Del Pasqua A, de Zorzi A, Sanders S P, Rinelli G
Cardiology Department, Università degli Studi di Siena, Siena, Italy.
Pediatr Cardiol. 2008 Jan;29(1):217-9. doi: 10.1007/s00246-007-9097-8. Epub 2007 Sep 14.
Ebstein's anomaly is a rare congenital heart defect. Associated lesions are uncommon, and the mortality rates can be as high as 54% during the first month of life. Two cases of severe Ebstein's anomaly with ventricular septal defect are described. It is speculated that this rare association, allowing adequate forward pulmonary blood flow in the neonate, permitted the reported patients to survive the neonatal period, which is the most life-threatening time. The authors propose that the presence of a small ventricular septal defect can be beneficial for such patients, averting the need for surgery during early infancy when the risk is highest.
埃布斯坦畸形是一种罕见的先天性心脏缺陷。相关病变并不常见，在出生后的第一个月死亡率可达54%。本文描述了两例伴有室间隔缺损的严重埃布斯坦畸形病例。据推测，这种罕见的关联使得新生儿期有足够的肺前向血流，从而使报告的患者在最具生命危险的新生儿期存活下来。作者提出，小室间隔缺损的存在可能对这类患者有益，避免了在婴儿早期风险最高时进行手术的必要性。
