Division of Cardiology, Mount Sinai Heart, Mount Sinai Hospital, Icahn School of Medicine at Mount Sinai, New York, NY 10029, USA.
Department of Systems Medicine, Division of Cardiology, University of Rome "Tor Vergata", 00133 Rome, Italy.
Int J Environ Res Public Health. 2022 Jan 29;19(3):1554. doi: 10.3390/ijerph19031554.
Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare coronary artery anomaly that carries 90% mortality in the first year of life when left untreated. The diagnosis of ALCAPA is rare in adulthood, and it includes a broad spectrum of clinical manifestations, including sudden cardiac death (SCD).
We report a rare case of resuscitated sudden cardiac arrest in a 55-year-old female, who was diagnosed with ALCAPA and underwent successful surgical correction and implantable cardioverter defibrillator (ICD) implantation for secondary prevention.
ALCAPA diagnosis is not confined to childhood, and it represents a rare cause of life-threatening arrhythmias and SCD in the adult population. Surgical correction is recommended, regardless of age, presence of symptoms or inducible myocardial ischemia. Multimodality imaging is crucial for diagnosis, management planning and follow up. Assessment of the risk of recurrent ventricular arrhythmias, despite full revascularization, should be performed in all adults with ALCAPA. Myocardial scar detected via late gadolinium enhancement represents a potential irreversible substrate for ventricular arrhythmias, and it provides additional information to evaluate indication of an ICD for secondary prevention.
起源于肺动脉的左冠状动脉异常（ALCAPA）是一种罕见的冠状动脉异常，如果不治疗，90%的患者会在生命的第一年死亡。ALCAPA 在成年期的诊断较为罕见，它包括广泛的临床表现，包括心源性猝死（SCD）。
我们报告了一例罕见的 55 岁女性复苏性心搏骤停病例，该患者被诊断为 ALCAPA，并接受了成功的手术矫正和植入式心律转复除颤器（ICD）植入术以进行二级预防。
ALCAPA 的诊断并不局限于儿童时期，它在成年人群中也是一种罕见的、危及生命的心律失常和 SCD 的病因。无论年龄、症状或可诱导性心肌缺血如何，均推荐进行手术矫正。多模态成像对于诊断、管理计划和随访至关重要。应在所有患有 ALCAPA 的成年人中评估尽管完全血运重建后是否仍存在复发性室性心律失常的风险。通过钆延迟增强检测到的心肌瘢痕代表了室性心律失常的潜在不可逆基质，它为评估 ICD 进行二级预防的适应证提供了额外信息。
