A63-24 Successful Delivery of a Pregnant Patient With Pulmonary Hypertension From Rheumatic Heart Disease

Abstract A 28-year-old female (G2P0) with rheumatic heart disease (RHD) presented at 28-weeks gestation for evaluation and management of high-risk pregnancy. She was diagnosed with RHD in the Philippines 3 years prior and begun medical management. On evaluation, the patient was afebrile with unlabored breathing. She had pitting edema to her bilateral thighs and an elevated jugular venous pressure. An updated TTE confirmed sequelae of RHD, with bi-atrial enlargement, severe MS, severe MR, moderate TR, a normal ejection fraction and a right ventricular (RV) systolic pressure of 82mmHg. The RV was normal in size with a mild decrease in function when measured by tricuspid annular pulmonary systolic excursion (TAPSE) and fractional area change with an enlarged left atrium. A right heart catheterization demonstrated a mean pulmonary artery pressure of 59mmHg and a pulmonary capillary wedge pressure of 17mmHg with a Fick cardiac output estimate of 2.2L/min correlating to a pulmonary vascular resistance of 19 wood units (WU). She was diagnosed with combined pre- and post-capillary pulmonary hypertension (PH), with a severe component of pre-capillary disease. A multidisciplinary team (MDT) evaluation recommended optimization of PH and planned preterm delivery at 32 weeks, however, worsening placental insufficiency necessitated urgent cesarian section at 28 weeks. Intravenous epoprostenol and inhaled nitric oxide were initiated and up-titrated for afterload reduction and loop diuretics for preload reduction. Arterial line and Swan-Ganz catheter were inserted for hemodynamic monitoring, and a femoral sheath was placed for extracorporeal membrane oxygenation backup.The operative course was unremarkable. Sildenafil was initiated postoperatively to provide additional afterload reduction, inotropic support was not required. The patient’s perioperative course was complicated by severe acute thrombocytopenia suspected to be secondary to epoprostenol therapy, which improved following transition from IV epoprostenol to IV treprostinil. On follow up, the patient remains clinically stable on treprostinil infusion 20ng/kg/min, sotatercept, macitentan, and sildenafil. Although our patient had severe disease, advances in pulmonary hypertension management have allowed patients to approach pregnancy with an individualized, risk-based assessment. This case illustrates the importance of shared decision-making and counseling rather than the strict avoidance that was recommended in the past. Women with PH who elect to become pregnant should be evaluated in academic centers with multidisciplinary teams, including PH specialists experienced in high-risk pregnancy and the complex challenges during and after pregnancy in the PH population. This abstract is funded by: None