Research & Literature

Initial Use of Ambrisentan plus Tadalafil in Pulmonary Arterial Hypertension.

Galiè N, Barberà JA, Frost AE, et al.

The New England journal of medicine 2015; (373(9)):834-44 doi:10.1056/NEJMoa1413687.

Managing the Patient with Pulmonary Hypertension: Specialty Care Centers, Coordinated Care, and Patient Support.

Chakinala MM, Duncan M, Wirth J

Cardiology clinics 2016; (34(3)):489-500.

Pilot Study of Endothelin Receptor Blockade in Heart Failure with Diastolic Dysfunction and Pulmonary Hypertension (BADDHY-Trial).

Koller B, Steringer-Mascherbauer R, Ebner CH, et al.

Heart, lung & circulation 2017; (26(5)):433-441 doi:10.1016/j.hlc.2016.09.004.

Heritable pulmonary hypertension: from bench to bedside.

Girerd B, Weatherald J, Montani D, Humbert M

European respiratory review : an official journal of the European Respiratory Society 2017; (26(145)) doi:10.1183/16000617.0037-2017.

Age, risk and outcomes in idiopathic pulmonary arterial hypertension.

Hoeper MM, Boucly A, Sitbon O

The European respiratory journal 2018; (51(5)) doi:10.1183/13993003.00629-2018.

Germline BMP9 mutation causes idiopathic pulmonary arterial hypertension.

Wang XJ, Lian TY, Jiang X, et al.

The European respiratory journal 2019; (53(3)) doi:10.1183/13993003.01609-2018.

Risk Reduction and Right Heart Reverse Remodeling by Upfront Triple Combination Therapy in Pulmonary Arterial Hypertension.

D'Alto M, Badagliacca R, Argiento P, et al.

Chest 2020; (157(2)):376-383 doi:10.1016/j.chest.2019.09.009.

Novel risk genes and mechanisms implicated by exome sequencing of 2572 individuals with pulmonary arterial hypertension.

Zhu N, Pauciulo MW, Welch CL, et al.

Genome medicine 2019; (11(1)):69 doi:10.1186/s13073-019-0685-z.

Matrix metalloproteinase 7 in diagnosis and differentiation of pulmonary arterial hypertension.

Arvidsson M, Ahmed A, Bouzina H, Rådegran G

Pulmonary circulation 2019; (9(4)):2045894019895414 doi:10.1177/2045894019895414.

TBX4 variants and pulmonary diseases: getting out of the 'Box'.

Haarman MG, Kerstjens-Frederikse WS, Berger RMF

Current opinion in pulmonary medicine 2020; (26(3)):277-284 doi:10.1097/MCP.0000000000000678.

Pulmonary Hypertension: A Brief Guide for Clinicians.

Mandras SA, Mehta HS, Vaidya A

Mayo Clinic proceedings 2020; (95(9)):1978-1988 doi:10.1016/j.mayocp.2020.04.039.

Development and Validation of an Abridged Version of the REVEAL 2.0 Risk Score Calculator, REVEAL Lite 2, for Use in Patients With Pulmonary Arterial Hypertension.

Benza RL, Kanwar MK, Raina A, et al.

Chest 2021; (159(1)):337-346 doi:10.1016/j.chest.2020.08.2069.

Prevalence and clinical features of bone morphogenetic protein receptor type 2 mutation in Korean idiopathic pulmonary arterial hypertension patients: The PILGRIM explorative cohort.

Jang AY, Kim BG, Kwon S, et al.

PloS one 2020; (15(9)):e0238698 doi:10.1371/journal.pone.0238698.

Idiopathic pulmonary arterial hypertension phenotypes determined by cluster analysis from the COMPERA registry.

Hoeper MM, Pausch C, Grünig E, et al.

The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation 2020; (39(12)):1435-1444 doi:10.1016/j.healun.2020.09.011.

Pulmonary hypertension in connective tissue diseases, new evidence and challenges.

Vonk MC, Vandecasteele E, van Dijk AP

European journal of clinical investigation 2021; (51(4)):e13453 doi:10.1111/eci.13453.

Long-Term Outcomes in Patients With Connective Tissue Disease-Associated Pulmonary Arterial Hypertension in the Modern Treatment Era: Meta-Analyses of Randomized, Controlled Trials and Observational Registries.

Khanna D, Zhao C, Saggar R, et al.

Arthritis & rheumatology (Hoboken, N.J.) 2021; (73(5)):837-847 doi:10.1002/art.41669.

Pulmonary Artery Pressure as a Treatment Target to Improve the Prognosis of Idiopathic Pulmonary Arterial Hypertension　- Insight From a Cohort From Two Japanese Pulmonary Hypertension Centers.

Ishiguro M, Takeuchi K, Kikuchi H, et al.

Circulation reports 2020; (2(4)):249-254 doi:10.1253/circrep.CR-20-0006.

Efficacy and safety of novel-targeted drugs in the treatment of pulmonary arterial hypertension: a Bayesian network meta-analysis.

Fu W, He W, Li Y, et al.

Drug delivery 2021; (28(1)):1007-1019 doi:10.1080/10717544.2021.1927243.

