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一位 70 多岁的女性,因双心室心肌致密化不全和房间隔缺损导致急性心力衰竭。

Acute-onset heart failure secondary to biventricular non-compaction cardiomyopathy and atrial septal defect in a woman presenting in the seventh decade.

机构信息

Department of Oncology, East Tennessee State University, Johnson City, Tennessee, USA

Department of Internal Medicine, University of Miami Miller School of Medicine Palm Beach Regional Campus, Atlantis, Florida, USA.

出版信息

BMJ Case Rep. 2022 Feb 28;15(2):e246385. doi: 10.1136/bcr-2021-246385.

Abstract

We present a case of a previously asymptomatic 63-year-old woman who presented with worsening dyspnoea for 3 weeks. Initial transthoracic and later transoesophageal echocardiography confirmed biventricular non-compaction cardiomyopathy and a large secundum atrial septal defect (ASD) measuring 1.4 cm. Additionally, there was a haemodynamically significant left to right shunt causing acute decompensated systolic heart failure. She eventually underwent closure of the septal defect using a AMPLATZER Septal Occluder device. Decision to close the defect was made as the left to right shunt was causing severe pulmonary hypertension and acute heart failure. Since most heart failure treatments involve lowering of the LV afterload there was consideration that this could cause right to left shunting and could cause an Eisenmenger physiology. Hence the AMPLATZER Septal Occluder device was placed to eliminate the shunt through the ASD. The ASD combined with the non-compaction posed significant treatment challenge in this case.

摘要

我们报告一例此前无症状的 63 岁女性,因呼吸困难加重 3 周就诊。最初的经胸超声心动图和后来的经食管超声心动图证实为双心室心肌致密化不全和 1.4cm 大的继发房间隔缺损(ASD)。此外,存在引起急性失代偿性收缩性心力衰竭的具有血流动力学意义的左向右分流。她最终使用 AMPLATZER 房间隔封堵器成功关闭了房间隔缺损。决定关闭该缺损是因为左向右分流导致严重的肺动脉高压和急性心力衰竭。由于大多数心力衰竭治疗都涉及降低左心室后负荷,因此有考虑到这可能会导致右向左分流,并可能导致艾森曼格生理。因此,放置了 AMPLATZER 房间隔封堵器以消除通过 ASD 的分流。该 ASD 与非致密化一起在该病例中构成了重大治疗挑战。

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本文引用的文献

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Left ventricular non-compaction cardiomyopathy.左心室心肌致密化不全心肌病。
Lancet. 2015 Aug 22;386(9995):813-25. doi: 10.1016/S0140-6736(14)61282-4. Epub 2015 Apr 9.

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