Adult Type Anomalous Origin of the Left Coronary Artery from the Main Pulmonary Artery: Report of One Case

成人型左冠狀動脈異常起端於肺動脈

李龍鳳(Long-Feng Lee) ； 張正成(Jeng-Sheng Chang) ； 洪瑞鬆(Re-Son Hung) ； 吳怡良(Yi-Liang Wu)

《Acta Paediatrica Sinica》 40卷2期 (1999/04) Pp. 112-115

https://doi.org/10.7097/APS.199904.0153

摘要

左冠狀動脈異常起端於肺動脈爲一罕見的疾病。大部份的病例於嬰兒早期即患有心肌梗塞，鬰血性心衰竭，甚至殘廢，此稱爲嬰兒型。本篇我們報告一位五歲患有此症成人型的女孩，起初被診斷爲非典型川崎症併發冠狀動脈瘤。在一年的藥物治療後，冠狀動脈瘤仍維持原來的大小，而且首次從心臓超音波見到左冠狀動脈與主肺動脈之間有血流的交通。雖然心臓超音波顯示左冠狀動脈與主動脈之間有連接，但仍安排了進一步心導管檢查才確定診斷。之後病人接受了開心手術矯正。
此疾病可經由從心臓超音波診斷，但較不可靠。確定的診斷還是需要冠狀動脈血管攝影。成人型的病人雖然較無症狀，但由於有潛在的危險，在確定診斷後需盡快接受手術矯正。

關鍵字

左冠狀動脈異常起端於肺動脈 ； 非典型川崎症 ； 冠狀動脈瘤 ； 心臓超音波 ； 血管攝影

並列摘要

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA syndrome) is a rare disease. Most of the cases suffer from myocardial infarction, congestive heart failure or even death during the early infantile period, and thus are called “infantile type”. We present one five-year-old girl with ALCAPA syndrome, adult type, who was initially suspected to be a case of atypical Kawasaki disease (KD) complicated with coronary arterial aneurysms. Due to persistent dilatation of the coronary artery after medical treatment for one year and a turbulent flow noticed between the left coronary artery (LCA) and the main pulmonary artery (MPA), cardiac catheterization was arranged. The diagnosis was established from the coronary cineangiography in spite of a direct connection between the aorta and LCA revealed from the echocardiogram. Thereafter open heart surgery was undertaken. Diagnosis of ALCAPA syndrome can be made by echocardiogram, but this is not fully satisfactory. Selective coronary cineangiography remains the definitive diagnosis. Although the patients of adult type are more asymptomatic, surgical correction should be undertaken as soon as the diagnosis is established since a substantial risk is still present.

並列關鍵字

ALCAPA syndrome ； atypical Ka wasaki disease ； coronary aneurysm ； echocardiogram ； cineangiography