Pediatric Pulmonary Vascular Disease or Adult Pulmonary Vascular Disease

Differential biventricular adaption to pulmonary vascular disease in patients with idiopathic/heritable and congenital heart disease: a prospective cardiac magnetic resonance and invasive study

Zhuoyuan Xu, Ruiyu Dou, Zhen Zhou, Hongsheng Zhang, Chen Zhang, Qiangqiang Li, Lei Xu, Hong GuBeijing Anzhen Hospital and Capital Medical University. China European Heart Journal Cardiovascular ImagingEur Heart J Cardiovasc Imag 2023; DOI: 10.1093/ehjci/jead106 AbstractAims: Despite shared pathophysiological mechanisms, patients with idiopathic/heritable pulmonary arterial hypertension (IPAH/HPAH) have a poorer prognosis than those with PAH after congenital […]

Differential biventricular adaption to pulmonary vascular disease in patients with idiopathic/heritable and congenital heart disease: a prospective cardiac magnetic resonance and invasive study Read More »

Endothelin inhibitors lower pulmonary vascular resistance and improve functional capacity in patients with Fontan circulation

Gabriella Agnoletti, Simona Gala, Francesca Ferroni, Roberto Bordese, Lorenzo Appendini, Carlo Pace Napoleone, Laura BergamascoCitta’ della Salute e della Scienza. Italy Journal of Thoracic and Cardiovascular SurgeryJ Thorac Cardiovasc Surg 2017; 153: 1468-1475DOI: 10.1016/j.jtcvs.2017.01.051 AbstractObjectives: To evaluate the effects of endothelin inhibitors (ERAs) on hemodynamic and functional parameters in patients post-Fontan procedure with high pulmonary vascular resistance

Endothelin inhibitors lower pulmonary vascular resistance and improve functional capacity in patients with Fontan circulation Read More »

WNT7A deficit is associated with dysfunctional angiogenesis in pulmonary arterial hypertension

Ananya Chakraborty, Abinaya Nathan, Mark Orcholski, Stuti Agarwal, Elya A. Shamskhou, Natasha Auer, Ankita Mitra, Eleana Stephanie Guardado, Gowri Swaminathan, David F. Condon, Joyce Yu, Matthew McCarra, Nicholas H. Juul, Alden Mallory, Roberto A. Guzman-Hernandez, Ke Yuan, Vanesa Rojas, Joseph T. Crossno, Lai-Ming Yung, Paul B. Yu, Thomas Spencer, Robert A. Winn, Andrea Frump, Vijaya

WNT7A deficit is associated with dysfunctional angiogenesis in pulmonary arterial hypertension Read More »

Pathological Roles for Endothelial Colony-Forming Cells in Neonatal and Adult Lung Disease

Jason O. Robertson, Serpil C. Erzurum, Kewal AsosinghCleveland ClinicUnited States American Journal of Respiratory Cell and Molecular BiologyAm J Respir Cell Mol Biol 2023; 68: 13-22DOI: 10.1165/rcmb.2022-0318PS AbstractEndothelial colony-forming cells (ECFCs) are vascular resident and circulating endothelial cell subtypes with potent angiogenic capacity, a hierarchy of single-cell clonogenic potentials, and the ability to participate in de novo blood

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Molecular Function and Contribution of TBX4 in Development and Disease

Justyna A. Karolak, Carrie L. Welch, Christian Mosimann, Katarzyna Bzdega, James D. West, David Montani, Melanie Eyries, Mary P. Mullen, Steven H. Abman, Matina Prapa, Stefan Graf, Nicholas W. Morrell, Anna R. Hemnes, Frederic Perros, Rizwan Hamid, Malcolm P. O. Logan, Jeffrey Whitsett, Csaba Galambos, Pawel Stankiewicz, Wendy K. Chung, Eric D. AustinMultiple InstitutionsPoland, France,

Molecular Function and Contribution of TBX4 in Development and Disease Read More »

RASA3 is a candidate gene in sickle cell disease-associated pulmonary hypertension and pulmonary arterial hypertension

Clare C. Prohaska, Xu Zhang, Tae‐Hwi L. Schwantes‐An, Robert S. Stearman, Stanley Hooker, Rick A. Kittles, Micheala A. Aldred, Katie A. Lutz, Michael W. Pauciulo, William C. Nichols, Ankit A. Desai, Victor R. Gordeuk, Roberto F. MachadoIndiana University. University of Illinois at Chicago. City of Hope. Morehouse School of Medicine. Cincinnati Children’s Hospital Medical Center

RASA3 is a candidate gene in sickle cell disease-associated pulmonary hypertension and pulmonary arterial hypertension Read More »

Lymphatic anomalies during lifetime in patients with Noonan syndrome: Retrospective cohort study

Jessie W. Swarts, Lotte E. R. Kleimeier, Erika K. S. M. Leenders, Tuula Rinne, Willemijn M. Klein, Jos M. T. DraaismaAmalia Children’s Hospital, Radboud Institute for Health Sciences and Radboud University Medical CenterNetherlands American Journal of Medical Genetics AAm J Med Genet A 2022; 188: 3242-3261DOI: 10.1002/ajmg.a.62955 AbstractNoonan syndrome (NS) has been associated with an increased

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MR lymphangiography of lymphatic abnormalities in children and adults with Noonan syndrome

C. C. Pieper, J. Wagenpfeil, A. Henkel, S. Geiger, T. Köster, K. Hoss, J. A. Luetkens, C. Hart, U. I. Attenberger, A. MüllerUniversity of Bonn and Children’s Hospital.Germany Scientific ReportsSci Rep 2022; 12: DOI: 10.1038/s41598-022-13806-w AbstractNoonan syndrome is associated with complex lymphatic abnormalities. We report dynamic-contrast enhanced MR lymphangiography (DCMRL) findings in children and adults with

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The spectrum of imaging manifestations of Gorham-Stout disease: a novel dynamic contrast-enhanced MR lymphangiography

Yuna Lee, Seunghyun Lee, Saebeom Hur, Yun Soo Jeong, Dong In Suh, Jangsup Moon, Man Jin Kim, Young Hun Choi, Jung‑Eun CheonSeoul National University Hospital. Seoul National University College of Medicine. Republic of Korea Orphanet Journal of Rare DiseasesOrphanet J Rare Dis 2023; 18: DOI: 10.1186/s13023-023-02704-7 AbstractBackground: To describe the radiological features of Gorham-Stout disease (GSD) as

The spectrum of imaging manifestations of Gorham-Stout disease: a novel dynamic contrast-enhanced MR lymphangiography Read More »

Brain Abscess and Stroke in Children and Adults With Hereditary Hemorrhagic Telangiectasia: Analysis of a Large National Claims Database

Andrew J. White, Itay Marmor, Kate M. Peacock, Katelin B. Nickel, Jessica Zavadil, Margaret A. OlsenSaint Louis University School of Medicine. Washington University School of Medicine.United States NeurologyNeurology 2023; DOI: 10.1212/WNL.0000000000207269 AbstractObjective: Hereditary hemorrhagic telangiectasia (HHT) is an inherited disease associated with pathogenic variants in TGF-β signaling pathway-related genes, resulting in abnormal vascular development in various organs.

Brain Abscess and Stroke in Children and Adults With Hereditary Hemorrhagic Telangiectasia: Analysis of a Large National Claims Database Read More »

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