Bilateral lung transplantation after caesarean section in pregnancy with severe pulmonary arterial hypertension: A case report

RATIONALE: Pulmonary arterial hypertension (PAH) can lead to an increase in right ventricular load and subsequently heart failure, making severe PAH a contraindication for pregnancy. In addition, PAH may worsen during pregnancy and puerperium, which requires high-quality critical care. This report is the first instance in which a patient with severe PAH, survived a successful atrial septal defect (ASD) repair and bilateral lung transplantation during puerperium. PATIENT CONCERNS: A 42-year-old pregnant woman with congenital heart disease (CHD) and severe PAH was admitted to our hospital for the management of pregnancy and delivery. The patient was diagnosed with severe PAH in 2013, and no significant improvements or deteriorations were found until this pregnancy-related hospital admission. DIAGNOSIS: The patient was diagnosed with CHD and severe PAH in 2013 with color Doppler echocardiography, right cardiac catheterization, and pulmonary perfusion imaging. The patient's mean pulmonary arterial pressure increased to 140 mm Hg during pregnancy, suggesting an exacerbated PAH with high risks to both her and the unborn child. INTERVENTIONS: The patient was treated with PAH-targeting treprostinil injection to reduce pulmonary artery pressure. Caesarean section was performed at 27 weeks and 5 days of gestation. The patient was put under extracorporeal membrane oxygenation (ECMO) with the help of local anesthesia before the operation. The investigators finally conducted a bilateral lung transplantation with a shell incision of the sternum under cardiopulmonary bypass. OUTCOMES: The mother and the neonate survived and recovered well after the operation, and were discharged from the hospital on the fourth month post-hospitalization. LESSONS: Severe PAH is an absolute contraindication for pregnancy. However, for patients who insist on a pregnancy, it could be plausible to proceed with a targeted drug therapy and ECMO after conducting a cesarean section, and finally, a lung transplantation. Multidisciplinary diagnosis and treatment is the key to the successful treatment of a PAH-complicated pregnancy.