Rosenkranz S, Howard LS, Gomberg-Maitland M, Hoeper MM. Systemic consequences of pulmonary hypertension and right-sided heart failure. Circulation. 2020;141:678–93.
Leber L, Beaudet A, Muller A. Epidemiology of pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: identification of the most accurate estimates from a systematic literature review. Pulm Circ. 2021;11:2045894020977300.
Article PubMed PubMed Central Google Scholar
D’Alonzo GE, Barst RJ, Ayres SM, Bergofsky EH, Brundage BH, Detre KM, Fishman AP, Goldring RM, Groves BM, Kernis JT, et al. Survival in patients with primary pulmonary hypertension. results from a national prospective registry. Ann Intern Med. 1991;115:343–9.
Article CAS PubMed Google Scholar
Barst RJ, McGoon M, Torbicki A, Sitbon O, Krowka MJ, Olschewski H, Gaine S. Diagnosis and differential assessment of pulmonary arterial hypertension. J Am Coll Cardiol. 2004;43:40s–7s.
Humbert M, Kovacs G, Hoeper MM, Badagliacca R, Berger RMF, Brida M, Carlsen J, Coats AJS, Escribano-Subias P, Ferrari P, et al. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2022;43:3618–731.
Article CAS PubMed Google Scholar
Barst RJ, Rubin LJ, Long WA, McGoon MD, Rich S, Badesch DB, Groves BM, Tapson VF, Bourge RC, Brundage BH, et al. A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. N Engl J Med. 1996;334:296–301.
Article CAS PubMed Google Scholar
Sitbon O, Vonk Noordegraaf A. Epoprostenol and pulmonary arterial hypertension: 20 years of clinical experience. Eur Respir Rev. 2017;26: 160055.
Article PubMed PubMed Central Google Scholar
UPTRAVI (selexipag) [prescribing information]. Food and Drug Administration [https://www.accessdata.fda.gov/drugsatfda_docs/label/2015/207947s000lbl.pdf]
OPSUMIT (macitentan) [prescribing information]. Food and Drug Administration [https://www.accessdata.fda.gov/drugsatfda_docs/label/2015/207947s000lbl.pdf]
ADEMPAS (riociguat) [prescribing information]. Food and Drug Administration [https://www.accessdata.fda.gov/drugsatfda_docs/label/2017/204819s006lbl.pdf]
Ghofrani HA, Galiè N, Grimminger F, Grünig E, Humbert M, Jing ZC, Keogh AM, Langleben D, Kilama MO, Fritsch A, et al. Riociguat for the treatment of pulmonary arterial hypertension. N Engl J Med. 2013;369:330–40.
Article CAS PubMed Google Scholar
Jing ZC, Parikh K, Pulido T, Jerjes-Sanchez C, White RJ, Allen R, Torbicki A, Xu KF, Yehle D, Laliberte K, et al. Efficacy and safety of oral treprostinil monotherapy for the treatment of pulmonary arterial hypertension: a randomized, controlled trial. Circulation. 2013;127:624–33.
Article CAS PubMed Google Scholar
Pulido T, Adzerikho I, Channick RN, Delcroix M, Galiè N, Ghofrani HA, Jansa P, Jing ZC, Le Brun FO, Mehta S, et al. Macitentan and morbidity and mortality in pulmonary arterial hypertension. N Engl J Med. 2013;369:809–18.
Article CAS PubMed Google Scholar
Sitbon O, Channick R, Chin KM, Frey A, Gaine S, Galiè N, Ghofrani HA, Hoeper MM, Lang IM, Preiss R, et al. Selexipag for the treatment of pulmonary arterial hypertension. N Engl J Med. 2015;373:2522–33.
Article CAS PubMed Google Scholar
REMODULIN (treprostinil) [prescribing information]. Food and Drug Administration [https://www.accessdata.fda.gov/drugsatfda_docs/label/2011/021272s015lbl.pdf. ]
Lau EMT, Giannoulatou E, Celermajer DS, Humbert M. Epidemiology and treatment of pulmonary arterial hypertension. Nat Rev Cardiol. 2017;14:603–14.
Article CAS PubMed Google Scholar
Galiè N, Humbert M, Vachiery JL, Gibbs S, Lang I, Torbicki A, Simonneau G, Peacock A, Vonk Noordegraaf A, Beghetti M, et al. 2015 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension: the joint task force for the diagnosis and treatment of pulmonary hypertension of the european society of cardiology (ESC) and the european respiratory society (ers): endorsed by: association for european paediatric and congenital cardiology (AEPC), international society for heart and lung transplantation (ISHLT). Eur Respir J. 2015;46:903–75.
