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一例罕见的心尖肥厚型心肌病(AHCM)。

A rare case of apical hypertrophic cardiomyopathy (AHCM).

作者信息

Mirabbasi Seyed Abbas, Khalighi Koroush, Mukkamala Suresh, Kodali Archana

机构信息

Department of Medicine, Easton Hospital, Drexel University School of Medicine, Easton, PA, USA.

Easton Cardiovascular Associates, Electrophysiology Department, Easton, PA, USA.

出版信息

J Community Hosp Intern Med Perspect. 2017 Jun 6;7(2):122-125. doi: 10.1080/20009666.2017.1324238. eCollection 2017 Mar.

Abstract

Apical hypertrophic cardiomyopathy is a rare form of hypertrophic cardiomyopathy that involves thickening of the distal portion of the left ventricular wall. Most commonly seen in the Japan, with a prevalence rate of about 15% of all HCM patient, its incidence in the USA is approximately 3% of HCM cases. We report a case of a 46-year-old woman with history of hypertension who presented to emergency department with worsening dyspnea and orthopnea with features of left ventricular hypertrophy (LVH) and diffuse large T-wave inversions in the lateral leads on a 12-lead ECG. Further work up revealed severe concentric LVH, with near obliteration of the LV cavity. Ventriculogram showed severe symmetric hypertrophy of the mid to lower septum, extending to the apex of left ventricle with significant pressure gradient of at least 160 mmHg across the apex to mid septal cavity, with no significant gradient across the left ventricular outflow tract. These findings were consistent with apical hypertrophic cardiomyopathy. She was treated with verapamil and metoprolol and has remained asymptomatic over last 2.5 years of follow-up. Although the clinical presentation of AHCM can be variable and nonspecific; however, hallmark findings on ECG and echo can be extremely important in its diagnosis. : AHCM: Apical hypertrophic cardiomyopathy; ECG: Electrocardiogram; LVH: Left ventricular hypertrophy; LVOT: Left ventricular outflow tract.

摘要

心尖肥厚型心肌病是肥厚型心肌病的一种罕见形式,累及左心室壁远端增厚。最常见于日本,在所有肥厚型心肌病患者中的患病率约为15%,在美国其发病率约占肥厚型心肌病病例的3%。我们报告一例46岁有高血压病史的女性,因呼吸困难和端坐呼吸加重就诊于急诊科,12导联心电图显示有左心室肥厚(LVH)特征及侧壁导联广泛大T波倒置。进一步检查发现严重的向心性LVH,左心室腔几乎闭塞。心室造影显示中下部室间隔严重对称性肥厚,延伸至左心室心尖,心尖至室间隔中部腔室间存在至少160 mmHg的显著压力阶差,而左心室流出道无明显压力阶差。这些发现符合心尖肥厚型心肌病。她接受了维拉帕米和美托洛尔治疗,在过去2.5年的随访中一直无症状。虽然心尖肥厚型心肌病的临床表现可能多变且不具特异性;然而,心电图和超声心动图的典型表现对其诊断极为重要。:AHCM:心尖肥厚型心肌病;ECG:心电图;LVH:左心室肥厚;LVOT:左心室流出道

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b6f9/5473187/ae3c3b541332/zjch_a_1324238_f0001_oc.jpg

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