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Biallelic variants of ATP13A3 cause dose-dependent childhood-onset pulmonary arterial hypertension characterised by extreme morbidity and mortality

Rajiv D. Machado, Carrie L. Welch, Matthias Haimel, Marta Bleda, Elizabeth Colglazier, John D. Coulson, Marusa Debeljak, Josef Ekstein, Jeffrey R. Fineman, William Christopher Golden, Emily L. Griffin, Charaka Hadinnapola, Michael A. Harris, Yoel Hirsch, Julie Elizabeth Hoover- Fong, Lawrence Nogee, Lewis H. Romer, Samo Vesel, NIHR Bioresource – Rare Diseases, Stefan Gräf, Nicholas W. […]

Biallelic variants of ATP13A3 cause dose-dependent childhood-onset pulmonary arterial hypertension characterised by extreme morbidity and mortality Read More »

Genetics dictating therapeutic decisions in pediatric pulmonary hypertension? A case report suggesting we are getting closer

Leah Stevens, Elizabeth Colglazier, Claire Parker, Elena K. Amin, Hythem Nawaytou, David Teitel, Vadiyala M. Reddy, Carrie L. Welch, Wendy K. Chung, Jeffrey R. FinemanUniversity of California San Francisco. Columbia University Irving Medical Center. United States Pulmonary CirculationPulm Circ 2022; 12: DOI: 10.1002/pul2.12033 AbstractDespite therapeutic advances over the past decades, pulmonary arterial hypertension (PAH) and related

Genetics dictating therapeutic decisions in pediatric pulmonary hypertension? A case report suggesting we are getting closer Read More »

Possible association between a polymorphism of EPAS1 gene and persistent pulmonary hypertension of the newborn: a case-control study

Narongsak Nakwan, Surakameth Mahasirimongkol, Nusara Satproedprai, Tassamonwan Chaiyasung, Punna Kunhapan, Cheep Charoenlap, KumonnutSingkhamanan, Chariyawan CharalsawadiPrince of Songkla University. Nonthaburi Ministry of Public Heath. Hat Yai Hospital. Thailand Jornal de PediatriaJ Pediatr 2022; 98: 383-389DOI: 10.1016/j.jped.2021.09.003 AbstractObjective: To explore possible genes related to the development of persistent pulmonary hypertension of the newborn (PPHN).Methods: The authors identified 285 single nucleotide

Possible association between a polymorphism of EPAS1 gene and persistent pulmonary hypertension of the newborn: a case-control study Read More »

Extracellular vesicles derived from endothelial cells in hypoxia contribute to pulmonary artery smooth muscle cell proliferation in-vitro and pulmonary hypertension in mice

Tianji Chen, Miranda R. Sun, Qiyuan Zhou, Alyssa M. Guzman, Ramaswamy Ramchandran, Jiwang Chen, Balaji Ganesh, J. Usha RajUniversity of Illinois at Chicago. United States Pulmonary CirculationPulm Circ 2022; 12: DOI: 10.1002/pul2.12014 AbstractIn the lung, communication between pulmonary vascular endothelial cells (PVEC) and pulmonary artery smooth muscle cells (PASMC) is essential for the maintenance of vascular

Extracellular vesicles derived from endothelial cells in hypoxia contribute to pulmonary artery smooth muscle cell proliferation in-vitro and pulmonary hypertension in mice Read More »

High haemoglobin levels at the time of occlusion predict worse outcome for patients with patent ductus arteriosus and pulmonary hypertension

Kanta Kishi, Jirayut Jarutach, Yinn Khurn Wong, Marhisham Che Mood, Geetha Kandhavello, Mazeni Alwi, Ming Chern Leong Osaka Medical and Pharmaceutical University. Prince of Songkla University. Serdang Hospital. Sarawak General Hospital. Institut Jantung Negara.Japan, Thailand and Malaysia Cardiology in the YoungCardiol Young 2022; DOI: 10.1017/S104795112200244X AbstractIntroduction: Current guidelines discourage shunt closure in patients with pulmonary vascular resistance

High haemoglobin levels at the time of occlusion predict worse outcome for patients with patent ductus arteriosus and pulmonary hypertension Read More »

