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先天性四叶式主动脉瓣异常合并肥厚型非梗阻性心肌病:一例报告并文献复习

Congenital quadricuspid aortic valve anomaly associated with hypertrophic non-obstructive cardiomyopathy: a case report and review of the literature.

作者信息

Janssens U, Klues H G, Hanrath P

机构信息

Medical Clinic I, University of Aachen, Germany.

出版信息

Heart. 1997 Jul;78(1):83-7. doi: 10.1136/hrt.78.1.83.

Abstract

A case is reported of a 38 year old woman without known cardiac congenital abnormality but a history of well controlled arterial hypertension who was admitted to hospital after successful resuscitation at home following cardiac arrest. There was no evidence of myocardial infarction on 12-lead electrocardiogram but there were signs of left ventricular hypertrophy. Transoesophageal echocardiography revealed a rare quadricuspid aortic valve (QAV) malformation with concomitant mild aortic regurgitation. The left ventricle showed a massive concentric hypertrophy without obstruction. The patient was eventually transferred in a persistent vegetative state to a home care facility. A review of the literature revealed 70 cases of QAV diagnosed by transthoracic or transoesophageal echocardiography (26 cases), at necropsy (25), during surgery (15), and during angiography (4). The present case is the first report of QAV associated with non-obstructive hypertrophic cardiomyopathy.

摘要

报告了一例38岁女性病例,该女性无已知心脏先天性异常,但有动脉高血压且控制良好的病史,在家中发生心脏骤停后成功复苏后入院。12导联心电图无心肌梗死证据,但有左心室肥厚迹象。经食管超声心动图显示罕见的四叶式主动脉瓣(QAV)畸形并伴有轻度主动脉瓣反流。左心室显示出巨大的同心性肥厚且无梗阻。该患者最终以持续植物状态被转至家庭护理机构。文献回顾显示,经胸或经食管超声心动图诊断出70例QAV(26例),尸检诊断出25例,手术中诊断出15例,血管造影术中诊断出4例。本病例是QAV与非梗阻性肥厚型心肌病相关的首例报告。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c19e/484869/4a2b27bc35cf/heart00008-0106-a.jpg

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