Diagnostic Testing for Pulmonary Vascular Disease. Non-invasive Testing

Brain and lung arteriovenous malformation rescreening practices for children and adults with hereditary hemorrhagic telangiectasia

Lauren A. Beslow, Helen Kim, Steven W. Hetts, Felix Ratjen, Marianne S. Clancy, James R. Gossage, Marie E. FaughnanChildren’s Hospital of Philadelphia and Perelman School of Medicine at the University of Pennsylvania. University of California San Francisco. Hospital for Sick Children and University of Toronto. Cure HHT Foundation. Augusta University. St. Michael’s Hospital.United States and […]

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Glenn circulation causes early and progressive shunting in a surgical model of pulmonary arteriovenous malformations

Tina C. Wan, Henry Rousseau, Carol Mattern, Madeline Tabor, Matthew R. Hodges, Ramani Ramchandran, Andrew D. SpearmanMedical College of Wisconsin and Children’s Wisconsin.United States Physiological ReportsPhysiol Rep 2024; 12: DOI: 10.14814/phy2.70123 AbstractPulmonary arteriovenous malformations (PAVMs) universally develop in patients with single ventricle congenital heart disease. Single ventricle PAVMs have been recognized for over 50 years but

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Persistent pulmonary hypertension in children after apparent resolution of ultrasound-defined pulmonary hypertension associated with bronchopulmonary dysplasia

Mami Takeoka, Hirofumi Sawada, Yoshihide Mitani, Hiroyuki Ohashi, Noriko Yodoya, Kazunobu Ohya, Naoki Tsuboya, Tomoya Harada, Masahiro HirayamaMie University Graduate School of Medicine. Japan European Journal of PediatricsEur J Pediatr 2024; 184: DOI: 10.1007/s00431-024-05843-6 AbstractTo evaluate pulmonary hemodynamics in patients who had been followed up for bronchopulmonary dysplasia (BPD)-associated pulmonary hypertension (PH) in the mid-term by

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Asymmetric right ventricular myocardial work correlates with gold standard measurements of cardiac function in pulmonary hypertension

Simone G. Diab, Ryota Ebata, Dariusz Mroczek, Wei Hui, Espen W. Remme, Thomas Möller, Mark K. FriedbergOslo University Hospital and University of Oslo. Chiba University. Hospital for Sick Children.Norway and Japan Pulmonary CirculationPulm Circ 2024; 14: DOI: 10.1002/pul2.70014 AbstractRight ventricular (RV) (dys)function determines outcomes in pulmonary hypertension (PH). We previously found that asymmetric RV myocardial work

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Magnetic resonance imaging of pulmonary hypertension

Christian Johannes KellenbergerUniversity Children’s Hospital Zurich.Switzerland Pediatric RadiologyPediatr Radiol 2024; DOI: 10.1007/s00247-024-06099-w AbstractPulmonary hypertension is a rare but serious disease in children with potentially poor prognosis in the long term. Direct measurement of increased pressure in the pulmonary circulation requires right-heart catheterisation. Echocardiography is the imaging modality commonly used for suggesting the presence of pulmonary hypertension

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Utility of Serum Matrix Metalloproteinase-7 as a Biomarker in Cholestatic Infants with Congenital Heart Disease

Sindhu Pandurangi, Michael E. Kim, Nicolas Noriega, Bradley Conant, JangDong Seo, Reena Mourya, Pranavkumar Shivakumar, Anna L. Peters, Andrew Misfeldt, Meghan Chlebowski Children’s Medical Center of Dallas and University of Texas Southwestern Medical Center. Hospital for Sick Children. University of Cincinnati College of Medicine and Cincinnati Children’s Hospital Medical Center. United States Pediatric CardiologyPediatr Cardiol

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Actigraphy Study Endpoints to Reduce Sample Size and Facilitate Drug Development for Pediatric Pulmonary Arterial Hypertension

Haihao Sun, Norman Stockbridge, D. Dunbar Ivy, Jennifer Clark, Angela Bates, Stephanie S. Handler, Usha S. Krishnan, Mary P. Mullen, Delphine Yung, Rachel K. Hopper, Nidhy P. Varghese, Catherine M. Avitabile, Jeff Fineman, Eric D. Austin, Grace FreireU.S. Food and Drug Administration. University of Colorado School of Medicine. Stollery Children’s Hospital and University of Alberta.

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The Pulmonary Artery Pulsatility Index Provides No Additional Prognostic Information in Pediatric Pulmonary Arterial Hypertension

Faye E. Smits, Chantal Lokhorst, Marlies G. Haarman , Mark-Jan Ploegstra , Rolf M. F. Berger, Johannes M. DouwesBeatrix Children’s Hospital, University Medical Center Groningen and University of Groningen.Netherlands ChildrenChildren 2024; 11: DOI: 10.3390/children11101152 AbstractBackground/objectives: The pulmonary artery pulsatility index (PAPi, calculated as (SPAP – DPAP)/mRAP) has been suggested as a measure of right ventricular-vascular coupling (RVVC)

The Pulmonary Artery Pulsatility Index Provides No Additional Prognostic Information in Pediatric Pulmonary Arterial Hypertension Read More »

Clinical Implications of Pretest Probability of HFpEF on Outcomes in Precapillary Pulmonary Hypertension

Yogesh N. V. Reddy, Robert P. Frantz, Paul M. Hassoun, Anna R. Hemnes, Evelyn Horn, Jane A. Leopold, Franz Rischard, Erika B. Rosenzweig, Nicholas S. Hill, Serpil C. Erzurum, Gerald J. Beck, J. Emmanuel Finet, Christine L. Jellis, StephenC. Mathai, W. H. Wilson Tang, Barry A BorlaugMayo Clinic. Johns Hopkins University. Vanderbilt University Medical Center. Weill

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Ventricular Dysfunction in Patients With Congenital Diaphragmatic Hernia Who Die After Repair

Catherine M. Avitabile, Yang Wang, Devon Ash, Sabrina J. Flohr, Leny Mathew, Natalie Rintoul, Holly L. HedrickPerelman School of Medicine at the University of Pennsylvania and Children’s Hospital of Philadelphia. United States Journal of Pediatric SurgeryJ Pediatr Surg 2024; 60: DOI: 10.1016/j.jpedsurg.2024.162002 AbstractBackground: Quantitative echocardiographic (echo) measures of ventricular function predict mortality in pediatric pulmonary hypertension (PH),

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