重度肺动脉高压并发自发性三尖瓣腱索断裂2例并文献复习

Spontaneous tricuspid valve chordal rupture in severe pulmonary hypertension:two cases report and literature review

目的分析重度肺动脉高压并发自发性三尖瓣腱索断裂患者的临床特点和治疗方法。方法对北京协和医院收治的2例重度肺动脉高压并发自发性三尖瓣腱索断裂患者的临床资料进行回顾性分析;结合文献中其他3例病例资料,总结该病临床特点,探讨治疗方法。结果临床表现:5例患者均表现为原有病情急性加重并快速进展,4例出现心原性休克,1例表现为顽固的重度右心功能不全。治疗及转归:1例患者行急诊三尖瓣置换术,术后临床症状缓解;1例患者行房间隔球囊造口术,术后临床症状轻度减轻;1例患者行肺移植术,围术期死亡。临床随访:2例患者分别在发病4周和8个月死于心原性休克和多脏器功能衰竭。结论自发性三尖瓣腱索断裂是重度肺动脉高压患者的少见并发症,患者病情危重,多进展为心原性休克,预后不佳;部分患者可以通过三尖瓣手术治疗缓解病情;心肺移植可能是此类患者最终的治疗方案,球囊房间隔造口术可以作为一种姑息治疗的方法。

Objective To analyze the clinical characteristics and treatment of spontaneous tricuspid valve chordal rupture in severe pulmonary hypertension patients.Methods The clinical data of 2 patients with spontaneous rupture of tricuspid valve chordal in severe pulmonary hypertension treated in Peking Union Medical College Hospital were analyzed retrospectively.Combined with the data of other 3 cases in the literature,the clinical characteristics and the management of this disease were discussed.Results Clinical manifestations:all patients showed acute aggravation and rapid progression,four had cardiogenic shock,one showed intractable right cardiac heart failure.Treatment and outcome:one patient relieved after an emergency tricuspid valve replacement,one patient made a slightly progress after balloon atrial septostomy,one patient died after lung transplantation.Clinical follow-up:two patients died at 4 weeks and 8 months later because of cardiogenic shock and multiple organ failure.Conclusions Spontaneous rupture of tricuspid valve chordal is a rare complication in patients with severe pulmonary hypertension.Most patients progressed to cardiogenic shock with a poor prognosis.Some patients can be relieved by tricuspid valve surgery.Cardiopulmonary transplantation may be the final treatment for such patients,and balloon atrial septostomy can be used as a palliative treatment.