Eric Austin

Department of Pulmonary Medicine, Vanderbilt University Medical Center

Affiliated withVanderbilt University Medical Center

Research Area

Biography

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JoVE Journal Publications

ArticleTotal : 1	Year
Publication title	2026

Other Publications

Article	Year
EIF2AK4 mutations in pulmonary capillary hemangiomatosis. Chest\| PubMed ID: 24135949	2014
Abnormal trafficking of endogenously expressed BMPR2 mutant allelic products in patients with heritable pulmonary arterial hypertension. PloS one\| PubMed ID: 24224048	2013
Genetics and mediators in pulmonary arterial hypertension. Clinics in chest medicine\| PubMed ID: 17338927	2007
Penetrance of pulmonary arterial hypertension is modulated by the expression of normal BMPR2 allele. Human mutation\| PubMed ID: 19206171	2009
End tidal CO(2) tension: pulmonary arterial hypertension vs pulmonary venous hypertension and response to treatment. Chest\| PubMed ID: 21622547	2011
Oxidative injury is a common consequence of BMPR2 mutations. Pulmonary circulation\| PubMed ID: 21904662	2011
Idiopathic and heritable PAH perturb common molecular pathways, correlated with increased MSX1 expression. Pulmonary circulation\| PubMed ID: 22140629	2011
Connectivity map analysis of nonsense-mediated decay-positive BMPR2-related hereditary pulmonary arterial hypertension provides insights into disease penetrance. American journal of respiratory cell and molecular biology\| PubMed ID: 22312021	2012
BMPR2 expression is suppressed by signaling through the estrogen receptor. Biology of sex differences\| PubMed ID: 22348410	2012
Whole exome sequencing to identify a novel gene (caveolin-1) associated with human pulmonary arterial hypertension. Circulation. Cardiovascular genetics\| PubMed ID: 22474227	2012
Role of BMPR2 alternative splicing in heritable pulmonary arterial hypertension penetrance. Circulation\| PubMed ID: 22923426	2012
A novel channelopathy in pulmonary arterial hypertension. The New England journal of medicine\| PubMed ID: 23883380	2013
Prostanoids But Not Oral Therapies Improve Right Ventricular Function in Pulmonary Arterial Hypertension. JACC. Heart failure\| PubMed ID: 24015376	2013
Interaction between bone morphogenetic protein receptor type 2 and estrogenic compounds in pulmonary arterial hypertension. Pulmonary circulation\| PubMed ID: 24618541
Shorter survival in familial versus idiopathic pulmonary arterial hypertension is associated with hemodynamic markers of impaired right ventricular function. Pulmonary circulation\| PubMed ID: 24618543	2013
Rescuing the BMPR2 signaling axis in pulmonary arterial hypertension. Drug discovery today\| PubMed ID: 24794464
The genetics of pulmonary arterial hypertension. Circulation research\| PubMed ID: 24951767	2014
Impact of diabetes on survival and right ventricular compensation in pulmonary arterial hypertension. Pulmonary circulation\| PubMed ID: 25006450	2014
Genetics of pulmonary hypertension. Current opinion in cardiology\| PubMed ID: 25159282	2014
Peripheral blood signature of vasodilator-responsive pulmonary arterial hypertension. Circulation\| PubMed ID: 25361553	2015
When to offer genetic testing for pulmonary arterial hypertension. The Canadian journal of cardiology\| PubMed ID: 25840103	2015
Letter by Mosley Regarding Article, "Iron Homeostasis and Pulmonary Hypertension: Iron Deficiency Leads to Pulmonary Vascular Remodeling in the Rat". Circulation research\| PubMed ID: 26316607	2015
Pulmonary Arterial Hypertension: A Current Perspective on Established and Emerging Molecular Genetic Defects. Human mutation\| PubMed ID: 26387786	2015
Estrogen Metabolite 16α-Hydroxyestrone Exacerbates Bone Morphogenetic Protein Receptor Type II-Associated Pulmonary Arterial Hypertension Through MicroRNA-29-Mediated Modulation of Cellular ... Circulation\| PubMed ID: 26487756
Toward Precision Medicine in Pulmonary Arterial Hypertension. American journal of respiratory and critical care medicine\| PubMed ID: 26623685	2015
Recommendations for the Use of Inhaled Nitric Oxide Therapy in Premature Newborns with Severe Pulmonary Hypertension. The Journal of pediatrics\| PubMed ID: 26703869	2016
Fatty Acid Metabolic Defects and Right Ventricular Lipotoxicity in Human Pulmonary Arterial Hypertension. Circulation\| PubMed ID: 27006481
Hepatoma-derived Growth Factor Predicts Disease Severity and Survival in Pulmonary Arterial Hypertension. American journal of respiratory and critical care medicine\| PubMed ID: 27254543	2016
Translational Advances in the Field of Pulmonary Hypertension Molecular Medicine of Pulmonary Arterial Hypertension. From Population Genetics to Precision Medicine and Gene Editing. American journal of respiratory and critical care medicine\| PubMed ID: 27398627
