How to manage moderate pulmonary hypertension (PH) diagnosed on 2D echocardiogram (2D echo) during pregnancy?

Management of Moderate Pulmonary Hypertension in Pregnancy

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

Maternal and Fetal Risks

• 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 1
• Risk factors for maternal mortality include late hospitalization, severity of pulmonary hypertension, and general anesthesia 1
• Even moderate forms of pulmonary vascular disease can worsen during pregnancy due to decreased systemic vascular resistance and right ventricular overload 1
• Neonatal survival rates are reported to be 87-89% 1

Initial Assessment and Monitoring

• Comprehensive echocardiographic assessment to confirm diagnosis and severity of pulmonary hypertension 1
• If echocardiography is insufficient, MRI without gadolinium should be considered 1
• Cardiac catheterization may be considered with strict indications and appropriate fetal shielding 1
• Regular monitoring of maternal vital signs, symptoms of right heart failure, and fetal well-being 1

Management Approach

Hospitalization and Care Setting

• Early hospitalization in a center with expertise in pulmonary hypertension management 1
• Management by a multidisciplinary team including pulmonary hypertension specialists, high-risk obstetrics, and cardiovascular anesthesiology 1

Medical Management

• For patients already on pulmonary hypertension therapy, continuation should be considered, with awareness of potential teratogenic effects of some medications (e.g., bosentan) 1
• Supplemental oxygen therapy if hypoxemia is present 1
• Consider intravenous prostacyclin or aerosolized iloprost if hemodynamic deterioration occurs 1
• Anticoagulation should be considered in patients with:
  • Established indication for anticoagulation outside pregnancy
  • Pulmonary artery thrombosis
  • Signs of heart failure 1

Delivery Planning

• Caesarean delivery should be considered for patients with Eisenmenger syndrome or severe heart failure 1
• If vaginal delivery is chosen (which may be appropriate in some cases), epidural analgesia and elective instrumental delivery should be considered 1
• Avoid general anesthesia if possible, as it is associated with higher mortality 2
• Regional anesthesia appears superior to general anesthesia for cesarean section 2

Post-Delivery Care

• The immediate postpartum period carries the highest risk due to dramatic volume shifts and increased cardiac filling pressures 1
• Close hemodynamic monitoring should continue for at least 72 hours after delivery 1
• Maintain vigilance for pulmonary hypertensive crises and right heart failure 1

Important Considerations

• Pregnancy termination should be offered, especially in early pregnancy, due to the significant risks 1
• If the patient chooses to continue pregnancy, every effort should be made to maintain circulating volume and avoid systemic hypotension, hypoxia, and acidosis 1
• Maternal mortality appears to correlate with PH classification, severity, delayed diagnosis, and NYHA functional class 2

Pitfalls to Avoid

• Delayed diagnosis and management - early referral to specialized centers is crucial 1
• General anesthesia should be avoided when possible 2
• Abrupt hemodynamic changes during delivery and postpartum period can precipitate right heart failure 1
• Underestimating the risk of moderate pulmonary hypertension - even moderate forms can worsen significantly during pregnancy 1