Chattranukulchai Pairoj, Namchaisiri Jule, Tumkosit Monravee, Puwanant Sarinya, Vorasettakarnkij Yongkasem, Srimahachota Suphot, Boonyaratavej Smonporn
Division of Cardiovascular Medicine, Department of Medicine, Faculty of Medicine, Chulalongkorn University, Cardiac Center, King Chulalongkorn Memorial Hospital, Bangkok, 10330, Thailand.
Division of Cardiovascular and Thoracic Surgery, Department of Surgery, Faculty of Medicine, Chulalongkorn University, Cardiac Center, King Chulalongkorn Memorial Hospital, Bangkok, Thailand.
J Cardiothorac Surg. 2018 Jun 18;13(1):70. doi: 10.1186/s13019-018-0751-4.
Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital coronary anomaly. The enlarged right coronary artery provides retrograde collaterals to supply the left ventricle then preferentially directs into the lower pressure pulmonary artery system causing coronary steal phenomenon. Few patients who survive through adulthood without surgery must have abundant, well-formed functioning collaterals with adequate perfusion of the left ventricle. We present the oldest reported patient with ALCAPA to undergo corrective surgery.
A 79-year-old woman presented with a 3-months history of worsening shortness of breath and orthopnea. Physical examination discovered a soft continuous murmur at the left upper chest. Transthoracic echocardiography demonstrated an unusual, tubular-like structure inside the interventricular septum with a turbulent flow from color Doppler. Moreover, there was a severe mitral regurgitation from posterior mitral leaflet restriction associated with ventricular remodeling in combination with mitral annular dilatation. Coronary angiography and coronary computed tomography angiography established the diagnostic hallmark of ALCAPA syndrome. Stress cardiovascular magnetic resonance perfusion imaging demonstrated no myocardial ischemia suggesting adequate collateral circulation. Remarkably, there was a left coronary ostial stenosis, which served as a protective mechanism against myocardia ischemia by limiting the steal effect. The patient successfully underwent the ligation of anomalous artery at its origin in combination with bioprosthetic mitral valve replacement. Her postoperative course was uneventful.
This case utilized multimodality imaging for delineating the course of abnormal vessels and helping to formulate therapeutic decision.
左冠状动脉起源于肺动脉（ALCAPA）是一种罕见的先天性冠状动脉异常。扩大的右冠状动脉提供逆行侧支循环以供应左心室，然后优先进入低压肺动脉系统，导致冠状动脉窃血现象。少数未经手术成年后存活的患者必须有丰富、结构良好且功能正常的侧支循环，以保证左心室的充分灌注。我们报告了有记录以来接受矫正手术年龄最大的ALCAPA患者。
一名79岁女性，有3个月的气短和端坐呼吸加重病史。体格检查发现左上胸部有柔和的连续性杂音。经胸超声心动图显示室间隔内有异常的管状结构，彩色多普勒显示有湍流。此外，因二尖瓣后叶受限及二尖瓣环扩张伴心室重塑导致严重二尖瓣反流。冠状动脉造影和冠状动脉计算机断层血管造影确定了ALCAPA综合征的诊断特征。应激心血管磁共振灌注成像显示无心肌缺血，提示侧支循环充分。值得注意的是，存在左冠状动脉开口狭窄，通过限制窃血效应起到了预防心肌缺血的保护机制。患者成功接受了异常动脉起始处结扎术并联合生物人工二尖瓣置换术。术后恢复顺利。
本病例利用多模态成像来描绘异常血管的走行，并有助于制定治疗决策。
