Bonello Béatrice, Renard Sébastien, Dragulescu Andréea, Habib Gilbert, Fraisse Alain
Cardiologie pédiatrique et congénitale, hôpital d'Enfants de la Timone, Marseille, France.
Presse Med. 2009 Dec;38 Suppl 1:1S10-3. doi: 10.1016/S0755-4982(09)73418-9.
Eisenmenger syndrome is the last-stage of congenital heart diseases with untreated left to right shunt. Tolerance and prognosis are better than with idiopathic pulmonary hypertension. However, patients follow-up is often performed in inappropriate non-specialized care centres which makes the collection of epidemiological data difficult. Consequently, population registries are necessary and would increase knowledge of natural history. Finally, complications are often multiple and unexpected with lack of specialized management. Such patients require expert supervision with a multidisciplinary approach in experienced and specialized centres.
艾森曼格综合征是先天性心脏病左向右分流未治疗的终末期阶段。耐受性和预后优于特发性肺动脉高压。然而，患者随访往往在不合适的非专科护理中心进行，这使得流行病学数据的收集变得困难。因此，有必要建立人群登记系统，这将增加对自然病史的了解。最后，并发症往往是多发且意想不到的，同时缺乏专科管理。这类患者需要在经验丰富的专科中心接受多学科方法的专家监督。
