Class 1. Heritable Pulmonary Hypertension

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 […]

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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

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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

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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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Cardiac Magnetic Resonance Derived Left Ventricular Eccentricity Index and Right Ventricular Mass Measurements Predict Outcome in Children with Pulmonary Arterial Hypertension

Meindina G. Haarman, Iris Coenraad, Quint A. J. Hagdorn, Hans L. Hillege, Tineke P. Willems, Rolf M. F. Berger, Johannes M. DouwesBeatrix Children’s Hospital and University Medical Center Groningen. Netherlands ChildrenChildren 2023; 10DOI: 10.3390/children10040756 AbstractPulmonary arterial hypertension (PAH) is associated with increased right ventricular (RV) afterload, affecting RV remodeling and RV performance, a major determinant of

Cardiac Magnetic Resonance Derived Left Ventricular Eccentricity Index and Right Ventricular Mass Measurements Predict Outcome in Children with Pulmonary Arterial Hypertension Read More »

Pediatric pulmonary arterial hypertension due to a novel homozygous GDF2 missense variant affecting BMP9 processing and activity

L. Chomette, E. Hupkens, M. Romitti, L. Dewachter, J. L.Vachiéry, S. Bailly, S. Costagliola, G. Smits, E. Tillet, Antoine BondueHôpital Erasme, Hôpital Universitaire de Bruxelles and Université Libre de Bruxelles. Laboratory BioSanté and Université Grenoble Alpes.Belgium and France American Journal of Medical GeneticsAm J Med Genet 2023; 191(8):2064-2073DOI: 10.1002/ajmg.a.63236 AbstractPulmonary arterial hypertension (PAH) is a rare

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The cascade screening in heritable forms of pulmonary arterial hypertension

Nidhy P. Varghese, Akhilesh A. Padhye, Pilar L. Magoulas, George B. Mallory, Fadel E. Ruiz, Sandeep SahayBaylor College of Medicine Texas Children’s Hospital Houston. Houston Methodist Hospital. Weill Cornell Medical College.United States Pulmonary CirculationPulm Circ 2023;DOI: 10.1002/pul2.12259 AbstractHeritable pulmonary artery hypertension (HPAH) is an increasingly recognized type of pulmonary arterial hypertension, in both pediatric and adult

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Clinical heterogeneity of Pulmonary Arterial Hypertension associated with variants in TBX4

Ignacio Hernandez-Gonzalez, Jair Tenorio, Julian Palomino-Doza, Amaya Martinez Meñaca, Rafael Morales Ruiz, Mauro Lago-Docampo, Marı´a Valverde Gomez, Javier Gomez Roman, Ana Bele´n Enguita Valls, Carmen Perez-Olivares, Diana Valverde, Joan Gil Carbonell, Elvira Garrido-Lestache Rodrı´guez-Monte, Maria Jesus del Cerro, Pablo Lapunzina, Pilar Escribano-SubiasHospital Universitario Río Hortega. Hospital Universitario La Paz. Centro de Investigación Biomédica en Red

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Customized Massive Parallel Sequencing Panel for Diagnosis of Pulmonary Arterial Hypertension

Jair Antonio Tenorio Castaño, Ignacio Hernández-Gonzalez, Natalia Gallego, Carmen Pérez-Olivares, Nuria Ochoa Parra, Pedro Arias, Elena Granda, Gonzalo Gómez Acebo, Mauro Lago-Docampo, Julian Palomino-Doza, Manuel López Meseguer, María Jesús del Cerro, Spanish PAH Consortium, Diana Valverde, Pablo Lapunzina, Pilar Escribano-SubíasHospital Universitario La Paz-UAM Paseo de La Castellana. Centro de Investigación Biomédica en Red de Enfermedades

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