Aberrant Caveolin-1-Mediated Smad Signaling and Proliferation Identified by Analysis of Adenine 474 Deletion Mutation (c.474delA) in Patient Fibroblasts: A New Perspective in the Mechanism o... Molecular biology of the cell\| PubMed ID: 28298490	2017
Disruption of lineage specification in adult pulmonary mesenchymal progenitor cells promotes microvascular dysfunction. The Journal of clinical investigation\| PubMed ID: 28463231
Aberrant caveolin-1-mediated Smad signaling and proliferation identified by analysis of adenine 474 deletion mutation (c.474delA) in patient fibroblasts: a new perspective on the mechanism o... Molecular biology of the cell\| PubMed ID: 28468941	2017
Learning a Comorbidity-Driven Taxonomy of Pediatric Pulmonary Hypertension. Circulation research\| PubMed ID: 28611076	2017
Dysfunctional BMPR2 signaling drives an abnormal endothelial requirement for glutamine in pulmonary arterial hypertension. Pulmonary circulation\| PubMed ID: 28680578
Accelerometry: Improving Objective Assessments of Therapeutic Impact in Pediatric Pulmonary Arterial Hypertension. American journal of respiratory and critical care medicine\| PubMed ID: 28707968	2017
Oestrogen inhibition reverses pulmonary arterial hypertension and associated metabolic defects. The European respiratory journal\| PubMed ID: 28775043
Y Not? Sex Chromosomes May Modify Sexual Dimorphism in Pulmonary Hypertension. American journal of respiratory and critical care medicine\| PubMed ID: 28968140	2018
Diabetes Mellitus Associates with Increased Right Ventricular Afterload and Remodeling in Pulmonary Arterial Hypertension. The American journal of medicine\| PubMed ID: 29421689	2018
Update in Pulmonary Vascular Disease 2016 and 2017. American journal of respiratory and critical care medicine\| PubMed ID: 29533671	2018
The Left Ventricle in Congenital Diaphragmatic Hernia: Implications for the Management of Pulmonary Hypertension. The Journal of pediatrics\| PubMed ID: 29628412	2018
A potential therapeutic role for angiotensin-converting enzyme 2 in human pulmonary arterial hypertension. The European respiratory journal\| PubMed ID: 29903860
Beyond oestrogens: towards a broader evaluation of the hormone profile in pulmonary arterial hypertension. The European respiratory journal\| PubMed ID: 29954927	2018
FHIT, a Novel Modifier Gene in Pulmonary Arterial Hypertension. American journal of respiratory and critical care medicine\| PubMed ID: 30107138	2019
The Y Chromosome Regulates BMPR2 Expression via SRY: A Possible Reason "Why" Fewer Males Develop Pulmonary Arterial Hypertension. American journal of respiratory and critical care medicine\| PubMed ID: 30252494	2018
Low-grade albuminuria in pulmonary arterial hypertension. Pulmonary circulation\| PubMed ID: 30632900	2019
End Stage Takes Center Stage in Pulmonary Arterial Hypertension. American journal of respiratory cell and molecular biology\| PubMed ID: 30726110	2019
Novel early life risk factors for adult pulmonary hypertension. Pulmonary circulation\| PubMed ID: 30880574	2019
United States Pulmonary Hypertension Scientific Registry (USPHSR): rationale, design, and clinical implications. Pulmonary circulation\| PubMed ID: 31099303	2019
Phenotype characterisation of mutation and deletion carriers with neonatal and paediatric pulmonary hypertension. The European respiratory journal\| PubMed ID: 31151956	2019
Racial and Ethnic Differences in Pediatric Pulmonary Hypertension: An Analysis of the Pediatric Pulmonary Hypertension Network Registry. The Journal of pediatrics\| PubMed ID: 31176455	2019
A multifaceted investigation into molecular associations of chronic thromboembolic pulmonary hypertension pathogenesis. JRSM cardiovascular disease\| PubMed ID: 32110389	2020
Sex hormone exposure and reproductive factors in pulmonary arterial hypertension: a case-control study. Pulmonary circulation\| PubMed ID: 32166018	2020
Estrogen metabolites in a small cohort of patients with idiopathic pulmonary arterial hypertension. Pulmonary circulation\| PubMed ID: 32206305	2020
4PBA Restores Signaling of a Cysteine-substituted Mutant BMPR2 Receptor Found in Patients with Pulmonary Arterial Hypertension. American journal of respiratory cell and molecular biology\| PubMed ID: 32255665	2020
TBX4 syndrome: a systemic disease highlighted by pulmonary arterial hypertension in its most severe form. The European respiratory journal\| PubMed ID: 32409426	2020
Elevated Interleukin-6 Levels Predict Clinical Worsening in Pediatric Pulmonary Arterial Hypertension. The Journal of pediatrics\| PubMed ID: 32711743	2020
United States Pulmonary Hypertension Scientific Registry: Baseline Characteristics. Chest\| PubMed ID: 32858008	2021
Pediatric pulmonary hypertension: insulin-like growth factor-binding protein 2 is a novel marker associated with disease severity and survival. Pediatric research\| PubMed ID: 32927467	2020
