Class 1. Idiopathic Pulmonary Hypertension

Cardiopulmonary Exercise Testing With Forehead and Popliteal Oximetry in Evaluating Efficacy of Reverse Potts Shunt

Keti Mamillo, Robert P. Frantz, Jason H. Anderson, Thomas G. AllisonMayo Clinic.United States Journal of the American College of Cardiology Case ReportsJACC Case Rep 2025; 30: DOI: 10.1016/j.jaccas.2025.103409 AbstractReverse Potts shunt (rPS) is a surgical procedure that creates an anastomosis between the left pulmonary artery and descending aorta to decompress the right ventricle in suprasystemic pulmonary […]

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Estrogen and Cyp1b1 Regulate Pparγ in Pulmonary Hypertension Through a Ubiquitin-Dependent Mechanism

Jingyuan Chen, Xinping Chen, Vineet Agrawal, Christy S. Moore, Tom Blackwell, Nivedita Rathaur, Santhi Gladson, Anandharajan Rathinasabapathy, Anna Hemnes, Eric Austin, James WestVanderbilt University Medical Center. Second Xiangya Hospital of Central South University. Wistar Institute.United States and China Pulmonary CirculationPulm Circ 2025; 15: DOI: 10.1002/pul2.70054 AbstractFemale sex increases risk of Group I pulmonary arterial hypertension by

Estrogen and Cyp1b1 Regulate Pparγ in Pulmonary Hypertension Through a Ubiquitin-Dependent Mechanism Read More »

Safety and Efficacy of Selexipag for Pediatric Pulmonary Arterial Hypertension in Japanese Patients – An Open-Label Phase 2 Study

Toru Iwasa, Ryo Inuzuka, Hiroshi Ono, Yuichiro Sugitani, Hirokuni Yamazawa, Chihiro Hiraishi, Naoki Shiota, Shinichi Tanaka, Chieko Yamamoto, Ken-ichi Kurosaki, Masaru Miura, Satoshi YasukochiNational Cerebral and Cardiovascular Center. University of Tokyo. National Center for Child Health and Development. Japan Community Health Care Organization Kyushu Hospital. Hokkaido University. Nippon Shinyaku Company. Tokyo Metropolitan Children’s Medical Center.

Safety and Efficacy of Selexipag for Pediatric Pulmonary Arterial Hypertension in Japanese Patients – An Open-Label Phase 2 Study Read More »

Pediatric Pulmonary Arterial Hypertension; is it Possible to Predict its Outcome?

Hala M. Aghaa, Ranya Hegazy, Ahmed Gamal, Ranya Essam, Antoine A. Elmessih, Ahmed Behairy,Nagib Dahdah, Ahmed Abdelwahed, Nesrine Sobhy, Noha AliSpecialized Pediatric Hospital and Cairo University. CHU Sainte Justine and University of Montreal.Egypt and Canada Journal of the Saudi Heart AssociationJ Saudi Heart Assoc 2024; 36: 408-419DOI: 10.37616/2212-5043.1398 AbstractObjectives: To assess the outcome of pediatric pulmonary arterial

Pediatric Pulmonary Arterial Hypertension; is it Possible to Predict its Outcome? Read More »

Safety, tolerability, and efficacy of an in-class combination therapy switch from bosentan plus sildenafil to ambrisentan plus tadalafil in children with pulmonary arterial hypertension

Cara Morgan, Nikmah Idris, Kathy Elterefi, Luca Di Ienno, Andrew Constantine, Sadia Quyam Roberta Bini, Shahin MoledinaGreat Ormond Street Hospital for Children. Queen Elizabeth Hospital. Institute of Cardiovascular Sciences University College London. United Kingdom Pulmonary CirculationPulm Circ 2024;DOI: 10.1002/pul2.70011 AbstractThe aim of this single-centre retrospective observational study was to evaluate the safety, tolerability, and efficacy of

