813: THE NATURAL PROGRESSION OF PULMONARY HYPERTENSION AND SICKLE CELL DISEASE IN PREGNANCY

Abstract

Learning Objectives: Pulmonary hypertension (PH) presents a particular challenge to obstetricians. With its associated maternal mortality rate of 30–50%, it is considered a relative contraindication to pregnancy, and thus encountered with increasing rarity. Most obstetricians recommend pregnancy avoidance and/or termination for the condition; and very sparse modern data exist regarding the natural progression of the disease during gestation and in subsequent pregnancies. Methods: We describe a case series of five pregnancies in the same patient, managed between the ages of 18 to 33 years. She has a long-standing history of sickle cell disease and developed what is believed to be group 5 PH in her second pregnancy. Pregnancy 1 was notable for sickle cell pain crises, encountered mostly in the third trimester and postpartum period. Pregnancy 2 was notable for more frequent sickle cell crises including acute chest syndrome and diagnosis of pulmonary embolus in the first postpartum week. Work-up at that time included right-heart catheterization (RHC) noting mean pulmonary artery pressure (PAP) of 35 mmHg. In pregnancies 3 and 4, she was managed with prophylactic anticoagulation and required serial exchange transfusion for very frequent sickle cell pain crises. RHC in the second trimesters demonstrated apparent worsening of disease with PAP of 51 and 60 mmHg respectively. Pregnancy 5 was further complicated by nearly daily visits to the emergency room for sickle cell pain exacerbated by opioid use disorder. RHC in the first trimester demonstrated PAP of 70 mmHg and the patient was counseled regarding pregnancy termination. She was managed in concert with Pulmonology and Hematology and consideration was given to targeted prostaglandin vasodilator therapy. Results:Sickle cell disease, especially with pulmonary involvement can contribute to development of PH affecting pregnancy. As the majority of maternal deaths related to PH occur during delivery and the first postpartum month, diligence must be maintained when managing labor and the puerperium. Intrapartum management should be individualized and focus on preventing fluid overload, hypotension and hypoxia, which may elicit arrhythmia and exacerbate PH. Co-management with pulmonology is requisite and serial examination with echocardiography and/or RHC help to stratify disease progression.