Expression of a Human Caveolin-1 Mutation in Mice Drives Inflammatory and Metabolic Defect-Associated Pulmonary Arterial Hypertension. Frontiers in medicine\| PubMed ID: 33015095
Insulin-like growth factor binding protein-2: a new circulating indicator of pulmonary arterial hypertension severity and survival. BMC medicine\| PubMed ID: 33019943	2020
TBX4 Transcription Factor Is a Positive Feedback Regulator of Itself and Phospho-SMAD1/5. American journal of respiratory cell and molecular biology\| PubMed ID: 33385213	2021
Sex Hormones across the Menstrual Cycle in Pulmonary Arterial Hypertension: Adding a New Layer of Complexity. Annals of the American Thoracic Society\| PubMed ID: 33522875	2021
ST2 Is a Biomarker of Pediatric Pulmonary Arterial Hypertension Severity and Clinical Worsening. Chest\| PubMed ID: 33609516	2021
Characteristics of infants or children presenting to outpatient bronchopulmonary dysplasia clinics in the United States. Pediatric pulmonology\| PubMed ID: 33713587	2021
KCNK3 Mutation Causes Altered Immune Function in Pulmonary Arterial Hypertension Patients and Mouse Models. International journal of molecular sciences\| PubMed ID: 34065088	2021
Characterisation of Pediatric Pulmonary Hypertensive Vascular Disease from the PPHNet Registry. The European respiratory journal\| PubMed ID: 34140292	2021
Pulmonary hypertension in the child with bronchopulmonary dysplasia. Pediatric pulmonology\| PubMed ID: 34324276	2021
Angiostatic Peptide, Endostatin, Predicts Severity in Pediatric Congenital Heart Disease-Associated Pulmonary Hypertension. Journal of the American Heart Association\| PubMed ID: 34622662	2021
Biomarkers of Pulmonary Hypertension Are Altered in Children with Down Syndrome and Pulmonary Hypertension. The Journal of pediatrics\| PubMed ID: 34687693	2022
Cardiac Catheterization and Hemodynamics in a Multicenter Cohort of Children with Pulmonary Hypertension. Annals of the American Thoracic Society\| PubMed ID: 35049414	2022
Upfront Combination Therapy: Growing the Case to Get Ahead of Pediatric Pulmonary Arterial Hypertension. Annals of the American Thoracic Society\| PubMed ID: 35103566	2022
Insurance coverage and respiratory morbidities in bronchopulmonary dysplasia. Pediatric pulmonology\| PubMed ID: 35437911	2022
Is It Still "Idiopathic"? Features of Autoimmunity in Idiopathic Pulmonary Arterial Hypertension. American journal of respiratory and critical care medicine\| PubMed ID: 35442875	2022
Hepatoma-derived growth factor is associated with pulmonary vascular remodeling and PAH disease severity and survival. Pulmonary circulation\| PubMed ID: 35506100	2022
Leveraging Therapy-Specific Polygenic Risk Scores to Predict Restrictive Lung Defects in Childhood Cancer Survivors. Cancer research\| PubMed ID: 35713625	2022
Daycare Attendance is Linked to Increased Risk of Respiratory Morbidities in Children Born Preterm with Bronchopulmonary Dysplasia. The Journal of pediatrics\| PubMed ID: 35803300	2022
Caveolin-1 associated with severe (pediatric-onset) presentation of pulmonary arterial hypertension. Pulmonary circulation\| PubMed ID: 35864912	2022
Pulmonary Hypertension in Children with Down Syndrome: Results from the Pediatric Pulmonary Hypertension Network Registry. The Journal of pediatrics\| PubMed ID: 36027975	2023
Molecular Function and Contribution of in Development and Disease. American journal of respiratory and critical care medicine\| PubMed ID: 36367783	2023
Transpulmonary generation of cell-free hemoglobin contributes to vascular dysfunction in pulmonary arterial hypertension via dysregulated clearance mechanisms. Pulmonary circulation\| PubMed ID: 36743426	2023
Validation of an outpatient questionnaire for bronchopulmonary dysplasia control. Pediatric pulmonology\| PubMed ID: 36793145	2023
Respiratory Outcomes for Ventilator-Dependent Children With Bronchopulmonary Dysplasia. Pediatrics\| PubMed ID: 37122061	2023
Insulin-like growth factor binding Protein-4: A novel indicator of pulmonary arterial hypertension severity and survival. Pulmonary circulation\| PubMed ID: 37152104	2023
Developing a Broader Understanding of Pediatric PAH Worldwide. Chest\| PubMed ID: 37295875	2023
Measurement of Physical Activity by Actigraphy in Infants and Young Children with Pulmonary Arterial Hypertension. The Journal of pediatrics\| PubMed ID: 37517653	2023
Recent Advances in Pediatric Pulmonary Hypertension: Implications for Diagnosis and Treatment. Clinical therapeutics\| PubMed ID: 37517916	2023
Low-affinity insulin-like growth factor binding protein 7 and its association with pulmonary arterial hypertension severity and survival. Pulmonary circulation\| PubMed ID: 37674873	2023
Exploring the Association of Male Sex With Adverse Outcomes in Severe Bronchopulmonary Dysplasia: A Retrospective, Multicenter Cohort Study. Chest\| PubMed ID: 37879559	2024
