Class 3. Pulmonary Hypertension Associated with Developmental Diseases of the Lung

High-level gonosomal mosaicism for a pathogenic non-coding CNV deletion of the lung-specific FOXF1 enhancer in an unaffected mother of an infant with ACDMPV

Esra Yıldız Bölükbaşi, Tomasz Gambin, Justyna A. Karolak, Nicholas Willard, Przemyslaw Szafranski, Steven H. Abman, Csaba Galambos, John P. Kinsella, Paweł StankiewiczBaylor College of Medicine. Poznan University of Medical Sciences. Warsaw University of Technology. University of Colorado Anschutz Medical Campus.United States and Poland Molecular Genetics and Genomic MedicineMol Genet Genomic Med 2022; 10: DOI: 10.1002/mgg3.2062 AbstractBackground: Alveolar capillary dysplasia with misalignment of pulmonary veins (ACDMPV) results […]

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Congenital Surfactant C Deficiency with Pulmonary Hypertension – A Case Report

Wei Chard Chua, I-Chen Chen, Yi-Ching Liu, Yen-Hsien Wu, Shih-Hsing Lo, Jong-Hau Hsu, Peir-In Liang, Hsiu-Lin Chen, Zen-Kong DaiKaohsiung Medical University Hospital. Taiwan ChildrenChildren 2022; 9: DOI: 10.3390/children9101435 AbstractInterstitial lung diseases in children are a diverse group in terms of etiology and pathogenesis. With advances in genetic testing, mutations in surfactant protein have now been identified

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A Morphomolecular Approach to Alveolar Capillary Dysplasia

Jan C. Kamp, Lavinia Neubert, Maxmilian Ackermann, Helge Stark, Edith Plucinski, Harshit R. Shah, Sabina Janciauskiene, Anke K. Bergmann, Gunnar Schmidt, Tobias Welte, Axel Haverich, Christopher Werlein, Peter Braubach, Florian Laenger, Nicolaus Schwerk, Karen M. Olsson, Jan Fuge, Da-Hee Park, Jonas C. Schupp, Marius M. Hoeper, Mark P. Kuehnel, Danny D. JonigkHannover Medical School. University

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Patent ductus arteriosus and the risk of bronchopulmonary dysplasia-associated pulmonary hypertension

Hythem Nawaytou, Nancy K. Hills, Ronald I. ClymanUniversity of California San Francisco.United States Pediatric ResearchPediatr Res 2023; DOI: 10.1038/s41390-023-02522-4 AbstractBackground: The aim of the study was to determine whether prolonged exposure to a moderate/large patent ductus arteriosus left-to-right shunt (PDA) increases the risk of late (beyond 36 weeks) pulmonary hypertension (BPD-PH) and pulmonary vascular disease (BPD-PVD) during

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High-dose epoprostenol therapy in pediatric patients with pulmonary hypertension and developmental lung disease: A report of two cases

Yoshie Fukusawa, Hidenori Yamamoto, Miharu Ito, Akiko Saito, Kiyotaka Go, Yoshihito Morimoto, Kazushi Yasuda, Yoshiaki Sato, Masahiro Hayakawa, Taichi KatoNagoya University and Nagoya University Hospital. Ogaki Municipal Hospital. Japanese Red Cross Nagoya Daichi Hospital. Aichi Child Health and Medical General Center.Japan Frontiers in Pediatrics. Pediatric Cardiology SectionFront Pediatr 2023; 11: DOI: 10.3389/fped.2023.1116434 AbstractPulmonary hypertension (PH) with

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Multifactorial pulmonary hypertension in infantile scimitar syndrome

Damien Bonnet, Isabelle Szezepanski, Christophe Delacourt, Sophie Malkezadeh-Milani, Maryline LévyHôpital Necker-Enfants Malades, AP-HP, Université de ParisFrance Archives of Cardiovascular DiseaseArch Cardiovasc Dis 2022; 115: 142-150DOI: 10.1016/j.acvd.2022.01.004 AbstractBackground: Pulmonary hypertension in infantile scimitar syndrome is highly prevalent at diagnosis, and has a multifactorial origin.Aims: To analyse the constellation of anatomical anomalies and initial physiology, and their contribution to

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Synchrotron-based phase-contrast micro-CT as a tool for understanding pulmonary vascular pathobiology and the 3-D microanatomy of alveolar capillary dysplasia

Christian Norvik, Christian Karl Westöö, Niccolò Peruzzi, Goran Lovric, Oscar van der Have, Rajmund Mokso, Ida Jeremiasen, Hans Brunnström, Csaba Galambos, Martin Bech, Karin Tran-LundmarkLund University. École Polytechnique Fédérale de Lausanne. Swiss Light Source. University of Colorado.Sweden, Switzerland and United States American Journal of Physiology Lung cellular and Molecular PhysiologyAm J Physiol Lung Cell Mol

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A long-term survivor with alveolar capillary dysplasia

Chandler E. Yost, Angelica R. Putnam, Megan K. Dishop, Lynda O. Jorgenson, Paul E. Wirkus, Ronald W. DayUniversity of Utah, Primary Children’s Hospital, Phoenix Children’s Hospital and Wasatch PediatricsUnited States Journal of the American College of Cardiology Case ReportsJACC Case Rep 2020; 2: 1492-1495DOI: 10.1016/j.jaccas.2020.05.055 AbstractA patient with alveolar capillary dysplasia has survived more than 56

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