Abstract
Systemic lupus erythematosus (SLE)-associated severe pulmonary hypertension developing in pregnancy is a rare and clinically serious phenomenon. Mortality in this patient population is as high as 56%. We present a case of new onset SLE-associated pulmonary hypertension with in-vitro fertilization (IVF) twin pregnancy. This is a 44-year-old Chinese woman with past medical history significant for SLE who initially presented to outpatient pulmonology clinic with progressive shortness of breath during the 21st week of her IVF pregnancy. Initial work up included a transthoracic echocardiogram that estimated pulmonary artery systolic pressure to be 55 mm Hg. Further evaluation including right heart catheterization revealed mean pulmonary artery systolic pressure of 48 mm Hg. At 25 weeks into pregnancy, she was admitted to the medical intensive unit for epoprostenol infusion under the care of a multidisciplinary team including pulmonology, obstetrics-gynecology, neonatology, and cardiology. Additional management included oral furosemide, fluid restriction and sildenafil. Enoxaparin for thrombosis prophylaxis was discontinued due to significant thrombocytopenia that developed during hospitalization. She did not develop a lupus flare during her hospitalization while on hydroxychloroquine and prednisone. Patient delivered at 30 weeks via cesarean section under slow epidural infusion. A viable male infant and female infant were delivered without complications. She continued epoprostenol infusion until worsening thrombocytopenia led to transition to treprostinil therapy. She was discharged from the hospital in stable condition and is doing well on continued treprostinil infusion with sildenafil. Combination of immunosuppressive and intravenous-vasodilator therapy have been implemented and subsequently reported in available case studies of this rare phenomenon. A similar regimen aimed at management of profound hemodynamic changes and lupus control was successfully utilized in the care of this patient with twin pregnancy, which may suggest a potential standard of care in these rare cases. Patients in the postpartum are at particular risk for mortality due to the loss of pulmonary vasodilation that normally occurs, leading to right ventricular failure. Long term prognosis and complications of SLE-associated pulmonary hypertension are still under investigation.