Medical Therapy. Efficacy or Lack of Efficacy

An Adolescent Patient With Idiopathic Pulmonary Arterial Hypertension Weaned Off Intravenous Epoprostenol Following Treatment With Selexipag: A Case Report

Ayako Chida-Nagai, Takao Tsujioka, Daisuke Sasaki, Gaku Izumi, Hirokuni Yamazawa, Atsuhito TakedaHokkaido University Hospital.Japan Frontiers in PediatricsFront Pediatr 2022; DOI: 10.3389/fped.2022.909595 AbstractIdiopathic pulmonary arterial hypertension (PAH) is a rare, progressive disease affecting the pulmonary arteries. Epoprostenol, a synthetic prostaglandin analog, is the most potent pharmacological treatment modality used in patients with PAH. However, it requires continuous […]

An Adolescent Patient With Idiopathic Pulmonary Arterial Hypertension Weaned Off Intravenous Epoprostenol Following Treatment With Selexipag: A Case Report Read More »

Clinical impact of subcutaneous treprostinil in trisomy 21 patient with pulmonary arterial hypertension associated with CHD

Ryusuke Numata, Kiyohiro Takigiku, Kouta TakeiNagano Children’s HospitalJapan Cardiology in the YoungCardiol Young 2022; DOI: 10.1017/S1047951121005096 AbstractSubcutaneous treprostinil is commonly used to improve idiopathic pulmonary arterial hypertension in children. However, its effectiveness has not been reported in trisomy 21. We report the case of 9-year-old boy in trisomy 21 with CHD-pulmonary artery hypertension after surgical correction

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Outpatient prescription of pulmonary vasodilator therapy to preterm children with bronchopulmonary dysplasia

Ida Jeremiasen, Karin Tran-Lundmark, Mikaela Dolk, Estelle NaumburgLund University and Skåne University Hospital. Umeå University.Sweden Acta PaediatricaActa Paediatr 2023; 112: 409-416DOI: 10.1111/apa.16615 Abstract Aim: The use of pulmonary vasodilator therapy in children born preterm is largely unknown. Our aim was to map prescription patterns in children with bronchopulmonary dysplasia in Sweden.Methods: This was a descriptive national registry-based study

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Efficacy of sildenafil and high-dose anakinra in an MIS-C patient with pulmonary vasculitis: A case report

Francesco La Torre, Gerolmina Calabrese, Katia Signorile, Francesca Bizzoco, Carla Mastrorilli, Antonella Strippoli, Doriana Amato, Francesco Carella, Ugo Vairo, Paola Giordano, Leonardo Milella, Fabio CardinaleGiovanni XXIII Pediatric Hospital and University of Bari. Italy Frontiers in PediatricsFront Pediatr 2023; DOI: 10.3389/fped.2022.1015617 AbstractMultisystem inflammatory syndrome in children (MIS-C) is a newly identified clinical entity still not very well

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Worsened short-term clinical outcomes in a cohort of patients with iNO-unresponsive PPHN: a case for improving iNO responsiveness

Julie Dillard, Leeann R. Pavlek, Saichidroopi Korada, Bernadette ChenSt. Louis University. Nationwide Children’s Hospital and Ohio State University.United States Journal of PerinatologyJ Perinatol 2022; 42: 37-44DOI: 10.1038/s41372-021-01228-x AbstractObjectives: To identify distinguishing characteristics of neonates with persistent pulmonary hypertension of the newborn (PPHN) unresponsive to inhaled nitric oxide (iNO) and evaluate the use of milrinone in this cohort.Study

Worsened short-term clinical outcomes in a cohort of patients with iNO-unresponsive PPHN: a case for improving iNO responsiveness Read More »

Adenosine reverses life-threatening persistent pulmonary hypertension of the neonate refractory to triple vasodilator therapy

Lucie Genet, Daniele De Luca“A.Béclère” Medical Center and Paris Saclay University Hospitals.France Cardiology in the YoungCardiol Young 2022; 32: 996-997DOI: 10.1017/S1047951121004157 AbstractPersistent pulmonary hypertension of the neonate can cause acute and life-threatening hypoxia, but preterm neonates are not suitable candidate to extra-corporeal life support. We report the unique case of an extremely preterm neonates with life-threatening

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Effects of inhaled nitric oxide (iNO) in pulmonary hypertension secondary to arteriovenous malformations: a retrospective cohort study from the European iNO registry

Aravanan Anbu Chakkarapani, Samir Gupta, Asma Jamil, Santosh Kumar Yadav, Nim Subhedar, Helmut D. HummlerSidra Medicine. Weill Cornell Medicine-Qatar. Durham University. Johns Hopkins University School of Medicine. Liverpool Women’s Hospital. University of Tuebingen.Qatar, United Kingdom, United States and Germany European Journal of PediatricsEur J Pediatr 2022; 181: 3915-3922DOI: 10.1007/s00431-022-04602-9 AbstractThis study aims to assess the effects of inhaled nitric oxide (iNO) on oxygenation in the

Effects of inhaled nitric oxide (iNO) in pulmonary hypertension secondary to arteriovenous malformations: a retrospective cohort study from the European iNO registry Read More »

Randomized Clinical and Biochemical Study Comparing the Effect of L-arginine and Sildenafil in Beta Thalassemia Major Children With High Tricuspid Regurgitant Jet Velocity

Eman El-Khateeb, Sahar Mohamed El-Haggar, Osama El-Razaky, Mohamed Ramadan El-Shanshory, Tarek Mohamed Mostafa68904Tanta University.United Kingdom and Egypt Journal of Cardiovascular Pharmacology and TherapeuticsJ Cardiovasc Pharmacol Ther 2022; DOI: 10.1177/10742484221132671 AbstractBackground: Pulmonary hypertension (PHT) is common in β-thalassemia patients due to hemolysis, iron overload and diminished nitric oxide (NO) levels. Biochemical markers can help to understand the pathophysiology and to introduce new therapies for this condition.Aim: This study

Randomized Clinical and Biochemical Study Comparing the Effect of L-arginine and Sildenafil in Beta Thalassemia Major Children With High Tricuspid Regurgitant Jet Velocity Read More »

Use of vasopressin in persistent pulmonary hypertension of the newborn: A case series

Swosti Joshi, Vilmaris Quinones Cardona, Ogechukwu R. MenkitiDrexel University College of Medicine and St. Christopher’s Hospital for Children.United States SAGE Medical Case ReportsSAGE Med Cas Rep 2022; DOI: 10.1177/2050313X221102289 AbstractTreatment of neonates with persistent pulmonary hypertension of newborn includes optimization of ventilatory support, use of pulmonary vasodilators, and/or inotropic support. If refractory to this management, some

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Acute Pulmonary Vasodilator Testing and Long-Term Clinical Course in Segmental Pulmonary Vascular Disease

Liezl Domingo, H. Sonali Magdo, Ronald W. DayUniversity of Utah and Primary Children’s Hospital.United States Pediatric CardiologyPediatr Cardiol 2018; 39: 501-508DOI: 10.1007/s00246-017-1780-9 AbstractResults of acute pulmonary vasodilator testing (AVT) and the outcome of medical therapy have not been described in patients with segmental pulmonary vascular disease (SPVD). We sought to compare the pulmonary vasodilatory effects of

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