Sudden Death as a Complication of Anomalous Left Coronary Origin From the Anterior Sinus of Valsalva: A Not‐So‐Minor Congenital Anomaly
Sudden Death as a Complication of Anomalous Left Coronary Origin From the Anterior Sinus of Valsalva: A Not‐So‐Minor Congenital Anomaly
复制标题
猝死是源自瓦尔萨尔瓦前窦的左冠状动脉异常的并发症：一种不那么轻微的先天性异常
作者：
M. Cheitlin;C. D. De Castro;H. Mcallister
来自同一瓦氏窦的两条冠状动脉作为单血管或双血管被认为是不影响寿命的轻微先天性异常。文献中很少有关于患有这种冠状动脉起源异常的年轻男性猝死的病例报告。我们审查了来自武装部队病理研究所先天性心脏病样本的 51 例此类病例。有 33 名患者的双侧冠状动脉均起源于 Valsalva 前窦，无论是单支还是双血管，18 名患者的冠状动脉起源于左冠状动脉 Valsalva 窦。在 33 名冠状动脉起源于瓦尔萨尔瓦前窦的患者中，有 9 名（27.3%）死于不明原因的突然死亡。双侧冠状动脉均来自左瓦尔萨尔瓦窦的患者中，没有发生不明原因的突然死亡。显然，如果左冠状动脉不在肺动脉和主动脉之间急剧向后和向左通过，则不存在猝死的风险。所有突然死亡的患者均为男性。在这些病例中，推测的猝死机制是冠状动脉沿着主动脉壁急性向左通道导致左冠状系统的入口呈狭缝状。在心脏活动增加、肺动脉和主动脉随着运动而扩张的情况下，左冠状动脉会被拉伸，左冠状动脉口会出现瓣状闭合，从而导致突然致命的心肌缺血。我们还介绍了第一个病例，在一名 14 岁男孩中发现了这种异常，并尝试通过手术在左冠状动脉中创建一个不可塌陷的漏斗状开口来纠正该问题。
Both coronary arteries arising as a single or double vessel from the same sinus of Valsalva has been considered a minor congenital anomaly not affecting longevity. There are rare case reports in the literature of sudden death in young males with this anomaly of coronary origin. We have reviewed 51 such cases from the Armed Forces Institute of Pathology congenital heart disease accessions. There were 33 patients in whom both coronaries arose from the anterior sinus of Valsalva either as a single or double vessel and 18 in whom they arose from the left coronary sinus of Valsalva. Out of the 33 patients in whom the coronaries arose from the anterior sinus of Valsalva, 9 (27.3%) died sudden, unexplained deaths. There were no sudden unexplained deaths among the patients in whom both coronaries arose from the left sinus of Valsalva. It is evident that where the left coronary artery does not pass acutely posterior and leftward between the pulmonary artery and the aorta there is no risk of sudden death. All patients who died suddenly were male. The suggested mechanism for sudden death in these cases is that the acute leftward passage of the coronary artery along the aortic wall causes the entrance into the left coronary svstem to be slit-like. Under circumstances of increased cardiac activity with increased expansion of the pulmonary artery and aorta with exercise, there is stretching of the left coronary artery and a flap-like closure of the orifice of the left coronary with sudden, fatal myocardial ischemia.We also present the first case where this anomaly was recognized in a 14-year-old boy and a surgical attempt was made to correct the problem by creating a non-collapsible funnel-like opening into the left coronary artery.