Class 1. Heritable Pulmonary Hypertension

Pulmonary Hypertension Induced by Right Pulmonary Artery Occlusion: Hemodynamic Consequences of Bmpr2 Mutation

Alban Todesco, Julien Grynblat, Kouamé Kan Firmin Akoumia, Damien Bonnet, Pedro Mendes‐Ferreira, Stéphane Morisset, Denis Chemla, Marilyne Levy, Mathilde Méot, Sophie‐Guiti Malekzadeh‐Milani, Birger Tielemans, Benoit Decante, Carine Vastel‐Amzallag, Paul Habert, Maria‐Rosa Ghigna, Marc Humbert, David Montani, David Boulate, Frédéric PerrosNorth Hospital Aix Marseille University, Assistance Publique-Hôpitaux de Marseille. INSERM UMR_S 999 Pulmonary Hypertension: Pathophysiology and Novel Therapies Le Plessis. Hôpital Necker-Enfants malades AP-HP Université de Paris Cité. Le Kremlin-Bicêtre Université Paris-Saclay. Faculty of Medicine of […]

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Early identification of SOX17 deficiency in infants to guide management of heritable pulmonary arterial hypertension using PDA stent to create reverse Potts shunt physiology

Heidi Ostler, Carolyn Fall, Howaida El‐Said, Henri Justino, Shylah Haldeman, Jeanne Carroll, Rohit RaoRady Children’s Hospital and University of California San Diego California.United States Pulmonary CirculationPulm Circ 2024; 14:DOI: 10.1002/pul2.12366 AbstractHeritable pulmonary arterial hypertension (HPAH) is a rare progressive condition that includes patients with an identified genetic cause of pulmonary arterial hypertension (PAH). HPAH and idiopathic

Early identification of SOX17 deficiency in infants to guide management of heritable pulmonary arterial hypertension using PDA stent to create reverse Potts shunt physiology Read More »

Extracorporeal membrane oxygenation support for balloon atrial septostomy in a BMPR2 variant-associated pulmonary arterial hypertension

Shoji Fukuoka, Noriyuki Kaku, Hazumu Nagata, Yusaku Nagatomo, Kanako Higashi, Daisuke Toyomura, Yuichiro Hirata, Keiichi Hirono, Kenichiro Yamamura, Shouichi OhgaKyushu University. University of Toyama.Japan Pediatric PulmonologyPediatr Pulmonol 2024; DOI: 10.1002/ppul.26973 AbstractAbstract Not Available CategoryClass I. Heritable Pulmonary HypertensionSurgical and Catheter-mediated Interventions for Pulmonary Vascular Disease Age Focus: Pediatric Pulmonary Vascular Disease Fresh or Filed Publication: Fresh

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Hemodynamic and Clinical Profiles of Pulmonary Arterial Hypertension Patients with GDF2 and BMPR2 Variants

Mei-Tzu Wang, Ken-Pen Weng, Sheng-Kai Chang, Wei-Chun Huang, Lee-Wei ChenNational Yang Ming Chiao Tung University. Kaohsiung Veterans General Hospital. Excelsior Biopharma Inc. Fooyin University. National Sun Yat-Sen University.Taiwan International Journal of Molecular SciencesInt J Mol Sci 2024; 25:DOI: 10.3390/ijms25052734 AbstractAsians have a higher carrier rate of pulmonary arterial hypertension (PAH)-related genetic variants than Caucasians do. This

Hemodynamic and Clinical Profiles of Pulmonary Arterial Hypertension Patients with GDF2 and BMPR2 Variants Read More »

Long-term safety and tolerability of ambrisentan treatment for pediatric patients with pulmonary arterial hypertension: An open-label extension study

Dunbar Ivy, Maurice Beghetti, Ernesto Juaneda‑Simian, Ramiya Ravindranath, Mary Ann Lukas, Sandra Machlitt‑Northen, Nicola Scott, Jun Narita, Rolf M. F. BergerChildren’s Hospital Colorado. University Children’s Hospital HUG and University of Geneva. Hospital de Niños de la Santísma Trinidad. Glaxo-Smith-Kline. Osaka University Graduate School of Medicine. Beatrix Children’s Hospital, University Medical Center Groningen and University of

