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. Author manuscript; available in PMC: 2023 Apr 29.
Published in final edited form as: Circ Res. 2022 Apr 28;130(9):1404–1422. doi: 10.1161/CIRCRESAHA.121.319970

Table 5.

Clinical trials evaluating pulmonary vasodilators in Group 3 pulmonary hypertension

Drug Class Author (year) Number of Participants PH Definition Lung Disease Treatment Duration Primary Outcome Result
ERA Stolz et al246 (2008) 30 Echo PASP ≥ 30mmHg COPD Bosentan 125mg BID 12 weeks Change in 6MWD No difference
Valerio et al247 (2009) 32 RHC mPAP ≥ 25mmHg COPD Bosentan 125mg BID 18 months PFTs, hemodynamics, 6MWD, dyspnea ratings/quality of life Improvement in mPAP, PVR, 6MWD, dyspnea ratings
Raghu et al195 (2013) 492 RHC mPAP ≥ 25mmHg ILD Ambrisentan 10mg daily 16 weeks Time to ILD progression No difference; stopped early due to increased adverse events
Corte et al194 (2014) 60 RHC mPAP ≥ 25mmHg ILD Bosentan 125mg BID 16 weeks Decrease in PVRi from baseline ≥20% No difference
PDE5 inhibitor Collard et al248 (2007) 11 Echo PASP > 35mmHg or RHC mPAP ≥ 25mmHg ILD Sildenafil 20 – 50mg TID 12 weeks Change in 6MWD Significant improvement
Han et al198, 199 (2010) 180 N/A ILD Sildenafil 20mg TID 12 weeks 20% improvement in 6MWD No difference except in subgroup with RV systolic dysfunction
Rao et al249 (2011) 33 Echo PASP > 40mmHg COPD Sildenafil 20mg TID 12 weeks Change in 6MWD Significant improvement
Goudie et al196 (2014) 120 Echo PASP > 30mmHg or PA-AT < 120ms COPD Tadalafil 10mg daily 12 weeks Change in 6MWD No difference
Vitulo et al197 (2017) 28 RHC mPAP ≥ 35mmHg and post-bronchodilator FEV1 < 30% or mPAP ≥ 30mmHg and post-bronchodilator FEV1 > 30% COPD Sildenafil 20mg TID 16 weeks Reduction in PVR Significant improvement
Behr et al200 (2021) 177 RHC mPAP ≥ 20mmHg ILD Sildenafil 20mg TID + Pirfenidone 801mg TID 52 weeks Disease progression No difference
Prostacyclin analogue Saggar et al202 (2014) 15 RHC mPAP ≥ 35mmHg ILD Treprostinil 34 ± 21ng/kg/min 12 weeks Hemodynamics, 6MWD, PFTs, ECHO, dyspnea ratings/quality of life Improvement in hemodynamics, 6MWD, dyspnea ratings
Waxman et al56, 203 (2021) 326 RHC mPAP ≥ 25mmHg ILD Treprostinil 72 μg QID 16 weeks Change in 6MWD Significant improvement; Improvement in FVC in IPF subgroup
sGC stimulator Nathan et al201 (2019) 147 RHC mPAP ≥ 25mmHg ILD Riociguat 0.5 – 2.5mg TID 26 weeks Change in 6MWD No difference; stopped early due to increased adverse events

PH = pulmonary hypertension. ERA = endothelin receptor antagonist. RHC = right heart catheterization. mPAP = mean pulmonary artery pressure. ILD = interstitial lung disease. BID = two times per day. PVRi = pulmonary vascular resistance index. PVR = pulmonary vascular resistance. PFT = pulmonary function test. 6MWD = 6 minute walk distance. ECHO = echocardiogram. PDE5 = phosphodiesterase type-5. PASP = pulmonary artery systolic pressure. TID = three times per day. PA-AT = pulmonary artery acceleration time. FEV1 = forced expiratory volume in the first second. QID = four times per day. sGC = soluble guanyl cyclase.