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. 2020 Aug 26;159(1):311–327. doi: 10.1016/j.chest.2020.07.088

Table 6.

PAH-Specific Medications Among Patients at Enrollment in the PAH Biobank

Variable Oral Therapies
Prostacyclin Pathway
CCB ERA PDE Type 5 Inhibitor Riociguat Oral Treprostinil Selexipag Inhaled Treprostinil SC Treprostinil IV Epoprostenol/Treprostinil
Overall, N = 491 34 (6.9) 264 (53.8) 366 (74.5) 11 (2.2) 21 (4.3) 11 (2.2) 45 (9.1) 23 (4.7) 101 (20.6)
Monotherapy 10 (2) 33 (6.7) 89 (18.1) 3 (0.6) 3 (0.6) ... 2 (0.4) 3 (0.6) 24 (4.8)
Combination, oral therapya 14 (2.6) 122 (24.8) 130 (26.5) 2 (0.4) 8 (1.6) 4 (0.8) 19 (3.9) 11 (2.2) 50 (10.2)
Combination, prostacyclin analogue 1 (0.2) 26 (5.3) 64 (13) 1 (0.2) ... ... ... ... ...
Combination, 3 agentsb ... 75 (15.3) 72 (14.7) 3 (0.6) 10 (2) 7 (1.4) 24 (4.9) 7 (1.4) 26 (5.3)
Combination, PAH blinded study drugc ... 5 (1) 9 (1.8) 1 (0.2) ... ... ... 1 (0.2) ...
Combination, “other PH” drugd 1 (0.2) 3 (0.6) 2 (0.4) 1 (0.2) ... ... ... 1 (0.2) 1 (0.2)

Data are presented as No. (%). CCB = calcium channel blocker (amlodipine, diltiazem, other); ERA = endothelin receptor antagonist (ambrisentan, bosentan, macitentan); PDE = phosphodiesterase (sildenafil, tadalafil); SC = subcutaneous. See Table 1 and 3 legends for expansion of other abbreviations.

Combinations with oral therapy, one prostacyclin analogue, three agents, PAH-blinded study drug, and “other PH” drug are mutually exclusive for each medication class.

a

Oral therapies include CCB, ERA, PDE type 5 inhibitor, and riociguat.

b

Three agents or triple therapy includes ERA, nitric oxide pathway agent, and prostacyclin analogue; excludes CCB. One patient was on triple combination therapy with inhaled iloprost.

c

Eighteen patients were listed as being on PAH-blinded study drug; eight of them were listed as being on PAH blinded study drug only.

d

Four patients were listed as being on combination therapy including an “other” pulmonary hypertension medication that was not further identified. One patient was listed as being on inhaled nitric oxide therapy.