Kids Mod PAH trial: A multicenter trial comparing mono- versus duo-therapy for initial treatment of pediatric pulmonary hypertension. Pulmonary circulation\| PubMed ID: 37915400	2023
Number of children in the household influences respiratory morbidities in children with bronchopulmonary dysplasia in the outpatient setting. Pediatric pulmonology\| PubMed ID: 37937888	2024
Genetic Testing in Pulmonary Arterial Hypertension Evaluation: A Patient and Clinician Survey. Chest\| PubMed ID: 38008306	2024
Development of a Peripherally Restricted 5-HT Partial Agonist for Treatment of Pulmonary Arterial Hypertension. JACC. Basic to translational science\| PubMed ID: 38094686	2023
Actigraphy methodology in the Kids Mod PAH trial: Physical activity as a functional endpoint in pediatric clinical trials. Pulmonary circulation\| PubMed ID: 38464344	2024
Quantifying the impact of post-acute sequelae of coronavirus on the cardiopulmonary endurance of athletes. Pulmonary circulation\| PubMed ID: 39022309	2024
Pulmonary Hypertension in Children. Clinics in chest medicine\| PubMed ID: 39069331	2024
Factors associated with liberation from home mechanical ventilation and tracheostomy decannulation in infants and children with severe bronchopulmonary dysplasia. Journal of perinatology : official journal of the California Perinatal Association\| PubMed ID: 39085436	2026
An interdisciplinary consensus approach to pulmonary hypertension in developmental lung disease. The European respiratory journal\| PubMed ID: 39147412	2024
Parental Report of Indoor Air Pollution Is Associated with Respiratory Morbidities in Bronchopulmonary Dysplasia. The Journal of pediatrics\| PubMed ID: 39151604	2024
Equivalency of Multiple Biomarkers to Clinical Pulmonary Arterial Hypertension Survival Risk Models. Chest\| PubMed ID: 39154795	2024
Outcomes of infants and children with bronchopulmonary dysplasia-associated pulmonary hypertension who required home ventilation. Pediatric research\| PubMed ID: 39181986	2025
Genetics and precision genomics approaches to pulmonary hypertension. The European respiratory journal\| PubMed ID: 39209481	2024
A multimodal approach identifies lactate as a central feature of right ventricular failure that is detectable in human plasma. Frontiers in medicine\| PubMed ID: 39346939	2024
Outpatient clinical care for bronchopulmonary dysplasia: A survey of the BPD collaborative. Pediatric pulmonology\| PubMed ID: 39392254	2025
SOX17-Associated Pulmonary Hypertension in Children: A Distinct Developmental and Clinical Syndrome. The Journal of pediatrics\| PubMed ID: 39603521	2025
Estrogen and Cyp1b1 Regulate Pparγ in Pulmonary Hypertension Through a Ubiquitin-Dependent Mechanism. Pulmonary circulation\| PubMed ID: 39958970	2025
RNA-Seq and ChIP-Seq Identification of Unique and Overlapping Target Genes and Pathways Regulated by TBX4 in Human Pulmonary Fibroblasts and Pericytes. Pulmonary circulation\| PubMed ID: 39980707	2025
X-linked filaminopathy: another pulmonary hypertension syndrome. The European respiratory journal\| PubMed ID: 40049735	2025
Cellular Communication Network Protein 2 in the Right Ventricle of Pulmonary Arterial Hypertension. Pulmonary circulation\| PubMed ID: 40177499	2025
Outcomes of Ventilator-Dependent Children With Severe Bronchopulmonary Dysplasia and Tracheobronchomalacia. Pediatric pulmonology\| PubMed ID: 40243389	2025
Procedural Variation May Contribute to 6-Minute Walk Distance Variability in Real-World Pediatric Pulmonary Arterial Hypertension Study. The AAPS journal\| PubMed ID: 40579609	2025
Measuring Activity in Pediatric Pulmonary Hypertension: Expanding Opportunities to Advance Clinical Trial Design and Impact. Chest\| PubMed ID: 40639886	2025
Allergy and atopic phenotype are associated with earlier gestation and severity of respiratory symptoms in bronchopulmonary dysplasia. Pediatric research\| PubMed ID: 40646282	2026
Enhancing Drug Development for Paediatric Pulmonary Hypertension-An Integrative Perspective. Pulmonary circulation\| PubMed ID: 40672980	2025
Circular RNA Profiling Identifies as a -Derived Regulator of Endothelial Proliferation and Stress Responses. Arteriosclerosis, thrombosis, and vascular biology\| PubMed ID: 40772817	2025
International Society for Heart and Lung Transplantation (ISHLT) Consensus Statement on Risk Stratification in Pulmonary Arterial Hypertension. The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation\| PubMed ID: 40910964	2025
Authors' Perspective on the International Society for Heart and Lung Transplantation Consensus Statement on Risk Stratification in Pulmonary Arterial Hypertension. The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation\| PubMed ID: 41136169	2025
