RESUMEN
BACKGROUND: Pulmonary arterial hypertension (PAH) in pregnancy is one of the major obstetric complications and is considered a contraindication to pregnancy as it is classified as a class IV risk in the revised risk classification of pregnancy by the World Health Organisation. Pregnancy, with its adaptive and expectant mechanical and hormonal changes, negatively affects the cardiopulmonary circulation in pregnant women. Do patients with repaired simple congenital heart disease (CHD) develop other pulmonary and cardiac complications during pregnancy? Can pregnant women with sudden pulmonary hypertension be treated and managed in time? In this paper, we present a case of a 39-year-old woman who underwent cesarean section at 33 wk' gestation and developed PAH secondary to repaired simple CHD. Our research began by a PubMed search for "pulmonary hypertension" and "pregnancy" and "CHD" case reports. Three cases were selected to review PAH in pregnancy after correction of CHD defects. These studies were reviewed, coupled with our own clinical experience. CASE SUMMARY: Herein, a case involving a woman who underwent atrial septal defect repair at the age of 34, became pregnant five years later, and had a sudden onset of PAH and right heart failure secondary to symptoms of acute peripheral edema in the third trimester of her pregnancy. As a result, the patient underwent a cesarean section and gave birth to healthy twins. Within three days after cesarean delivery, her cardiac function deteriorated as the pulmonary artery pressure increased. Effective postpartum management, including diuresis, significant oxygen uptake, vasodilators, capacity and anticoagulants management, led to improvements in cardiac function and oxygenation. The patient was discharged from hospital with a stable recovery and transferred to local hospitals for further PAH treatment. CONCLUSION: This case served as a reminder to obstetricians of the importance of pregnancy after repair of CHD. It is crucial for patients with CHD to receive early correction. It suggests doctors should not ignore edema of twin pregnancy. Also, it provides a reference for the further standardization of antenatal, intrapartum and postpartum management for patients with CHD worldwide.