Adult Pulmonary Vascular Disease

Early portopulmonary hypertension predicts mortality in patients with cirrhosis: insights from the PORTO-DETECT cohort

Luis Téllez, Jesús Donate, Antonio Guerrero, Idoia Agulleiro Beraza, Diego San Martín, Edilmar Alvarado, Victor Echevarría, Ángela Puente, Javier Zamora, Susana Del Prado, Andrés Tenes, Guillem Balagué, Barbara Vidal, Cristina Geli, Miguel Ángel Rodríguez-Gandía, Anna Baiges, Anna Brujats, Daniel Lopéz-Herrera Rodríguez, Santiago Fernández-Gordón Sánchez, José Ignacio Fortea, Maria Jesús Del Cerro, Candid Villanueva, Juan Carlos

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Large Patent Ductus Arteriosus in an Adult With Severe Pulmonary Hypertension and Polycythemia: PDA With Severe Pulmonary Hypertension

Tyler L. Langenfeld, Maram Sati, Martin L. Bocks, Arpit K. AgarwalRainbow Babies and Children’s Hospital. United States Journal of the American College of CardiologyJACC Case Rep 2025; 30: DOI: 10.1016/j.jaccas.2025.104677 AbstractBackground: Patent ductus arteriosus (PDA) is one of the most common cardiac abnormalities in children, but in rare cases it can go undiagnosed into adulthood until symptoms

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Cardio-Respiratory Complications in Adult Monozygotic Twins With Myhre Syndrome

Hyoungjun Sim, Forrest Wilke, Emily Hamburger, Charlie J. Sang 3rd, Clara HildebrandtUniversity of North Carolina. United States American Journal of Medical Genetics Part C Seminars in GeneticsAm J Med Genet C Semin Med Genet 2025; DOI: 10.1002/ajmg.c.32150 AbstractMyhre syndrome is a rare connective tissue disorder characterized by skeletal, cardiopulmonary, dermatologic, neurocognitive changes, and a predisposition to

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Echocardiographic correlation of right ventricular-Pulmonary artery coupling to exercise tolerance in congenital heart disease-associated pulmonary hypertension

Anudya Kartika Ratri, Ovin Nada Saputri, David Nugraha, Ricardo Adrian Nugraha, Alisia Yuana Putri, Meity Ardiana, Budi Susetyo Pikir, I. Gde Rurus SuryawanUniversitas Airlangga. Dr. Soetomo General Academic Hospital. Indonesia Annals of Pediatric CardiologyAnn Pediatr Cardiol 2025; 18: 26-32DOI: 10.4103/apc.apc_230_24 AbstractBackground: Pulmonary arterial hypertension (PAH) is a chronic, progressive disease affecting the pulmonary vasculature, with a high

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Shone’s Syndrome and Transplant: A Road Less Traveled

Lyana Labrada, Tripti Gupta, Christiane Haeffele, Adam D. DeVore, Jonathan MenachemTemple University Hospital. University of San Diego. Stanford University. Duke University School of Medicine. Vanderbilt University Medical Center.United States Journal of the American College of Cardiology Case ReportsJACC Case Rep 2025; DOI: 10.1016/j.jaccas.2025.105000 Abstract“Shone’s syndrome” was identified in 1963 as a constellation of 4 coexisting lesions

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Reassessment of the Current Iron Deficiency Definition in Pulmonary Hypertension

Aurelia E. Reiser, Markus Thiersch, Max Gassmann, Martina U. Muckenthaler, Thomas Geiser, Mona Lichtblau, Silvia UlrichUniversity of Zürich. University of Heidelberg. University of Berne.Switzerland and Germany Pulmonary CirculationPulm Circ 2025; 15: DOI: 10.1002/pul2.70142 AbstractIron deficiency (ID) is prevalent in pulmonary hypertension(PH), but there is no consensus on ID definition and its possible correlation to prognostic markers.

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Rare variants in STAB2 in patients with chronic thromboembolic pulmonary hypertension

Mark W. Dodson, Kristina Allen-Brady, Jeffrey Stevens, Meghan M. Cirulis, Mona Alotaibi, Timothy M. Fernandes, Nick H. Kim, Kim M. Kerr, Demosthenes G. Papamatheakis, David S. Poch, Julianna Desmarais, D. Hunter Best, Nathan D. Hatton, John J. Ryan, C. Gregory Elliott, Lisa A. Cannon-AlbrightIntermountain Medical Center. University of Utah and University of Utah School of

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Distinctive hemodynamic phenotype in Fontan-type circulation patients with distal esophageal varices

Luis Téllez, Luis, María Toledano, María Álvarez, Elvira Garrido-Lestache, Elena Garrido, Antonio Guerrero, Jesús Donate, Lorenzo Canova, María Torres, Cristian Perna, María Jesús del Cerro, Agustín AlbillosHospital Universitario Ramón y Cajal, IRYCIS, CIBERehd, Universidad de Alcalá. Spain HepatologyHepatology 2025;  DOI: 10.1097/HEP.0000000000001472 AbstractBackground aims: Esophageal varices (EV) are common in adults with Fontan-type circulation and may arise via

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The Impact of Cardiac Output Methods on the Classification of Pulmonary Hypertension

Léon Genecand, Gaëtan Simian, Mona Lichtblau, Jean‐Marc Fellrath, Julian Klug, Hugues Turbé, Christian Lovis, Stéphane Noble, Julie Wacker, Julian Müller, Roberto Desponds, Maurice Beghetti, Benoit Lechartier, David Montani, Olivier Sitbon, Silvia Ulrich, Frédéric LadorGeneva University Hospitals and University of Geneva. University Hospital of Zurich and University of Zurich. Hopital Pourtales, Reseau Hospitalier Neuchâtelois. Lausanne University

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Dysregulated Tricarboxylic Acid Cycle Metabolism Is Associated With Right Ventricular Maladaptation in Pulmonary Vascular Disease

Darin T. Rosen, Todd M. Kolb, Stephen C. Mathai, Karthik Suresh, Rachel Damico, Steven Hsu, Ryan J. Tedford, Anna R. Hemnes, Jane A. Leopold, Evelyn M. Horn, Erika S. Berman‐Rosenzweig, Franz Rischard, Robert P. Frantz, Serpil C. Erzurum, Gerald J. Beck, Nicholas S. Hill, John Barnard, Samar Farha, Gabriele Grunig, Christine Jellis, Deborah H. Kwon, Reena Mehra, Margaret M. Park, W. H. Wilson Tang, Paul M. Hassoun, Catherine E. Simpson, the PVDOMICS Study GroupJohns Hopkins University. University of Miami, Medical University of South Carolina. Vanderbilt University. Brigham and Women’s Hospital. Cornell University Medical Center. Westchester Medical Center and New

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