Pulmonary Hemodynamics and Long-Term Outcomes in Children with Pulmonary Hypertension-Associated Bronchopulmonary Dysplasia. The Journal of pediatrics\| PubMed ID: 41139016	2026

Article	Year
EIF2AK4 mutations in pulmonary capillary hemangiomatosis. Chest\| PubMed ID: 24135949	2014
Abnormal trafficking of endogenously expressed BMPR2 mutant allelic products in patients with heritable pulmonary arterial hypertension. PloS one\| PubMed ID: 24224048	2013
Genetics and mediators in pulmonary arterial hypertension. Clinics in chest medicine\| PubMed ID: 17338927	2007
Penetrance of pulmonary arterial hypertension is modulated by the expression of normal BMPR2 allele. Human mutation\| PubMed ID: 19206171	2009
End tidal CO(2) tension: pulmonary arterial hypertension vs pulmonary venous hypertension and response to treatment. Chest\| PubMed ID: 21622547	2011
Oxidative injury is a common consequence of BMPR2 mutations. Pulmonary circulation\| PubMed ID: 21904662	2011
Idiopathic and heritable PAH perturb common molecular pathways, correlated with increased MSX1 expression. Pulmonary circulation\| PubMed ID: 22140629	2011
Connectivity map analysis of nonsense-mediated decay-positive BMPR2-related hereditary pulmonary arterial hypertension provides insights into disease penetrance. American journal of respiratory cell and molecular biology\| PubMed ID: 22312021	2012
BMPR2 expression is suppressed by signaling through the estrogen receptor. Biology of sex differences\| PubMed ID: 22348410	2012
Whole exome sequencing to identify a novel gene (caveolin-1) associated with human pulmonary arterial hypertension. Circulation. Cardiovascular genetics\| PubMed ID: 22474227	2012
Role of BMPR2 alternative splicing in heritable pulmonary arterial hypertension penetrance. Circulation\| PubMed ID: 22923426	2012
A novel channelopathy in pulmonary arterial hypertension. The New England journal of medicine\| PubMed ID: 23883380	2013
Prostanoids But Not Oral Therapies Improve Right Ventricular Function in Pulmonary Arterial Hypertension. JACC. Heart failure\| PubMed ID: 24015376	2013
Interaction between bone morphogenetic protein receptor type 2 and estrogenic compounds in pulmonary arterial hypertension. Pulmonary circulation\| PubMed ID: 24618541
Shorter survival in familial versus idiopathic pulmonary arterial hypertension is associated with hemodynamic markers of impaired right ventricular function. Pulmonary circulation\| PubMed ID: 24618543	2013
Rescuing the BMPR2 signaling axis in pulmonary arterial hypertension. Drug discovery today\| PubMed ID: 24794464
The genetics of pulmonary arterial hypertension. Circulation research\| PubMed ID: 24951767	2014
Impact of diabetes on survival and right ventricular compensation in pulmonary arterial hypertension. Pulmonary circulation\| PubMed ID: 25006450	2014
Genetics of pulmonary hypertension. Current opinion in cardiology\| PubMed ID: 25159282	2014
Peripheral blood signature of vasodilator-responsive pulmonary arterial hypertension. Circulation\| PubMed ID: 25361553	2015
When to offer genetic testing for pulmonary arterial hypertension. The Canadian journal of cardiology\| PubMed ID: 25840103	2015
Letter by Mosley Regarding Article, "Iron Homeostasis and Pulmonary Hypertension: Iron Deficiency Leads to Pulmonary Vascular Remodeling in the Rat". Circulation research\| PubMed ID: 26316607	2015
Pulmonary Arterial Hypertension: A Current Perspective on Established and Emerging Molecular Genetic Defects. Human mutation\| PubMed ID: 26387786	2015
Estrogen Metabolite 16α-Hydroxyestrone Exacerbates Bone Morphogenetic Protein Receptor Type II-Associated Pulmonary Arterial Hypertension Through MicroRNA-29-Mediated Modulation of Cellular ... Circulation\| PubMed ID: 26487756
Toward Precision Medicine in Pulmonary Arterial Hypertension. American journal of respiratory and critical care medicine\| PubMed ID: 26623685	2015
Recommendations for the Use of Inhaled Nitric Oxide Therapy in Premature Newborns with Severe Pulmonary Hypertension. The Journal of pediatrics\| PubMed ID: 26703869	2016
Fatty Acid Metabolic Defects and Right Ventricular Lipotoxicity in Human Pulmonary Arterial Hypertension. Circulation\| PubMed ID: 27006481
Hepatoma-derived Growth Factor Predicts Disease Severity and Survival in Pulmonary Arterial Hypertension. American journal of respiratory and critical care medicine\| PubMed ID: 27254543	2016
Translational Advances in the Field of Pulmonary Hypertension Molecular Medicine of Pulmonary Arterial Hypertension. From Population Genetics to Precision Medicine and Gene Editing. American journal of respiratory and critical care medicine\| PubMed ID: 27398627
Aberrant Caveolin-1-Mediated Smad Signaling and Proliferation Identified by Analysis of Adenine 474 Deletion Mutation (c.474delA) in Patient Fibroblasts: A New Perspective in the Mechanism o... Molecular biology of the cell\| PubMed ID: 28298490	2017
