Arow Ziad, Zilberman Liaz, Koifman Edward, Assali Abid, Arnson Yoav
Cardiology Department, Meir Medical Center, Tchernichovsky St 59, Kfar Saba 4418001, Israel.
Sackler Faculty of Medicine, Tel-Aviv University, Kiryat HaUniversita, Ramat Aviv, Tel Aviv 6139001, Israel.
Eur Heart J Case Rep. 2024 Dec 18;9(1):ytae672. doi: 10.1093/ehjcr/ytae672. eCollection 2025 Jan.
Anomalous origin of the left coronary artery (LCA) from the pulmonary artery (PA) (ALCAPA) is a rare congenital abnormality. We present a case of an ALCAPA in a 25-year-old man.
A 25-year-old male with no past medical history was admitted to our intensive cardiac care unit after sudden cardiac arrest due to ventricular fibrillation and suspected acute coronary syndrome. After being treated with shock by automated external defibrillator, an electrocardiogram (ECG) demonstrated sinus tachycardia with antero-lateral ST-segment elevation. Initial transthoracic echocardiography showed severe and diffuse left ventricular dysfunction and dilatation. Coronary angiography revealed anomalous origin of the LCA from the PA and extensive collateral circulation from a giant RCA. An ECG-gated cardiac computed tomography confirmed the diagnosis of anomalous left main originating from the left PA. Cardiac magnetic resonance demonstrated an enlarged left ventricle with globally reduced function and extensive sub-endocardial scarring of the anterior, antero-lateral, and lateral walls. Following a multidisciplinary heart team discussion, the patient successfully underwent repair of aberrant LCA with direct LCA re-implantation to the aorta and subcutaneous implantable cardioverter defibrillator implantation. Optimal medical therapy for heart failure with reduced ejection fraction was initiated, and the patient was discharged home for a close clinical and echocardiographic follow-up.
In conclusion, ALCAPA in the adulthood is a very rare congenital anomaly that clinicians should be aware of. The preferred treatment, when diagnosed in time, is direct re-implantation of the LCA to the aorta.
左冠状动脉(LCA)起源于肺动脉(PA)(ALCAPA)是一种罕见的先天性异常。我们报告一例25岁男性的ALCAPA病例。
一名无既往病史的25岁男性因室颤导致心脏骤停并怀疑急性冠状动脉综合征后入住我们的心脏重症监护病房。经自动体外除颤器电击治疗后,心电图(ECG)显示窦性心动过速伴前侧壁ST段抬高。最初的经胸超声心动图显示严重且弥漫性的左心室功能障碍和扩张。冠状动脉造影显示LCA起源于PA异常,且有来自巨大右冠状动脉(RCA)的广泛侧支循环。心电图门控心脏计算机断层扫描证实了左主干起源于左PA的诊断。心脏磁共振显示左心室扩大,整体功能降低,前壁、前侧壁和侧壁广泛的心内膜下瘢痕形成。经过多学科心脏团队讨论后,患者成功接受了异常LCA修复,将LCA直接重新植入主动脉,并植入皮下植入式心律转复除颤器。开始对射血分数降低的心力衰竭进行最佳药物治疗,患者出院回家进行密切的临床和超声心动图随访。
总之,成年期的ALCAPA是一种非常罕见的先天性异常,临床医生应予以关注。及时诊断时,首选的治疗方法是将LCA直接重新植入主动脉。