Transition from intravenous treprostinil to enteral selexipag in an infant with pulmonary arterial hypertension

2019 ◽  
Vol 29 (06) ◽  
pp. 849-851 ◽  
Author(s):  
Rachel Koo ◽  
Jennifer Lo ◽  
Matthew J. Bock
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Adverse Effects ◽  
Arterial Hypertension ◽  
Receptor Agonist ◽  
Ip Receptor ◽  
Pulmonary Arterial

AbstractSelexipag is an enteral, selective prostacyclin IP receptor agonist approved for pulmonary hypertension in adults. There are few reports of its use in children and none in infants. We report the first transition of an infant (11.5 months, 8.6 kg) from intravenous treprostinil (40 ng/kg/minute) to enteral selexipag (400 mcg twice daily) with a good response and no adverse effects.

scholarly journals Hemodynamic Effects of the Oral Prostacyclin (IP) Receptor Agonist Ralinepag in Pulmonary Arterial Hypertension

CHEST Journal ◽  
2017 ◽  
Vol 152 (4) ◽  
pp. A738
Author(s):  
Fernando Torres ◽  
Harrison Farber ◽  
Arsen Ristić ◽  
Vallerie McLaughlin ◽  
John Adams ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Receptor Agonist ◽  
Hemodynamic Effects ◽  
Ip Receptor ◽  
Pulmonary Arterial

scholarly journals Should we use the oral selective IP receptor agonist selexipag off-label in children with pulmonary arterial hypertension?

2018 ◽  
Vol 8 (3) ◽  
pp. 204589401879358 ◽  
Author(s):  
Martin Koestenberger ◽  
Georg Hansmann
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Children And Adolescents ◽  
Receptor Agonist ◽  
Off Label ◽  
Ip Receptor ◽  
Targeted Agent ◽  
Pulmonary Arterial ◽  
Prostacyclin Analog

We discuss the currently available data on the use of the prostacyclin mimetic selexipag in children and adolescents with pulmonary arterial hypertension (PAH). Future indications may include transitioning from intravenous prostacyclin/prostacyclin analog to oral selexipag, and vice versa, or adding selexipag as a third oral PAH-targeted agent in children not responding well to dual PAH therapy.

Pulmonary Arterial Hypertension: Epidemiology and Registries

2014 ◽  
Vol 13 (1) ◽  
pp. 21-26 ◽  
Author(s):  
Michael D. McGoon ◽  
Marc Humbert
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Task Force ◽  
The World ◽  
Important Means ◽  
Pulmonary Arterial

Registries of pulmonary arterial hypertension (PAH) are important means by which to characterize the presentation and outcome of patients and to provide a basis for predicting the course of the disease. This article summarizes the published conclusions of the World Symposium of Pulmonary Hypertension task force that addressed registries and epidemiology of PAH.

scholarly journals Heart and lung transplantation in pulmonary arterial hypertension related to congenital heart disease: an unusual indication

2020 ◽  
Vol 4 (S1) ◽  
Author(s):  
Rosaria Barracano ◽  
Heba Nashat ◽  
Andrew Constantine ◽  
Konstantinos Dimopoulos
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Lung Transplantation ◽  
Atrial Septal Defect ◽  
Septal Defect ◽  
Pulmonary Vascular Disease ◽  
Eisenmenger Syndrome ◽  
Pulmonary Arterial ◽  
Recurrent Haemoptysis

Abstract Background Eisenmenger syndrome is a multisystem disorder, characterised by a significant cardiac defect, severe pulmonary hypertension and long-standing cyanosis. Despite the availability of pulmonary hypertension therapies and improved supportive care in specialist centres, Eisenmenger patients are still faced with significant morbidity and mortality. Case presentation We describe the case of a 44-year-old woman with Eisenmenger syndrome secondary to a large secundum atrial septal defect. Her pulmonary vascular disease was treated with pulmonary vasodilators, but she experienced a progressive decline in exercise tolerance, increasing atrial arrhythmias, resulting in referral for transplantation. Her condition was complicated by significant recurrent haemoptysis in the context of extremely dilated pulmonary arteries and in-situ thrombosis, which prompted successful heart and lung transplantation. She made a slow recovery but remains well 3 years post-transplant. Conclusions Patients with Eisenmenger syndrome secondary to a pre-tricuspid lesion, such as an atrial septal defect have a natural history that differs to patients with post-tricuspid shunts; the disease tends to present later in life but is more aggressive, prompting early and aggressive medical intervention with pulmonary arterial hypertension therapies. This case illustrates that severe recurrent haemoptysis can be an indication for expediting transplantation in Eisenmenger syndrome patients.

scholarly journals Sex Dimorphism in Pulmonary Hypertension: The Role of the Sex Chromosomes

Antioxidants ◽  
2021 ◽  
Vol 10 (5) ◽  
pp. 779
Author(s):  
Daria S. Kostyunina ◽  
Paul McLoughlin
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Pulmonary Vascular Resistance ◽  
Vascular Resistance ◽  
Sex Chromosomes ◽  
Bone Morphogenetic Protein 2 ◽  
Sex Dimorphism ◽  
Pulmonary Arterial ◽  
The Impact

Pulmonary hypertension (PH) is a condition characterised by an abnormal elevation of pulmonary artery pressure caused by an increased pulmonary vascular resistance, frequently leading to right ventricular failure and reduced survival. Marked sexual dimorphism is observed in patients with pulmonary arterial hypertension, a form of pulmonary hypertension with a particularly severe clinical course. The incidence in females is 2–4 times greater than in males, although the disease is less severe in females. We review the contribution of the sex chromosomes to this sex dimorphism highlighting the impact of proteins, microRNAs and long non-coding RNAs encoded on the X and Y chromosomes. These genes are centrally involved in the cellular pathways that cause increased pulmonary vascular resistance including the production of reactive oxygen species, altered metabolism, apoptosis, inflammation, vasoconstriction and vascular remodelling. The interaction with genetic mutations on autosomal genes that cause heritable pulmonary arterial hypertension such as bone morphogenetic protein 2 (BMPR2) are examined. The mechanisms that can lead to differences in the expression of genes located on the X chromosomes between females and males are also reviewed. A better understanding of the mechanisms of sex dimorphism in this disease will contribute to the development of more effective therapies for both women and men.

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