VALENCIA, Spain—The management of patients with pulmonary arterial hypertension (PAH) during pregnancy was outlined by Constantin von Kaisenberg, MD, PhD, from the departments of obstetrics, gynecology, and reproductive medicine at Hannover Medical School in Germany during the 20th World Congress in Fetal Medicine.

PAH is a rare and progressive disorder characterized by high blood pressure in the pulmonary arteries. Pregnancy may accelerate the progression of PAH and guidelines recommend patients with PAH to avoid pregnancy.

“Our study challenges the dogma,” Dr. von Kaisenberg said.

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He reported results from 16 patients with the disease. Of these, 13 had a total number of 17 successful pregnancies and delivered 18 offspring. Five patients had a total number of 8 abortions; 4 recovered while 1 patient developed right ventricular failure and required lung transplantation.

Dr. von Kaisenberg gave a detailed overview of managing patients with PAH during and after pregnancy. Once a patient with PAH reports a pregnancy, an urgent visit should be scheduled he said, endothelial receptor antagonist therapy should be stopped, and her medications should be reviewed for potentially embryotoxic substances.

At the initial visit, a clinical assessment and echocardiography should be performed, followed by N-terminal pro-b-type natriuretic peptide (NT-proBNP) and blood gas analysis. The patients should be counseled on short-term and long-term risks and outcomes of the pregnancy.

Clinical assessment and echocardiography as well as NT-proBNP and blood gas analysis should be repeated during the follow-up visits, which should take place at 18 to 23 weeks and then every 6 to 12 weeks in the first and second trimester and every 4 weeks in the third trimester. Evaluation for possible lung transplantation in case of postpartum complications should be evaluated. Delivery should be done at 37 to 38 weeks by Cesarean section under spinal or epidural anesthesia.

Delivery preparation should include a multidisciplinary team meeting between pneumologists, intensivists, anesthetists, cardiothoracic surgeons, obstetricians, and neonatologists for final preparations, Dr. von Kaisenberg said.

During the postpartum period, hemodynamic monitoring—including echocardiography and hourly assessment of venous oxygen saturation—should be performed for 6 to 12 hours. On Day 1, lactation should be suppressed with cabergoline or bromocriptine and endothelin receptor antagonists should be reintroduced. If the patient is stable, discharge from the hospital should be done on Day 5.

Phone visits should be performed at Weeks 2 and 4 after discharge and visits at the center should occur every 3 to 6 months in stable patients, according to the presentation.


von Kaisenberg C. Pregnancy in pulmonary arterial hypertension: midterm outcomes of mothers and offspring. Oral presentation at: 20th World Congress in Fetal Medicine; June 27, 2023; Valencia, Spain.