Class 1. Pulmonary Hypertension Associated with Congenital Cardiovascular Disease

Eccentricity Index Is Associated With Pulmonary Arterial Hypertension in Children After Repair of Complete Atrioventricular Septal Defects

Charles T. Simpkin, Marissa DeLima, Morgan MacBeth, Dale A. Burkett, D. Dunbar Ivy, Benjamin S. FrankUniversity of Colorado Medical Campus and Children’s Hospital Colorado.United States Echocardiography Echocardiography 2026; DOI: 10.1111/echo.70451 AbstractIntroduction: Eccentricity Index is an echocardiographic predictor of disease severity in children with pulmonary arterial hypertension (PAH). Its utility after atrioventricular septal defect (AVSD) repair remains unclear […]

Eccentricity Index Is Associated With Pulmonary Arterial Hypertension in Children After Repair of Complete Atrioventricular Septal Defects Read More »

Multimodality imaging in a child with complete atrioventricular canal defect, left superior vena cava, and airway compression: Echocardiography, bronchoscopy, and lung ultrasound insights

Rajesh Madavathazathil Gopalakrishnan, Shashikapoor Yadav, Ashish KatewaAmrita Institute of Medical Sciences and Research Centre.India Annals of Pediatric CardiologyAnn Pediatr Cardiol 2025; 18: 628-631 DOI: 10.4103/apc.apc_261_25 AbstractCongenital heart disease may coexist with airway abnormalities, increasing perioperative risk. We report a 2-year-old girl with a complete atrioventricular canal defect (CAVCD), left superior vena cava (LSVC), and severe pulmonary

Multimodality imaging in a child with complete atrioventricular canal defect, left superior vena cava, and airway compression: Echocardiography, bronchoscopy, and lung ultrasound insights Read More »

Pulmonary Hypertension in Down Syndrome Versus Non-syndromic Pediatric Populations With Congenital Heart Disease: A Comparative Study

Tariqul Islam, Tahmina Karim, Sadia Afrin Mony, Mostafizur Rahman Bhuyan, Faizah IslamBangladesh Medical University.Bangladesh CureusCureus 2026; 18: DOI: 10.7759/cureus.104634 AbstractBackground: Down syndrome (DS), or trisomy 21, is the most common chromosomal disorder associated with congenital heart disease (CHD), profoundly affecting disease progression and management. While 4-10% of all CHD cases occur in DS, 40-60% of individuals with

Pulmonary Hypertension in Down Syndrome Versus Non-syndromic Pediatric Populations With Congenital Heart Disease: A Comparative Study Read More »

Congenital Heart Disease and Pulmonary Arterial Hypertension: Current Perspectives

Enrique Blanca-Jover, Francisco Contreras-Chova, Antonio Jerez-Calero, Jose Uberos-Fernandez, Laura Pérez-LaraUniversity of Granada and niversity Hospital of Granada.Spain Reviews in Cardiovascular MedicineRev Cardiovasc Med 2026; 27: DOI: 10.31083/RCM48337 AbstractPulmonary arterial hypertension (PAH) is the most serious complication of congenital heart disease (CHD), constituting a heterogeneous clinical entity classified within Group 1 of the Clinical Classification of Pulmonary

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The Postnatal Lung Maturation Disrupted by Increased Pulmonary Blood Flow and Its Clinical Implications

Sixie Zheng, Zheng Wang, Yiting Xue, He Zhang, Yingying Xiao, Yuqing Hu, Debao Li, Qing Cui, Chenxi Liu, Jing Wang, Lincai Ye, Lisheng QiuShanghai Children’s Medical Center and Shanghai Jiao Tong University School of Medicine. Women and Children’s Hospital of Ningbo University. Children’s Hospital of Fudan University and National Children’s Medical Center. China Journal of

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Neonatal pulmonary vascular remodeling induced by increased blood flow is associated with an antiviral-like immune signature