Hoeper MM, Kramer T, Pan Z, Eichstaedt CA, Spiesshoefer J, Benjamin N, Olsson KM, Meyer K, Vizza CD, Vonk-Noordegraaf A, et al. Mortality in pulmonary arterial hypertension: prediction by the 2015 European pulmonary hypertension guidelines risk stratification model. Eur Respir J. 2017;50:1700740.
Hoeper MM, Pausch C, Olsson KM, Huscher D, Pittrow D, Grunig E, Staehler G, Vizza CD, Gall H, Distler O, et al. COMPERA 20: A refined 4-strata risk assessment model for pulmonary arterial hypertension. Eur Respir J. 2021;1:210.
Benza RL, Gomberg-Maitland M, Elliott CG, Farber HW, Foreman AJ, Frost AE, McGoon MD, Pasta DJ, Selej M, Burger CD, Frantz RP. Predicting survival in patients with pulmonary arterial hypertension: the REVEAL risk score calculator 20 and comparison with ESC/ERS-based risk assessment strategies. Chest. 2019;156:323–37.
McLaughlin VV, Suissa S. Prognosis of pulmonary arterial hypertension: the power of clinical registries of rare diseases. Circulation. 2010;122:106–8.
Chung WJ, Park YB, Jeon CH, Jung JW, Ko KP, Choi SJ, Seo HS, Lee JS, Jung HO. Baseline characteristics of the korean registry of pulmonary arterial hypertension. J Korean Med Sci. 2015;30:1429–38.
Article PubMed PubMed Central Google Scholar
Hachamovitch R, Peña JM, Xie J, Shaw LJ, Min JK. Imaging registries and single-center series. JACC Cardiovasc Imaging. 2017;10:276–85.
Emmons-Bell S, Johnson C, Boon-Dooley A, Corris PA, Leary PJ, Rich S, Yacoub M, Roth GA. Prevalence, incidence, and survival of pulmonary arterial hypertension: A systematic review for the global burden of disease 2020 study. Pulm Circ. 2022;12: e12020.
Article CAS PubMed PubMed Central Google Scholar
Chen H, Taichman DB, Doyle RL. Health-related quality of life and patient-reported outcomes in pulmonary arterial hypertension. Proc Am Thorac Soc. 2008;5:623–30.
Article PubMed PubMed Central Google Scholar
Favoccia C, Kempny A, Yorke J, Armstrong I, Price LC, McCabe C, Harries C, Wort SJ, Dimopoulos K. EmPHasis-10 score for the assessment of quality of life in various types of pulmonary hypertension and its relation to outcome. Eur J Prev Cardiol. 2019;26:1338–40.
Mathai SC, Suber T, Khair RM, Kolb TM, Damico RL, Hassoun PM. Health-related quality of life and survival in pulmonary arterial hypertension. Ann Am Thorac Soc. 2016;13:31–9.
Article PubMed PubMed Central Google Scholar
DuBrock HM, Reddy YN, Durst LA, Schroeder DR, Park G, Cajigas HR, Kane GC, Kushwaha SS, McCully RB, Murphy JG, et al. The feasibility and value of assessing patient-reported outcomes in pulmonary arterial hypertension. Pulm Circ. 2022;12: e12143.
Article CAS PubMed PubMed Central Google Scholar
McCabe C, Bennett M, Doughty N, MacKenzie Ross R, Sharples L, Pepke-Zaba J. Patient-reported outcomes assessed by the CAMPHOR questionnaire predict clinical deterioration in idiopathic pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension. Chest. 2013;144:522–30.
Article PubMed PubMed Central Google Scholar
Skride A, Sablinskis K, Lejnieks A, Rudzitis A, Lang I. Characteristics and survival data from Latvian pulmonary hypertension registry: comparison of prospective pulmonary hypertension registries in Europe. Pulm Circ. 2018;8:2045894018780521.
Article PubMed PubMed Central Google Scholar
Swinnen K, Quarck R, Godinas L, Belge C, Delcroix M. Learning from registries in pulmonary arterial hypertension: pitfalls and recommendations. Eur Respir Rev. 2019;28: 190050.
Article PubMed PubMed Central Google Scholar
Engauge Digitizer Software [http://markummitchell.github.io/engauge-digitizer]
Hedges LV. A random effects model for effect sizes. Psychol Bull. 1983;93:388–95.
Miller JJ. The inverse of the freeman – tukey double arcsine transformation. Am Stat. 1978;32:138–138.
Chazova IE, Arkhipova OA, Martynyuk TV. Pulmonary arterial hypertension in russia: six-year observation analysis of the national registry. Ter Arkh. 2019;91:24–31.
留言 (0)