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一例不寻常的心绞痛病例:一名患有孤立性左心室心肌致密化不全的患者。

An unusual case of angina pectoris: a patient with isolated non-compaction of the left ventricular myocardium.

作者信息

Dabarian André L, Mady Charles, Rochitte Carlos E, Shiozaki Afonso A, Lemos Pedro A, Salemi Vera Maria Cury

机构信息

Cardiomyopathy Unit, Heart Institute InCor, University of Sao Paulo Medical School, São Paulo, Brazil.

出版信息

Eur J Echocardiogr. 2008 Sep;9(5):728-30. doi: 10.1093/ejechocard/jen151. Epub 2008 Apr 28.

Abstract

A 29-year-old white woman with typical angina pectoris presented diastolic dysfunction and was suggestive of isolated non-compaction of the ventricular myocardium (INCM) by echocardiography. Cardiac catheterization disclosed normal coronary arteries. Cardiovascular magnetic resonance (CMR) depicted prominent left ventricular INCM areas with non-compaction/compaction ratio of 3.7, and dipyridamole CMR demonstrated global perfusion defect at stress and normal perfusion at rest. Adenosine-induced vasodilation showed subnormal coronary velocity flow reserve in the right, left circumflex, and left anterior descending coronary arteries. The evidence of our case indicates that patients with INCM may present angina pectoris and, probably, relative chronic myocardial ischaemia related to an impaired microvascular function is responsible for this symptom as demonstrated invasively here. It is a possible mechanism for progressive myocardial dysfunction seen in these patients.

摘要

一名29岁患有典型心绞痛的白人女性出现舒张功能障碍,经超声心动图检查提示存在孤立性心室心肌致密化不全(INCM)。心导管检查显示冠状动脉正常。心血管磁共振成像(CMR)显示左心室INCM区域明显,非致密化/致密化比率为3.7,双嘧达莫CMR显示负荷状态下整体灌注缺损,静息状态下灌注正常。腺苷诱导的血管舒张显示右冠状动脉、左旋支冠状动脉和左前降支冠状动脉的冠状动脉血流储备低于正常。我们这例病例的证据表明,INCM患者可能出现心绞痛,并且如在此通过侵入性检查所证实的,可能是与微血管功能受损相关的相对慢性心肌缺血导致了这一症状。这是这些患者出现进行性心肌功能障碍的一种可能机制。

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