Normal Echocardiographic Reference Values of the Right Ventricular to Left Ventricular Endsystolic Diameter Ratio and the Left Ventricular Endsystolic Eccentricity Index in Healthy Children and in Children With Pulmonary Hypertension

Sabrina Schweintzger, Stefan Kurath-Koller, Ante Burmas, Gernot Grangl, Andrea Fandl, Nathalie Noessler, Alexander Avian, Andreas Gamillscheg, Philippe Chouvarine, Georg Hansmann, Martin KoesnbergerMedical University Graz. Hannover Medical School. European Pediatric Pulmonary Vascular Disease Network. Austria and Germany Frontiers in Cardiovascular MedicineFront Cardiovasc Med 2022; DOI: 10.3389/fcvm.2022.950765 AbstractBackground: An accurate assessment of the right and left ventricle and their

Normal Echocardiographic Reference Values of the Right Ventricular to Left Ventricular Endsystolic Diameter Ratio and the Left Ventricular Endsystolic Eccentricity Index in Healthy Children and in Children With Pulmonary Hypertension Read More »

Large animal preclinical investigation into the optimal extracorporeal life support configuration for pulmonary hypertension and right ventricular failure

Rei Utika, John W. Stokes, W. Kelly Wu, Yatrik J. Patel, Jennifer R. Talackine, Nancy Cardwell, Clayne Benson, Ryan J. Lefevre, Susan Eagle, Caitlin Demarest, Elizabeth Simonds, Yuliya Tipograf, Michael Cortelli, David J. Skoog, Keith Cook, Erika B. Rosenzweig, Matthew BachettaVanderbilt University Medical Center. Advanced Respiratory Technologies LLC. Carnegie Mellon University. Columbia University Medical Center.United

Large animal preclinical investigation into the optimal extracorporeal life support configuration for pulmonary hypertension and right ventricular failure Read More »

Safety and tolerability of combination therapy with ambrisentan and tadalafil for the treatment of pulmonary arterial hypertension in children: Real-world experience

Azadeh Issapour, Benjamin Frank, Sarah Crook, Michelle D. Hite, Michelle L. Dorn, Erika B. Rosenzweig, D. Dunbar Ivy, Usha S. KrishnanColumbia University Irving Medical Center. University of Colorado and Children’s Hospital Colorado.United States Pediatric PulmonologyPediatr Pulmonol 2022; 57: 724-733DOI: 10.1002/ppul.25796 AbstractObjective: To describe the safety and tolerability of treatment with ambrisentan and tadalafil in pediatric pulmonary hypertension

Safety and tolerability of combination therapy with ambrisentan and tadalafil for the treatment of pulmonary arterial hypertension in children: Real-world experience Read More »

Distinguishing exercise intolerance in early-stage pulmonary hypertension with invasive exercise hemodynamics: Rest VE /VCO2 and ETCO2 identify pulmonary vascular disease

Farhan Raza, Naga Dharmavaram MD, Timothy Hess, Ravi Dhingra, James Runo, Amy Chybowski, Callyn Kozitza, Supria Batra, Evelyn M. Horn, Naomi Chesler, Marlowe EldridgeUniversity of Wisconsin-Madison. Weill Cornell Medicine. University of California-Irvine.United States Clinical CardiologyClin Cardiol 2022; 45: 742-751DOI: 10.1002/clc.23831 AbstractBackground: Among subjects with exercise intolerance and suspected early-stage pulmonary hypertension (PH), early identification of pulmonary vascular

Distinguishing exercise intolerance in early-stage pulmonary hypertension with invasive exercise hemodynamics: Rest VE /VCO2 and ETCO2 identify pulmonary vascular disease Read More »

Channelopathy Genes in Pulmonary Arterial Hypertension

Carrie L. Welch, Wendy K. ChungColumbia University Irving Medical Center.United States BiomoleculesBiomolecules 2022; 12: DOI: 10.3390/biom12020265 AbstractPulmonary arterial hypertension (PAH) is a rare, progressive vasculopathy with significant cardiopulmonary morbidity and mortality. The underlying pathogenetic mechanisms are heterogeneous and current therapies aim to decrease pulmonary vascular resistance but no curative treatments are available. Causal genetic variants can

Channelopathy Genes in Pulmonary Arterial Hypertension Read More »

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