Class 1. Pulmonary Hypertension Associated with Congenital Cardiovascular Disease

Case report: Multisystemic smooth muscle dysfunction syndrome: a rare genetic cause of infantile interstitial lung disease

Qianying Li, Lidan Cui, Jun Su, Yuelin ShenChildren’s Hospital Affiliated to Zhengzhou University, Henan Children’s Hospital and Zhengzhou Children’s Hospital. Beijing Children’s Hospital and Capital Medical University. China Frontiers in PharmacologyFront Pharmacol 2025; DOI: 10.3389/fphar.2024.1510969 AbstractMultisystemic smooth muscle dysfunction syndrome (MSMDS) is an autosomal dominant disorder caused by mutations in the ACTA2 gene, resulting in variable clinical manifestation […]

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Perioperative extra corporeal membrane oxygenation in neonates with transposition of the great arteries: 15 years of experience

Jesse A. Weeda, Roel L. F. van der Palen, Heleen E. Bunker-Wiersma, Lena Koers, Eelco van Es, Mark G. Hazekamp, Arjan B. te Pas, Peter Paul RoeleveldWillem-Alexander Children’s Hospital and Leiden University Medical Centre. Netherlands European Journal of Cardiothoracic SurgeryEur J Cardiothorac Surg 2025; DOI: 10.1093/ejcts/ezae442 AbstractBackground: Extracorporeal membrane oxygenation (ECMO) can act as a bridge to

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Safety and Efficacy of Selexipag for Pediatric Pulmonary Arterial Hypertension in Japanese Patients – An Open-Label Phase 2 Study

Toru Iwasa, Ryo Inuzuka, Hiroshi Ono, Yuichiro Sugitani, Hirokuni Yamazawa, Chihiro Hiraishi, Naoki Shiota, Shinichi Tanaka, Chieko Yamamoto, Ken-ichi Kurosaki, Masaru Miura, Satoshi YasukochiNational Cerebral and Cardiovascular Center. University of Tokyo. National Center for Child Health and Development. Japan Community Health Care Organization Kyushu Hospital. Hokkaido University. Nippon Shinyaku Company. Tokyo Metropolitan Children’s Medical Center.

Safety and Efficacy of Selexipag for Pediatric Pulmonary Arterial Hypertension in Japanese Patients – An Open-Label Phase 2 Study Read More »

Pediatric Pulmonary Arterial Hypertension; is it Possible to Predict its Outcome?

Hala M. Aghaa, Ranya Hegazy, Ahmed Gamal, Ranya Essam, Antoine A. Elmessih, Ahmed Behairy,Nagib Dahdah, Ahmed Abdelwahed, Nesrine Sobhy, Noha AliSpecialized Pediatric Hospital and Cairo University. CHU Sainte Justine and University of Montreal.Egypt and Canada Journal of the Saudi Heart AssociationJ Saudi Heart Assoc 2024; 36: 408-419DOI: 10.37616/2212-5043.1398 AbstractObjectives: To assess the outcome of pediatric pulmonary arterial

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Independent prognostic value of lipocalin-2 in congenital heart disease-associated pulmonary artery hypertension

Zhang-Ke Guo, Ping-Gui Chen, Yao-Xuan Li, Hong Jiao, Xiao-Hui Kong, Song Bai, Xiao-Feng Li, Ai-Jun Liu, Guo-Liang WangBeijing Children’s Hospital, Capital Medical University, Beijing Anzhen Hospital and National Center for Children’s Health. Third Hospital of Shanxi Medical University, Shanxi Bethune Hospital and Shanxi Academy of Medical Sciences. China World Journal of CardiologyWorld J Cardiol 2024;

Independent prognostic value of lipocalin-2 in congenital heart disease-associated pulmonary artery hypertension Read More »

High Shear Stress Reduces ERG Causing Endothelial-Mesenchymal Transition and Pulmonary Arterial Hypertension

Tsutomo Shinohara, Jan-Ranier Moonen, Yoon Hong Chun, Yannick C. Lee-Yow, Kenichi Okamura, Jason M. Szafron, Jordan Kaplan, Aiqin Cao, Lingli Wang, Divya Guntur, Shalina Taylor, Sarasa Isobe, Melody Dong, Weiguang Yang, Katherine Guo, Benjamin D. Franco, Cholawat Pacharinsak, Laura J. Pisani, Shinji Saitoh, Yoshihide Mitani, Alison L. Marsden, Jesse M. Engreitz, Jakob Körbelin, Marlene RabinovitchLucile

High Shear Stress Reduces ERG Causing Endothelial-Mesenchymal Transition and Pulmonary Arterial Hypertension Read More »

Fetal-to-Neonatal Transition of an Infant with Transposition of the Great Arteries and Intact Ventricular Septum: A Case Report

Jesse A. Weeda, Arjan B. te Pas, Monique C. Haak, Nico A.Blom, Roel L.F. van der PalenWillem-Alexander Children’s Hospital and Leiden University Medical Centre. Netherlands Biomedicine HubBiomed Hub 2024;DOI: 10.1159/000542723 AbstractIntroduction: Transposition of the great arteries (TGA), especially with intact ventricular septum (TGA-IVS), presents unique challenges during fetal-to-neonatal transition, which can contribute to developing persistent pulmonary hypertension

Fetal-to-Neonatal Transition of an Infant with Transposition of the Great Arteries and Intact Ventricular Septum: A Case Report Read More »

Update on Eisenmenger syndrome – Review of pathophysiology and recent progress in risk assessment and management

Ranjan Banerjee, Alexander R. OpotowskyCincinnati Children’s Hospital and University of Cincinnati College of MedicineUnited States International Journal of Cardiology Congenital Heart DiseaseInt J Cardiol Congenit Heart Dis 2024; DOI: 10.1016/j.ijcchd.2024.100520 AbstractLongstanding left-to-right shunting associated with congenital heart disease (CHD) can ultimately lead to pulmonary vascular remodeling, pulmonary arterial hypertension, and shunt reversal, the hallmark feature of

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Survival and Risk Factor for Mortality of Infants with Trisomy-21 and Pulmonary Hypertension: A Population-Based Study from a Middle-Income Country

Mohd Nizam Mat Bah, Norazah Zahari, Noor Adibah Abdullah, Mohd Hanafi Sapian, EmieliyuzaYusnita AliasMinistry of Health Malaysia and Hospital Sultanah Aminah.  University of Malaya.Malaysia Pediatric CardiologyPediatr Cardiol 2024; DOI: 10.1007/s00246-024-03732-1 AbstractLimited studies are available on the outcome of infants with trisomy-21 and pulmonary hypertension (PHT) in lower- and middle-income countries. This population-based cohort study aims to

Survival and Risk Factor for Mortality of Infants with Trisomy-21 and Pulmonary Hypertension: A Population-Based Study from a Middle-Income Country Read More »

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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