COMPERA 2.0: a refined four-stratum risk assessment model for pulmonary arterial hypertension.

Hoeper MM, Pausch C, Olsson KM, et al.

The European respiratory journal 2022; (60(1)) doi:10.1183/13993003.02311-2021.

External validation of a refined four-stratum risk assessment score from the French pulmonary hypertension registry.

Boucly A, Weatherald J, Savale L, et al.

The European respiratory journal 2022; (59(6)) doi:10.1183/13993003.02419-2021.

Strategies for optimizing intravenous prostacyclin-analog therapy in patients with pulmonary arterial hypertension.

Ewert R, Habedank D, Halank M, et al.

Expert review of respiratory medicine 2022; (16(1)):57-66 doi:10.1080/17476348.2022.2011220.

Clinical characteristics and prognosis analysis of idiopathic and hereditary pulmonary hypertension patients with ACVRL1 gene mutations.

Zhang X, Zhang C, Li Q, et al.

Pulmonary circulation 2021; (11(4)):20458940211044577 doi:10.1177/20458940211044577.

A Retrospective Population-Based Survival Study of Idiopathic Pulmonary Arterial Hypertension in Korea.

Jang SY, Kim EK, Huh J, et al.

Journal of Korean medical science 2022; (37(10)):e80 doi:10.3346/jkms.2022.37.e80.

Whole Exome Sequencing of Patients With Heritable and Idiopathic Pulmonary Arterial Hypertension in Central Taiwan.

Liang KW, Chang SK, Chen YW, et al.

Frontiers in cardiovascular medicine 2022; (9()):911649 doi:10.3389/fcvm.2022.911649.

Screening for Brain Metastases in Patients With NSCLC: A Qualitative Study on the Psychologic Impact of Being Diagnosed With Asymptomatic Brain Metastases.

Schoenmaekers JJAO, Bruinsma J, Wolfs C, et al.

JTO clinical and research reports 2022; (3(10)):100401 doi:10.1016/j.jtocrr.2022.100401.

CircGSAP alleviates pulmonary microvascular endothelial cells dysfunction in pulmonary hypertension via regulating miR-27a-3p/BMPR2 axis.

Sun Y, Jiang R, Hu X, et al.

Respiratory research 2022; (23(1)):322 doi:10.1186/s12931-022-02248-7.

Time to diagnosis of pulmonary hypertension and diagnostic burden: A retrospective analysis of nationwide US healthcare data.

Didden EM, Lee E, Wyckmans J, et al.

Pulmonary circulation 2023; (13(1)):e12188 doi:10.1002/pul2.12188.

Phase 3 Trial of Sotatercept for Treatment of Pulmonary Arterial Hypertension.

Hoeper MM, Badesch DB, Ghofrani HA, et al.

The New England journal of medicine 2023; (388(16)):1478-1490 doi:10.1056/NEJMoa2213558.

Feasibility and preliminary efficacy of a virtual reality intervention targeting distress and anxiety in primary brain tumor patients at the time of clinical evaluation: Study protocol for a phase 2 clinical trial.

King AL, Acquaye-Mallory AA, Vera E, et al.

BMC cancer 2023; (23(1)):262 doi:10.1186/s12885-023-10671-2.

Coping With 'Scanxiety': Within-Person Processes in Lung Cancer.

Dunsmore VJ, Neupert SD

Psychological reports 2025; (128(2)):702-722 doi:10.1177/00332941231164336.

Scanxiety Conversations on Twitter: Observational Study.

Bui KT, Li Z, Dhillon HM, et al.

JMIR cancer 2023; (9()):e43609 doi:10.2196/43609.

Refined risk stratification, current treatment, and new therapeutic approaches in pulmonary arterial hypertension.

Lange TJ

Herz 2023; (48(4)):259-265 doi:10.1007/s00059-023-05179-1.

Impact of targeted pulmonary arterial hypertension therapies in severe pulmonary hypertension in chronic lung diseases.

Naud R, Bermudez J, Resseguier N, et al.

ERJ open research 2023; (9(4)) doi:10.1183/23120541.00027-2023.

E2F1 Mediates SOX17 Deficiency-Induced Pulmonary Hypertension.

Yi D, Liu B, Ding H, et al.

Hypertension (Dallas, Tex. : 1979) 2023; (80(11)):2357-2371 doi:10.1161/HYPERTENSIONAHA.123.21241.

Long-Term Effect of TBX4 Germline Mutation on Pulmonary Clinico-Histopathologic Phenotype.

Doughty ES, Norvik C, Levin A, et al.

Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society 2024; (27(1)):83-89 doi:10.1177/10935266231199933.

ERS International Congress 2023: highlights from the Pulmonary Vascular Diseases Assembly.

Cullivan S, Boucly A, Jevnikar M, et al.

ERJ open research 2024; (10(1)) doi:10.1183/23120541.00847-2023.

Updated Clinical Classification and Hemodynamic Definitions of Pulmonary Hypertension and Its Clinical Implications.

Kularatne M, Gerges C, Jevnikar M, et al.

