Pediatric Pulmonary Vascular Disease or Adult Pulmonary Vascular Disease

Multimodality Imaging of Right Heart Function: JACC Scientific Statement

Rebecca T. Hahn, Stamatios Lerakis, Victoria Delgado, Karima Addetia, Daniel Burkhoff, Denisa Muraru, Sean Pinney, Mark K. FriedbergColumbia University Medical Center and NewYork-Presbyterian Hospital. Mount Sinai Icahn School of Medicine. Hospital University Germans Trias i Pujol Hospital. University of Chicago. University of Milano-Bicocca. Hospital for Sick Children.United States, Spain, Italy and Canada Journal of the […]

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Tricuspid Annular Plane Systolic Excursion (TAPSE) correlates with mean pulmonary artery pressure especially 10 years after pediatric heart transplantation

Morgana Michalski, Nikolaus Haas, Robert Dalla Pozza, Sebastian Michel, Marcus Fischer, Anja Lehner, Laura Rosenthal, Andre Jakob, Madaleine Orban, Sarah UlrichLudwig-Maximilians-University of Munich.Germany Clinical TransplantationClinic Transplant 2023; 37: DOI: 10.1111/ctr.14710 AbstractTricuspid annular plane systolic excursion (TAPSE) is important in the noninvasive echocardiographic assessment of right heart function. This retrospective observational study shows correlations of TAPSE with

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Pearls & Oy-sters: Late-Onset Cobalamin C Deficiency Presenting With Subacute Combined Degeneration

Christopher Goyne, Leena KansalUniversity of California San Diego.United States NeurologyNeurology 2023; 100: 486-489DOI: 10.1212/WNL.0000000000201695 AbstractCobalamin C (CblC) deficiency is a rare inborn error in cobalamin (vitamin B12) metabolism which results in impaired intracellular processing of dietary vitamin B12. This leads to a wide range of clinical manifestations including cognitive impairment, psychiatric symptoms, myelopathy, thrombotic events, glomerulonephritis,

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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

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Digenic Inheritance in a Case of Pulmonary Arterial Hypertension Associated with Two Incidental Septal Defects and Multiple Thoracic Collaterals

Alejandro Cruz-Utrilla, María Pilar Escribano Subias, Jair Antonio Tenorio Castaño, María Jesús del Cerro MarínHospital Universitario 12 de Octubre, Madrid.  Instituto de Salud Carlos III.  Hospital Universitario La Paz.  European Reference Network on Rare Congenital Malformations and Rare Intellectual Disability. Ramón y Cajal University Hospital.Spain and Belgium Archivos de BronconeumologiaArch Bronconeumol 2023; 59: 169-170DOI: 10.1016/j.arbres.2022.09.006 AbstractNo

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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

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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

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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,

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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

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