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首页> 外文期刊>Journal of Medical Case Reports >Successful pregnancy in pulmonary arterial hypertension associated with systemic lupus erythematosus: a case report
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Successful pregnancy in pulmonary arterial hypertension associated with systemic lupus erythematosus: a case report

机译：合并系统性红斑狼疮的肺动脉高压成功妊娠：一例

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Introduction Pulmonary arterial hypertension is a complication of systemic lupus erythematosus. Mortality in pregnant patients with pulmonary arterial hypertension related to connective tissue disease is as high as 56%. The authors report the first case of a successful maternal-fetal outcome in a pregnant patient with systemic lupus erythematosus-associated pulmonary arterial hypertension treated with sildenafil and inhaled iloprost during pregnancy and until several weeks after caesarean section. Case presentation The case presented is of a 29-year-old woman with systemic lupus erythematosus and associated severe pulmonary arterial hypertension. Vasodilator therapy with bosentan and sildenafil, immunosuppressive therapy with prednisone, hydroxychloroquine and azathioprine and oral anticoagulation (phenprocoumon) had normalized her right ventricular over right atrial pressure when she was diagnosed in her 5th week of pregnancy. The teratogenic drugs bosentan and phenprocoumon were stopped, the latter replaced by low molecular weight heparin. During the 35th week, a slight increase in pulmonary pressure was found. Therapy with inhaled iloprost was established. A caesarean section was performed in the 37th week and a healthy baby was delivered. The patient remained stable until 11 weeks after delivery, when an increase in right ventricular over right atrial pressure was noted. Bosentan was reintroduced and prednisone and azathioprine doses were increased. The patient has remained stable until the present time. Conclusion Pulmonary arterial hypertension has been considered a contraindication for pregnancy. Novel vasodilator therapy, combined with immunosuppressants in this patient with systemic lupus erythematosus, may "cure" pulmonary arterial hypertension and permit pregnancy with successful outcome. However, postpartum exacerbation of systemic lupus erythematosus and pulmonary arterial hypertension have to be considered.
机译：引言肺动脉高压是系统性红斑狼疮的并发症。与结缔组织疾病有关的肺动脉高压孕妇的死亡率高达56％。作者报告了在妊娠期直至剖腹产后几周接受西地那非和吸入伊洛前列素治疗的系统性红斑狼疮相关性肺动脉高压孕妇的成功母胎结果。病例介绍该病例为一名29岁系统性红斑狼疮并伴有严重肺动脉高压的妇女。当在怀孕的第5周被确诊时，波生坦和西地那非的血管舒张药疗法，泼尼松，羟氯喹和硫唑嘌呤的免疫抑制疗法以及口服抗凝剂（苯丙香酚）使她的右心室压力恢复正常，超过了右心房压力。终止了致畸药波生坦和苯普鲁蒙，后者被低分子量肝素代替。在第35周，发现肺动脉压力略有增加。建立了吸入伊洛前列素的治疗方法。在第37周进行了剖腹产，并分娩了健康的婴儿。当观察到右心室压力超过右心房压力增加时，患者保持稳定直至分娩后11周。重新引入波生坦，并增加了泼尼松和硫唑嘌呤的剂量。病人一直保持稳定直到现在。结论肺动脉高压被认为是妊娠的禁忌症。对于患有系统性红斑狼疮的患者，新颖的血管扩张药疗法与免疫抑制剂相结合可以“治愈”肺动脉高压，并成功妊娠。但是，必须考虑全身性红斑狼疮的产后病情加重和肺动脉高压。

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