Heifetz S A, Robinowitz M, Mueller K H, Virmani R
Pediatr Cardiol. 1986;7(1):11-8. doi: 10.1007/BF02315476.
The clinicopathologic features of four patients with total anomalous origin of the coronary arteries from the pulmonary artery (TCAPA) are presented and compared with 21 previously reported cases. Patients with TCAPA usually present with left ventricular heart failure, angina, or associated cardiovascular anomalies. Of the 19 patients in whom a clinical history was available, 16 were symptomatic before three days of age. All patients died with 60% dying before two weeks of age. Longer survival was associated with additional cardiovascular anomalies that increased pulmonary arterial perfusion pressure, oxygen saturation, or both. Seventeen (68%) patients had additional cardiovascular anomalies, most commonly atrial (nine cases) or ventricular (eight cases) septal defects and tetralogy of Fallot or other variants of pulmonary atresia (four cases). Only five (22%) of 23 had noncardiovascular anomalies. The coronary arteries arose equally from either one ostium or from two, and the number of ostia was not related to either anomalous coronary artery distribution or to the presence of additional cardiovascular anomalies. Cardiomegaly was present in 56% of cases and the majority of patients had myocardial fibrosis or infarction. Embryology is reviewed and evidence is presented to support the theory of involution-persistence of coronary artery anlagen as the pathogenetic mechanism of TCAPA.
本文介绍了4例冠状动脉起源于肺动脉（TCAPA）患者的临床病理特征，并与之前报道的21例病例进行了比较。TCAPA患者通常表现为左心室心力衰竭、心绞痛或相关心血管异常。在有临床病史的19例患者中，16例在3日龄前出现症状。所有患者均死亡，60%在2周龄前死亡。较长的生存期与增加肺动脉灌注压、血氧饱和度或两者的其他心血管异常有关。17例（68%）患者有其他心血管异常，最常见的是房间隔（9例）或室间隔缺损（8例）以及法洛四联症或其他肺动脉闭锁变体（4例）。23例中只有5例（22%）有非心血管异常。冠状动脉起源于一个开口或两个开口的情况相同，开口数量与异常冠状动脉分布或其他心血管异常的存在均无关。56%的病例存在心脏扩大，大多数患者有心肌纤维化或梗死。本文回顾了胚胎学，并提供证据支持冠状动脉原基退化-持续理论作为TCAPA的发病机制。
