{"title":"No coronary artery in the aorta: Severe congenital malformation under-diagnosed","authors":"J. Karila, L. Houyel","doi":"10.1016/j.acvdsp.2023.07.032","DOIUrl":null,"url":null,"abstract":"<div><h3>Introduction</h3><p><span>Absence of connection of both coronary arteries<span> to the aorta is an extremely rare congenital malformation. Most cases reported are anatomic variants of anomalous </span></span>left coronary artery to pulmonary artery.</p></div><div><h3>Objective</h3><p>We describe here four cases of patients born without any coronary artery connecting connected to the aorta (<span>Figure 1</span>).</p></div><div><h3>Results/Expected results</h3><p><span><span>Case 1: a male infant was diagnosed with aortic coarctation and perimembranous </span>ventricular septal defect<span><span> (VSD). Left ventricular dysfunction appeared four months after coarctation repair and </span>pulmonary artery banding<span>. Heart catheterization showed no coronary artery in the aorta and a single coronary artery connected to the </span></span></span>right pulmonary artery.</p><p><span>Case 2: a female neonate born with multiples VSD and aortic coarctation died during coarctation repair. At autopsy, there were no </span>epicardial coronary arteries<span> 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.</span></p><p><span><span>Case 3: a male neonate was born with pulmonary atresia<span> with intact ventricular septum. Cardiac </span></span>angiography revealed multiple coronary-ventricular </span>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.</p><p>Case 4: a coronarography<span><span> revealed a single coronary artery originating from the left ventricle just below the </span>aortic valve<span> in a 64-year old with syncope at exercise. Surgical reimplantation was performed with success.</span></span></p></div><div><h3>Conclusion/Perspectives</h3><p>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.</p></div>","PeriodicalId":8140,"journal":{"name":"Archives of Cardiovascular Diseases Supplements","volume":null,"pages":null},"PeriodicalIF":18.0000,"publicationDate":"2023-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Archives of Cardiovascular Diseases Supplements","FirstCategoryId":"1085","ListUrlMain":"https://www.sciencedirect.com/science/article/pii/S1878648023002537","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"Medicine","Score":null,"Total":0}
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.
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.