Baufreton Christophe, Bruneval Patrick, Rousselet Marie-Christine, Ennezat Pierre-Vladimir, Fouquet Olivier, Giraud Raphael, Banfi Carlo
aDepartment of Cardiovascular Surgery, Centre Hospitalier Universitaire d'Angers bDepartment of Pathology, Hôpital Européen Georges Pompidou, Paris Descartes University cDepartment of Pathology, Centre Hospitalier Universitaire d'Angers dDepartment of Cardiology, Centre Hospitalier Universitaire de Grenoble, France eIntensive Care Service, Geneva University Hospitals fHemodynamic Research Group Geneva, Faculty of Medicine, University of Geneva gDivision of Cardiovascular Surgery, Geneva University Hospitals, Geneva, Switzerland.
Medicine (Baltimore). 2017 Jan;96(2):e4985. doi: 10.1097/MD.0000000000004985.
Drug-induced valvular heart disease (DI-VHD) remains an under-recognized entity.
This report describes a heart valve replacement which was complicated by intractable systemic pulmonary arterial hypertension in a 61-year-old female with severe restrictive mitral and aortic disease. The diagnosis of valvular disease was preceded by a history of unexplained respiratory distress. The patient had been exposed to benfluorex for 6.5 years.
The diagnostic procedure documented specific drug-induced valvular fibrosis.
Surgical mitral and aortic valve replacement was performed.
Heart valve replacement was postoperatively complicated by unanticipated disproportionate pulmonary hypertension. This issue was fatal despite intensive care including prolonged extracorporeal life support.
Benfluorex is a fenfluramine derivative which has been marketed between 1976 and 2009. Although norfenfluramine is the common active and toxic metabolite of all fenfluramine derivatives, the valvular and pulmonary arterial toxicity of benfluorex was much less known than that of fenfluramine and dexfenfluramine. The vast majority of benfluorex-induced valvular heart disease remains misdiagnosed as hypothetical rheumatic fever due to similarities between both etiologies. Better recognition of DI-VHD is likely to improve patient outcome.
药物性瓣膜性心脏病（DI-VHD）仍是一个未得到充分认识的病症。
本报告描述了一名61岁患有严重限制性二尖瓣和主动脉疾病的女性，其心脏瓣膜置换术后并发难治性系统性肺动脉高压。在瓣膜疾病诊断之前，患者有无法解释的呼吸窘迫病史。该患者曾服用苄氟雷司6.5年。
诊断程序记录了特定的药物性瓣膜纤维化。
进行了二尖瓣和主动脉瓣置换手术。
心脏瓣膜置换术后出现意外的不成比例的肺动脉高压并发症。尽管进行了包括延长体外生命支持在内的重症监护，这个问题还是导致了患者死亡。
苄氟雷司是一种氟苯丙胺衍生物，于1976年至2009年上市。虽然去甲氟苯丙胺是所有氟苯丙胺衍生物常见的活性和有毒代谢物，但苄氟雷司的瓣膜和肺动脉毒性比氟苯丙胺和右芬氟拉明要少为人知得多。由于两种病因之间存在相似性，绝大多数苄氟雷司引起的瓣膜性心脏病仍被误诊为疑似风湿热。更好地认识DI-VHD可能会改善患者的预后。
