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一例合并右心室流出道及左心室中部梗阻的肥厚型心肌病。

A case of hypertrophic cardiomyopathy with right ventricular outflow tract and left midventricular obstruction.

作者信息

Okada Natsumi, Shibata Atsushi, Tanihata Akiko, Kitada Ryoko, Ehara Shoichi, Izumiya Yasuhiro

机构信息

Department of Cardiovascular Medicine, Osaka City University Graduate School of Medicine, Osaka, Japan.

出版信息

J Cardiol Cases. 2022 Mar 26;26(1):66-69. doi: 10.1016/j.jccase.2022.02.010. eCollection 2022 Jul.

Abstract

UNLABELLED

We describe a case of a 59-year-old woman with hypertrophic cardiomyopathy who remained with right ventricular outflow tract obstruction after the pressure gradient in the left midventricle was resolved by a drug with a negative inotropic effect. The patient was diagnosed with hypertrophic cardiomyopathy 30 years previously and was only on low-dose beta-blocker therapy. She presented at our hospital with suspected exacerbation of heart failure because of the development and exacerbation of dyspnea and chest tightness. Transthoracic echocardiography showed an accelerated blood flow of 3 m/s in the middle of the left ventricle; thus, she was started on , a drug with a negative inotropic effect. After admission, intracardiac pressure measurement showed no pressure gradient in the left chamber. However, there was a pressure gradient of 18 mmHg between the apex of the right ventricle and the right ventricular outflow tract, and right ventricular outflow tract obstruction was confirmed on cardiac magnetic resonance imaging. We decided to reinforce the negative inotropic effect by adding , and the subjective symptoms and auscultatory systolic murmur were eliminated 2 months later.

LEARNING OBJECTIVE

Hypertrophy of the right ventricular myocardium can occur in patients with hypertrophic cardiomyopathy (HCM). However, right ventricular outflow tract obstruction remains a rare finding in patients with HCM, despite the presence of morphological abnormalities such as right ventricular hypertrophy. In patients with HCM, obstruction of the right ventricle should be considered if the symptoms and auscultatory findings do not match the left ventricular imaging findings.

摘要

未标注

我们描述了一例59岁肥厚型心肌病女性患者,其左心室中部压力梯度通过具有负性肌力作用的药物消除后,仍存在右心室流出道梗阻。该患者30年前被诊断为肥厚型心肌病,仅接受低剂量β受体阻滞剂治疗。她因呼吸困难和胸闷的出现及加重,以疑似心力衰竭加重为由就诊于我院。经胸超声心动图显示左心室中部血流速度加快至3m/s;因此,她开始使用一种具有负性肌力作用的药物。入院后,心内压力测量显示左心腔内无压力梯度。然而,右心室心尖与右心室流出道之间存在18mmHg的压力梯度,心脏磁共振成像证实存在右心室流出道梗阻。我们决定通过加用另一种药物来增强负性肌力作用,2个月后主观症状和听诊收缩期杂音消失。

学习目标

肥厚型心肌病(HCM)患者可发生右心室心肌肥厚。然而,尽管存在右心室肥厚等形态学异常,但右心室流出道梗阻在HCM患者中仍然是一种罕见的发现。在HCM患者中,如果症状和听诊结果与左心室影像学表现不相符,应考虑右心室梗阻。

相似文献

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Hypertrophic cardiomyopathy.肥厚型心肌病
Cardiol Clin. 1988 May;6(2):233-88.

本文引用的文献

4
Right ventricular involvement in hypertrophic cardiomyopathy.肥厚型心肌病中的右心室受累
Am J Cardiol. 2007 Oct 15;100(8):1293-8. doi: 10.1016/j.amjcard.2007.05.061. Epub 2007 Aug 9.

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