Disruption of lineage specification in adult pulmonary mesenchymal progenitor cells promotes microvascular dysfunction. The Journal of clinical investigation\| PubMed ID: 28463231
Aberrant caveolin-1-mediated Smad signaling and proliferation identified by analysis of adenine 474 deletion mutation (c.474delA) in patient fibroblasts: a new perspective on the mechanism o... Molecular biology of the cell\| PubMed ID: 28468941	2017
Learning a Comorbidity-Driven Taxonomy of Pediatric Pulmonary Hypertension. Circulation research\| PubMed ID: 28611076	2017
Dysfunctional BMPR2 signaling drives an abnormal endothelial requirement for glutamine in pulmonary arterial hypertension. Pulmonary circulation\| PubMed ID: 28680578
Accelerometry: Improving Objective Assessments of Therapeutic Impact in Pediatric Pulmonary Arterial Hypertension. American journal of respiratory and critical care medicine\| PubMed ID: 28707968	2017
Oestrogen inhibition reverses pulmonary arterial hypertension and associated metabolic defects. The European respiratory journal\| PubMed ID: 28775043
Y Not? Sex Chromosomes May Modify Sexual Dimorphism in Pulmonary Hypertension. American journal of respiratory and critical care medicine\| PubMed ID: 28968140	2018
Diabetes Mellitus Associates with Increased Right Ventricular Afterload and Remodeling in Pulmonary Arterial Hypertension. The American journal of medicine\| PubMed ID: 29421689	2018
Update in Pulmonary Vascular Disease 2016 and 2017. American journal of respiratory and critical care medicine\| PubMed ID: 29533671	2018
The Left Ventricle in Congenital Diaphragmatic Hernia: Implications for the Management of Pulmonary Hypertension. The Journal of pediatrics\| PubMed ID: 29628412	2018
A potential therapeutic role for angiotensin-converting enzyme 2 in human pulmonary arterial hypertension. The European respiratory journal\| PubMed ID: 29903860
Beyond oestrogens: towards a broader evaluation of the hormone profile in pulmonary arterial hypertension. The European respiratory journal\| PubMed ID: 29954927	2018
FHIT, a Novel Modifier Gene in Pulmonary Arterial Hypertension. American journal of respiratory and critical care medicine\| PubMed ID: 30107138	2019
The Y Chromosome Regulates BMPR2 Expression via SRY: A Possible Reason "Why" Fewer Males Develop Pulmonary Arterial Hypertension. American journal of respiratory and critical care medicine\| PubMed ID: 30252494	2018
Low-grade albuminuria in pulmonary arterial hypertension. Pulmonary circulation\| PubMed ID: 30632900	2019
End Stage Takes Center Stage in Pulmonary Arterial Hypertension. American journal of respiratory cell and molecular biology\| PubMed ID: 30726110	2019
Novel early life risk factors for adult pulmonary hypertension. Pulmonary circulation\| PubMed ID: 30880574	2019
United States Pulmonary Hypertension Scientific Registry (USPHSR): rationale, design, and clinical implications. Pulmonary circulation\| PubMed ID: 31099303	2019
Phenotype characterisation of mutation and deletion carriers with neonatal and paediatric pulmonary hypertension. The European respiratory journal\| PubMed ID: 31151956	2019
Racial and Ethnic Differences in Pediatric Pulmonary Hypertension: An Analysis of the Pediatric Pulmonary Hypertension Network Registry. The Journal of pediatrics\| PubMed ID: 31176455	2019
A multifaceted investigation into molecular associations of chronic thromboembolic pulmonary hypertension pathogenesis. JRSM cardiovascular disease\| PubMed ID: 32110389	2020
Sex hormone exposure and reproductive factors in pulmonary arterial hypertension: a case-control study. Pulmonary circulation\| PubMed ID: 32166018	2020
Estrogen metabolites in a small cohort of patients with idiopathic pulmonary arterial hypertension. Pulmonary circulation\| PubMed ID: 32206305	2020
4PBA Restores Signaling of a Cysteine-substituted Mutant BMPR2 Receptor Found in Patients with Pulmonary Arterial Hypertension. American journal of respiratory cell and molecular biology\| PubMed ID: 32255665	2020
TBX4 syndrome: a systemic disease highlighted by pulmonary arterial hypertension in its most severe form. The European respiratory journal\| PubMed ID: 32409426	2020
Elevated Interleukin-6 Levels Predict Clinical Worsening in Pediatric Pulmonary Arterial Hypertension. The Journal of pediatrics\| PubMed ID: 32711743	2020
United States Pulmonary Hypertension Scientific Registry: Baseline Characteristics. Chest\| PubMed ID: 32858008	2021
Pediatric pulmonary hypertension: insulin-like growth factor-binding protein 2 is a novel marker associated with disease severity and survival. Pediatric research\| PubMed ID: 32927467	2020
Expression of a Human Caveolin-1 Mutation in Mice Drives Inflammatory and Metabolic Defect-Associated Pulmonary Arterial Hypertension. Frontiers in medicine\| PubMed ID: 33015095
