Suzuki H, Nakasato M, Sato S, Komatsu H, Hayasaka K
Department of Pediatrics, Yamagata University School of Medicine, Japan.
Tohoku J Exp Med. 1997 Apr;181(4):459-65. doi: 10.1620/tjem.181.459.
Ebstein's anomaly is a rare congenital cardiac anomaly showing significant clinical manifestations with a high mortality rate in the neonatal period. The prognosis of the patient is essentially determined by the severity in morphological changes, however, high pulmonary vascular resistance in the neonatal period may aggravate tricuspid regurgitation and lead to functional pulmonary atresia. We describe a critically ill neonate with morphologically mild Ebstein's anomaly who was successfully managed with intensive care including isoproterenol administration for functional pulmonary atresia. Isoproterenol is a potent pulmonary vasodilator with inotropic and chronotropic effects, and seemed to decrease the pulmonary vascular resistance allowing increased antegrade blood flow to the pulmonary artery and improved cardiac output. If tachycardia is not present, isoproterenol administration is recommended in critically ill neonates with anatomically mild Ebstein's anomaly and no associated cardiac defects.
埃布斯坦畸形是一种罕见的先天性心脏畸形，在新生儿期表现出显著的临床表现且死亡率很高。患者的预后基本上由形态学改变的严重程度决定，然而，新生儿期高肺血管阻力可能会加重三尖瓣反流并导致功能性肺动脉闭锁。我们描述了一名患有形态学上轻度埃布斯坦畸形的危重新生儿，通过包括使用异丙肾上腺素治疗功能性肺动脉闭锁在内的重症监护成功得到救治。异丙肾上腺素是一种强效的肺血管扩张剂，具有正性肌力和变时性作用，似乎能降低肺血管阻力，使肺动脉前向血流增加并改善心输出量。如果不存在心动过速，建议对患有解剖学上轻度埃布斯坦畸形且无相关心脏缺陷的危重新生儿使用异丙肾上腺素。
