Figure 1
Overview of the unique challenges faced by women with pulmonary hypertension. PH: pulmonary hypertension; PAH: pulmonary arterial hypertension; IV: intravenous
Table 1
Pulmonary hypertension groups. PAH: pulmonary arterial hypertension; PH: pulmonary hypertension; CCB: calcium channel blocker; LHD: left heart disease; PA: pulmonary artery
| GROUP | SUBTYPES | GENERAL PREVALENCE | TREATMENT STRATEGIES |
|---|---|---|---|
| 1 PAH |
| Rare |
|
| 2 PH associated with LHD |
| Very common |
|
| 3 PH associated with lung disease and/or hypoxia |
| Common |
|
| 4 PH associated with pulmonary artery obstructions |
| Rare |
|
| 5 PH with unclear/multifactorial etiology |
| Rare |
|
Table 2
Registries of pulmonary hypertension. PAH: pulmonary arterial hypertension; CTEPH: chronic thromboembolic pulmonary hypertension; iPAH: idiopathic pulmonary artery hypertension; PoPH: portopulmoanry hypertension; CHD: congenital heart disease; CTD: connective tissue disorders; hPAH: heritable pulmonary arterial hypertension
| REGISTRY | # OF CENTERS # OF PATIENTS | STUDY COHORT | FEMALE TO MALE PREDOMINANCE | OUTCOMES BY GENDER (95% CI) |
|---|---|---|---|---|
| REVEAL US (2006-2009)9 | 55 PAH: 3515 | PAH, > 3 months | 4.:1 F:M ratio | Not reported |
| Spanish REHAP (Retrospective 1998-2006, prospective 2007-2008)10 | 31 PAH: 866 CTEPH: 162 | PAH, CTEPH, > 14 years | PAH: 51-90% female CTEPH: 60% female | Males: HR 1.38* (1.03-1.83) |
| Latvian (2007-2016)11 | 1 PAH: 130 CTEPH: 44 | PAH, CTEPH, > 18 years | PAH: 73% female CTEPH: 61% female | Not reported |
| COMPERA (2007-2011)13 | 28 iPAH: 587 | iPAH, > 18 years | 18-65 years: 2.3:1 F:M ratio > 65 years: 1.2:1 F:M ratio | Males: HR 1.952* (1.264-3.016) |
| Japanese (2008-2013)14 | 8 PAH: 189 | PAH, > 18 years | PAH: 76.2% female | Not reported |
| UK (2001-2009)15 | 7 PoPH: 110 | PoPH, > 18 years | PoPH: 50.9% female | No significant difference in survival between genders |
| Scottish (1986-2001)16 | All Scottish hospitals PAH: 374 | PAH, > 16 years | PAH: 1.5-3:1 F:M ratio | Median survival: iPAH: males: 3.8 y; females: 5.6 y CHD-PAH: males: 5.9 y; females 4.9 y CTD-PAH: males: 4.5 y; females 2.6 y |
| French (2002-2003)17 | 17 PAH: 354 | iPAH, hPAH, anorexigen PAH, > 18 years | Overall: Female: 61% 1.5:1 F:M ratio | Male: HR 1* Female: HR 0.375 (0.212-0.662, female) |
[i] *risk factor for mortality, all patients
Table 3
Outcomes of pregnant patients with PAH but with a low risk profile and followed by multidisciplinary team. PAH: pulmonary arterial hypertension; a/w: associated with; C: combination therapy; CHD: congenital heart disease; CS: C-section; CTD: connective tissue disease; GA: general anesthesia; G5: group 5; IS: intraspinal; LHD: left heart disease; MDT: multidisciplinary team; M: monotherapy; NA: no anesthesia; N/A: not applicable; iPAH: idiopathic PAH; oPAH: other PAH; oPH: other PH; PH: pulmonary hypertension; RA: regional anesthesia; SLE: systemic lupus erythematous; T: targeted therapy; VD: vaginal delivery
| STUDY + NUMBER OF PREGNANT PH PATIENTS | PH ETIOLOGY | PH TREATMENT DURING PREGNANCY | DELIVERY WEEK (RANGE) AND MODE OF DELIVERY (PERCENTAGE) | TYPE OF ANESTHESIA | MATERNAL AND FETAL MORTALITY |
|---|---|---|---|---|---|
