Table 1.
Baseline characteristics of patients initiated on sequential triple combination therapy.
| Variable (n = 55) | Mean ± standard deviation/median (interquartile range)/count (%) |
|---|---|
| Demographics | |
| Age (years) | 57 ± 14 |
| Gender (Female) | 47 (85.5%) |
| Race/ethnicity | |
| Black | 19 (34.5%) |
| White | 14 (25.5%) |
| Hispanic | 12 (21.8%) |
| Asian | 6 (10.9) |
| Native American | 1 (1.8%) |
| Unknown | 3 (5.5%) |
| Body mass index (kg/m2) | 26.6 (22.6, 30.1) |
| Etiology of pulmonary arterial hypertension | |
| Idiopathic/Heritable | 23 (41.8%) |
| Connective tissue disorder | 17 (30.9%) |
| Human immunodeficiency virus infection | 5 (9.1%) |
| Congenital heart disease | 5 (9.1%) |
| Multiple risk factors for PAH | 5 (9.1%) |
| All-cause hospitalization (1-year) | 20 (36.4%) |
| All-cause mortality | 6 (10.9%) |
| Duration to first follow-up on triple therapy (weeks) | 68 (43, 122) |
| Time from diagnosis to initiation of triple therapy (weeks) | 120 (39, 296) |
| Initial therapy | |
| ERA and prostacyclin combination | 8 (14.5%) |
| PDE-5i/sGCs and parenteral prostacyclin combination | 2 (3.6%) |
| ERA and PDE-5i/sGCs combination | 45 (81.8%) |
| Final triple combination therapy | |
| ERA with PDE-5i/sGCs and parenteral prostacyclin | 14 (25.5%) |
| ERA and PDE-5i/sGCs and oral prostacyclin | 9 (16.4%) |
| ERA and PDE-5i/sGCs and inhaled prostacyclin | 8 (14.5%) |
| ERA and PDE-5i/sGCs and prostacyclin (IP2) receptor agonist | 24 (43.6%) |
| Initial functional status | |
| World health organization functional class (WHO FC) | |
| I | 0 (0%) |
| II | 10 (18.2%) |
| III | 42 (76.4%) |
| IV | 3 (5.5%) |
| REVEAL 2.0 score | 7 (5, 10) |
| REVEAL 2.0 risk category | |
| Low | 21 (38.2%) |
| Intermediate | 15 (27.3%) |
| High | 19 (34.5%) |
| ERS/ESC 2022 simplified four-strata risk category | |
| Low-risk | 6 (10.9%) |
| Intermediate-low-risk | 26 (47.3%) |
| Intermediate-high risk | 18 (32.7%) |
| High risk | 5 (9.1%) |
| Six-min walk distance (meters) | 282 ± 126 |
| Diffusion capacity of carbon monoxide (%) | 48 ± 19 |
| Brain natriuretic peptide (pg/dL) | 45.7 (26.0, 158.0) |
| Initial echocardiographic parameters | |
| Tricuspid valve regurgitation velocity (m/s) | 4.01 ± 0.71 |
| Tricuspid annulus planar systolic excursion (low) | 24 (52.2%) |
| Right ventricular function (hypokinetic) | 35 (64.8%) |
| Right ventricular size | |
| Normal | 9 (16.7%) |
| Mildly dilated | 12 (22.2%) |
| Moderately dilated | 19 (34.5%) |
| Severely dilated | 14 (25.5%) |
| Estimated right atrial pressure | 8 (3, 15) |
| Pericardial effusion | 5 (9.3%) |
| Initial hemodynamics | |
| Mean right atrial pressure (mm Hg) | 9 ± 4 |
| Systolic pulmonary artery pressure (mm Hg) | 79 ± 19 |
| Diastolic pulmonary artery pressure (mm Hg) | 31 ± 8 |
| Mean pulmonary artery pressure (mm Hg) | 47 ± 11 |
| Pulmonary artery wedge pressure (mm Hg) | 10 (7, 13) |
| Pulmonary vascular resistance (woods unit) | 8.08 (5.66, 12.00) |
| Cardiac output (L/min) | 4.50 ± 1.38 |
| Cardiac index (L/min/m2) | 2.52 ± 0.72 |
| Mixed venous oxygen saturation (%) | 63 ± 8 |
ERA, endothelin receptor antagonist; ERS/ESC, European Respiratory Society/European Society of Cardiology; PAH, pulmonary arterial hypertension; PDE-5i, phosphodiesterase 5 inhibitor; REVEAL 2.0, Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management; sGCs, soluble guanylate cyclase stimulator.