Moura C, Guimarães H, Areias J C, Moreira J
Serviço de Cardiologia Pediátrica e de Neonatologia Departamento de Pediatria do Hospital de S. João, Porto.
Rev Port Cardiol. 2001 Sep;20(9):865-73.
Ebstein's anomaly is a rare congenital heart disease abnormality in which the tricuspid valve leaflets do not attach normally to the tricuspid valve annulus. The effective tricuspid valve orifice is displaced apically into the right ventricle (RV), near the junction of the inlet and the trabecular parts of the RV. The authors present a retrospective study of the patients with Ebstein's anomaly admitted to a neonatal intensive care unit, in the period between January 1993 and March 2000. There were ten patients, representing 0.24% of total neonates and 1.99% of total congenital heart disease admitted to the institution in the same period. Fifty per cent were male and only one case had prenatal diagnosis. Holosystolic murmur (100%) from tricuspid regurgitation and cyanosis (80%) were the most frequent clinical findings. Chest X-ray was abnormal in 90% of the neonates, with a "balloon-shaped" enlarged heart. The main electrocardiographic findings were right atrial enlargement (70%) and arrhythmias (40%). Apical displacement of the septal leaflet of the tricuspid valve, to a maximum of 20 mm, and leaflets tethering to underlying RV myocardium were found in all patients. Tricuspid valve regurgitation was found in 90% (severe form in four cases). An atrial intracardiac shunt, mostly right-to-left, was also found in 50%. Digoxin was used (40%) to restore sinus rhythm. Fifty per cent of the neonates received intravenous prostaglandins. Two patients required a surgical procedure. Two patients died in the neonatal period. During the follow-up period (range 0.3-74.6 months), only one episode of supraventricular tachycardia was recorded. At present seven patients are clinically stable, three of them on medication.
埃布斯坦畸形是一种罕见的先天性心脏病异常，其中三尖瓣小叶不能正常附着于三尖瓣环。有效的三尖瓣口向心尖移位至右心室，靠近右心室流入道与小梁部的交界处。作者对1993年1月至2000年3月期间入住新生儿重症监护病房的埃布斯坦畸形患者进行了一项回顾性研究。共有10例患者，占同期该机构收治新生儿总数的0.24%，先天性心脏病总数的1.99%。50%为男性，仅有1例产前诊断。三尖瓣反流的全收缩期杂音（100%）和发绀（80%）是最常见的临床表现。90%的新生儿胸部X线检查异常，心脏呈“气球样”增大。主要心电图表现为右心房增大（70%）和心律失常（40%）。所有患者均发现三尖瓣隔叶向心尖移位，最大达20mm，小叶与下方右心室心肌相连。90%发现三尖瓣反流（4例为重度）。50%还发现心内房水平分流，大多为右向左分流。40%使用地高辛恢复窦性心律。50%的新生儿接受了静脉注射前列腺素。2例患者需要手术治疗。2例患者在新生儿期死亡。在随访期（0.3 - 74.6个月），仅记录到1次室上性心动过速发作。目前7例患者临床稳定，其中3例正在服药。