Insulin-like growth factor binding protein-2: a new circulating indicator of pulmonary arterial hypertension severity and survival. BMC medicine\| PubMed ID: 33019943	2020
TBX4 Transcription Factor Is a Positive Feedback Regulator of Itself and Phospho-SMAD1/5. American journal of respiratory cell and molecular biology\| PubMed ID: 33385213	2021
Sex Hormones across the Menstrual Cycle in Pulmonary Arterial Hypertension: Adding a New Layer of Complexity. Annals of the American Thoracic Society\| PubMed ID: 33522875	2021
ST2 Is a Biomarker of Pediatric Pulmonary Arterial Hypertension Severity and Clinical Worsening. Chest\| PubMed ID: 33609516	2021
Characteristics of infants or children presenting to outpatient bronchopulmonary dysplasia clinics in the United States. Pediatric pulmonology\| PubMed ID: 33713587	2021
KCNK3 Mutation Causes Altered Immune Function in Pulmonary Arterial Hypertension Patients and Mouse Models. International journal of molecular sciences\| PubMed ID: 34065088	2021
Characterisation of Pediatric Pulmonary Hypertensive Vascular Disease from the PPHNet Registry. The European respiratory journal\| PubMed ID: 34140292	2021
Pulmonary hypertension in the child with bronchopulmonary dysplasia. Pediatric pulmonology\| PubMed ID: 34324276	2021
Angiostatic Peptide, Endostatin, Predicts Severity in Pediatric Congenital Heart Disease-Associated Pulmonary Hypertension. Journal of the American Heart Association\| PubMed ID: 34622662	2021
Biomarkers of Pulmonary Hypertension Are Altered in Children with Down Syndrome and Pulmonary Hypertension. The Journal of pediatrics\| PubMed ID: 34687693	2022
Cardiac Catheterization and Hemodynamics in a Multicenter Cohort of Children with Pulmonary Hypertension. Annals of the American Thoracic Society\| PubMed ID: 35049414	2022
Upfront Combination Therapy: Growing the Case to Get Ahead of Pediatric Pulmonary Arterial Hypertension. Annals of the American Thoracic Society\| PubMed ID: 35103566	2022
Insurance coverage and respiratory morbidities in bronchopulmonary dysplasia. Pediatric pulmonology\| PubMed ID: 35437911	2022
Is It Still "Idiopathic"? Features of Autoimmunity in Idiopathic Pulmonary Arterial Hypertension. American journal of respiratory and critical care medicine\| PubMed ID: 35442875	2022
Hepatoma-derived growth factor is associated with pulmonary vascular remodeling and PAH disease severity and survival. Pulmonary circulation\| PubMed ID: 35506100	2022
Leveraging Therapy-Specific Polygenic Risk Scores to Predict Restrictive Lung Defects in Childhood Cancer Survivors. Cancer research\| PubMed ID: 35713625	2022
Daycare Attendance is Linked to Increased Risk of Respiratory Morbidities in Children Born Preterm with Bronchopulmonary Dysplasia. The Journal of pediatrics\| PubMed ID: 35803300	2022
Caveolin-1 associated with severe (pediatric-onset) presentation of pulmonary arterial hypertension. Pulmonary circulation\| PubMed ID: 35864912	2022
Pulmonary Hypertension in Children with Down Syndrome: Results from the Pediatric Pulmonary Hypertension Network Registry. The Journal of pediatrics\| PubMed ID: 36027975	2023
Molecular Function and Contribution of in Development and Disease. American journal of respiratory and critical care medicine\| PubMed ID: 36367783	2023
Transpulmonary generation of cell-free hemoglobin contributes to vascular dysfunction in pulmonary arterial hypertension via dysregulated clearance mechanisms. Pulmonary circulation\| PubMed ID: 36743426	2023
Validation of an outpatient questionnaire for bronchopulmonary dysplasia control. Pediatric pulmonology\| PubMed ID: 36793145	2023
Respiratory Outcomes for Ventilator-Dependent Children With Bronchopulmonary Dysplasia. Pediatrics\| PubMed ID: 37122061	2023
Insulin-like growth factor binding Protein-4: A novel indicator of pulmonary arterial hypertension severity and survival. Pulmonary circulation\| PubMed ID: 37152104	2023
Developing a Broader Understanding of Pediatric PAH Worldwide. Chest\| PubMed ID: 37295875	2023
Measurement of Physical Activity by Actigraphy in Infants and Young Children with Pulmonary Arterial Hypertension. The Journal of pediatrics\| PubMed ID: 37517653	2023
Recent Advances in Pediatric Pulmonary Hypertension: Implications for Diagnosis and Treatment. Clinical therapeutics\| PubMed ID: 37517916	2023
Low-affinity insulin-like growth factor binding protein 7 and its association with pulmonary arterial hypertension severity and survival. Pulmonary circulation\| PubMed ID: 37674873	2023
Exploring the Association of Male Sex With Adverse Outcomes in Severe Bronchopulmonary Dysplasia: A Retrospective, Multicenter Cohort Study. Chest\| PubMed ID: 37879559	2024
