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右冠状动脉单支畸形作为应激性心肌病和急性心力衰竭的偶然发现:病例报告及文献复习

Right single coronary artery as an incidental finding in Takotsubo syndrome and acute heart failure: Case report and review of the literature.

作者信息

Neiva Joana, Passos Silva Marisa, Pires-Morais Gustavo, Dias Adelaide, Ponte Marta, Caeiro Daniel, Braga José Pedro, Ferreira Nuno, Ribeiro Vasco

机构信息

Department of Cardiology, Hospital de Vila Franca de Xira, Vila Franca de Xira, Portugal.

Department of Cardiology, Centro Hospitalar de Vila Nova de Gaia Espinho, Vila Nova de Gaia, Portugal.

出版信息

Rev Port Cardiol (Engl Ed). 2019 Mar;38(3):215-223. doi: 10.1016/j.repc.2018.06.013. Epub 2019 Apr 20.

Abstract

INTRODUCTION

Single coronary artery (SCA) with no associated congenital heart disease is a rare congenital anomaly. Most cases are asymptomatic and incidental findings, but SCA can cause ischemia, congestive heart failure, and sudden cardiac death (SCD).

CASE REPORT

A 44-year-old woman presented with Takotsubo cardiomyopathy and cardiogenic shock. Selective cannulation of the left coronary artery (LCA) was not possible on coronary angiography (CA); an SCA was revealed arising from the right sinus, continuing distally as the circumflex artery and thereafter as the left anterior descending artery. Coronary computed tomography angiography (CCTA) confirmed left main atresia and no coronary stenosis. Cardiac magnetic resonance imaging (MRI) showed diffuse myocardial edema and no perfusion defects. The patient's clinical course was favorable under conservative management.

DISCUSSION

Our paper describes an incidental finding of right SCA. We report a Lipton type R-I, in which a dominant right SCA supplies the entire myocardium. It is the rarest SCA presentation, with an incidence of 0.0008%; only 15 cases have been reported in the literature, all of which were studied by CA. Of these 15, one had SCD, five angina, one ventricular arrhythmia and one complicated acute coronary syndrome. CCTA confirmed the diagnosis in seven patients, MRI in one and transesophageal echocardiography in another. Nine patients had coronary lesions. Two underwent coronary artery bypass grafting, one percutaneous intervention and 11 conservative treatment.

CONCLUSION

Right SCA with congenital absence of the LCA is one of the rarest coronary artery anomalies. In a significant percentage of patients it is associated with ischemia and can be life-threatening. CCTA and MRI are the modalities of choice for diagnosis and risk stratification.

摘要

引言

单冠状动脉(SCA)且无相关先天性心脏病是一种罕见的先天性异常。大多数病例无症状,为偶然发现,但SCA可导致缺血、充血性心力衰竭和心源性猝死(SCD)。

病例报告

一名44岁女性出现应激性心肌病和心源性休克。冠状动脉造影(CA)时无法对左冠状动脉(LCA)进行选择性插管;结果显示一条SCA发自右窦,向远端延续为回旋支动脉,之后为左前降支动脉。冠状动脉计算机断层扫描血管造影(CCTA)证实左主干闭锁且无冠状动脉狭窄。心脏磁共振成像(MRI)显示弥漫性心肌水肿且无灌注缺损。在保守治疗下,患者的临床病程良好。

讨论

我们的论文描述了一例右SCA的偶然发现。我们报告的是Lipton R-I型,即一条优势右SCA供应整个心肌。这是SCA最罕见的表现形式,发病率为0.0008%;文献中仅报道了15例,所有这些病例均通过CA进行研究。在这15例中,1例发生SCD,5例有胸痛,1例有室性心律失常,1例并发急性冠状动脉综合征。CCTA在7例患者中证实了诊断,MRI在1例中证实,经食管超声心动图在另1例中证实。9例患者有冠状动脉病变。2例行冠状动脉旁路移植术,1例行经皮介入治疗,11例接受保守治疗。

结论

先天性LCA缺如的右SCA是最罕见的冠状动脉异常之一。相当一部分患者与之相关的缺血可能危及生命。CCTA和MRI是诊断和风险分层的首选检查方法。

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