Diagnosis and treatment of hypertrophic cardiomyopathy based on the guideline

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1 * ADDF/AHA HCM HCM HCM HCM R542.2 A Diagnosis and treatment of hypertrophic cardiomyopathy based on the guideline ZHANG Yan, WANG Jun*, WANG Ming-he Department of Cardiology, Huashan Hospital, Fudan University Jing an Branch, Shanghai , China Abstract: 2011 ACCF/AHA guideline for the diagnosis and treatment of hypertrophic cardiomyopathy HCM provides a reference for the diagnosis and treatment of HCM, to which clinicians should pay more attention. This review interprets the diagnostic technology and treatment methods of HCM as a highlight of the new guideline. Key words: hypertrophic cardiomyopathy; disease gene; diagnosis; treatment HCM 0.2% 0.5% 1 1% 2 HCM 0.16% 100 HCM 3 1/4 HCM HCM SCD 1 HCM /3 HCM 4-5 HCM 900 β MYH7 C MYBPC3 T TNT 13 6 MYH7 MYBPC3 TNT 3 HCM 3/4 2 MYBPC3 HCM 7 HCM 8 HCM HCM

2 236 HCM 2 HCM LVOT 9 HCM LVOT HCM 10 HCM 1/3 30 mmhg 1/3 HCM 30 mmhg 30 mmhg 11 1/3 HCM 30 mmhg 50 mmhg LVOT Venturi SAM LVOT LVOT LVOT LVOT 9 HCM 13 HCM LOVT SAM LVOT HCM NYHA 14 - LVOT HCM SCD 35% SCD AF

3 3.2 HCM 75% 95% 12 ECG 17 ST T Q ECG T V3 V5 ST-T AHCM ECG HCM 24 AF NSVT SCD HCM NYHA 3.3 HCM 2011 / ACCF/AHA HCM 9 TTE HCM HCM HCM TEE HCM 15 mm mm SCD HCM =1.03± CMR CMR HCM Maron CMR 6% HCM AHCM HCM 3.5 CT HCM CHD HCM HCM CHD CHD HCM HCM HCM 40 HCM CT 3.6 HCM HCM HCM 22 HCM 4 HCM SCD 237

4 238 SCD 12 10% SCD 2 SCD 6 SCD NSVT Monserrat 23 NSVT SCD mm SCD 20 mmhg 20 mmhg 10% 20% 85% 95% 3% HCM SCD SCD LVH LVH , HCM LVOT HCM /min 9 CCB 480 mg 9 CCB HOCM 24 HOCM CCB a mg 3 HOCM 5.3 DDD LVOT 30 mmhg 50 mmhg DDD DDD LVOT Megevand DDD % 44% 17% 28% P NYHA

5 1.8 P=0.02 LVOT 82± 35 mmhg 32±23 mmhg P ICD 2011 ACCF/AHA HCM HCM ICD B 1 ICD a 9 SCD ICD HCM 5.5 HOCM 26 HCM LVOT 50 mmhg Mcleod 27 Mayo Clinic 125 ICD ICD LVOT HCM 5.6 PTSMA PTSMA HOCM PTSMA MCE PTSMA HOCM 15 mm / / LVOT LVOTG 50 mmhg mmhg 70 mmhg PTSMA Brown HCM 12 LVOT 65 mmhg 16 mmhg P mm 14 mm P PTSMA HCM PTSMA 5% 30% 1 Maron BJ, Towbin JA, Thiene G, et al. Contemporary definitions and classification of the cardiomyopathies: an American Heart Association Scientific Statement from the Council on Clinical Cardiology, Heart Failure and Transplantation Com ittee; Quality of Care and Outcomes Research and Functional Genomics and Translational Biology Interdisciplinary W orking Groups; and Coun cil on Epidemiology and Prevention J. Circulation, 2006, : Ho CY. Hypertrophic cardiomyopathy in 2012 J. Circulation, 2012, : Zou Y, Song L, Wang Z, et al. Prevalence of idiopathic hypertrophic cardiomyopathy in China: a population-based echocardiographic analysis of 8080 adults J. Am J Med, 2004, : Marian AJ, Roberts R. The molecular genetic basis for hypertrophic cardiomyopathy J. J Mol Cell Cardiol, 2001, 33 4 : ,,,. DNA J., 1998, 38 7 : Geisterfer-Lowrance AAT, Kass S, Tanigawa G, et al. A molecular basis for familial hypertrophic cardiomyopathy: a p cardiac myosin heavy chain gene missense mutation J. Cell, 1990, 62 5 : Oliva-Sandoval MJ, Ruiz-Espejo F, Monserrat L, et al. 239

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