Comparison of the Therapeutic and Side Effects of Tadalafil and Sildenafil in Children and Adolescents with Pulmonary Arterial Hypertension

2013 ◽  
Vol 35 (4) ◽  
pp. 699-704 ◽  
Author(s):  
Mohammad Reza Sabri ◽  
Elham Beheshtian
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Side Effects ◽  
Arterial Hypertension ◽  
Children And Adolescents ◽  
Pulmonary Arterial

scholarly journals Contemporary use of Selexipag in pulmonary arterial hypertension associated with congenital heart disease: a case series

2020 ◽  
Author(s):  
Sarah Blissett ◽  
David Blusztein ◽  
Vaikom S Mahadevan
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Pulmonary Arterial Hypertension ◽  
Side Effects ◽  
Arterial Hypertension ◽  
Exercise Capacity ◽  
Congenital Heart ◽  
Pulmonary Veins ◽  
Case Series ◽  
Pulmonary Arterial

Abstract Background There are significant risks of parenteral prostacyclin use in patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD), which may limit their use. Selexipag is an oral, selective prostacyclin analogue that has been shown to reduce disease progression and improve exercise capacity in patients with PAH-CHD. Administering Selexipag in patients with PAH-CHD could potentially overcome some of the risks of parenteral therapy while improving clinical outcomes. Case summary We report five cases highlighting the clinical uses of Selexipag in patients with PAH-CHD. In the first two cases, Selexipag was initiated as part of a Treat-to-close strategy. In the third case, initiation of Selexipag improved symptoms and objective exercise capacity in a patient with Eisenmenger syndrome. In the fourth and fifth cases, rapid cross-titration protocols were used to transition from parenteral prostacyclins to Selexipag. In the fourth case, Selexipag was initiated in the context of significant side effects limiting parenteral prostacyclin use. In the fifth case, Selexipag was used to down-titrate from parenteral prostacyclins following closure of a sinus venosus atrial septal defect and redirection of anomalous pulmonary veins. Discussion Selexipag is a promising oral therapy for patients with at various stages of the spectrum of PAH-CHD to improve symptoms, exercise capacity and, in some cases, haemodynamics. Our cases also highlight practical aspects of Selexipag use including targeting the individualized maximally tolerated dose for each patient, managing side effects and managing dose interruptions.

Safety and Long-Term Efficacy of Transition from Sildenafil to Tadalafil due to Side Effects in Patients with Pulmonary Arterial Hypertension

Lung ◽  
2014 ◽  
Vol 193 (1) ◽  
pp. 105-112 ◽  
Author(s):  
Mona Lichtblau ◽  
Dominik Harzheim ◽  
Nicola Ehlken ◽  
Alberto Marra ◽  
Fabiola Pena Pinado ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Side Effects ◽  
Arterial Hypertension ◽  
Term Efficacy ◽  
Pulmonary Arterial

scholarly journals Chemical and biological assessment of metal organic frameworks (MOFs) in pulmonary cells and in an acute in vivo model: relevance to pulmonary arterial hypertension therapy

2017 ◽  
Vol 7 (3) ◽  
pp. 643-653 ◽  
Author(s):  
Nura A. Mohamed ◽  
Robert P. Davies ◽  
Paul D. Lickiss ◽  
Blerina Ahmetaj-Shala ◽  
Daniel M. Reed ◽  
...  
Keyword(s):  
Endothelial Cells ◽  
Pulmonary Arterial Hypertension ◽  
Side Effects ◽  
Arterial Hypertension ◽  
Therapeutic Window ◽  
Endothelin 1 ◽  
Wide Range ◽  
Metal Organic ◽  
Pulmonary Arterial

Pulmonary arterial hypertension (PAH) is a progressive and debilitating condition. Despite promoting vasodilation, current drugs have a therapeutic window within which they are limited by systemic side effects. Nanomedicine uses nanoparticles to improve drug delivery and/or reduce side effects. We hypothesize that this approach could be used to deliver PAH drugs avoiding the systemic circulation. Here we report the use of iron metal organic framework (MOF) MIL-89 and PEGylated MIL-89 (MIL-89 PEG) as suitable carriers for PAH drugs. We assessed their effects on viability and inflammatory responses in a wide range of lung cells including endothelial cells grown from blood of donors with/without PAH. Both MOFs conformed to the predicted structures with MIL-89 PEG being more stable at room temperature. At concentrations up to 10 or 30 µg/mL, toxicity was only seen in pulmonary artery smooth muscle cells where both MOFs reduced cell viability and CXCL8 release. In endothelial cells from both control donors and PAH patients, both preparations inhibited the release of CXCL8 and endothelin-1 and in macrophages inhibited inducible nitric oxide synthase activity. Finally, MIL-89 was well-tolerated and accumulated in the rat lungs when given in vivo. Thus, the prototypes MIL-89 and MIL-89 PEG with core capacity suitable to accommodate PAH drugs are relatively non-toxic and may have the added advantage of being anti-inflammatory and reducing the release of endothelin-1. These data are consistent with the idea that these materials may not only be useful as drug carriers in PAH but also offer some therapeutic benefit in their own right.

