TABLE 3

Published registry data for pulmonary arterial hypertension therapies in patients with chronic thromboembolic pulmonary hypertension (CTEPH)

Country (year) [ref.]	Functional class	Therapies		Main outcomes
UK (2008)^# [44]	WHO II–IV	Surgical n=321^¶ Targeted therapy 65	Nonsurgical n=148^¶ Targeted therapy 90 ERA 56 PDE-5 inhibitor 33 Prostanoid 11	Survival at 1 and 3 years⁺: 88% and 76%, respectively, for surgical patients; 82% and 70%, respectively for nonsurgical patients (p=0.023)
International (2016) [10]	NYHA I–IV	Operated n=404 Overall 29 ERA 13 PDE-5 inhibitor 15 Prostanoid 1	Nonoperated n=275 Overall 61 ERA^§ 24 PDE-5 inhibitor 17 ERA + PDE-5 inhibitor 18 Prostanoid 2	Estimated survival at 1, 2 and 3 years Operated 93% (95% CI 90–95%), 91% (87–93%), and 89% (86–92%), respectively Nonoperated 88% (95% CI 83–91%), 79% (74–83%) and 70% (64–76%), respectively
Latvia (2016) [48]	NYHA II–IV	Overall n=31 Sildenafil 90 Ambrisentan 7 Bosentan 1		Cross-sectional analysis only
Portugal (2013) [43]	WHO II–IV	Overall n=33 Any 67 Monotherapy 36 Two drugs 15 Three drugs 7 Clinical trial 3 ERA 52 Sildenafil 39 Prostanoid 6		Overall Kaplan–Meier survival estimate at 1 year 94%; nonoperated 92.9%; PEA 100%
Spain (2016) [45]	WHO I–IV	PEA n=122 Any 43	Non-PEA n=269 Any 82 ERA 38 PDE-5 inhibitor 31 ERA/PDE-5 inhibitor 8 Prostanoid (oral) 3 Prostanoid (i.v.) 1 Prostanoid (inhaled) <1	1-, 3- and 5-year survival: nonoperated 93%, 81% and 65%, respectively, and operated 97%, 91% and 86%, respectively (p=0.003) In nonoperated patients at 1 year: 39% improved WHO FC; 6MWD increased by 28±92 m; mPAP decreased by 1.1±11.8 mmHg; PVR decreased by 3.5±4.6 Wood units
Switzerland (2008) [46]	NYHA II–IV	Baseline n=70 Any 31 Iloprost (inhaled) 22 Bosentan 5 Bosentan/sildenafil 1 Iloprost (i.v.) 1 Last visit n=46 Any 81 Iloprost (inhaled) 24 Bosentan 21 Sildenafil 7 Bosentan/iloprost 6 Sildenafil/iloprost 11 Bosentan/sildenafil 6 ≥3 drugs 2		Significant increase in 6MWD from baseline (377 m) to best response (436 m; p<0.001) At last assessment, 6MWD was significantly lower than best response (380 m; p<0.001) Functional class distribution (I/II/III/IV) improved from 0/14/60/26% at baseline to 6/20/40/34% at best 6MWD response and 4/14/44/38% at last assessment
Switzerland (2015) [47]	NYHA 3.0±0.7	Overall (n=100)^ƒ Started within 3 months Any 74 Monotherapy 64 Two drugs 9 Three drugs 0 ERA 63 PDE-5 inhibitor 20 Prostanoid 0 Maximal therapy Any 82 Monotherapy 49 Two drugs 30 Three drugs 2		Overall 1-, 2-, 3- and 4-year transplant-free survival in patients with nonoperated CTEPH was 91%, 84%, 77% and 73%, respectively Survival was significantly better in patients treated after 2004 (p<0.05)

Data are presented as % or mean±sd, unless otherwise stated. WHO: World Health Organization; ERA: endothelin receptor antagonist; PDE: phosphodiesterase; New York Heart Association; PEA: pulmonary endarterectomy; 6MWD: 6-min walking distance; mPAP: mean pulmonary arterial pressure; PVR: pulmonary vascular resistance. ^#: study from UK national PEA surgery centre; ^¶: data from 2003 onward; ⁺: data from 2001 onward; ^§: mostly bosentan, with some sitaxsentan; ^ƒ: data for 2009–2012.