SUPPLEMENTARY DATA
References of table 1 Currently available randomized controlled trials on disease targeting therapies in patients with
pulmonary arterial hypertension due to congenital heart disease or mixed pulmonary arterial hypertension groups
1.
Simonneau G, Barst RJ, Galie N, et al. Continuous subcutaneous infusion of treprostinil, a prostacyclin analogue, in
patients with pulmonary arterial hypertension: a double-blind, randomized, placebo-controlled trial. Am. J. Respir.
Crit. Care Med. 2002;165:800–4.
2.
Galiè N, Humbert M, Vachiéry J-L, et al. Effects of beraprost sodium, an oral prostacyclin analogue, in patients with
pulmonary arterial hypertension: a randomized, double-blind, placebo-controlled trial. J. Am. Coll. Cardiol.
2002;39:1496–502.
3.
Barst RJ, McGoon M, McLaughlin V, et al. Beraprost therapy for pulmonary arterial hypertension. J. Am. Coll.
Cardiol. 2003;41:2119–25.
4.
McLaughlin V V, Oudiz RJ, Frost A, et al. Randomized study of adding inhaled iloprost to existing bosentan in
pulmonary arterial hypertension. Am. J. Respir. Crit. Care Med. 2006;174:1257–63.
5.
Tapson VF, Torres F, Kermeen F, et al. Oral treprostinil for the treatment of pulmonary arterial hypertension in
patients on background endothelin receptor antagonist and/or phosphodiesterase type 5 inhibitor therapy (the
FREEDOM-C study): a randomized controlled trial. Chest. 2012;142:1383–90.
6.
Tapson VF, Jing Z-C, Xu K-F, et al. Oral treprostinil for the treatment of pulmonary arterial hypertension in patients
receiving background endothelin receptor antagonist and phosphodiesterase type 5 inhibitor therapy (the
FREEDOM-C2 study): a randomized controlled trial. Chest. 2013;144:952–8.
7.
Jing Z-C, Parikh K, Pulido T, et al. Efficacy and safety of oral treprostinil monotherapy for the treatment of
pulmonary arterial hypertension: a randomized, controlled trial. Circulation. 2013;127:624–633.
8.
Barst RJ, Langleben D, Frost A, et al. Sitaxsentan therapy for pulmonary arterial hypertension. Am. J. Respir. Crit.
Care Med. 2004;169:441–7.
9.
Galiè N, Beghetti M, Gatzoulis MA, et al. Bosentan therapy in patients with Eisenmenger syndrome: a multicenter,
double-blind, randomized, placebo-controlled study. Circulation. 2006;114:48–54.
10.
Barst RJ, Langleben D, Badesch D, et al. Treatment of pulmonary arterial hypertension with the selective
endothelin-A receptor antagonist sitaxsentan. J. Am. Coll. Cardiol. 2006;47:2049–56.
11.
Galiè N, Rubin L, Hoeper M, et al. Treatment of patients with mildly symptomatic pulmonary arterial hypertension
with bosentan (EARLY study): a double-blind, randomised controlled trial. Lancet. 2008;371:2093–100.
12.
Sandoval J, Torbicki A, Souza R, et al. Safety and efficacy of sitaxsentan 50 and 100 mg in patients with pulmonary
arterial hypertension. Pulm. Pharmacol. Ther. 2012;25:33–9.
13.
Pulido T, Adzerikho I, Channick RN, et al. Macitentan and morbidity and mortality in pulmonary arterial
hypertension. N. Engl. J. Med. 2013;369:809–18.
14.
Galiè N, Barberà JA, Frost AE, et al. Initial Use of Ambrisentan plus Tadalafil in Pulmonary Arterial Hypertension.
N. Engl. J. Med. 2015;373:834–44.
15.
Galiè N, Ghofrani HA, Torbicki A, et al. Sildenafil citrate therapy for pulmonary arterial hypertension. N. Engl. J.
Med. 2005;353:2148–57.
16.
Singh TP, Rohit M, Grover A, et al. A randomized, placebo-controlled, double-blind, crossover study to evaluate the
efficacy of oral sildenafil therapy in severe pulmonary artery hypertension. Am. Heart J. 2006;151:851.e1–5.
17.
Simonneau G, Rubin LJ, Galiè N, et al. Addition of sildenafil to long-term intravenous epoprostenol therapy in
patients with pulmonary arterial hypertension: a randomized trial. Ann. Intern. Med. 2008;149:521–30.
18.
Galiè N, Brundage BH, Ghofrani HA, et al. Tadalafil therapy for pulmonary arterial hypertension. Circulation.
2009;119:2894–903.
19.
Jing Z-C, Yu Z-X, Shen J-Y, et al. Vardenafil in pulmonary arterial hypertension: a randomized, double-blind,
placebo-controlled study. Am. J. Respir. Crit. Care Med. 2011;183:1723–9.
