scholarly journals Transitions between infused and oral prostacyclin pathway agents in pulmonary arterial hypertension: key considerations

2020 ◽  
Vol 10 (3) ◽  
pp. 204589402093132
Author(s):  
Therese Sargent ◽  
Lillian Hansen ◽  
Robin Hohsfield
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Patient Selection ◽  
Side Effect ◽  
Case Reports ◽  
Optimal Dose ◽  
Clinical Guidance ◽  
Pulmonary Arterial

Prostacyclin pathway agents are a critical treatment for patients with pulmonary arterial hypertension. Seven prostacyclin pathway agents are available, including agents administered by parenteral infusion, by inhalation, and orally. Pulmonary arterial hypertension patients are now transitioned from one prostacyclin pathway agent to another with increasing frequency. Such transitions require careful downtitration and uptitration to avoid decompensation from rapid withdrawal and to achieve a patient's optimal dose based on efficacy and tolerability. Clinical guidance is especially lacking for transitions involving the newer, oral prostacyclin pathway agents; specifically, selexipag and oral treprostinil. We present three case reports of patients with pulmonary arterial hypertension who underwent one or more transition between parenteral and oral prostacyclin pathway agents, including some transitions that were successful and some that were not. These cases illustrate key considerations, such as titration protocols, patient selection, side effect management, and pharmacokinetics.

scholarly journals Impact of Nutrition on Pulmonary Arterial Hypertension

Nutrients ◽  
2020 ◽  
Vol 12 (1) ◽  
pp. 169
Author(s):  
María Callejo ◽  
Joan Albert Barberá ◽  
Juan Duarte ◽  
Francisco Perez-Vizcaino
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Animal Models ◽  
Gut Microbiota ◽  
Arterial Hypertension ◽  
Case Reports ◽  
Host Immune System ◽  
Dietary Polyphenols ◽  
Pulmonary Arterial ◽  
Near Future

Pulmonary arterial hypertension (PAH) is characterized by sustained vasoconstriction, vascular remodeling, inflammation, and in situ thrombosis. Although there have been important advances in the knowledge of the pathophysiology of PAH, it remains a debilitating, limiting, and rapidly progressive disease. Vitamin D and iron deficiency are worldwide health problems of pandemic proportions. Notably, these nutritional alterations are largely more prevalent in PAH patients than in the general population and there are several pieces of evidence suggesting that they may trigger or aggravate disease progression. There are also several case reports associating scurvy, due to severe vitamin C deficiency, with PAH. Flavonoids such as quercetin, isoflavonoids such as genistein, and other dietary polyphenols including resveratrol slow the progression of the disease in animal models of PAH. Finally, the role of the gut microbiota and its interplay with the diet, host immune system, and energy metabolism is emerging in multiple cardiovascular diseases. The alteration of the gut microbiota has also been reported in animal models of PAH. It is thus possible that in the near future interventions targeting the nutritional status and the gut dysbiosis will improve the outcome of these patients.

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.

Ambulatory Hemodynamic Monitoring in the Management of Pulmonary Arterial Hypertension

2014 ◽  
Vol 13 (2) ◽  
pp. 81-85 ◽  
Author(s):  
Amresh Raina ◽  
Raymond L. Benza
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Case Reports ◽  
Right Heart Failure ◽  
Ambulatory Setting ◽  
Heart Catheterization ◽  
Right Heart ◽  
Small Series ◽  
Hemodynamic Assessment ◽  
Pulmonary Arterial

Background: Pulmonary arterial hypertension (PAH) is characterized by pulmonary vascular remodeling, rise in pulmonary arterial pressures, and if left untreated, right heart failure. Invasive hemodynamic assessment with right heart catheterization (RHC) has been the gold standard for the diagnosis and serial assessment of patients with PAH. However, RHC has important limitations and might be supplemented by newer technologies in the management of PAH patients. Implications for Clinicians: Implantable hemodynamic monitors (IHM) hold the promise of being able to provide accurate pulmonary artery pressure measurements, with frequent or continuous remote monitoring in the home or ambulatory setting. As such, IHMs may provide a more complete understanding of a patient's hemodynamic profile and burden of disease. IHM data may also help to provide ongoing feedback in terms of a PAH patient's response to medical therapy and other interventions and might be valuable in specific subsets of patients with borderline or exercise-induced PAH. Conclusions: Though clinical studies using IHMs in PAH patients have been limited to small series and case reports, these devices hold a great deal of promise to supplement RHC in the management of PAH patients and warrant further investigation and clinical experience.

