Medical Therapy. Efficacy or Lack of Efficacy

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 »

Macitentan vs Standard of Care in Pediatric Pulmonary Arterial Hypertension (TOMORROW): A Randomized Clinical Trial

Rolf M. F. Berger, D. Dunbar Ivy, Julian I. Borissoff, Sofija Cerovic, Dénes Csonka, Tatiana Remenová, Dominik Richard, Matthieu Villeneuve, Maurice BeghettiBeatrix Children’s Hospital, University Medical Center Groningen and University of Groningen. Children’s Hospital Colorado. Johnson & Johnson. University Hospitals of Geneva.Netherlands, United States and Switzerland Journal of PediatricsJ Pediatr 2026; DOI: 10.1016/j.jpeds.2026.115057 AbstractObjectives: To evaluate pharmacokinetics

Macitentan vs Standard of Care in Pediatric Pulmonary Arterial Hypertension (TOMORROW): A Randomized Clinical Trial Read More »

Hypercapnia as a Key Mechanism in the Development of Pulmonary Hypertension in Malignant Pertussis: A Case Report

Juan Ignacio Muñoz-Bonet, Francisco José Sebastián-Cuevas, Eva María Flor-Macián, María del Carmen Ortega-Sánchez, Andrés Rodríguez-Sacristán, Juan BrinesHospital Clínico Universitario. University of Valencia. Hospital Dr. Peset Valencia. Hospital Universitario Virgen Macarena.Spain Respirology Case ReportsRespirol Case Rep 2026; 14: DOI: 10.1002/rcr2.70528 AbstractMalignant pertussis (MP) is a highly lethal complication of pertussis in young infants, typically associated with hyperleukocytosis, pulmonary

Hypercapnia as a Key Mechanism in the Development of Pulmonary Hypertension in Malignant Pertussis: A Case Report Read More »

Lamp Oil Poisoning: Pulmonary Hypertension Following Oil Aspiration Successfully Treated with Inhaled Nitric Oxide

Demet Tosun, Ilyas Bingöl Nihal AkçayIstanbul Kanuni Sultan Suleyman Training and Research Hospital.Turkey Klinische PädiatrieKlin Padiatr 2026; DOI: 10.1055/a-2814-3185 AbstractAbstract Not Available CategoryClass I. Drug-induced and Toxin-induced Pulmonary HypertensionMedical Therapy. Efficacy or Lack of Efficacy Age Focus: Pediatric Pulmonary Vascular Disease Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication Article Access Free

Lamp Oil Poisoning: Pulmonary Hypertension Following Oil Aspiration Successfully Treated with Inhaled Nitric Oxide Read More »

Clinical Application of Inhaled Nitric Oxide in Conditions of Excessive Right Heart Load: A Review from Neonatal Pulmonary Hypertension to Perioperative Cardiac Surgery Management

Chengming Hu, Zhe Chen, Lizhi Lv, Yan Zhu, Yan Chen, Qiang WangBeijing Anzhen Hospital and Capital Medical University.China Journal of Cardiovascular Development and DiseaseJ Cardiovasc Drv Dis 2026; 13: DOI: 10.3390/jcdd13020081 AbstractExcessive right heart load imposes an acute or chronic injury on the right ventricle (RV), predisposing critically ill neonates and cardiac surgical patients to RV

Clinical Application of Inhaled Nitric Oxide in Conditions of Excessive Right Heart Load: A Review from Neonatal Pulmonary Hypertension to Perioperative Cardiac Surgery Management Read More »

Decreased AMP-Kinase Function in Pulmonary Artery Smooth Muscle Cells Contributes to Pulmonary Artery Remodeling in Persistent Pulmonary Hypertension of Newborn

Emily A. Mooers, Ujala Rana, Chintamani N. Joshi, Adeleye J. Afolayan, Ru-Jeng Teng, Girija Ganesh KonduriMedical College of Wisconsin. United States American Journal of Physiology Lung Cellular and Molecular PhysiologyAm J Physiol Lung Cell Mol Physiol 2026; DOI: 10.1152/ajplung.00229.2025 AbstractPulmonary vascular remodeling contributes to Persistent Pulmonary Hypertension of the Newborn (PPHN); the mechanisms remain unknown. 5’AMP-Activated

Decreased AMP-Kinase Function in Pulmonary Artery Smooth Muscle Cells Contributes to Pulmonary Artery Remodeling in Persistent Pulmonary Hypertension of Newborn Read More »

A Rare Case of Reversible Pulmonary Hypertension Phenotype in a Child with Scurvy: Aetiologies Insights

Mattia Pasquinucci, Luisa Bonsembiante, Sofia Mezzalira, Martina Locallo, Davide MeneghessoAULSS 7 Pedemontana-San Bassiano Hospital. IRCCS Istituto Giannina Gaslini. University of Padua.Italy ReportsReports 2026; DOI: 10.3390/reports9010044 AbstractBackground and Clinical Significance: Scurvy, caused by chronic vitamin C deficiency, is re-emerging in Western countries, particularly among pediatric patients with highly selective diets. While its musculoskeletal and mucocutaneous manifestations are well-known,

A Rare Case of Reversible Pulmonary Hypertension Phenotype in a Child with Scurvy: Aetiologies Insights Read More »

Systematic Review of the Application of Pulmonary Hypertension Treatments in Ventricular Septal Defect, Pulmonary Atresia, and Major Aortopulmonary Collateral Arteries

Keiichi Hirono, Keiko Uchida, Taku Ishii, Hidekazu Ishida, Shinichi Takatsuki, Hiroyuki Fukushima, Kei Inai, Susumu Hosokawa, Reina Ishizaki, Hirofumi Sawada, Naofumi F. Sumitomo, Ayako Chida-Nagai, Yuichi Ishikawa, Hirohiko Motoki, Atsushi Yao, Shigetoyo Kogaki, Hiroyuki Yamagishi, Shozaburo DoiToyama University Hospital. Tokyo Medical University. Institute of Science Tokyo. Osaka University Graduate School of Medicine. Toho University, Omori

Systematic Review of the Application of Pulmonary Hypertension Treatments in Ventricular Septal Defect, Pulmonary Atresia, and Major Aortopulmonary Collateral Arteries Read More »

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