Medical Therapy. Efficacy or Lack of Efficacy

Macitentan in the Young-Mid-term Outcomes of Patients with Pulmonary Hypertensive Vascular Disease treated in a Pediatric Tertiary Care Center

Sulaima Albinni, Julian Heno, Imre Pavo, Erwin Kitzmueller, Manfred Marx, Ina Michel‑BehnkeMedical University of Vienna.Austria Paediatric DrugsPaediatr Drugs 2023; 25: 467-481DOI: 10.1007/s40272-023-00573-y AbstractBackground: Pulmonary hypertension (PH) is a severe hemodynamic condition with high morbidity and mortality. Approved targeted therapies are limited for pediatric subjects, and treatments are widely adopted from adult algorithms. Macitentan is a safe and effective drug used for adult PH, […]

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Efficacy of Sildenafil in Infants with Bronchopulmonary Dysplasia-Associated Pulmonary Hypertension

Kacie Dillon, Vineet Lamba, Ranjit R. Philip, Mark F. Weems, Ajay J. Talati University of Tennessee Health Science Center.United States ChildrenChildren 2023; DOI: 10.3390/children10081397 AbstractBackground: Pulmonary hypertension (PH) is a common comorbidity in infants with bronchopulmonary dysplasia (BPD). Sildenafil is a widely recognized therapy for PH, but its efficacy in infants with BPD is questionable. We propose

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The role of rhIGF-1/BP3 in the prevention of pulmonary hypertension in bronchopulmonary dysplasia and its underlying mechanism

Sehua Qu, Lianqiang Shan, Xin Chen, Zhen Zhang, Yumeng Wu, Yun Chen, Feixiang Zhuo, Yitong Wang, Huaifu DongThe First Affiliated Hospital of Bengbu Medical College. China BioMed Central Pulmonary MedicineBMC Pulm Med 2023; 23: DOI: 10.1186/s12890-023-02498-1 AbstractBackground: This study aimed to determine whether postnatal treatment with recombinant human IGF-1 (rhIGF-1)/binding peptide 3 (BP3) ameliorates lung injury and

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The perioperative use of inhaled prostacyclins in cardiac surgery: a systematic review and meta-analysis

Berend Marcus, Frederik Marynen, Steffen Fieuws, Dieter Van Beersel, Filip Rega, Steffen RexUniversitair Ziekenhuis Leuven. Katholieke Universiteit Leuven. Belgium Canadian Journal of AnaesthesiaCan J Anaesth 2023; 70: 1381-1393DOI: 10.1007/s12630-023-02520-4 AbstractPurpose: Perioperative pulmonary hypertension (PH) is an independent risk factor for morbidity and mortality in cardiac surgery. While inhaled prostacyclins (iPGI2s) are an established treatment of chronic PH,

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Intravenous prostacyclins for right ventricular failure following left ventricular assist device in paediatric heart failure

Laura J. Radel, Mehreen Iqbal, Megan GriffithsUniversity of Texas Southwestern.United States Cardiology in the YoungCardiol Young 2023; DOI: 10.1017/S1047951123001725 AbstractRight ventricular failure after placement of left ventricular assist device in paediatric heart failure is associated with increased mortality. We report successful use of intravenous prostacyclin for right ventricular support and pulmonary hypertension after initiation of left

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Cost-utility of ambrisentan and bosentan for pediatric pulmonary arterial hypertension

Jefferson Antonio Buendia, Diana Guerrero Patino, Erika Fernanda LindarteUniversity of Antioquia. University of Oxford. Columbia and United Kingdom Expert Review of Pharmacoeconomic and Outcomes ResearchExpert Rev Pharmacoecon Outcomes Res 2023; DOI: 10.1080/14737167.2023.2233698 AbstractIntroduction: Despite the increasing evidence supporting the efficacy of ambrisentan and bosentan in improving functional classes among pediatric patients with pulmonary arterial hypertension (PAH), there

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Retrospective on global pulmonary hypertension clinical trials: 1999-2021

Lu Zheng, Jun Tan, Yi Yan, Shang Wang, Ping Yuan, Cheng Wu, Yin-Tao Zhao, Hai-Bo Yang, Francesco Nappi, Adriano R. Tonelli, Lan Wang, Qing-Hua Hu, Rong JiangFirst Affiliated Hospital of Zhengzhou University. Yeda Hospital of Yantai. Shanghai Children’s Medical Center and National Children’s Medical Center. Shanghai Pulmonary Hospital and Tongji University. Naval Medical University. Centre

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Factors leading to supranormal cardiac index in pediatric pulmonary hypertension patients treated with parenteral prostanoid therapy

Kimberley G. Miles Paul J. Critser, Patrick D. Evers, Michelle Cash, Melissa Magness, Elizabeth Geers Meredith O’Neil, Zhiqian Gao, Nicholas J. Ollberding, Russel HirschCincinnati Children’s Hospital Medical Center and Cincinnati College of Medicine. Oregon Health and Sciences University. United States Pulmonary CirculationPulm Circ 2023;DOI: 10.1002/pul2.12264 AbstractParenteral prostanoid therapy (PPT) can result in supranormal cardiac index (SCI;

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Echocardiographic Assessment of Pulmonary Arterial Hypertension Following Inhaled Nitric Oxide in Infants with Severe Bronchopulmonary Dysplasia

Maria V. Fraga, Kevin C. Dysart, Jason Z. Stroller, Matthew Huber, Anysia Fedec, Laura Mercer-Rosa, Haresh KirpalaniThe Children’s Hospital of Philadelphia and University of Pennsylvania Perelman School of Medicine. Nemours Children’s Health and duPont Hospital for Children. McMaster University.United States and Canada NeonatologyNeonatology 2023; DOI: 10.1159/000531586 AbstractObjectives: Inhaled nitric oxide (iNO) is an effective pulmonary vasodilator. However,

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Acid ceramidase gene therapy ameliorates pulmonary arterial hypertension with right heart dysfunction

Michael G. Katz, Yoav Hadas, Adam Vincek, Lina Freage‑Kahn, Nataly Shtraizent, Jeko M. Madjarov, Peter Pastuszko, Efrat Eliyahu Icahn School of Medicine at Mount Sinai. SeneX Therapeutics Inc. Atrium Health Sanger Heart and Vascular Institute. Wake Forest School of Medicine. United States Respiratory ResearchRespir Res 2023; 24DOI: 10.1186/s12931-023-02487-2 AbstractBackground: Up-regulation of ceramides in pulmonary hypertension (PH), contributing to

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