Journal of cardiovascular development and disease 2024; (11(3)) doi:10.3390/jcdd11030078.

SPECTRA Phase 2b Study: Impact of Sotatercept on Exercise Tolerance and Right Ventricular Function in Pulmonary Arterial Hypertension.

Waxman AB, Systrom DM, Manimaran S, et al.

Circulation. Heart failure 2024; (17(5)):e011227 doi:10.1161/CIRCHEARTFAILURE.123.011227.

Positive Vasoreactivity Testing in Pulmonary Arterial Hypertension: Therapeutic Consequences, Treatment Patterns, and Outcomes in the Modern Management Era.

Gerhardt F, Fiessler E, Olsson KM, et al.

Circulation 2024; (149(20)):1549-1564 doi:10.1161/CIRCULATIONAHA.122.063821.

Calcium channel blockers in patients with pulmonary arterial hypertension receiving PAH-specific treatment.

Hirakawa K, Asano R, Ueda J, et al.

International journal of cardiology 2024; (406()):132043 doi:10.1016/j.ijcard.2024.132043.

Factors associated with discontinuation of treatment for pulmonary arterial hypertension in the United States.

Farber HW, Germack HD, Croteau NS, et al.

Pulmonary circulation 2024; (14(2)):e12326 doi:10.1002/pul2.12326.

A review regarding the article `Right heart catheterization in idiopathic pulmonary hypertension: An all-inclusive necessity'.

Wu J, Jiang Y

Current problems in cardiology 2024; (49(9)):102673 doi:10.1016/j.cpcardiol.2024.102673.

Application of REVEAL Lite 2 and COMPERA 2.0 risk scores to patients with pulmonary arterial hypertension switching to riociguat in the REPLACE study.

Benza RL, Simonneau G, Ghofrani HA, et al.

The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation 2024; (43(10)):1756-1760 doi:10.1016/j.healun.2024.06.002.

Surveillance-Associated Anxiety After Curative-Intent Cancer Surgery: A Systematic Review.

Khatri R, Quinn PL, Wells-Di Gregorio S, et al.

Annals of surgical oncology 2025; (32(1)):47-62 doi:10.1245/s10434-024-16287-5.

Cardiopulmonary Exercise Testing in Pulmonary Hypertension.

Dmytriiev K, Stickland MK, Weatherald J

Heart failure clinics 2025; (21(1)):51-61 doi:10.1016/j.hfc.2024.05.002.

SOX17-Associated Pulmonary Hypertension in Children: A Distinct Developmental and Clinical Syndrome.

Mullen MP, Ivy DD, Varghese NP, et al.

The Journal of pediatrics 2025; (278()):114422 doi:10.1016/j.jpeds.2024.114422.

The Role of Lung Ventilation/Perfusion Scan in the Management of Chronic Thromboembolic Pulmonary Hypertension.

Ha S, Han S

Nuclear medicine and molecular imaging 2024; (58(7)):449-458 doi:10.1007/s13139-023-00830-5.

Hemodynamic and Genetic Associations with the Risk of Idiopathic Pulmonary Arterial Hypertension Development in an Ethnic Cohort of Kazakhs.

Taizhanova D, Nurpissova T, Abildinova G, et al.

Diagnostics (Basel, Switzerland) 2024; (14(23)) doi:10.3390/diagnostics14232687.

Emerging therapies and new directions in the treatment of pulmonary arterial hypertension.

Kopeć G, Skride A, Ereminiene E, et al.

Kardiologia polska 2025; (83(1)):18-26 doi:10.33963/v.phj.104053.

Sotatercept and pulmonary arterial hypertension.

Tan Y, Chen Y, Li J

Zhong nan da xue xue bao. Yi xue ban = Journal of Central South University. Medical sciences 2024; (49(9)):1503-1508 doi:10.11817/j.issn.1672-7347.2024.240093.

Sotatercept in Patients with Pulmonary Arterial Hypertension at High Risk for Death.

Humbert M, McLaughlin VV, Badesch DB, et al.

The New England journal of medicine 2025; (392(20)):1987-2000 doi:10.1056/NEJMoa2415160.

Reaching for the summit in pulmonary arterial hypertension with sotatercept: Results of the ZENITH study.

Condliffe R, Kiely DG

Med (New York, N.Y.) 2025; (6(7)):100750 doi:10.1016/j.medj.2025.100750.

Diagnosis of chronic thromboembolic pulmonary hypertension.

Jais X, Tapson V, Fernandes TM, et al.

The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation 2025; (44(7S)):S1-S7 doi:10.1016/j.healun.2025.02.1688.

Prospective safety assessment of the acute vasoreactivity test using an inhaled nitric oxide delivery system in pulmonary hypertension.

Minatsuki S, Hatano M, Ishii S, et al.

International journal of cardiology 2026; (444()):134009 doi:10.1016/j.ijcard.2025.134009.

THBS4 Regulates Pulmonary Hypertension via TGF-β/SMAD2 Signaling.

Zeng J, Li D, Wang J, et al.

Hypertension (Dallas, Tex. : 1979) 2026; (83(6)):e25968 doi:10.1161/HYPERTENSIONAHA.125.25968.