PDF(Pubmed)
Abstract：
BACKGROUND: Anomalous aortic origin of a coronary artery from the inappropriate sinus of Valsalva (AAOCA) is a rare congenital heart lesion. It is uncommon for patients with AAOCA to present with severe symptoms at a very young age.
METHODS: We describe a very rare but critical presentation in a young infant with AAOCA that requires surgical repair and pacemaker placement. A three-month-old infant was referred because of syncope. Cardiac arrest occurred shortly after admission. The electrocardiogram indicated a complete atrioventricular block and a transvenous temporary pacemaker was implanted. A further coronary computed tomographic angiography (CTA) showed the anomalous origin of the right coronary artery from the left sinus of Valsalva. Coronary artery unroofing was performed due to an interarterial course with the intramural component, and a permanent epicardial pacemaker was implanted. The postoperative recovery was uneventful, and this patient was thriving and asymptomatic at the nine-month follow-up. However, the electrocardiogram still indicated a complete pacing rhythm.
CONCLUSIONS: By timely diagnosis and treatment, this patient is successfully rescued. Although rare, AAOCA may be fatal even in infants.
METHODS: We describe a very rare but critical presentation in a young infant with AAOCA that requires surgical repair and pacemaker placement. A three-month-old infant was referred because of syncope. Cardiac arrest occurred shortly after admission. The electrocardiogram indicated a complete atrioventricular block and a transvenous temporary pacemaker was implanted. A further coronary computed tomographic angiography (CTA) showed the anomalous origin of the right coronary artery from the left sinus of Valsalva. Coronary artery unroofing was performed due to an interarterial course with the intramural component, and a permanent epicardial pacemaker was implanted. The postoperative recovery was uneventful, and this patient was thriving and asymptomatic at the nine-month follow-up. However, the electrocardiogram still indicated a complete pacing rhythm.
CONCLUSIONS: By timely diagnosis and treatment, this patient is successfully rescued. Although rare, AAOCA may be fatal even in infants.
摘要：
背景：主动脉异常起源于Valsalva不适当窦（AAOCA）是一种罕见的先天性心脏病。AAOCA患者在很小的时候就出现严重的症状是不常见的。
方法：我们描述了一名AAOCA患儿的非常罕见但严重的表现，该患儿需要手术修复和起搏器放置。一名三个月大的婴儿因晕厥被转诊。入院后不久发生心脏骤停。心电图显示完全房室传导阻滞，并植入了经静脉临时起搏器。进一步的冠状动脉计算机断层扫描血管造影（CTA）显示右冠状动脉异常起源于Valsalva的左窦。由于与壁内组件的动脉间过程，进行了冠状动脉去顶，植入了永久性心外膜起搏器.术后恢复顺利,该患者在9个月的随访中表现良好且无症状。然而,心电图仍显示完整的起搏节律.
结论：通过及时诊断和治疗，这个病人成功获救。虽然罕见，AAOCA甚至在婴儿中也可能是致命的。
方法：我们描述了一名AAOCA患儿的非常罕见但严重的表现，该患儿需要手术修复和起搏器放置。一名三个月大的婴儿因晕厥被转诊。入院后不久发生心脏骤停。心电图显示完全房室传导阻滞，并植入了经静脉临时起搏器。进一步的冠状动脉计算机断层扫描血管造影（CTA）显示右冠状动脉异常起源于Valsalva的左窦。由于与壁内组件的动脉间过程，进行了冠状动脉去顶，植入了永久性心外膜起搏器.术后恢复顺利,该患者在9个月的随访中表现良好且无症状。然而,心电图仍显示完整的起搏节律.
结论：通过及时诊断和治疗，这个病人成功获救。虽然罕见，AAOCA甚至在婴儿中也可能是致命的。
