Left Ventricular Noncompaction and Cardiogenic Shock.
Left Ventricular Noncompaction and Cardiogenic Shock.
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DOI：
10.1161/circulationaha.119.043716
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发表时间：
2020-02
期刊：
影响因子：
37.8
通讯作者：
Felipe Kazmirczak;Cindy M. Martin;C. Shenoy
中科院分区：
文献类型：
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作者：
Felipe Kazmirczak;Cindy M. Martin;C. Shenoy
患者表现：一名23岁女性，因干咳和进行性呼吸困难2周就诊于急诊室。脉搏130次/分钟，血压84/60毫米汞柱，室内空气氧饱和度92%。她的病史是值得注意的，只有一个自律性闭合性室间隔缺损（VSD）在儿童时期。3代家族史对先天性心脏病、遗传性心肌病和心源性猝死无显著影响。她因肺炎接受了治疗。在门诊随访2周后，她描述了持续的用力呼吸困难和新发的矫形呼吸。她的体格检查没有显示充血性心力衰竭的迹象。胸部x光片显示心脏肿大。心电图（图1）显示窦性心律伴左心室肥厚，非特异性室内传导延迟，偶有室性早搏。超声心动图（图2）显示轻度左室扩张，严重的整体运动不足，左室射血分数为20%至25%，胸骨旁短轴位过度小梁，收缩末期非紧致心肌与紧致心肌（NC/C）之比为bbb2.0。右心室大小和收缩功能正常。同时注意到轻度至中度二尖瓣反流，中度三尖瓣反流，估计右室收缩压为40 mm Hg，未见室间隔缺损。
Patient presentation: A 23-year-old woman presented to the emergency room with a dry cough and progressive dyspnea for 2 weeks. Her pulse was 130 bpm, blood pressure was 84/60 mm Hg , and oxygen saturation was 92% on room air. Her medical history was remarkable only for a spontaneously closed ventricular septal defect (VSD) in childhood. A 3-generation family history was unremarkable for congenital heart disease, inherited cardiomyopathies, and sudden cardiac death. She was treated for pneumonia. In outpatient follow-up 2 weeks later, she described continuing exertional dyspnea and new-onset orthopnea. Her physical examination did not show signs of congestive heart failure. A chest x-ray revealed cardiomegaly. An electrocardiogram (Figure 1) showed sinus rhythm with left ventricular (LV) hypertrophy, nonspecific intraventricular conduction delay, and occasional premature ventricular beats. An echocardiogram (Figure 2) demonstrated mild LV dilatation, severe global hypokinesis with an LV ejection fraction of 20% to 25%, and excessive trabeculations with an end-systolic ratio of noncompacted to compacted (NC/C) myocardium of >2.0 in the parasternal short-axis view. The right ventricle (RV) had normal size and systolic function. Also noted were mild-to-moderate mitral regurgitation, moderate tricuspid regurgitation, and an estimated RV systolic pressure of 40 mm Hg. A VSD was not seen.