Ma Fan, Zhou Kaiyu, Shi Xiaoqing, Wang Xiaoqing, Zhang Yi, Li Yifei, Hua Yimin, Wang Chuan
Department of Pediatric, West China Second University Hospital, Sichuan University West China Medical School of Sichuan University The Cardiac Development and Early Intervention Unit, West China Institute of Women and Children's Health, West China Second University Hospital, Sichuan University Key Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University), Ministry of Education, Chengdu, Sichuan, China.
Medicine (Baltimore). 2017 Jun;96(24):e7199. doi: 10.1097/MD.0000000000007199.
Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare but severe congenital cardiac malformation. The prognosis mainly depends on the early and accurate diagnosis and treatment. However, without a typical and specific clinical manifestation in early stage, ALCAPA has a higher rate of false initial diagnosis.
Three infants with impaired left ventricle (LV) function, LV enlargement, mitral valve regurgitation (MR), and LV endocardium thickness were initially diagnosed as endocardial fibroelastosis (EFE). Due to the treatment effectiveness with prednisone acetate and digoxin, abnormal Q waves with T inversion, and dilated right coronary artery (RCA), the diagnosis of ALCAPA was suspected. Lastly, cardiac angiography confirmed the diagnosis. All of them were transferred to the cardiac surgery department and received a successful surgical repair. The follow-up results showed that abnormal Q waves with T waves inversion on electrocardiogram gradually regressed and disappeared, LV ejection fraction and LV dilation returned to a normal range after surgery, with alleviation of MR. Besides, endocardial thickness secondary to ischemia also returned to normal.
ALCAPA should be suspected when confronted with patients with left heart enlargement, impaired left ventricular function, and signs of myocardial ischemia, particularly in infancy. EFE is an important differential diagnosis and may also arise as a result of ALCAPA. Abnormal Q waves with T waves inversion, particularly in avL, dilated RCA and increased ratio of RCA/AO are important differential key points for the identification of ALCAPA and EFE. Awareness of this condition is essential for prompt recognition and referral to a tertiary cardiac center to enable early surgical intervention and improved prognosis for these children.
起源于肺动脉的异常左冠状动脉（ALCAPA）是一种罕见但严重的先天性心脏畸形。其预后主要取决于早期准确的诊断与治疗。然而，由于早期缺乏典型且特异的临床表现，ALCAPA的初始误诊率较高。
3例左心室（LV）功能受损、LV扩大、二尖瓣反流（MR）及LV心内膜增厚的婴儿最初被诊断为心内膜弹力纤维增生症（EFE）。因醋酸泼尼松和地高辛治疗有效、异常Q波伴T波倒置以及右冠状动脉（RCA）扩张，怀疑为ALCAPA。最后，心脏血管造影确诊。所有患儿均转入心脏外科并成功接受手术修复。随访结果显示，心电图上异常Q波伴T波倒置逐渐消退并消失，术后LV射血分数及LV扩张恢复至正常范围，MR减轻。此外，缺血所致的心内膜厚度也恢复正常。
对于左心扩大、左心室功能受损及心肌缺血征象的患者，尤其是婴儿，应怀疑ALCAPA。EFE是重要的鉴别诊断，也可能是ALCAPA的结果。异常Q波伴T波倒置，尤其是avL导联，RCA扩张及RCA/AO比值增加是鉴别ALCAPA和EFE的重要关键点。认识这种疾病对于及时识别并转诊至三级心脏中心至关重要，以便能早期进行手术干预并改善这些患儿的预后。
