Alghamdi Fatima Abdullah, Shah Muhammad Azam, Alshalash Saleh, Alkhodair Abdullah Mohammed
Adult Cardiology Department, King Fahad Medical City, Dabab street, Sulaimaniya, PO Box 221124, Riyadh 11311, Saudi Arabia.
Eur Heart J Case Rep. 2023 May 2;7(5):ytad230. doi: 10.1093/ehjcr/ytad230. eCollection 2023 May.
Anomalous origin of the coronary artery from the pulmonary artery (ALCAPA) is a rare congenital disease. Surgical re-implantation of the left main coronary artery (LMCA) to the aorta is a definitive treatment with a good prognosis.
A 9-year-old boy was admitted with a complaint of exertional chest pain and dyspnoea. At 13 months of age, he was diagnosed to have ALCAPA as a workup of severe left ventricular systolic dysfunction and underwent coronary re-implantation of ALCAPA. Coronary angiogram displayed the high takeoff of re-implanted LMCA with significant ostial stenosis, and echocardiogram showed significant supravalvular pulmonary stenosis (SVPS) with a peak gradient of 74 mmHg. After a multidisciplinary team discussion, he underwent percutaneous coronary intervention with stenting to ostial LMCA. On follow-up, he was asymptomatic and a cardiac computed tomography scan showed a patent stent in LMCA with an under-expanded area in the mid-segment. The proximal part of the LMCA stent was located very close to the stenotic segment of the main pulmonary artery making it a high risk for balloon angioplasty. The surgical intervention of SVPS is delayed to allow the somatic growth of the patient.
Percutaneous coronary intervention in re-implanted LMCA is a feasible option. If stenosis of re-implanted LMCA is accompanied by SVPS, the latter can be best treated surgically and staged to decrease the operative risk. Our case also demonstrates the importance of long-term follow-up of post-operative complications of patients with ALCAPA.
冠状动脉起源于肺动脉(ALCAPA)是一种罕见的先天性疾病。将左冠状动脉主干(LMCA)手术重新植入主动脉是一种确定性治疗方法,预后良好。
一名9岁男孩因活动时胸痛和呼吸困难入院。13个月大时,他因严重左心室收缩功能障碍检查被诊断为ALCAPA,并接受了ALCAPA冠状动脉重新植入术。冠状动脉造影显示重新植入的LMCA高位起始且开口处有明显狭窄,超声心动图显示有明显的肺动脉瓣上狭窄(SVPS),峰值压差为74 mmHg。经过多学科团队讨论,他接受了经皮冠状动脉介入治疗并在LMCA开口处植入支架。随访时,他无症状,心脏计算机断层扫描显示LMCA支架通畅,但中段有未充分扩张区域。LMCA支架近端非常靠近主肺动脉狭窄段,这使得球囊血管成形术风险很高。SVPS的手术干预推迟,以便患者身体生长。
对重新植入的LMCA进行经皮冠状动脉介入治疗是一种可行的选择。如果重新植入的LMCA狭窄伴有SVPS,后者最好通过手术分期治疗以降低手术风险。我们的病例还证明了对ALCAPA患者术后并发症进行长期随访的重要性。