Sixie Zheng, Hao Li, Siqi She, Yiting Xue, Debao Li, Jiapei Wang, Jing Wang, Yuqing Hu, Lincai YeShanghai Children’s Medical Center and Shanghai Jiao Tong University School of Medicine. Children’s Hospital of Fudan University and National Children’s Medical Center. Affiliated Women and Children’s Hospital of Ningbo University. China Frontiers in ImmunologyFront Immunol 2026; 17: DOI: 10.3389/fimmu.2026.1780303

Neonatal pulmonary vascular remodeling induced by increased blood flow is associated with an antiviral-like immune signature Read More »

The Impact of Acute COVID-19 Infection and Long COVID in Patients with Congenital Heart Disease: A Longitudinal Study by the German National Register for Congenital Heart Disease

Cornelia Tremblay, Ulrike M. M. Bauer, Jens Beudt, Stefan Orwat, Gerhard-Paul Diller, Constanze Pfitzer, Paul C. HelmNational Register for Congenital Heart Defects. Competence Network for Congenital Heart Defects. University Hospital Muenster. Deutsches Herzzentrum der Charité. Freie Universität Berlin and Humboldt-Universität zu Berlin. Germany Journal of Clinical MedicineJ Clin Med 2026; 15: DOI: 10.3390/jcm15051986 AbstractBackground: Patients with congenital

The Impact of Acute COVID-19 Infection and Long COVID in Patients with Congenital Heart Disease: A Longitudinal Study by the German National Register for Congenital Heart Disease Read More »

Protocol for an open-label, randomised, controlled trial to evaluate the efficacy and safety of sotatercept add-on therapy compared with pulmonary vasodilator-based standard of care for pulmonary vasodilator-resistant pulmonary arterial hypertension associated with unrepaired congenital shunts (atrial septal defect, ventricular septal defect or patent ductus arteriosus), including Eisenmenger syndrome: the SuMILE trial

Keimei Yoshida, Kazuya Hosokawa, Takahiro Hiraide, Satoshi Akagi, Kentaro Ejiri, Yu Taniguchi, Shiro Adachi, Takumi Inami, Naohiko Nakanishi, Masaharu Kataoka, Taijyu Satoh, Shunsuke Tatebe, Toshiro Shinke, Hideshi Tomita, Yusuke Akazawa, Takashi Higaki, Koshiro Tagawa, Ayako Ishikita, Soshun Asakawa, Kohtaro AbeKyushu University and Kyushu University Hospital. Keio University School of Medicine. Okayama University. Kobe University Hospital.

Protocol for an open-label, randomised, controlled trial to evaluate the efficacy and safety of sotatercept add-on therapy compared with pulmonary vasodilator-based standard of care for pulmonary vasodilator-resistant pulmonary arterial hypertension associated with unrepaired congenital shunts (atrial septal defect, ventricular septal defect or patent ductus arteriosus), including Eisenmenger syndrome: the SuMILE trial Read More »

Sotatercept in Patients With Eisenmenger Syndrome

Oktay Tutarel, Oliver Miera, Felix BergerDeutsches Herzzentrum der Charité, Freie Universität Berlin and Humboldt-Universität zu Berlin.Germany Pulmonary CirculationPulm Circ 2026; 16: DOI: 10.1002/pul2.70283 AbstractEisenmenger syndrome represents a complex and severe form of pulmonary arterial hypertension (PAH) associated with congenital heart disease. Sotatercept, a first-in-class activin-signaling inhibitor, offers a novel therapeutic approach in the treatment of PAH,

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Reversing the irreversible? A case of successful surgical repair in a late-presenting aortopulmonary window with severe pulmonary hypertension

Rido Mulawarman, Ericko Ongko Joyo, Muhamad Adrin Aefiansyah Putra, Aditya Agita Sembiring, Sisca Natalia Siagian, Oktavia LilyasarUniversitas Indonesia. Indonesia Egyptian Heart JournalEgypt Heart J 2026; 78: DOI: 10.1186/s43044-026-00726-7 AbstractBackground: Aortopulmonary window (APW) is a rare congenital heart defect, accounting for only 0.2-0.6% of all congenital cardiac anomalies, and is usually identified and surgically corrected within the first

Reversing the irreversible? A case of successful surgical repair in a late-presenting aortopulmonary window with severe pulmonary hypertension Read More »

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