Korean Circ J. 2022;52(8):563-575.
Major Clinical Issues in Hypertrophic Cardiomyopathy.
제1저자 이현정 · 교신저자 김형관, 이상철
소속 (4)
- Division of Cardiology, Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea.
- Division of Cardiology, Department of Medicine, Samsung Medical Center, Heart Vascular Stroke Institute, Sungkyunkwan University School of Medicine, Seoul, Korea.
- Division of Cardiology, Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea. cardiman73@gmail.com, hkkim73@snu.ac.kr.
- Division of Cardiology, Department of Medicine, Samsung Medical Center, Heart Vascular Stroke Institute, Sungkyunkwan University School of Medicine, Seoul, Korea. sc.lea@samsung.com.
Abstract
Hypertrophic cardiomyopathy (HCM) is one of the most common inheritable cardiomyopathies. Contemporary management strategies, including the advent of implantable cardioverter-defibrillators and effective anticoagulation, have substantially improved the clinical course of HCM patients; however, the disease burden of HCM is still high in Korea. Sudden cardiac death (SCD), atrial fibrillation and thromboembolic risk, dynamic left ventricular outflow tract (LVOT) obstruction, and heart failure (HF) progression remain important issues in HCM. SCD in HCM can be effectively prevented with implantable cardioverter-defibrillators. However, appropriate patient selection is important for primary prevention, and the 5-year SCD risk score and the presence of major SCD risk factors should be considered. Anticoagulation should be initiated in all HCM patients with atrial fibrillation regardless of the CHA2DS2-VASc score, and non-vitamin K antagonist oral anticoagulants are the first option. Symptomatic dynamic LVOT obstruction is first treated medically with negative inotropes, and if symptoms persist, septal reduction therapy is considered. The recently approved myosin inhibitor mavacamten is promising. HF in HCM is usually related to diastolic dysfunction, while about 5% of HCM patients show reduced left ventricular ejection fraction <50%, also referred to as "end-stage" HCM. Myocardial fibrosis plays an important role in the progression to advanced HF in patients with HCM. Patients who do not respond to guideline-directed medical therapy can be considered for heart transplantation. The development of imaging techniques, such as myocardial deformation on echocardiography and late gadolinium enhancement on cardiac magnetic resonance, can provide better risk evaluation and decision-making for management strategies in HCM.
Copyright © 2022. The Korean Society of Cardiology.
Keywords
Atrial fibrillationHeart failureHypertrophic cardiomyopathySudden cardiac deathVentricular outflow obstruction
Conflict of interest statement
HKK reports research grants from Actelion, Handok, GSK, Dae-Woong, Hanmi, Ildong, JW Pharmaceutical, Samjin, and Norvatis. The remaining authors declare no conflict of interest.
그 밖의 정보
- 출판 형태
- Journal Article, Review
- 저널
- Korean circulation journal · ISSN 1738-5520
- 참고문헌
- 94편