Safety, tolerability, and efficacy of an in-class combination therapy switch from bosentan plus sildenafil to ambrisentan plus tadalafil in children with pulmonary arterial hypertension Read More »

Case report: A finding of PVOD and PAH in first degree relatives suggests shared heritable risk and overlapping features of both pulmonary vascular diseases

Roger Winters, Lindsay M. Forbes, Dunbar Ivy, Carlyne Cool, Bryan D. Park, Peter Hountras, David Badesch, Sue Gu, Edda Spiekerkoetter, Roham Zamanian, Stacey Leier Gluck, Todd M. BullUniversity of Colorado and University of Colorado Children’s Hospital. Stanford University.United States Respirology Case ReportsRespirol Case Rep 2024; 12: DOI: 10.1002/rcr2.70064 AbstractPulmonary veno-occlusive disease (PVOD) is a rare form

Case report: A finding of PVOD and PAH in first degree relatives suggests shared heritable risk and overlapping features of both pulmonary vascular diseases Read More »

Genetic burden in neonatal and pediatric-onset pulmonary hypertension: A single-center retrospective study using exome sequencing in a Chinese population

Chen Chen Fucheng Li, Hang Zhou, Dan Wang, Fang Fu, Qiuxia Yu, Ruibin Huang, Yan Lu, You Wang, Guilan Chen, Fei Guo, Tingying Lei, Chunlin Ma, Ru LiGuangzhou Women and Children’s Medical Center. Guangzhou Medical University. China Pediatrics and NeonatologyPediatr Neonatol 2024; DOI: 10.1016/j.pedneo.2024.06.010 AbstractObjective: This single-center retrospective study aimed to investigate the genetic factors contributing to

Genetic burden in neonatal and pediatric-onset pulmonary hypertension: A single-center retrospective study using exome sequencing in a Chinese population Read More »

Collapse of an early adolescent girl. Syncope? Simple or sinister?

Graham Martin, Chiara Cavaliere, Nikila Pandya, Krishnan BalasubramanianMaidstone and Tunbridge Wells NHS Trust.United Kingdom British Medical Journals Case ReportsBMJ Case Rep 2024; 17: DOI: 10.1136/bcr-2023-259246 AbstractAn early adolescent girl presented to the emergency department (ED) of her local hospital following a syncopal episode after a warm bath on a background of increasing breathlessness for 1-year duration. On examination, she

Collapse of an early adolescent girl. Syncope? Simple or sinister? Read More »

PPARγ/ETV2 axis regulates endothelial-to-mesenchymal transition in pulmonary hypertension

Dong Hun Lee, Andrew J. Jang, Minseong Kim, Sarah S. Chang, Raham Lee, Juyoung Kim, Jing Ma, Michael J. Passineau, Raymond L. Benza, Harry Karmouty‐Quintana, Benjamin T. Kopp Roy L. Sutliff, Wilbur A. Lam, C. Michael Hart, Changwon Park, Bum‐Yong KangEmory University School of Medicine. Chonnam National University. Louisiana State University Health Science Center. Atlanta

PPARγ/ETV2 axis regulates endothelial-to-mesenchymal transition in pulmonary hypertension Read More »

A Single Center Experience of Pulmonary Arterial Hypertension Management in Korea: A 25-Year Comparative Analysis Following the Introduction of Targeted Therapy

Ji Hyun Cha I-Seok Kang, Shin Yi Jang, June Huh Jeong Hoon Yang, Jinyoung Song, Taek Kyu Park, Seung Woo Park, Hojoong Kim, Duk-Kyung Kim Sung-A ChangSamsung Medical Center, Samsung Changwon Hospital and Sungkyunkwan University School of Medicine. Republic of Korea Korean Circulation JournalKorean Circ J 2024; DOI: 10.4070/kcj.2023.0316 AbstractBackground and objectives: The transformation of pulmonary arterial

A Single Center Experience of Pulmonary Arterial Hypertension Management in Korea: A 25-Year Comparative Analysis Following the Introduction of Targeted Therapy Read More »

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