Streit Michael, Speich Rudolf, Fischler Manuel, Ulrich Silvia
Department of Internal Medicine, University Hospital of Zurich Raemistrasse 100, 8091 Zurich Switzerland.
J Med Case Rep. 2009 Jun 9;3:7255. doi: 10.4076/1752-1947-3-7255.
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.
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.
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.
肺动脉高压是系统性红斑狼疮的一种并发症。患有与结缔组织病相关的肺动脉高压的孕妇死亡率高达56%。作者报告了首例成功实现母婴良好结局的病例，该孕妇患有系统性红斑狼疮相关的肺动脉高压，在孕期及剖宫产术后数周接受了西地那非和吸入用伊洛前列素治疗。
该病例为一名29岁患有系统性红斑狼疮及相关严重肺动脉高压的女性。在她怀孕第5周被诊断时，使用波生坦和西地那非进行血管扩张剂治疗、使用泼尼松、羟氯喹和硫唑嘌呤进行免疫抑制治疗以及口服抗凝剂（苯丙香豆素）已使她的右心室与右心房压力恢复正常。致畸药物波生坦和苯丙香豆素停用，后者被低分子量肝素取代。在第35周时，发现肺动脉压力略有升高。于是开始吸入用伊洛前列素治疗。在第37周进行了剖宫产，产下一名健康婴儿。患者在分娩后11周内保持稳定，之后右心室与右心房压力出现升高。重新引入波生坦，并增加泼尼松和硫唑嘌呤的剂量。患者至今一直保持稳定。
肺动脉高压一直被视为妊娠的禁忌证。在这名系统性红斑狼疮患者中，新型血管扩张剂疗法与免疫抑制剂联合使用，可能“治愈”肺动脉高压并使妊娠获得成功结局。然而，必须考虑系统性红斑狼疮和肺动脉高压在产后的病情加重情况。
