DłużNiewska Natalia, Kostkiewicz Magdalena, Tomkiewicz-Pająk Lidia, Krupiński Maciej, Podolec Jakub, Olszowska Maria, Podolec Piotr
a Department of Cardiac and Vascular Disease.
b Department of Radiology and Diagnostic Imaging.
Acta Cardiol. 2017 Feb;72(1):41-46. doi: 10.1080/00015385.2017.1281559.
Background Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital cardiac anomaly which requires surgical treatment, which improves left ventricular function and clinical outcomes. The definitive diagnosis of ALCAPA is based on coronary angiography, however, noninvasive visualization of the origin of the coronary artery is often helpful in the diagnosis of ALCAPA. Objectives The purpose of this study was to evaluate noninvasive techniques for the diagnosis of ALCAPA, and present the clinical outcomes in adults after surgical correction of ALCAPA. Methods Medical charts of five patients with ALCAPA treated at the John Paul II Hospital in Cracow between 2004 and 2012 were analyzed retrospectively. Noninvasive imaging techniques were used to visualize coronary vessels preoperatively. Patients were followed one year after the operation with echocardiograms and assessment of the New York Heart Association functional class. Results Computed tomography showed ALCAPA in four patients. Magnetic resonance imaging showed abnormal left ventricle remodeling suggestive of ischemia of the anterior cardiac wall. Myocardial perfusion imaging revealed ischemia of the middle and periapical segments of the anterior and anterolateral wall of the left ventricle in two patients. Postoperatively, patients had symptomatic improvement, but there was no statistically significant difference in mean ejection fraction. Conclusions Noninvasive techniques can be used to visualize the origin of the coronary arteries and characterize their morphology. Long-term follow-up of adult patients after surgical correction of ALCAPA is not well documented in the literature, but surgical treatment revealed beneficial short-term effects and symptoms relief.
背景 左冠状动脉起源于肺动脉（ALCAPA）是一种罕见的先天性心脏异常，需要手术治疗，手术可改善左心室功能和临床结局。ALCAPA的确诊基于冠状动脉造影，然而，冠状动脉起源的无创可视化通常有助于ALCAPA的诊断。目的 本研究的目的是评估诊断ALCAPA的无创技术，并介绍成人患者ALCAPA手术矫正后的临床结局。方法 回顾性分析2004年至2012年在克拉科夫约翰·保罗二世医院接受治疗的5例ALCAPA患者的病历。术前使用无创成像技术可视化冠状动脉血管。术后1年对患者进行超声心动图检查并评估纽约心脏协会功能分级。结果 计算机断层扫描显示4例患者存在ALCAPA。磁共振成像显示左心室异常重塑，提示前壁心肌缺血。心肌灌注成像显示2例患者左心室前壁和前侧壁中层及心尖段缺血。术后，患者症状改善，但平均射血分数无统计学显著差异。结论 无创技术可用于可视化冠状动脉起源并描述其形态。成人患者ALCAPA手术矫正后的长期随访在文献中记录较少，但手术治疗显示出有益的短期效果和症状缓解。
