肥厚性梗阻性心肌病伴SAM现象1例报告并文献复习。

Mei-Lian Cai, Guo-Qiang Zhong
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摘要

背景:肥厚性心肌病(HCM)的定义是在没有其他潜在的心脏、全身、综合征或代谢性疾病的情况下存在左心室肥厚(LVH)[1]。它是最常见的心肌遗传异常,麻醉后患病率从1:500到高达1:200[2-4]。它是青少年和运动员心脏性猝死(SCD)的主要原因。患者:56岁男性,活动后出现胸闷心悸6个月。建议病人住院。他接受了超声心动图、心脏磁共振(CMR)、冠状动脉造影(CAG)检查和左心室造影。诊断为肥厚性梗阻性心肌病(HOCM)伴收缩期前运动(SAM)现象。结果:超声心动图显示室间隔厚度14 ~ 16mm,二尖瓣有2度的SAM。这导致严重的左心室流出道狭窄(LVOT)和中度至重度二尖瓣功能不全。左心室造影证实二尖瓣反流(MR)与HOCM和SAM现象相关。在永久性起搏器的保护下,患者接受酒精室间隔消融术(ASA)治疗。出院后无胸闷、心悸症状复发。结论:在永久性起搏器的条件下,对HOCM和SAM患者应用ASA治疗均有良好的效果。
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Hypertrophic Obstructive Cardiomyopathy with SAM Phenomenon: A Case Report and Literature Review.

Background: Hypertrophic cardiomyopathy (HCM) is defined by the presence of left ventricular hypertrophy (LVH) in the absence of other potentially causative cardiac, systemic, syndromic, or metabolic diseases [1]. It is the most common genetic abnormality of the myocardium, with an anaesthetized prevalence ranging from 1:500 to as high as 1:200 [2-4]. It is the primary cause of sudden cardiac death (SCD) among teenagers and athletes.

Patient: A 56-year-old man presented with chest tightness and palpitations which had been occurring post-activity for the previous 6 months. The patient was advised to be admitted. He underwent echocardiography, cardiac magnetic resonance (CMR), coronary angiography (CAG) examination, and left ventriculography. He was diagnosed with hypertrophic obstructive cardiomyopathy (HOCM) with systolic anterior motion (SAM) phenomenon.

Results: Echocardiography results showed that the interventricular septal thickness was 14-16 mm and that there were 2 degrees of SAM of the mitral valve. This resulted in severe stenosis of the left ventricular outflow tract (LVOT) and moderate to severe mitral insufficiency. Left ventriculography confirmed mitral regurgitation (MR) associated with HOCM with SAM phenomenon. Under the protection of a permanent pacemaker, the patient was treated with alcohol septal ablation (ASA). After discharge, the symptoms of chest tightness and palpitation did not recur.

Conclusion: Beneficial effects were observed when patients with HOCM and SAM were treated with ASA under the condition of a permanent pacemaker.

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