Crépin D, Jimenez M, Thambo J B, Girardot R, Choussat A
Service des maladies cardiovasculaires congénitales de l'adulte et de l'enfant, hôpital cardiologique du Haut-Lévêque, Pessac.
Arch Mal Coeur Vaiss. 2004 May;97(5):535-9.
Nearly 150 years after the description of Ebstein's anomaly the prognostic factors are not well known. Besides the foetal and neonatal forms which carry a poor prognosis the adult forms are often well tolerated and pose the difficult problem of management. This was a retrospective study of 73 patients with this malformation followed up between 1967 and 2002 at the University Hospital of Bordeaux. There were 14 deaths (19%) half of which (7) occurred during the first 3 months of life. The factors predictive of mortality (p<0.05) were: age, associated malformations, Classes III-IV of the New York Heart Association, cardiac failure, malaises, cyanosis, a raised cardiothoracic index, and a high mitro-tricuspid displacement indexed to body surface area. Supraventricular arrhythmia and the Wolff-Parkinson-White syndome do not seem to be associated with extramortality. Surgery, with reference to published studies, seems to improve survival, functional status and decrease the prevalence of arrhythmias. It would appear to be justified to propose surgery when functional status declines or when signs of poor prognosis are observed.
在埃布斯坦畸形被描述近150年后,其预后因素仍不为人所知。除了预后较差的胎儿和新生儿形式外,成人形式通常耐受性良好,但带来了管理难题。这是一项对73例患有这种畸形的患者进行的回顾性研究,这些患者于1967年至2002年在波尔多大学医院接受随访。有14例死亡(19%),其中一半(7例)发生在生命的前3个月。预测死亡率的因素(p<0.05)有:年龄、合并畸形、纽约心脏协会III-IV级、心力衰竭、不适、发绀、心胸指数升高以及根据体表面积计算的高二尖瓣-三尖瓣移位。室上性心律失常和预激综合征似乎与额外死亡率无关。参照已发表的研究,手术似乎可提高生存率、改善功能状态并降低心律失常的发生率。当功能状态下降或观察到预后不良迹象时,建议进行手术似乎是合理的。