Statement on Pregnancy in Pulmonary Hypertension

Hemnes AR, Kiely DG, Cockrill BA, et al.
Statement on Pregnancy in Pulmonary Hypertension From the Pulmonary Vascular Research Institute. Pulm Circ 2015;5:435-465.

The following are 10 points to remember about pregnancy and pulmonary hypertension. It is important to remember that these recommendations are based on expert consensus recommendations; no literature found met a prespecified standard definition for high-level recommendation.

  1. In general, patients with pulmonary hypertension (PH), especially pulmonary arterial hypertension (PAH), should be counseled to avoid pregnancy. The risk of maternal death was identified to be in the range of 30-56% in the pre-prostacyclin era. Although more recent studies suggest improved outcomes in the modern era, maternal morbidity and mortality remains high. Moreover, these data are subject to publication bias and should be interpreted with caution.
  2. Permanent contraception should be strongly considered in PAH patients, with hysteroscopy sterilization being the preferred method because of the potential for lower procedural risk. This procedure entails the blockade of the fallopian tubes with the use of coils wound via a hysteroscopy. No incision is required and patients require minimal or no anesthesia.
  3. Estrogen-containing contraception is not recommended because of the increased risk of venous thromboembolism and the possible deleterious effects of estrogen on the pulmonary vasculature. Pulmonary embolism is particularly dangerous in PAH patients.
  4. Progestin-only implants and intrauterine devices (IUDs) are acceptable nonpermanent contraception methods. These devices are safe, effective, and there is no evidence that they increase risk of thromboembolism.
  5. The highest risk periods for maternal mortality are the peripartum period and immediate postpartum period (up to 2 month). This is probably due to the rapid change in fluids during these periods.
  6. Cesarean section is the preferred mode of delivery. This method avoids the multiple disadvantages that vaginal delivery carries, such as:
    • Valsalva maneuver, which increases intrathoracic pressure, thus, decreasing venous return
    • Labor-induced vasovagal response
    • Sympathetic nervous system activation due to pain
    • Autotransfusion: returning blood to venous circulation from contracting uterus after delivery
  7. Epidural or spinal epidural anesthesia is recommended over general anesthesia. General anesthesia is known to depress cardiac contractility and increase pulmonary vascular resistance via positive pressure ventilation.
  8. Parenteral and inhaled prostacyclin, as well as phosphodiesterase inhibitors are recommended for management of PH during pregnancy.
  9. Endothelin receptor blockers and soluble guanylate cyclase stimulators are pregnancy category X and should not be use in pregnancy.
  10. In patients receiving anticoagulation before pregnancy, the risk and benefits of this therapy should be re-evaluated and discussed with patients. Warfarin should be stopped and unfractionated or low molecular weight heparin should be used if anticoagulation is to be continued.
  11. Keywords: Anesthesia, Epidural, Anesthesia, General, Anticoagulants, Cesarean Section, Contraception, Heparin, Low-Molecular-Weight, Hypertension, Hypertension, Pulmonary, Hysteroscopy, Intrauterine Devices, Phosphodiesterase Inhibitors, Pregnancy, Pulmonary Embolism, Thromboembolism, Venous Thromboembolism, Vascular Resistance, Warfarin

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