Long-term safety and tolerability of ambrisentan treatment for pediatric patients with pulmonary arterial hypertension: An open-label extension study Read More »

Younger age at initiation of subcutaneous treprostinil is associated with better response in pediatric Group 1 pulmonary arterial hypertension

Justin J. Kochanski, Jeffrey A. Feinstein, Michelle Ogawa, Victor Ritter, Rachel K. Hopper, Gregory T. AdamsonStanford University School of Medicine.United States Pulmonary CirculationPulm Circ 2024; DOI: 10.1002/pul2.12328 AbstractChildren with severe Group 1 pulmonary arterial hypertension (PAH) have an unpredictable response to subcutaneous treprostinil (TRE) therapy, which may be influenced by age, disease severity, or other unknown

Younger age at initiation of subcutaneous treprostinil is associated with better response in pediatric Group 1 pulmonary arterial hypertension Read More »

Biallelic variants in the calpain regulatory subunit CAPNS1 cause pulmonary arterial hypertension

Alex V. Postma, Christina K. Rapp, Katrin Knoflach, Alexander E. Volk, Johannes R. Lemke, Maximilian Ackermann, Nicolas Regamey, Philipp Latzin, Lucas Celant, Samara M. A. Jansen, Harm J. Bogaard, AhoI lgun, Mariëlle Alders, Karin Y. van Spendonck-Zwarts, Danny Jonigk, Christoph Klein, Stefan Gräf, Christian Kubisch, Arjan C. Houweling, Matthias GrieseAmsterdam University Medical Centre. Dr. von

Biallelic variants in the calpain regulatory subunit CAPNS1 cause pulmonary arterial hypertension Read More »

Treprostinil effectiveness in higher-risk pediatric patients with Idiopathic and Heritable Pulmonary Arterial Hypertension

Yuan He, Qiangqiang Li, Chen Zhang, Bradley B. Keller, Hong GuBeijing Anzhen Hospital and Capital Medical University. Cincinnati Children’s Hospital Medical Center. China and United States Canadian Journal of CardiologyCan J Cardiol 2023; DOI: 10.1016/j.cjca.2023.11.004 AbstractIntroduction: Little is known about the effectiveness of treprostinil in higher-risk pediatric patients with various PAH genotypes. This study was designed to

Treprostinil effectiveness in higher-risk pediatric patients with Idiopathic and Heritable Pulmonary Arterial Hypertension Read More »

Single-Cell Imaging Maps Inflammatory Cell Subsets to Pulmonary Arterial Hypertension Vasculopathy

Selena Ferrian, Aiqin Cao, Erin McCaffrey, Toshie Saito, Noah F. Greenwald, Mark R. Nichols, Trevor Bruce, Roham T. Zamanian, Patricia Del Rosario, Marlene Rabinovitch, Michael AngeloStanford University School of Medicine. United States American Journal of Respiratory and Critical Care MedicineAm J Respir Crit Care Med 2023; DOI: 10.1164/rccm.202209-1761OC AbstractRationale: Elucidating the immune landscape within and surrounding pulmonary

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Corrigendum: Case report: Selexipag in pediatric pulmonary hypertension: initiation, transition, and titration

Jenna M. Faircloth, Neelam D. Bhatt, Corey A. Chartan, Ryan D. Coleman, Natalie Villafranco, Fadel E. Ruiz, Raysa Morales-Demori, Elise Whalen, Erin Ely, Rozmeen Fombin, Nidhy P. VargheseTexas Children’s Hospital and Baylor College of Medicine.United States Frontiers in PediatricsFront Pediatr 2023; 11DOI: 10.3389/fped.2023.1275389 AbstractThis corrects the article DOI: 10.3389/fped.2023.1050508. CategoryClass I. Idiopathic Pulmonary HypertensionClass I. Heritable

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