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一名患有左冠状动脉起源于肺动脉(ALCAPA综合征)且有13次活产经历的患者。

A patient with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA syndrome) and 13 live births.

作者信息

Yolcu Mustafa, Bilal Mehmet Salih, Avsar Mustafa Kemal, Yildirim Ozgur

机构信息

Department of Cardiology, Private Medicana International Istanbul Hospital, Arel University, Istanbul, Turkey. Email:

Department of Cardiovascular Surgery, Private Medicana International Istanbul Hospital, Istanbul, Turkey.

出版信息

Cardiovasc J Afr. 2019;30(3):e1-e2. doi: 10.5830/CVJA-2019-003. Epub 2019 Feb 6.

Abstract

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) or Bland-White-Garland syndrome is a rarely seen congenital anomaly. Adult and infantile types are defined according to the degree of collateral development between the left coronary artery (LCA) and right coronary artery (RCA). If left untreated, ALCAPA has a 90% mortality rate in the first year of life, primarily due to myocardial ischaemia and heart failure. The degree of collateral development and the related LCA perfusion in ALCAPA syndrome determine the occurrence of symptoms. Herein, we present a case of a female patient who had previously, without any symptoms, given live birth to 13 babies. She had been experiencing exertional angina, which started long after the delivery of her 13th child. Since our patient had well-developed collaterals to the LCA, she was asymptomatic and able to give birth to the children via the vaginal route without any problems. Having well-formed collateral vessels between the RCA and LCA may prevent patients from developing symptoms, and even stressful conditions such as pregnancy may be tolerable.

摘要

左冠状动脉起源于肺动脉(ALCAPA),即布兰德 - 怀特 - 加兰综合征,是一种罕见的先天性异常。根据左冠状动脉(LCA)和右冠状动脉(RCA)之间侧支循环发育程度,可分为成人型和婴儿型。若不进行治疗,ALCAPA在出生后第一年的死亡率为90%,主要原因是心肌缺血和心力衰竭。ALCAPA综合征中侧支循环发育程度及相关的LCA灌注情况决定了症状的出现。在此,我们报告一例女性患者,她此前未出现任何症状,已顺产13个孩子。在她第13个孩子出生很久之后,她开始出现劳力性心绞痛。由于我们的患者LCA侧支循环发育良好,她没有症状,能够经阴道顺利分娩这些孩子。RCA和LCA之间形成良好的侧支血管可能会使患者不出现症状,甚至像怀孕这样的应激情况也可能可以耐受。

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