scholarly journals Clinical trial design and new therapies for pulmonary arterial hypertension

2019 ◽  
Vol 53 (1) ◽  
pp. 1801908 ◽  
Author(s):  
Olivier Sitbon ◽  
Mardi Gomberg-Maitland ◽  
John Granton ◽  
Michael I. Lewis ◽  
Stephen C. Mathai ◽  
...  
Keyword(s):  
Clinical Trial ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Task Force ◽  
Case Reports ◽  
Clinical Trial Design ◽  
Epidemiological Studies ◽  
Novel Therapies ◽  
Small Series ◽  
Pulmonary Arterial

Until 20 years ago the treatment of pulmonary arterial hypertension (PAH) was based on case reports and small series, and was largely ineffectual. As a deeper understanding of the pathogenesis and pathophysiology of PAH evolved over the subsequent two decades, coupled with epidemiological studies defining the clinical and demographic characteristics of the condition, a renewed interest in treatment development emerged through collaborations between international experts, industry and regulatory agencies. These efforts led to the performance of robust, high-quality clinical trials of novel therapies that targeted putative pathogenic pathways, leading to the approval of more than 10 novel therapies that have beneficially impacted both the quality and duration of life. However, our understanding of PAH remains incomplete and there is no cure. Accordingly, efforts are now focused on identifying novel pathogenic pathways that may be targeted, and applying more rigorous clinical trial designs to better define the efficacy of these new potential treatments and their role in the management scheme. This article, prepared by a Task Force comprised of expert clinicians, trialists and regulators, summarises the current state of the art, and provides insight into the opportunities and challenges for identifying and assessing the efficacy and safety of new treatments for this challenging condition.

scholarly journals Incident pulmonary arterial hypertension associated with Bosutinib

2020 ◽  
Vol 10 (3) ◽  
pp. 204589402093691 ◽  
Author(s):  
Shaun Yo ◽  
John Thenganatt ◽  
Jeffrey Lipton ◽  
John Granton
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Chronic Myeloid Leukemia ◽  
Tyrosine Kinase ◽  
Arterial Hypertension ◽  
Myeloid Leukemia ◽  
Kinase Inhibitors ◽  
Case Reports ◽  
Drug Induced ◽  
Pulmonary Arterial ◽  
Partial Reversal

Pulmonary arterial hypertension is associated with tyrosine kinase inhibitors used in the treatment of chronic myeloid leukemia. Dasatinib is a known cause of drug-induced pulmonary arterial hypertension. There have been case reports linking Bosutinib with deterioration of pre-existing pulmonary arterial hypertension. Here, we present a case of a 37-year-old woman with chronic myeloid leukemia treated with Bosutinib who was diagnosed with pulmonary arterial hypertension. Prior to Bosutinib, she had received Dasatinib without documented cardiopulmonary toxicity. Withdrawal of Bosutinib led to partial reversal of pulmonary arterial hypertension, and with the addition of pulmonary arterial hypertension-targeted treatment, there was near normalization of hemodynamics.

Successful treatment with sildenafil in systemic sclerosis patients with isolated pulmonary arterial hypertension: two case reports

2005 ◽  
Vol 26 (3) ◽  
pp. 270-273 ◽  
Author(s):  
Ikuko Hayakawa ◽  
Fumiaki Shirasaki ◽  
Takashi Hirano ◽  
Naoto Oishi ◽  
Minoru Hasegawa ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Successful Treatment ◽  
Case Reports ◽  
Pulmonary Arterial
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