scholarly journals Management of prostacyclin side effects in adult patients with pulmonary arterial hypertension

2017 ◽  
Vol 7 (3) ◽  
pp. 598-608 ◽  
Author(s):  
Martha Kingman ◽  
Christine Archer-Chicko ◽  
Mary Bartlett ◽  
Joy Beckmann ◽  
Robin Hohsfield ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Side Effects ◽  
Arterial Hypertension ◽  
Side Effect ◽  
The United States ◽  
Management Program ◽  
Collaborative Effort ◽  
Health Providers ◽  
Extensive Experience ◽  
Pulmonary Arterial

Therapies that target the prostacyclin pathway are considered effective, yet are complex to dose and may cause dose-limiting side effects for patients with pulmonary arterial hypertension (PAH). Careful side effect management and the ability to discern side effects from worsening disease are essential in order for patients to continue, and benefit from, prostacyclin therapy. This manuscript was developed through a collaborative effort of allied health providers with extensive experience in managing patients with PAH who are treated with medications that target the prostacyclin pathway. This article provides an overview of individual prostacyclin pathway therapies approved in the United States, side effects most commonly associated with these therapies, and practical suggestions for side effect management. Most patients will experience significant side effects on prostacyclin therapy. Creating a proactive and careful side effect management program will increase the likelihood that patients are able to stay on therapy and receive the benefits afforded by prostacyclin therapy.

Home Exercise Training in Children and Adolescents with Pulmonary Arterial Hypertension: A Pilot Study

2016 ◽  
Vol 38 (1) ◽  
pp. 191-198 ◽  
Author(s):  
David Zöller ◽  
Jannos Siaplaouras ◽  
Anita Apitz ◽  
Peter Bride ◽  
Michael Kaestner ◽  
...  
Keyword(s):  
Pilot Study ◽  
Pulmonary Arterial Hypertension ◽  
Exercise Training ◽  
Arterial Hypertension ◽  
Children And Adolescents ◽  
Home Exercise ◽  
Pulmonary Arterial

scholarly journals Selexipag treatment in patients with systemic sclerosis–associated pulmonary arterial hypertension in clinical practice, a case series

2020 ◽  
Vol 5 (3) ◽  
pp. NP7-NP11
Author(s):  
Jacqueline MJ Lemmers ◽  
Håvard Fretheim ◽  
Hanneke KA Knaapen ◽  
Frank HJ van den Hoogen ◽  
Jolanda HGM van Haren-Willems ◽  
...  
Keyword(s):  
Risk Assessment ◽  
Systemic Sclerosis ◽  
Pulmonary Arterial Hypertension ◽  
Side Effects ◽  
Arterial Hypertension ◽  
Interquartile Range ◽  
Walking Distance ◽  
Expert Team ◽  
Pulmonary Arterial

Objective: To describe the efficacy and safety in all patients with systemic sclerosis–associated pulmonary arterial hypertension who started selexipag between 09-2016 and 06-2018 in two pulmonary arterial hypertension expert centers. Methods: All patients with systemic sclerosis–associated pulmonary arterial hypertension diagnosed by right heart catheterization and treated with selexipag were included. Every 12 weeks, treatment effect was assessed by (1) the opinion of the expert team and (2) the abbreviated risk assessment, consisting of functional class, six-minute walking distance, and N-terminal prohormone of brain natriuretic peptide level at baseline and during follow-up. Side effects and adverse events were registered. Results: We included 13 systemic sclerosis–associated pulmonary arterial hypertension patients, 10 patients were female, median age (interquartile range) of 68 (58–75) years, median systemic sclerosis disease duration of 7.4 (4.7–13.5) years, and median pulmonary arterial hypertension duration of 4 (2.5–7.5) years. Two patients discontinued selexipag within 4 weeks due to side effects. The remaining 11 patients had a median follow-up duration of 48 (interquartile range = 24–72) weeks. Two patients died (one pulmonary arterial hypertension–related, the other systemic sclerosis–related). According to the expert team, 8 of 11, 9 of 10, and 5 of 7 patients stabilized or improved at 12, 24, and 48 weeks, respectively. According to the abbreviated risk assessment at study end, 3 of 11 patients had 1 low-risk criterion. No previously unrecorded side effects were reported. Conclusion: Adding selexipag to background therapy in a high-risk cohort of systemic sclerosis–associated pulmonary arterial hypertension patients provided sustained stabilization of symptoms with an acceptable safety profile. Improvement was reached in only two of our patients. Further research should focus on systemic sclerosis–associated pulmonary arterial hypertension patients treated with multiple targeted treatments, preferably these patients should be prospectively followed in international registries.

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