Diagnostic Testing for Pulmonary Vascular Disease. Invasive Testing

Congenital localized stenosis of the pulmonary veins

M. Henry, J. C. Hoeffel, C. PernotHospital Jeanne d’Arc.France Pediatric RadiologyPediatr Radiol 1975; 4: 49-52DOI: 10.1007/BF00978821 AbstractOne case of localized stenosis of the extrapulmonary portion of pulmonary veins is reported in a 3 years old child. The chest X-Ray showed a rounded and pulsatile opacification over the right hilum and on angiocardiography the right superior pulmonary […]

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Unilateral pulmonary vein atresia

Leonard E. Swischuk, Philippe L’HeureuxUniversityb of Texas Medical Branch. University of Minnesota Medical School.United States American Journal of RoentgenologyAJR Am J Roentgenol 1980; 135: 667-672DOI: 10.2214/ajr.135.4.667 AbstractThree cases of unilateral pulmonary vein atresia in children are described. The findings in all three cases were similar and consisted of hemoptysis, recurrent pulmonary infections, and a normal size

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Esophageal varices in association with unilateral pulmonary vein atresia

J. Kevin Harrison, Steven E. Hearne, William M. Baker, Peter Van Trigt, Gil Leidig, Thomas M. Bashore, Martin P. O’LaughlinDuke University Medical Center.United States Catheterization and Cardiovascular DiagnosesCathet Cardiovasc Diagn 1996; 38: 387-392DOI: 10.1002/(SICI)1097-0304(199608)38:4<387::AID-CCD14>3.0.CO;2-C AbstractA 27-yr-old woman with recurrent episodes of hemoptysis (and hematemesis due to esophageal varices) was found to have unilateral pulmonary vein atresia.

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Congenital unilateral pulmonary vein atresia: radiologic findings in three adult patients

Laura E. Heyneman, Robert L. Nolan, J. Kevin Harrison, H. Page McAdamsDuke University Medical Center.United States American Journal of RoentgenologyAJR Am J Roentgenol 2001; 177: 681-685DOI: 10.2214/ajr.177.3.1770681 AbstractObjective: The purpose of our study is to describe the radiologic findings of adult patients presenting with congenital unilateral pulmonary vein atresia.Conclusion: Chest radiography in affected patients typically reveals a small

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Survival comparison in adults with congenital systemic to pulmonary shunt and borderline elevated pulmonary vascular resistance versus Eisenmenger syndrome

Jantaras Sengsim, Chodchanok Vijarnsorn, Prakul Chanthong, Paweena Chungsomprasong, Supaluck Kanjanauthai, Ploy Thammasate, Thita Pacharapakornpong, Nalin Chaiwangyen, Densiri Bositthipichet, Jarupim Soongswang, Teerapong Tocharoenchok, Ekarat Nitiyarom, Kriangkrai Tantiwongkosri, Thaworn Subtaweesin, Kritvikrom DurongpisitkulSiriraj Hospital and Mahidol University.Thailand Scientific ReportsSci Rep 2024; 14: DOI: 10.1038/s41598-024-81834-9 AbstractPulmonary arterial hypertension (PAH) associated with congenital heart disease (PAH-CHD) is a consequence of unrepaired

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SOX17 – Associated Pulmonary Hypertension in Children: A Distinct Developmental and Clinical Syndrome

Mary P. Mullen, D. Dunbar Ivy, Nidhy P. Varghese, Abbey J. Winant, Nahir Cortes-Santiago, Sara O. Vargas, Diego Porres, Nicola Maschietto, Paul J. Critser, Russel Hirsch, Catherine M. Avitabile, Rachel K. Hopper, Benkamin S. Frank, Ryan D. Coleman, Pankaj B. Agrawal, Jill A. Madden, Amy E. Roberts, Shane L. Collins, J. Usha Raj, Eric D.

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MicroRNA Profiling to Inform Disease Classification, Severity, and Treatment Response in Pediatric Pulmonary Hypertension

Michael A. Smith, Sam Chiacchia, Jason Boehme, Sanjeev A. Datar, Emily Morell, Roberta L. Keller, Amy Romer, Elizabeth Colglazier, Clair Parker, Jasmine Becerra, Jeffrey R. FinemanUniversity of California, San Francisco. Stanford University. Children’s Hospital of Philadelphia.United States American Journal of Physiology Heart and Circulatory PhysiologyAm J Physiol Heart Circ Physiol 2024; DOI: https://doi.org/10.1152/ajpheart.00622.2024 AbstractPediatric pulmonary

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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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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)

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