Medical Therapy. Efficacy or Lack of Efficacy

Persistent pulmonary hypertension of the newborn infant (PPHN) due to premature closure of the ductus arteriosus (DA)

Ayevbekpen Grace Okoye, Adrita Khawash, Mahesh Nanjundappa, Matthew Jones, Anne GreenoughKings College Hospital NHS Foundation Trust. Evelina Children’s Hospital and Guys and St Thomas Hospital. United Kingdom Case Reports in Perinatal MedicineCase Rep Perinat Med 2025; 13: DOI: 10.1515/crpm-2024-0001 AbstractObjectives: To describe the course of an infant with persistent pulmonary hypertension of the newborn (PPHN) secondary to […]

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Mechanism and Treatment of Right Ventricular Failure Due to Pulmonary Hypertension in Children

Bibhuti B. DasUniversity of Mississippi Medical Center.United States ChildrenChildren 2025; 12: DOI: 10.3390/children12040476 AbstractPulmonary hypertension (PH) is a progressive disorder characterized by obstructive changes in the pulmonary vasculature, leading to increased pulmonary vascular resistance (PVR), right ventricular (RV) strain, and eventual RV failure (RVF). Despite advancements in medical therapy, PH remains associated with significant morbidity and

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Different Effects of Riociguat and Vericiguat on Pulmonary Vessels and Airways

Katharina Nubbemeyer, Julia Krabbe, Svenja Böll, Anna Michely, Sebastian Kalverkamp, Jan Spillner, Christian MartinRWTH Aachen University. Ruhr University Bochum. Germany BiomedicinesBiomedicines 2025; 13: DOI: 10.3390/biomedicines13040856 AbstractBackground/Objectives: Pulmonary hypertension is a progressive disease leading to right heart failure. One treatment strategy is to induce vasodilation via the nitric oxide-soluble guanylate cyclase-cyclic guanosine monophosphate (NO-sGC-cGMP) signaling pathway. There

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Successful Treatment of Congenital Chylothorax by Early Use of Prednisolone: A Case Report

Kyoka Hirano , Koji Nakae , Manaka Matsunaga , Kentaro Ueno , Yasuhiro OkamotoKagoshima University Hospital.Japan CureusCureus 2024; 16:DOI: 10.7759/cureus.60628 AbstractCongenital chylothorax is the most common form of pleural effusion during the neonatal period; however, no treatment strategy exists. The pathogenesis and etiology of this disease are not fully understood; hence, several cases are difficult to

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Mas1 Receptor Activation is Necessary and Sufficient to Transduce ACE2 Effect in PAH, But Ang(1-7) Alone is Insufficient

James West, Megha Talati, Erica Carrier, Anandharajan Rathinasabapathy, Ibragim Gaidarov, Benjamin Vigl, Ying Cai, Hongpeng Jia, Tom Blackwell, Santhi Gladson, Christie Moore, Sheila Shay, Ethan Sevier, Anna HemnesVanderbilt University Medical Center. Eurofins Beacon Discovery. Alterras Therapeutics. Johns Hopkins University School of Medicine.United States and Austria Pulmonary CirculationPulm Circ 2025; 15: DOI: 10.1002/pul2.70083 AbstractACE2 has shown effectiveness

Mas1 Receptor Activation is Necessary and Sufficient to Transduce ACE2 Effect in PAH, But Ang(1-7) Alone is Insufficient Read More »

The sonic hedgehog signaling inhibitor cyclopamine improves pulmonary arterial hypertension via regulating the bone morphogenetic protein receptor 2 pathway

Youpeng Jin, Fei Mao, Xuehui Wang, Jie Zhang, Yanting Gao, Youfei FanShandong Provincial Hospital, First Affiliated Hospital and Shandong First Medical University.China Scientific ReportsSci Rep 2025; 15: DOI: 10.1038/s41598-025-97627-7 AbstractPulmonary arterial hypertension (PAH) is a severe and progressive disease with hallmarks of pulmonary vascular remodeling and bone morphogenetic protein receptor 2 (BMPR2) mutation. Recent studies indicate

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Novel Use of Drug-Coated Balloon Angioplasty to Treat Pulmonary Vein Stenosis

Saloni Agrawal, Kali A. Hopkins, Ali N. Zaidi, Barry LoveMount Sinai Hospital. Mount Sinai Fuster Heart Hospital.United States Journal of the American College of Cardiology Case ReportsJACC Case Rep 2024; 29: DOI: 10.1016/j.jaccas.2024.102613 AbstractPulmonary vein stenosis is challenging to treat due to high rate of recurrence. Multiple interventions exist but are limited by high rates of

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Paclitaxel-coated drug-eluting balloon for pulmonary vein stenosis after repair of total anomalous pulmonary venous return with asplenia

Hiroshi Ono, Ryo Mafune, Taiyu Hayashi, Yasushi Misaki, Yukihiro Kaneko, Jumpei Saito, Miki Nagai, Hitoshi KatoNational Medical Center for Child Health and Development. Japan Journal of Cardiology CasesJ Cardiol Cases 2020; 22: 107-109DOI: 10.1016/j.jccase.2020.05.008 AbstractWe used a paclitaxel-coated drug-eluting balloon (DEB) for pulmonary vein stenosis (PVS) after repair of total anomalous pulmonary venous return with asplenia.

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Paclitaxel drug-eluting stent placement for pulmonary vein stenosis as a bridge to heart-lung transplantation

Andreea Dragulescu, Olivier Ghez, Jacques Quilici, Alain FraisseCentre Hospitalo-Universitaire La Timone.France Pediatric CardiologyPediatr Cardiol 2009; 30: 1169-1171DOI: 10.1007/s00246-009-9511-5 AbstractCongenital pulmonary vein stenosis (PVS) presents as an isolated lesion or in association with other congenital heart anomalies. The most extensive forms of the disease are uniformly fatal because neither surgical repair nor transcatheter therapy results in long-term

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Eliglustat and cardiac comorbidities in Gaucher disease: a pharmacogenomic approach to safety and efficacy

Noor Ul Ain, Armaan Saith, Audrey Ruan, Ruhua Yang, Aaron Burton, Pramod K. MistryYale School of Medicine and Yale New Haven Hospital.United States Frontiers in MedicineFront Med 2025; 12: DOI: 10.3389/fmed.2025.1535099 AbstractIntroduction: Gaucher disease (GD), a lysosomal storage disorder, results from the accumulation of glycosphingolipids due to deficient lysosomal glucocerebrosidase activity. This pathological accumulation triggers immune activation,

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