Severe Pulmonary Arterial Hypertension in a 26-Week Pregnancy Complicated by Systemic Lupus Erythematosus

Abstract

Background: Pulmonary arterial hypertension is a rare condition characterized by vascular proliferation and remodeling of the pulmonary vessels. Despite seeing an improvement with modern management, pulmonary arterial hypertension continues to be associated with high rates of morbidity and mortality. This risk is known to increase even more during pregnancy, as the physiologic changes impose a significant threat to safety.

Case: A 26-year-old G2P0101 at 26+1 weeks gestation with severe pulmonary arterial hypertension, systemic lupus erythematosus, and chronic hypoxemic respiratory failure, presented with progressive dyspnea and hypoxemia. She required immediate ICU admission. Echocardiogram estimated a right ventricular systolic pressure of 100-110 mmHg. A multidisciplinary team managed her with advanced vasodilator therapy, diuresis, oxygen supplementation, and planned for controlled preterm surgical delivery with regional anesthesia.

Conclusion: This case highlights the unstable hemodynamic balance in pregnant patients with advanced pulmonary arterial hypertension and autoimmune disease.