• Long-term use of verapamil improved the clinical symptoms and LV function in patients with HOCM
Adelman A, et al. Br Heart J 1970;32:804–11. Maron BJ, et al. N Engl J Med 1987;316:780–9.
Calcium Antagonists
• Verapamil were used for the treatment of patients with HOCM
运动相关/心律失常相关，需要鉴别 • 慢性病容
临床体征
• 血压低 • 心脏听诊主动动脉瓣区收缩期杂音，二尖瓣区
收缩期杂音
Diagnosis of HCM
• 心电图，超声心动图，核磁共振等 • ①室间隔异常增厚，舒张期末的室间隔厚度＞
15mm。②室间隔运动幅度明显降低，一般≤5mm。 ③室间隔厚度／左室后壁厚度比值可达1.5:1有诊断 意义。④左心室收缩末内径比正常人小。⑤收缩起 始时间室隔与二尖瓣前叶的距离常明显缩小 • 左室流出道梗阻，压力结差>30mmHg
• 心肌细胞普遍显著肥大，肥大的心肌细胞直径 可达60μm（正常为15μm）。心肌细胞排列紊 乱，尤以室间隔深部及左室游离壁明显
• 心肌间质见多少不等的纤维化或大小不等的瘢 痕
Pathophysiology of HCM
• Ventricular hypertrophy • Left Ventricular Outflow （LVOT）Pressure
• Beta-blockers were effective in improving clinical symptoms in both HOCM and HNOCM
• Beta-blockers do not improve LV diastolic function in patients with HCM
肥厚型梗阻性心肌病的现代治疗 Current Therapy of Hypertrophic Obstructive Cardiomyopathy
Etiology of Hypertrophic Cardiomyopathy (HCM)
• HCM is inherited as a Mendelian autosomal （常 染色体） dominant trait.
(SAM) and mid-systolic contact with ventricular septum Mitral regurgitation due to incomplete leaflet coaptation
Obstructive Mechanism
Left ventricular outflow tract obstruction is present at rest in approximately 25% of HCM patients
Beta-blockers
• Beta-blockers were mainly used for the reduction of LVPG in patients with hypertrophic subaortic stenosis
• The effect of beta-blockers in attenuating resting LVPG was very weak
Gradient（PG) • Diastolic dysfunction • Arrhythmia • Myocardial ischemia
/LVOT obstruction
临床表现
• 劳力性心绞痛：活动时胸闷、胸痛、饱餐后明 显，休息后可以缓解；
• 活动耐量显著降低； • 脑供血不足症状：发作性头晕、黑蒙、晕厥；
In addition, 50% of patients without obstruction at rest can generate significant intraventricular gradients with exercise
激发试验—隐匿性梗阻
• 证实了激发试验全性和 有效性。
LVOTPG
RV
Septum
LV
AO
LVOT
SAM
Posterior wall
LA
SAM现象
LVOTPG
MRI诊断
• 室壁厚度及部位 • 流出道宽度
Obstructive Mechanism
Mechanical impedance at the subaortic level Owing to mitral valve systolic anterior motion
• Estimated prevalence of 0.2% (0.16% in China).
• It is caused by mutations in 29 genes coding for sarcomeric proteins(肌小节蛋白) .
Pathophysiology of HCM
33%
• 区分梗阻类型（静息型 和潜在型)。
• 明确介入和外科治疗适
应证。
30%
37% 静息梗阻
潜在梗阻 非梗阻
中 华 心 血 管 病 杂 志 2008;36:412-414.
HCM管理流程
Therapy
• Conservative medications: -- β-blockers, calcium-channel blockers, and anti-arrhythmic agents: Disopyramide（丙吡胺）， Cibenzoline（新型IA） • Invasive therapy: -- Surgical myectomy, -- septal eng -- radio frequency (RF), -- microwave (WMA), -- laser, and cryo-ablation • ICD