| Zhang et al. (1993-2016 retrospectively, 2017-2019 prospectively)39 2220 total pregnancies 729 had PH | Carried pregnancy: No PH: 1491 Mild PH: 398 CHD-PAH: 346 (86.9%) CHD-oPH: 52 (13.1%) Moderate-to-severe PH: 331 CHD-PAH: 273 (82.5%) CHD-oPH: 58 (17.5%) Terminated pregnancy: 535 Mild PH: 41 Moderate-to-severe PH: 494 | No PH: n/a Mild PH: T: 29 (7.33%) M: 26 (6.5%) C: 3 (0.8%) Moderate-to-severe PH: T: 99 (29.9%) M: 68 (20.5%) C: 31 (9.4%) | No PH: 37.8 + 1.98 weeks VD: 246 (16.8%) CS: 1218 (83.2%) Mild PH: 37.4 + 2.1 weeks VD: 44 (11.1%) CS: 354 (88.9%) Moderate-to-severe PH: 35.6 + 3.1 weeks VD: 26 (7.9%) CS: 305 (92.1%) | No PH: GA: 36 (2.5%) RA: 1209 (82.6%) NA: 219 (15%) Mild PH: GA: 9 (2.3%) RA: 347 (87.2%) NA: 42 (10.6%) Moderate-to-severe PH: GA: 38 (11.5%) RA: 273 (82.5%) NA: 19 (5.7%) | Maternal mortality: No PH: 1 (0.1%) Mild PH: 0 (0) Moderate-to-severe PH: 19 (5.7%) Fetal mortality: 535 (42.3%) miscarriages or terminations in PH patients Mortality: No PH: 7 (0.5%) Mild PH: 1 (0.3%) Moderate-to-severe PH: 4 (1.2%) |
| Lv C et al. (2011-2020)41 154 pregnant PH patients | Carried pregnancy: 139 iPAH: 6 (3.9%) CHD-PAH: 34 (82.9%) oPAH: 40 (88.9%) LHD-PH: 59 (95.2%) Terminated pregnancy: 15 iPAH: 0 CHD-PAH: 7 (17.1%) oPAH: 5 (11.1%) -LHD-PH (3 (4.8%) | NR | Delivery range: Total: 30.9 + 5.1 weeks iPAH: 28.7 + 4.5 weeks CHD-PAH: 30.6 + 4.6 weeks oPAH: 30.9 + 4.6 weeks LHD-PH: 31.5 + 5.7 weeks Mode: VD: 29 (18.8%) CS: 108 (70.1%) | Patients who underwent CS: GA 52 (33.8%) IS: 56 (36.4%) | Maternal mortality: Total: 5 (3.2%) iPAH: 3 (50%) CHD-PAH: 1 (2.4%) oPAH: 0 (0) LHD-PH: 1 (1.6%) Fetal mortality: Therapeutic abortion: 15 (9.7%) Missed abortion: 1 (0.6%) Intrauterine death: 7 (4.5%) Neonatal death (< 1 week): 3 (1.9%) |
| Chen et al. (2004-2020)35 103 pregnant PH patients | Pre-MDT: 49 iPAH: 1 (2.0%) CTD-PAH: 2 (4.1%) CHD-PAH: 30 (61.2%) LHD-PH: 14 (28.6%) G5-PH: 2 (4.1%) Post-MDT: 54 iPAH: 3 (5.6%) CTD-PAH: 1 (1.9%) CHD-PAH: 33 (61.2%) LHD-PH: 12 (22.2%) | Pre-MDT: T: 8 (24.2%) M: 7 (87.5%) C: 1 (12.5%) Post-MDT: T: 22 (59.5%) M: 12 (54.5%) C: 10 (45.5%) | Pre-MDT: 37 (31, 42) weeks VD: 2 (4.2%) CS: 46 (95.8%) Post-MDT: 36 (27, 40) weeks VD: 1 (1.9%) CS: 533 (98.1%) | Pre-MDT: GA: 18 (39.1%) IS: 28 (60.9%) Post-MDT: GA: 38 (71.7%) IS: 15 (28.3%) | Maternal mortality: Pre-MDT: 5 (10.2%) Post-MDT: 0 (00%) Fetal mortality: Abortions/terminations: not reported Mortality: Pre-MDT: 7 (14.2%) Post-MDT: 1 (1.9%) |
| Corbach et al. (2004-2020)42 5 patients, 7 pregnancies | iPAH: 3 (60%) PAH a/w SLE: 1 (20%) PAH a/w schistosomiasis: 1 (20%) | CCB: 4 pregnancies (57%) PDE-5: 7 pregnancies (100%) | 37.1 (33.7, 38.0) weeks CS: 7 (100%) | IS: 7: 100% | Maternal mortality: 0 (0%) Fetal mortality: Abortions/terminations: 0 Mortality: 0 (0%) |
| Vaidya et al. (2013-2021)36 6 patients, 10 pregnancies | Completed pregnancy: 6 iPAH: 3 (50%) CHD-PAH: 2 (33.3%) SLE-PAH: 1 (16.7%) Terminated pregnancy: 3 hPAH: 1 (33.3%) CHD-PAH: 1 (33.3%) oPAH: 1 (33.3%) | Prepregnancy: None: 2 (28.6%) Ambrisentan + sildenafil: 3 (42.8%) Treprostinil: 1 (14.3%) Tadalafil: 1 (14.3%) Pregnancy:Sildenafil/Tadalafil + treprostinil: 6 (85.7%) Tadalafil: 1 (14.3%) Epoprostenol at delivery: 2 (28.6%) | 36.9 (31.3, 40.4) weeks VD: 3 (42.9%) CS: 4 (57.1%) | GA: 1 (14.3%) IS: 6 (85.7%) | Maternal mortality: 0 (0%) Fetal mortality: Abortions: 3 (30%) Mortality: 0 (0%) |
| Duarte et al. (1999-2009)43 18 pregnant PH patients | Completed pregnancy: 12 iPAH: 4 (33.3%) CTD-PAH: 2 (16.7%) CHD-PAH: 6 (50%) Terminated pregnancy: 6 iPAH: 3 (50%) CHD-PAH: 2 (33.3%) Stimulant: 1 (16.7%) | Completed pregnancies: Prepregnancy: None: 7 (25%) ERA: 3 (25%) Prostaglandin: 1 (8.3%) CCB: 1 (8.3%) During pregnancy: None: 3 (25%) Sildenafil: 3 (25%) Prostanoid: 5 (41.7%) Combination: 1 (8.3%) Terminated pregnancies: None: 1 (16.7%) ERA + PD: 3 (50%) ERA: 2 (33.3%) | 34 (28, 36) weeks CS: 12 (100%) | GA: 3 (25%) IS: 8 (66.7%) N/A: 1 (8.3%) | Maternal mortality: 1 (8.3%) Fetal mortality: Abortions: 6 (33.33%) Mortality: 0 (0%) |