A case of postpartum pulmonary hypertension complicated with mixed connective tissue disease

A 34-year-old pregnant woman was diagnosed with mixed connective tissue disease (MCTD), complicated with postpartum pulmonary hypertension (PH). She was taking 10 mg oral prednisolone during pregnancy. At 29 weeks’ gestation, systolic blood pressure increased and proteinuria developed, and she was admitted for preeclampsia. However, at 32 and 3/7 weeks’ gestation, her blood pressure elevated to 195/117 mmHg, and her platelet count decreased to 96×103/μl. Thus, she was diagnosed as severe preeclampsia, and an emergency cesarean section was performed. At postpartum 1 day, she complained of dyspnea and palpitation. Pericardial effusion and right ventricle enlargement were detected on cardiac ultrasound. Her tricuspid regurgitation peak gradient was elevated (40 mmHg), indicating the presence of PH. Accordingly, prednisolone 30 mg/day was administered, and her dyspnea improved. Her blood pressure decreased and her proteinuria disappeared on day 10 postpartum. She was discharged on day 14 with oral prednisolone. Evaluation of cardiac and pulmonary functions before pregnancy in MCTD-complicated patients and careful observation for PH during and after pregnancy is recommended.