Management of Moderate Pulmonary Hypertension in Pregnancy

Maternal and Fetal Risks

The European Society of Cardiology recommends that moderate pulmonary hypertension diagnosed during pregnancy requires immediate referral to a specialized center with multidisciplinary expertise, as even moderate forms can worsen during pregnancy and carry significant maternal mortality risk (17-33% in recent studies) 1, 2
Maternal death most commonly occurs in the last trimester or first months after delivery due to pulmonary hypertensive crises, pulmonary thrombosis, or refractory right heart failure, with risk factors including late hospitalization, severity of pulmonary hypertension, and general anesthesia 2
Even moderate forms of pulmonary vascular disease can worsen during pregnancy due to decreased systemic vascular resistance and right ventricular overload 2
Neonatal survival rates are reported to be 87-89% 3

Comprehensive echocardiographic assessment is recommended to confirm diagnosis and severity of pulmonary hypertension, with MRI without gadolinium considered if echocardiography is insufficient, and cardiac catheterization considered with strict indications and appropriate fetal shielding 1
Regular monitoring of maternal vital signs, symptoms of right heart failure, and fetal well-being is essential 2

Early hospitalization in a center with expertise in pulmonary hypertension management is crucial, with management by a multidisciplinary team including pulmonary hypertension specialists, high-risk obstetrics, and cardiovascular anesthesiology 3, 4
For patients already on pulmonary hypertension therapy, continuation should be considered, with awareness of potential teratogenic effects of some medications (e.g., bosentan), and supplemental oxygen therapy should be provided if hypoxemia is present 3
Consider intravenous prostacyclin or aerosolized iloprost if hemodynamic deterioration occurs, and anticoagulation should be considered in patients with established indication for anticoagulation outside pregnancy, pulmonary artery thrombosis, or signs of heart failure 3

Caesarean delivery should be considered for patients with Eisenmenger syndrome or severe heart failure, with epidural analgesia and elective instrumental delivery considered if vaginal delivery is chosen 1

The immediate postpartum period carries the highest risk due to dramatic volume shifts and increased cardiac filling pressures, with close hemodynamic monitoring recommended for at least 72 hours after delivery 3, 4
Maintain vigilance for pulmonary hypertensive crises and right heart failure 2

Pregnancy termination should be offered, especially in early pregnancy, due to the significant risks, and if the patient chooses to continue pregnancy, every effort should be made to maintain circulating volume and avoid systemic hypotension, hypoxia, and acidosis 3

Delayed diagnosis and management can lead to poor outcomes, with early referral to specialized centers crucial, and abrupt hemodynamic changes during delivery and postpartum period can precipitate right heart failure 2, 4
Underestimating the risk of moderate pulmonary hypertension can lead to poor outcomes, as even moderate forms can worsen significantly during pregnancy 2

The American Thoracic Society recommends that pregnancy in the setting of pulmonary hypertension should be avoided, and when it does occur, immediate referral to a specialized pulmonary hypertension center with multidisciplinary expertise is mandatory, as maternal mortality remains 12-33% even with modern management 5, 6
Assess functional status using WHO functional class, as this impacts treatment intensity 5
Early hospitalization is crucial once the fetus is viable for closer hemodynamic monitoring 6, 7

The American College of Chest Physicians recommends avoiding endothelin receptor antagonists (bosentan, ambrisentan, macitentan) due to evidence of serious fetal abnormalities 6, 7, 8
Avoid riociguat due to its pregnancy category X classification 8

Diuretics (furosemide) may be used for pulmonary congestion, but use cautiously as they may decrease placental blood flow 6, 7
Dobutamine may be used if inotropic support is needed 6, 7

Administer corticosteroids for fetal lung maturity if delivery is anticipated before 34 weeks 9

Maintain intensive monitoring for at least 72 hours postpartum, as this is when most maternal deaths occur 6, 7, 10
Continue pulmonary vasodilator therapy and oxygen supplementation postpartum 6, 7, 10

Pulmonary hypertension may progress during pregnancy and remain worse postpartum, even with successful delivery 6, 7, 10
Dual mechanical barrier contraception is mandatory postpartum to prevent future pregnancies 6, 7, 8