Kids Mod PAH trial: A multicenter trial comparing mono- versus duo-therapy for initial treatment of pediatric pulmonary hypertension. Pulmonary circulation\| PubMed ID: 37915400	2023
Number of children in the household influences respiratory morbidities in children with bronchopulmonary dysplasia in the outpatient setting. Pediatric pulmonology\| PubMed ID: 37937888	2024
Genetic Testing in Pulmonary Arterial Hypertension Evaluation: A Patient and Clinician Survey. Chest\| PubMed ID: 38008306	2024
Development of a Peripherally Restricted 5-HT Partial Agonist for Treatment of Pulmonary Arterial Hypertension. JACC. Basic to translational science\| PubMed ID: 38094686	2023
Actigraphy methodology in the Kids Mod PAH trial: Physical activity as a functional endpoint in pediatric clinical trials. Pulmonary circulation\| PubMed ID: 38464344	2024
Quantifying the impact of post-acute sequelae of coronavirus on the cardiopulmonary endurance of athletes. Pulmonary circulation\| PubMed ID: 39022309	2024
Pulmonary Hypertension in Children. Clinics in chest medicine\| PubMed ID: 39069331	2024
Factors associated with liberation from home mechanical ventilation and tracheostomy decannulation in infants and children with severe bronchopulmonary dysplasia. Journal of perinatology : official journal of the California Perinatal Association\| PubMed ID: 39085436	2026
An interdisciplinary consensus approach to pulmonary hypertension in developmental lung disease. The European respiratory journal\| PubMed ID: 39147412	2024
Parental Report of Indoor Air Pollution Is Associated with Respiratory Morbidities in Bronchopulmonary Dysplasia. The Journal of pediatrics\| PubMed ID: 39151604	2024
Equivalency of Multiple Biomarkers to Clinical Pulmonary Arterial Hypertension Survival Risk Models. Chest\| PubMed ID: 39154795	2024
Outcomes of infants and children with bronchopulmonary dysplasia-associated pulmonary hypertension who required home ventilation. Pediatric research\| PubMed ID: 39181986	2025
Genetics and precision genomics approaches to pulmonary hypertension. The European respiratory journal\| PubMed ID: 39209481	2024
A multimodal approach identifies lactate as a central feature of right ventricular failure that is detectable in human plasma. Frontiers in medicine\| PubMed ID: 39346939	2024
Outpatient clinical care for bronchopulmonary dysplasia: A survey of the BPD collaborative. Pediatric pulmonology\| PubMed ID: 39392254	2025
SOX17-Associated Pulmonary Hypertension in Children: A Distinct Developmental and Clinical Syndrome. The Journal of pediatrics\| PubMed ID: 39603521	2025
Estrogen and Cyp1b1 Regulate Pparγ in Pulmonary Hypertension Through a Ubiquitin-Dependent Mechanism. Pulmonary circulation\| PubMed ID: 39958970	2025
RNA-Seq and ChIP-Seq Identification of Unique and Overlapping Target Genes and Pathways Regulated by TBX4 in Human Pulmonary Fibroblasts and Pericytes. Pulmonary circulation\| PubMed ID: 39980707	2025
X-linked filaminopathy: another pulmonary hypertension syndrome. The European respiratory journal\| PubMed ID: 40049735	2025
Cellular Communication Network Protein 2 in the Right Ventricle of Pulmonary Arterial Hypertension. Pulmonary circulation\| PubMed ID: 40177499	2025
Outcomes of Ventilator-Dependent Children With Severe Bronchopulmonary Dysplasia and Tracheobronchomalacia. Pediatric pulmonology\| PubMed ID: 40243389	2025
Procedural Variation May Contribute to 6-Minute Walk Distance Variability in Real-World Pediatric Pulmonary Arterial Hypertension Study. The AAPS journal\| PubMed ID: 40579609	2025
Measuring Activity in Pediatric Pulmonary Hypertension: Expanding Opportunities to Advance Clinical Trial Design and Impact. Chest\| PubMed ID: 40639886	2025
Allergy and atopic phenotype are associated with earlier gestation and severity of respiratory symptoms in bronchopulmonary dysplasia. Pediatric research\| PubMed ID: 40646282	2026
Enhancing Drug Development for Paediatric Pulmonary Hypertension-An Integrative Perspective. Pulmonary circulation\| PubMed ID: 40672980	2025
Circular RNA Profiling Identifies as a -Derived Regulator of Endothelial Proliferation and Stress Responses. Arteriosclerosis, thrombosis, and vascular biology\| PubMed ID: 40772817	2025
International Society for Heart and Lung Transplantation (ISHLT) Consensus Statement on Risk Stratification in Pulmonary Arterial Hypertension. The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation\| PubMed ID: 40910964	2025
Authors' Perspective on the International Society for Heart and Lung Transplantation Consensus Statement on Risk Stratification in Pulmonary Arterial Hypertension. The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation\| PubMed ID: 41136169	2025
Pulmonary Hemodynamics and Long-Term Outcomes in Children with Pulmonary Hypertension-Associated Bronchopulmonary Dysplasia. The Journal of pediatrics\| PubMed ID: 41139016	2026