Faculty of Medicine Ramathibodi Hospital Mahidol University
Bibliographic Citation
Ramathibodi Medical Journal. Vol. 37, No. 3 (Jul-Sep 2014), 153-160
Suggested Citation
Objoon Trachoo, Tarinee Tangcharoen, โอบจุฬ ตราชู, ธารินี ตั้งเจริญ Hypertrophic Cardiomyopathy: From Genes to Bedside. Ramathibodi Medical Journal. Vol. 37, No. 3 (Jul-Sep 2014), 153-160. Retrieved from: https://repository.li.mahidol.ac.th/handle/20.500.14594/79684
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Title
Hypertrophic Cardiomyopathy: From Genes to Bedside
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Abstract
Hypertrophic cardiomyopath (HCM) is the common hereditary cardiomyopathy found in clinical practice. The disease is defined by left ventricular hypertrophy in the absence of abnormal loading conditions. The individuals affected by HCM present with asymptomatic atatus, syncopr, congestive heart failure, arrthymia and sudden cardiac death. Common presentation often develops in teenagers and young adulthood with an autosomal dominant inheritance, with various age-dependent penetrance. Most of the genes resposible for these disorders are related to the cardiac sarcomere proteins. The diagnosis is established on cardiac imaging such as transthoracic echocardiography and/or cardiac magnatic resonance imaging. The treatment modalities include pharmacologic therapy, invasive septal reduction and implantation of pacemaker or cardiac defibrillators to prevent sudden cardiac death. Genetic counselling is highly recommended in the probands and family members sine the relatives at risk can have the benefit from longitudinal clinical surveillance to prevent further morbidity and mortality.