20.
Ghofrani H-A, Galiè N, Grimminger F, et al. Riociguat for the treatment of pulmonary arterial hypertension. N.
Engl. J. Med. 2013;369:330–40.
References of table 2 Independent predictors of mortality in PAH-CHD
21.
Barst RJ, Ivy DD, Foreman AJ, et al. Four- and seven-year outcomes of patients with congenital heart diseaseassociated pulmonary arterial hypertension (from the REVEAL Registry). Am. J. Cardiol. 2014;113:147–55.
22.
Diller G-P, Alonso-Gonzalez R, Kempny A, et al. B-type natriuretic peptide concentrations in contemporary
Eisenmenger syndrome patients: predictive value and response to disease targeting therapy. Heart. 2012;98:736–42.
23.
Schuuring MJ, van Riel ACMJ, Vis JC, et al. New predictors of mortality in adults with congenital heart disease and
pulmonary hypertension: Midterm outcome of a prospective study. Int. J. Cardiol. 2014;181C:270–276.
24.
Jansa P, Jarkovsky J, Al-Hiti H, et al. Epidemiology and long-term survival of pulmonary arterial hypertension in
the Czech Republic: a retrospective analysis of a nationwide registry. BMC Pulm. Med. 2014;14.:
25.
Daliento L, Somerville J, Presbitero P, et al. Eisenmenger syndrome. Factors relating to deterioration and death. Eur.
Heart J. 1998;19:1845–55.
26.
Scognamiglio G, Kempny A, Price LC, et al. C-reactive protein in adults with pulmonary arterial hypertension
associated with congenital heart disease and its prognostic value. Heart. 2014;100:1335–41.
27.
Van De Bruaene A, De Meester P, Voigt J-U, et al. Worsening in oxygen saturation and exercise capacity predict
adverse outcome in patients with Eisenmenger syndrome. Int. J. Cardiol. 2013;168:1386–92.
28.
Oya H, Nagaya N, Satoh T, et al. Haemodynamic correlates and prognostic significance of serum uric acid in adult
patients with Eisenmenger syndrome. Heart. 2000;84:53–8.
29.
Lopes A, Barreto A, Maeda N, et al. Plasma von Willebrand factor as a predictor of survival in pulmonary arterial
hypertension associated with congenital heart disease. Brazilian J. Med. Biol. Res. 2011;44:1269–1275.
30.
Yang T, Sun Y-J, Xiong C-M, et al. Red blood cell distribution width predicts survival in patients with Eisenmenger
syndrome. Clin. Chem. Lab. Med. 2014;52:743–50.
31.
Belly MJ, Tiede H, Morty RE, et al. HbA1c in pulmonary arterial hypertension: A marker of prognostic relevance?
J. Hear. Lung Transplant. 2012;31:1109–1114.
32.
Cantor WJ, Harrison DA, Moussadji JS, et al. Determinants of survival and length of survival in adults with
Eisenmenger syndrome. Am. J. Cardiol. 1999;84:677–81.
33.
Benza RL, Miller DP, Gomberg-Maitland M, et al. Predicting survival in pulmonary arterial hypertension: insights
from the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management
(REVEAL). Circulation. 2010;122:164–72.
34.
Salehian O, Schwerzmann M, Rambihar S, et al. Left ventricular dysfunction and mortality in adult patients with
Eisenmenger syndrome. Congenit. Heart Dis. 2007;2:156–64.
35.
Ramos RP, Arakaki JSO, Barbosa P, et al. Heart rate recovery in pulmonary arterial hypertension: Relationship with
exercise capacity and prognosis. Am. Heart J. 2012;163:580–588.
36.
Van De Bruaene A, De Meester P, Voigt J-U, et al. Right Ventricular Function in Patients With Eisenmenger
Syndrome. Am. J. Cardiol. 2012;109:1206–1211.
37.
Moceri P, Dimopoulos K, Liodakis E, et al. Echocardiographic predictors of outcome in eisenmenger syndrome.
Circulation. 2012;126:1461–8.
38.
Adachi S, Hirashiki A, Nakano Y, et al. Prognostic factors in pulmonary arterial hypertension with Dana Point
group 1. Life Sci. 2014;24:404–409.
39.
D’Alto M, Romeo E, Argiento P, et al. Pulmonary vasoreactivity predicts long-term outcome in patients with
Eisenmenger syndrome receiving bosentan therapy. Heart. 2010;96:1475–9.
40.
Dimopoulos K, Inuzuka R, Goletto S, et al. Improved survival among patients with Eisenmenger syndrome
receiving advanced therapy for pulmonary arterial hypertension. Circulation. 2010;121:20–5.
41.
Cenedese E, Speich R, Dorschner L, et al. Measurement of quality of life in pulmonary hypertension and its
significance. Eur. Respir. J. 2006;28:808–15.