Table 2.
Representative guideline recommendations for pregnant women with PAH.
| Organization (Year) | pregnancy avoidance Recommendation | Contraception approach recommendation | Delivery approach recommendation | disease-targeted therapies recommendation | Post-menopausal HRT recommendation | Genetic counseling recommendation |
|---|---|---|---|---|---|---|
| ESC/ERS(2022) | Yes | Barrier contraceptive methods; Progesterone-only contraceptives; | Planned elective delivery (epidural anesthesia preferred) | CCBs; Phosphodiesterase type 5 inhibitors; Prostacyclin analogues and prostacyclin receptor agonists |
Unclear | BMPR2 mutation carriers suggested to remain childless |
| 6th WSPH（2018） | Not mentioned | Not mentioned | Not mentioned | Endothelin receptor antagonists; Prostacyclin pathway agents; Endothelin receptor antagonists; Nitric oxide pathway agents; New drugs（FK506，olaparib，Imatinib，Elafin and so on） |
Not mentioned | Inform relevant gene mutation population about the possibility of a genetic condition and that family members could carry a mutation that increases the risk of PAH （55） |
| Chinese (2021) | Yes | Not addressed | Planned elective delivery (local anesthesia or epidural anesthesia preferred) | CCBs; Endothelin receptor antagonist; Phosphodiesterase type 5 inhibitors; Soluable guanylate cyclase; Prostacyclin analogues and prostacyclin receptor agonists |
Unclear | Pathogenic gene for PAH：BMPR2、BMP9、ALK1、Endoglin、SMAD9、 BMPR1B、TBX4、CAV1 and KCNK3 |
| CCS/CTS(2019) | Yes | Not addressed | Not addressed | Warfarin; CCBs; Prostacyclin pathway agents; Endothelin receptor antagonists; Nitric oxide pathway agents |
Not mentioned | Not mentioned |
| TSOC(2018) | Not mentioned | Not mentioned | Not mentioned | Prostacyclin analogues and prostacyclin receptor agonists; Endothelin receptor antagonists; SGC stimulator; Phosphodiesterase type 5 inhibitors |
Not mentioned | Not mentioned |
| TSOC(2018) | Not mentioned | Not mentioned | Not mentioned | Prostacyclin analogues and prostacyclin receptor agonists; Endothelin receptor antagonists; SGC stimulator; Phosphodiesterase type 5 inhibitors |
Not mentioned | Not mentioned |
Abbreviation：ESC/ERS: ESC: European Society of Cardiology, and ERS: European Respiratory Society; 6th WSPH：the 6th world symposia on pulmonary hypertension; CCS/CTS: CCS: Canadian Cardiovascular Society, and CTS: Canadian Thoracic Society; TSOC: Taiwan Society of Cardiology; CCBs: Calcium channel blockers; SGC: Soluble guanylate cyclase.