Class 1. Heritable Pulmonary Hypertension

Altered maturation and activation state of circulating monocytes is associated with their enhanced recruitment in pulmonary arterial hypertension

Rebecca L. Harper, Xin Zhou, David P. Marciano, Aiqin Cao, Lingli Wang, Guibin Chen, Mir S. Adil, Wenyu Zhou, Peter Maguire, Shanthi Deivanayagam, Quan Yu, Vignesh Viswanathan, Dan Yang, Marcy Martin, Sarasa Isobe, Shoichiro Otsuki, Jordan Burgess, Audrey Inglis, Devon Kelley, Patricia A. del Rosario, Andrew Hsi, Francois Haddad, Roham T. Zamanian, Manfred Boehm, Michael […]

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X-linked filaminopathy: another pulmonary hypertension syndrome

Eric D. AustinVanderbilt University Medical Center.United States European Respiratory JournalEur Respir J 2025; 65: DOI: 10.1183/13993003.00073-2025 AbstractAbstract Not Available CategoryClass I. Heritable Pulmonary HypertensionGenetic Factors Associated with Pulmonary Vascular Disease Age Focus: Pediatric Pulmonary Vascular Disease Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication Article Access Free PDF File or Full Text

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RNA-Seq and ChIP-Seq Identification of Unique and Overlapping Target Genes and Pathways Regulated by TBX4 in Human Pulmonary Fibroblasts and Pericytes

Ying Cai, Ling Yan, James West, Joy D. Cogan, Lora K. Hedges, Bethany Nunley, Nick Negretti, Jennifer M. S. Sucre, Eric D. Austin, Rizwan HamidVanderbilt University Medical Center Nashville. Biodevelopment Origins of Lung Disease (BOLD) Center. United States Pulmonary CirculationPulm Circ 2025; 15: DOI: 10.1002/pul2.70058 AbstractTranscription factor TBX4 rare variants associate with pulmonary arterial hypertension (PAH), particularly in

RNA-Seq and ChIP-Seq Identification of Unique and Overlapping Target Genes and Pathways Regulated by TBX4 in Human Pulmonary Fibroblasts and Pericytes 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

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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 »

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

Mary P. Mullen, D. Dunbar Ivy, Nidhy P. Varghese, Abbey J. Winant, Nahir Cortes-Santiago, Sara O. Vargas, Diego Porres, Nicola Maschietto, Paul J. Critser, Russel Hirsch, Catherine M. Avitabile, Rachel K. Hopper, Benkamin S. Frank, Ryan D. Coleman, Pankaj B. Agrawal, Jill A. Madden, Amy E. Roberts, Shane L. Collins, J. Usha Raj, Eric D.

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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 »

Genetics and precision genomics approaches to pulmonary hypertension

Eric D. Austin, Micheala A. Aldred, Mona Alotaibi, Stefan Gräf, William C. Nichols, Richard C. Trembath, Wendy K. ChungVanderbilt University Medical Center. Indiana University School of Medicine. University of California San Diego. University of Cambridge. Cincinnati Children’s Hospital Medical Center and University of Cincinnati College of Medicine. King’s College London. Boston Children’s Hospital. United States

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Safety and Effectiveness of Selexipag in Pediatric Pulmonary Hypertension: A Retrospective Multi-Center Cohort Study

Benjamin S. Frank, Eliza R. Gentzler, Catherine M. Avitabile, Kathleen Miller-Reed, Zhaoxing Pan, Erika B. Rosenzweig, D. Dunbar Ivy, Usha S. KrishnanUniversity of Colorado School of Medicine and Children’s Hospital Colorado. Columbia University Irving Medical Center and Morgan Stanley Childrens Hospital of New York Presbyterian Hospital. University of Pennsylvania School of Medicine and Children’s Hospital

Safety and Effectiveness of Selexipag in Pediatric Pulmonary Hypertension: A Retrospective Multi-Center Cohort Study Read More »

Pediatric Lung Transplantation for Pulmonary Vascular Diseases: Recent Advances and Challenges

Levent Midyat, Eleanor D. Muise, Gary A. VisnerUniversity of Pittsburgh School of Medicine and Children’s Hospital of Pittsburgh. NYU Grossman School of Medicine and Hassenfeld Children’s Hospital. Harvard Medical School and Boston Children’s Hospital.United states Clinics in Chest MedicineClin Chest Med 2024; 45: 761-769DOI: 10.1016/j.ccm.2024.02.023 AbstractPediatric lung transplantation for pulmonary vascular diseases has seen notable advancements

Pediatric Lung Transplantation for Pulmonary Vascular Diseases: Recent Advances and Challenges Read More »

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