Medical Therapy. Efficacy or Lack of Efficacy

Real-world effectiveness and safety of macitentan in patients with pulmonary artery hypertension: a multicenter, retrospective, observational study in China

Yu-Cheng Chen, Hai-Long Dai, Chun-Li Liu, Jiang Li, Qiu-Shang Ji, Yun-Shan Cao, Jing Xiao, Rong Jian, Jian-Min Zhuo, Xin-Chao Luo, Hong GuWest China Hospital of Sichuan University. Yan’an Affiliated Hospital of Kunming Medical University. First Affiliated Hospital of Guangzhou Medical University. Second Xiangya Hospital of Central South University. Qilu Hospital of Shandong University. Gansu Provincial […]

Real-world effectiveness and safety of macitentan in patients with pulmonary artery hypertension: a multicenter, retrospective, observational study in China Read More »

Contemporary survival outcomes of congenital systemic-to-pulmonary shunt in children with borderline pulmonary vascular resistant index compared to Eisenmenger syndrome

Nalin Chaiwangyen, Chodchanok Vijarnsorn, Paweena Chungsomprasong, Prakul Chanthong, Supaluck Kanjanauthai, Ploy Thammasate, Thita Pacharapakornpong, Densiri Bositthipichet, Jantaras Sengsim, Jarupim Soongswang, Teerapong Tocharoenchok, Ekarat Nitiyarom, Kriangkrai Tantiwongkosri, Thaworn Subtaweesin, Kritvikrom DurongpisitkuFaculty of Medicine Siriraj Hospital, Mahidol University.Thailand Scientific ReportsSci Rep 2024; 14: DOI: 10.1038/s41598-024-67899-6 AbstractPulmonary arterial hypertension (PAH) associated with congenital heart disease (PAH-CHD) is a complication

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Clinical phenotype of pulmonary vascular disease requiring treatment in extremely preterm infants

Ki Teak Hong, Seung Han Shin, Ee‑Kyung Kim, Han‑Suk KimSeoul National University Children’s Hospital.Republic of Korea BioMedical Central PediatricsBMC Pediatr 2024; 24: DOI: 10.1186/s12887-024-04943-4 AbstractBackground: Pulmonary vascular disease (PVD) and pulmonary hypertension (PH) is a significant disorder affecting prognosis of extremely preterm infants. However, there is still a lack of a consensus on the definition and optimal

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Trametinib for Refractory Chylous Effusions and Systemic Complications in Children with Noonan Syndrome

Taizo A. Nakano, Alexander W. Rankin, Aparna Annam, Ann M. Kulungowski, Leslie M. McCallen, Lauren R. Hill, Kathryn C. ChatfieldUniversity of Colorado Anschutz Medical Campus and Children’s Hospital Colorado. United States Journal of PediatricsJ Pediatr 2022; 248: 81-88DOI: 10.1016/j.jpeds.2022.05.030 AbstractObjective: To evaluate the effect of the RAS-MAPK pathway inhibitor trametinib on medically refractory chylous effusions in 3

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Trametinib restores the central conducting lymphatic flow in a premature infant with Noonan syndrome

Erika K. S. M. Leenders, Lotte E. R. Kleimeier, Lauren C. Weeke, Catelijne H. Coppens, Willemijn M. Klein, Jos M.T. DraaismaRadboud University Medical Center. Amalia Children’s Hospital.Netherlands Clinical Case ReportsClin Case Rep 2024; 12: DOI: 10.1002/ccr3.9164 AbstractWe describe a premature hydropic infant with Noonan syndrome and a therapy refractory chylothorax. This was shown to be due to a central conducting lymphatic anomaly.

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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 treat. Some patients with

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[Protective effects of 2-methoxyestradiol against hypoxic pulmonary hypertension in neonatal rats]

Ou Xie, Shan-Shan Li, Yang Luo, Le WangFirst Affiliated Hospital of Xinjiang Medical University.China Chinese Journal of Contemporary PediatricsZhongguo Dang Dai Er Ke Za Zhi 2024; 26: 757-764DOI: 10.7499/j.issn.1008-8830.2401078 AbstractObjectives: To investigate the protective effects of 2-methoxyestradiol (2ME) against hypoxic pulmonary hypertension (HPH) in neonatal rats.Methods: Ninety-six Wistar neonatal rats were randomly divided into a normoxia group, a

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Pharmacological Gq inhibition induces strong pulmonary vasorelaxation and reverses pulmonary hypertension

Alexander Seidinger, Richard Roberts, Yan Bai, Marion Müller, Eva Pfeil, Michaela Matthey, Sarah Rieck, Judith Alenfelder, Gabriele M. KUonig, Alexander Pfeifer, Evi Kostenis, Anna Klinke, Bernd K. Fleischmann, Daniela WenzelUniversity Hospital of the Ruhr University of Bochum and Ruhr University of Bochum. University Hospital of Nottingham. Massachusetts General Hospital and Harvard Medical School. University of

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High-intensity Focused Ultrasound-A New Choice to Conduct Pulmonary Artery Denervation

Yonghui Xie, Taoyue Yao, Xiaogang Zhu, Fan Yang, Haoqin Fan, Shirui Cao, Huaiyang Chen, Manzhen Liao, Yuanxi Xia, Jinqiao Liu, Zhenghui Xiao, Zhou Yang, Yunbin XiaoUniversity of South China. Affiliated Children’s Hospital of Xiangya School of Medicine. Third Xiangya Hospital. Second Xiangya Hospital. Central South University. Changsha Yali High School. China Journal of Cardiovascular Translational

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Vasopressin as adjunctive therapy in pulmonary hypertension associated with refractory systemic hypotension in term newborns

Felipe Santelices, Daniela Masoli, Javier Katten, Alberto Toso, Matias LucoPontificia Universidad Católica de Chile. Chile Journal of PerinatologyJ Perinatol 2024; DOI: 10.1038/s41372-024-02015-0 AbstractObjective: The use of vasopressin as an adjunctive therapy in pulmonary hypertension associated with refractory systemic hypotension has increased. The objective of our study is to describe its effects on term infants.Study design: Retrospective observational study.

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