Cardiovascular Sciences Research Centre, St. George's, University of London, London, United Kingdom.
Cardiovascular Sciences Research Centre, St. George's, University of London, London, United Kingdom; Istituto di Cardiologia, Ospedale Sant'Orsola-Malpighi, Alma Mater Studiorum University of Bologna, Bologna, Italy.
JACC Clin Electrophysiol. 2019 Apr;5(4):516-522. doi: 10.1016/j.jacep.2018.11.015. Epub 2019 Jan 30.
This study sought to describe the clinical and pathological features of anomalous origin of a coronary artery (AOCA) in sudden cardiac death (SCD) victims.
AOCA from the inappropriate sinus of Valsalva or from the pulmonary artery is increasingly diagnosed with current imaging techniques. AOCA is a possible cause of SCD.
We reviewed a database of 5,100 consecutive cases of SCD referred to our specialist cardiac pathology center between January 1994 and March 2017 and identified a subgroup of 30 cases (0.6%) with AOCA. All cases underwent detailed post-mortem evaluation including histological analysis by an expert cardiac pathologist. Clinical information was obtained from referring coroners.
The mean age was 28 ± 16 years and 23 individuals were male (77%). In 8 cases (27%), SCD occurred before 18 years of age. Cardiac symptoms were present in 11 individuals (37%), and syncope was the most common (n = 6, 20%). Anomalous left coronary artery arising from the right sinus of Valsalva (ALCA) with interarterial course (n = 11) and anomalous right coronary artery arising from the left sinus of Valsalva (ARCA) with interarterial course (n = 11) were the most common found. ALCA arising from pulmonary artery was present in 7 cases, whereas in 1 case, the left coronary artery arose from the noncoronary cusp. Left ventricular fibrosis was reported in 11 cases (37%) and was mainly subendocardial. There was evidence of acute infarction in 2 cases. Death occurred during exercise or emotional stress in 15 (50%) cases. The AOCA variant where death occurred more frequently during physical activity was ALCA (8 of 11, 73%), followed by ALCA arising from pulmonary artery (4 of 7, 57%) and ARCA (2 of 11, 18%).
AOCA is a rare cause of SCD. ALCA and ARCA with interarterial course are the most common anatomical variants recognized at the postmortem of SCD victims. ALCA is more commonly associated with death during exercise. Cardiac arrhythmias causing sudden death seem most likely in the cases without overt myocardial damage.
本研究旨在描述冠状动脉异常起源（AOCA）在心脏性猝死（SCD）患者中的临床和病理学特征。
目前的影像学技术越来越多地诊断出异常起源于瓦氏窦或肺动脉的 AOCA。AOCA 是 SCD 的一个可能原因。
我们回顾了 1994 年 1 月至 2017 年 3 月间我们专科心脏病病理中心收治的 5100 例连续 SCD 患者的数据库，从中确定了一个亚组 30 例（0.6%）有 AOCA。所有病例均进行了详细的尸检评估，包括由心脏病专家进行的组织学分析。临床信息来自转介的验尸官。
平均年龄为 28 ± 16 岁，23 名男性（77%）。8 例（27%）患者在 18 岁之前发生 SCD。11 例（37%）患者有心脏症状，最常见的是晕厥（n=6，20%）。最常见的是左冠状动脉异常起源于右瓦氏窦（ALCA）伴动脉间行程（n=11）和右冠状动脉异常起源于左瓦氏窦（ARCA）伴动脉间行程（n=11）。7 例为左冠状动脉异常起源于肺动脉，1 例为左冠状动脉异常起源于非冠状动脉瓣。11 例（37%）报告有左心室纤维化，主要为心内膜下。2 例有急性梗死证据。15 例（50%）在运动或情绪应激时死亡，其中 AOCA 变异型在体力活动时更易导致死亡，ALCA 占 8 例（11 例中的 73%），其次是左冠状动脉异常起源于肺动脉（7 例中的 4 例，57%）和 ARCA（11 例中的 2 例，18%）。
AOCA 是 SCD 的罕见原因。ALCA 和 ARCA 伴动脉间行程是 SCD 患者尸检中最常见的解剖变异。ALCA 更常与运动时死亡有关。在没有明显心肌损伤的情况下，心脏性心律失常引起的猝死似乎更有可能。
