Pediatric Pulmonary Vascular Disease

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 »

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 »

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 »

Long-Term Follow-Up of Pediatric Patients with Severe Postoperative Pulmonary Hypertension After Correction of Congenital Heart Defects

Lars LindbergLund University and Skane University HospitalSweden Pediatric CardiologyPediatr Cardiol 2022; 43: 827-836DOI: 10.1007/s00246-021-02794-9 AbstractThe surgical repair of congenital heart defects in children with preoperative pulmonary hypertension (PH) is to varying degree associated with the occurrence of postoperative PH. The objective of this study was to follow up children with severe postoperative PH (pulmonary arterial/aortic pressure

Long-Term Follow-Up of Pediatric Patients with Severe Postoperative Pulmonary Hypertension After Correction of Congenital Heart Defects Read More »

Fetal endoscopic tracheal occlusion and pulmonary hypertension in moderate congenital diaphragmatic hernia

Roopali Donepudi, Michael A. Belfort, Alireza A. Shamshirsaz, Timothy C. Lee, Sundeep G. Keswani, Alice King, Nancy A. Ayres, Caraciolo J. Fernandes, Magdalena Sanz-Cortes, Ahmed A. Nassr, Andres F. Espinoza, Candace C. Style, Jimmy EspinozaBaylor College of Medicine.United States Journal of Maternal Fetal and Neonatal MedicineJ Mat Fetal Neonat Med 2022; 35: 6967-6972DOI: 10.1080/14767058.2021.1932806 AbstractObjective: To study

Fetal endoscopic tracheal occlusion and pulmonary hypertension in moderate congenital diaphragmatic hernia Read More »

Anesthesia in Children With Pulmonary Hypertension: Clinically Significant Serious Adverse Events Associated With Cardiac Catheterization and Noncardiac Procedures

Mary Lyn Stein, Steven J. Staffa, Amy O’Brien Charles, Ryan Callahan, James A. DiNardo, Viviane G. Nasr, Morgan L. BrownHarvard Medical School and Boston Children’s Hospital.United States Journal of Cardiothoracic and Vascular AnesthesiaJ Cardiothorac Vasc Anesth 2022; 36: 1606-1616 DOI: 10.1053/j.jvca.2022.01.014 AbstractObjectives: To determine the incidence of clinically significant serious adverse events in a contemporary population of

Anesthesia in Children With Pulmonary Hypertension: Clinically Significant Serious Adverse Events Associated With Cardiac Catheterization and Noncardiac Procedures Read More »

Caveolin-1 associated with severe (pediatric-onset) presentation of pulmonary arterial hypertension

Rachel Farrell, Elizabeth Colglazier, Claire Parker, Leah Stevens, Eric D. Austin, Jeffrey R. FinemanUCSF Benioff Children’s Hospital. Vanderbilt University Medical Center.United States Pulmonary CirculationPulm Circ 2022; 12: DOI: 10.1002/pul2.12100 AbstractThere has been a growing interest in the role that genetic factors influence pediatric pulmonary vascular disease. In fact, data suggests that genetic factors contribute to ~42%

Caveolin-1 associated with severe (pediatric-onset) presentation of pulmonary arterial hypertension Read More »

Health-related quality of life and parental depression in children with pulmonary arterial hypertension

Fatma H. Canbeyli, Vildan Atasayan, Semiha Tokgoz, Fatma S. Tunaoglu, Ayse D. Oguz, Bulent Celik, Serdar KulaKırıkkale University. Umraniye Training and Research Hospital. Gazi University.Turkey Pediatric PulmonologyPediatr Pulmonol 2022; 57: 544-550DOI: 10.1002/ppul.25777 AbstractBackground: Impaired health-related quality of life (HRQoL) is a common problem in pulmonary arterial hypertension (PAH), but there is limited data on HRQoL in children

Health-related quality of life and parental depression in children with pulmonary arterial hypertension Read More »

Scroll to Top