肺动脉生冠状动脉异常(约08例)

G. Bennani, B.E.N.N.A. Zahri
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引用次数: 0

摘要

冠状动脉从肺动脉出生异常(ACAPA)是最常见的冠状动脉异常。这是一种罕见的先天性心脏病,在儿科中更为常见,有时是致命的。目的通过了解本病的患病率、临床及临床旁特征及预后,总结我院的服务经验。方法回顾性、描述性研究:2018年9月至2023年9月5年间,Ibn ROCHD医院心内科心儿科随访的08例ACAPA患儿。结果/预期结果在同一研究期间,ACAPA患病率与诊断出的先天性心脏病数量相比:0.16%。分布:2例ALCAPA, 6例ARCAPA; 5例男孩,3例女孩;平均年龄:1岁9个月。最常见的就诊原因是饮食困难和呼吸症状。2例患者心电图显示Q波坏死,这是该病的一个非常特殊的征象。TTE:表现为间接征象,如低运动扩张型心肌病,这在我们所有的患者中都存在,瓣膜泄漏也很频繁。直接征象不常见且难以客观观察:冠状动脉起源位于主动脉底部,或超折射心内膜可见,伴有冠状动脉异常生成的图像。在诊断有疑问的情况下进行心导管造影。确诊后5例患者行手术治疗,1例患者术后随访死亡,其余4例患者病情发展有利。结论/观点acapa是一种罕见但严重的缺血性心肌病,它关系到患者的生命预后。只有早期诊断和旨在恢复双冠状动脉系统的手术才能取得良好的效果,并允许心肌逐步恢复。
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The anomaly of birth of the coronary arteries from the pulmonary artery (about 08 cases)

Introduction

Anomaly of birth of the coronary artery from the pulmonary artery (ACAPA) is the most common coronary anomaly. More classic in pediatrics, it is a rare congenital heart disease that is sometimes fatal.

Objective

Share the experience of our service by determining the prevalence, the clinical and paraclinical characteristics as well as the prognosis of the disease.

Methods

This is a retrospective, descriptive study of 08 children with ACAPA followed in the unit of cardio-paediatrics of the cardiac department, Ibn ROCHD Hospital, over a period of 5 years from September 2018 to September 2023.

Results/Expected results

The prevalence of ACAPA compared to the number of congenital heart diseases diagnosed during the same study period: 0.16%.

Distribution:

– 2 ALCAPA, and 6 ARCAPA;

– 5 boys, 3 girls;

– Average age: 1 year 9 months.

The most frequent reason for consultation is a food difficulty with the signs respiratory.

ECG showed in 2 patients a Q wave of necrosis which is a very specific sign of the disease.

TTE: shows indirect signs such as hypokinetic dilated cardiomyopathy which is present in all our patients, valvular leaks are also frequent. Direct signs are less frequent and difficult to objectivise: absence of visualization of the origin of the coronary artery a the base of the aorta, or visualization of the hyper refractile endocardium, with image abnormal birth of a coronary artery.

Cardiac catheterization with angiography is performed in case of diagnostic doubt.

After the positive diagnosis, 5 of our patients underwent surgery, only 1 patient died in the post-operative follow-up, the evolution was favorable for the 4 other patients.

Conclusion/Perspectives

ACAPA is a rare but serious ischemic cardiomyopathy because it involves the patient's vital prognosis. Only early diagnosis and surgery aimed at restoring a dual coronary artery system have excellent results and allow progressive myocardial recovery.

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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.
期刊最新文献
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