No coronary artery in the aorta: Severe congenital malformation under-diagnosed

IF 18 Q4 Medicine
J. Karila, L. Houyel
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引用次数: 0

Abstract

Introduction

Absence of connection of both coronary arteries to the aorta is an extremely rare congenital malformation. Most cases reported are anatomic variants of anomalous left coronary artery to pulmonary artery.

Objective

We describe here four cases of patients born without any coronary artery connecting connected to the aorta (Figure 1).

Results/Expected results

Case 1: a male infant was diagnosed with aortic coarctation and perimembranous ventricular septal defect (VSD). Left ventricular dysfunction appeared four months after coarctation repair and pulmonary artery banding. Heart catheterization showed no coronary artery in the aorta and a single coronary artery connected to the right pulmonary artery.

Case 2: a female neonate born with multiples VSD and aortic coarctation died during coarctation repair. At autopsy, there were no epicardial coronary arteries except a short segment in the posterior atrioventricular sulcus. Two small dimples were seen in aortic root. No proximal coronary artery connected to the aorta or the pulmonary arteries. Several intramyocardial arterial vessels of different sizes were detected, without any communication with the ventricles.

Case 3: a male neonate was born with pulmonary atresia with intact ventricular septum. Cardiac angiography revealed multiple coronary-ventricular fistulae and no coronary arteries from the aorta. Autopsy confirmed 1) absence of epicardial coronary arteries except a very short segment of left anterior descending and small fistulous apical segments 2) two large coronary-ventricular fistulas. There were no coronary ostia within the aorta or the pulmonary tree.

Case 4: a coronarography revealed a single coronary artery originating from the left ventricle just below the aortic valve in a 64-year old with syncope at exercise. Surgical reimplantation was performed with success.

Conclusion/Perspectives

Those exceptional coronary malformations have a poor prognosis and are often diagnosed at autopsy. Total absence of epicardial coronary arteries, described only once in the literature, leads us to reconsider current knowledge of human coronary artery development.

主动脉无冠状动脉:诊断不足的严重先天性畸形
双冠状动脉与主动脉缺乏连接是一种极为罕见的先天性畸形。大多数病例报告为左冠状动脉到肺动脉异常的解剖变异。结果/预期结果病例1:一名男婴被诊断为主动脉缩窄和膜周室间隔缺损(VSD)。左心室功能障碍出现在缩窄修复和肺动脉绑扎术后4个月。心导管检查显示主动脉内无冠状动脉,仅有一根冠状动脉与右肺动脉相连。病例2:一名女性新生儿患有多发室间隔缺损和主动脉缩窄,在主动脉缩窄修复期间死亡。尸检时,除房室后沟一小段外,未见心外膜冠状动脉。主动脉根部可见两个小酒窝。没有近端冠状动脉与主动脉或肺动脉相连。心内动脉血管大小不一,与心室没有任何联系。病例3:一男新生儿先天性肺闭锁伴室间隔完整。心脏血管造影显示多个冠状室瘘,主动脉未见冠状动脉。尸检证实:1)心外膜冠状动脉缺失,除了左前降段很短和小的根尖段瘘管外;2)两个大的冠状室瘘。主动脉及肺动脉内未见冠状动脉开口。病例4:64岁运动时晕厥,冠状造影显示单冠状动脉起源于左心室主动脉瓣下方。手术再植成功。结论/观点那些特殊的冠状动脉畸形预后较差,通常在尸检时才被诊断出来。心外膜冠状动脉的完全缺失,在文献中只描述过一次,这使我们重新考虑目前对人类冠状动脉发育的认识。
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来源期刊
Archives of Cardiovascular Diseases Supplements
Archives of Cardiovascular Diseases Supplements CARDIAC & CARDIOVASCULAR SYSTEMS-
自引率
0.00%
发文量
508
期刊介绍: Archives of Cardiovascular Diseases Supplements is the official journal of the French Society of Cardiology. The journal publishes original peer-reviewed clinical and research articles, epidemiological studies, new methodological clinical approaches, review articles, editorials, and Images in cardiovascular medicine. The topics covered include coronary artery and valve diseases, interventional and pediatric cardiology, cardiovascular surgery, cardiomyopathy and heart failure, arrhythmias and stimulation, cardiovascular imaging, vascular medicine and hypertension, epidemiology and risk factors, and large multicenter studies. Additionally, Archives of Cardiovascular Diseases also publishes abstracts of papers presented at the annual sessions of the Journées Européennes de la Société Française de Cardiologie and the guidelines edited by the French Society of Cardiology.
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