Zeilinger G, Singer H, Deeg K H, Gutheil H
Klin Padiatr. 1986 Jan-Feb;198(1):53-7. doi: 10.1055/s-2008-1026853.
A cyanotic newborn is described to illustrate and discuss the rare transient tricuspid insufficiency together with functional pulmonary atresia. Etiologically the manifold causes of perinatal asphyxia are to be taken into consideration. The clinical appearance corresponds to type A of transient myocardial dysfunction according to the classification of Rowe together with respiratory distress syndrome and heart failure. Pathogenetically the disease is based on the different histological consequences of myocardial ischaemia of the right ventricle in connection with a delayed fall of the pulmonary arterial resistance. Fetal connections lead to a pronounced right-to-left-shunt. The diagnosis is primarily established by the way of clinical and echocardiographic investigation and pulsed doppler examination. The most important differential diagnosis are in Ebstein-anomaly, obstruction and atresia of the right ventricular outflow tract. Therapy is conservative. Its aim is to improve myocardial performance and to lower right ventricular afterload. Outcome of patients with neonatal tricuspid insufficiency is uncertain and depends on the severity of myocardial ischaemia. In our patient the course has been uneventful after initial difficulties.
描述了一名青紫型新生儿,以说明和讨论罕见的短暂性三尖瓣关闭不全合并功能性肺动脉闭锁。病因上,围产期窒息的多种原因都应予以考虑。临床表现符合罗伊(Rowe)分类中A型短暂性心肌功能障碍,同时伴有呼吸窘迫综合征和心力衰竭。发病机制上,该疾病基于右心室心肌缺血的不同组织学后果,以及肺动脉阻力延迟下降。胎儿期的连接导致明显的右向左分流。诊断主要通过临床、超声心动图检查和脉冲多普勒检查来确立。最重要的鉴别诊断是埃布斯坦畸形、右心室流出道梗阻和闭锁。治疗是保守的。其目的是改善心肌功能并降低右心室后负荷。新生儿三尖瓣关闭不全患者的预后不确定,取决于心肌缺血的严重程度。在我们的患者中,最初有困难之后病情一直平稳。