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.

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.

scholarly journals Use of Sildenafil in Pulmonary Arterial Hypertension: Findings from a U.S. Healthcare Claims Database

10.36469/9871 ◽  
2014 ◽  
Vol 1 (3) ◽  
pp. 254-265 ◽  
Author(s):  
Ariel Berger ◽  
John Edelsberg ◽  
Simon Teal ◽  
Marko A. Mychaskiw ◽  
Gerry Oster
Keyword(s):  
United States ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Medication Possession Ratio ◽  
The United States ◽  
The European Union ◽  
Claims Database ◽  
Pharmacy Claims ◽  
Pulmonary Arterial

Background: Pulmonary arterial hypertension (PAH) is a disease characterized by dyspnea, fatigue, chest pain and syncope. As there is no known cure for PAH, the goal of treatment is to control symptoms and slow disease progression. Sildenafil, a phosphodiesterase-5 inhibitor, has been indicated to improve exercise capacity in PAH in both the United States and the European Union since 2005; since 2009, it also has been indicated in the United States to delay clinical worsening. Patterns of sildenafil use in PAH patients have not been reported. Objectives: To describe patterns of treatment with sildenafil among commercially insured patients in the United States with PAH. Methods: Using a large U.S. healthcare claims database, we identified all patients with evidence of PAH (International Classification of Disease, 9th Revision, Clinical Modification [ICD-9-CM] diagnosis codes 416.0, 416.8) and receipt of sildenafil between January 1, 2005 and September 30, 2008. The date of each patient’s earliest pharmacy claim for sildenafil was designated as his or her “index date”; patients with <6 months of data prior to this date were excluded. Post-index use of sildenafil was then examined in terms of the numbers of pharmacy claims and therapy-days, the medication possession ratio (MPR), and the incidence of therapy switching. Results: We identified a total of 855 PAH patients who began sildenafil therapy and met all other entry criteria. Mean (standard deviation [SD]) follow-up was 423.4 (313.0) days. Over this period, these patients averaged 7.1 (6.8) (median, 5) pharmacy dispensings for sildenafil, representing 273.4 (254.8) therapy-days (median, 180). Mean MPR was 71% (median, 83%). Fourteen percent of sildenafil patients switched to another agent during follow-up. Conclusions: In “real-world” clinical practice, many PAH patients beginning treatment with sildenafil remain on therapy for extended periods and are relatively compliant with treatment.

scholarly journals Managing the Patient With Pulmonary Arterial Hypertension and Methamphetamine Use: A Practical Perspective for the Clinician

2018 ◽  
Vol 17 (2) ◽  
pp. 55-62 ◽  
Author(s):  
Nimaljeet Tarango ◽  
Andrea Gergay Baird
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
San Francisco ◽  
Medical Center ◽  
The United States ◽  
Methamphetamine Abuse ◽  
Methamphetamine Use ◽  
Personal Experiences ◽  
History Of ◽  
Pulmonary Arterial

Pulmonary arterial hypertension (PAH) is a serious, chronic, progressive cardiopulmonary disease. PAH is associated with several concomitant conditions, as well as drugs and toxins.12 Methamphetamine abuse is likely associated with the development of PAH.3 Methamphetamine abuse is epidemic in the United States and abroad, with rates of new users escalating since 2012. There are over 100,000 new users annually as young as 12 years old. Treating a patient with a history of methamphetamine abuse poses many challenges for a clinician, including nonadherence, therapeutic treatment selection, complex psychosocial issues, and relapse or continued drug abuse. Patients with methamphetamine-associated PAH (Meth-APAH) have higher mortality rates when compared to idiopathic PAH.3 Having a better understanding of the complexities of addiction and working with a multidisciplinary team that includes a social worker to provide care and counseling to these patients can improve their trajectory. In this article, we will offer insight and background into methamphetamine abuse and addiction, as well as discuss a practical approach for clinicians in treating a patient with Meth-APAH, based on the literature, as well as our personal experiences at University of California, San Francisco Medical Center.

scholarly journals The Burden of Illness of Pulmonary Arterial Hypertension: A Managed Care Perspective in the United States

2013 ◽  
Vol 16 (7) ◽  
pp. A521
Author(s):  
V.N. Joish ◽  
D. Muccino ◽  
C. Kreilick ◽  
R. Kamalakar ◽  
J. Yao ◽  
...  
Keyword(s):  
United States ◽  
Pulmonary Arterial Hypertension ◽  
Managed Care ◽  
Arterial Hypertension ◽  
Burden Of Illness ◽  
The United States ◽  
Pulmonary Arterial

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.

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