Practical considerations for therapies targeting the prostacyclin pathway

Therapies that target the prostacyclin pathway play a key role in the treatment of both early- and late-stage pulmonary arterial hypertension, and provide significant clinical benefits for patients. A number of agents have been approved, which are administeredviaintravenous, subcutaneous, inhaled or oral routes. The use of these therapies is associated with practical challenges, relating to the need for up-titration and their routes of administration. We discuss here a number of measures that can be taken to support patients and healthcare professionals in order to address the complexities of using these therapies and to encourage compliance. Providing patients with timely information and education, together with practical advice on managing their medication and associated equipment, assists patients with day-to-day management of therapy. Referral to patient associations and support groups can be of further benefit. With an effective management plan and an experienced multidisciplinary team, the use of therapies that target the prostacyclin pathway can be optimised.

Download Full-text

Use of eHealth in the management of pulmonary arterial hypertension: review of the literature

BMJ Health & Care Informatics ◽

10.1136/bmjhci-2020-100176 ◽

2020 ◽

Vol 27 (3) ◽

pp. e100176

Author(s):

Manuel C Gonzalez-Garcia ◽

Farhad Fatehi ◽

Marlien Varnfield ◽

Hang Ding ◽

Mohan Karunanithi ◽

...

Keyword(s):

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Small Sample Size ◽

Survival Rates ◽

Poor Quality ◽

Small Sample ◽

Haemodynamic Monitoring ◽

Clinical Benefits ◽

Pulmonary Arterial ◽

Diagnostic Technologies

BackgroundPulmonary arterial hypertension (PAH) is a severe chronic condition associated with poor quality of life and high risks of mortality and hospitalisation. The utilisation of novel diagnostic technologies has improved survival rates although the effectiveness of Electronic Health (eHealth) interventions in patients with a chronic cardiopulmonary disease remains controversial. As the effectiveness of eHealth can be established by specific evaluation for different chronic health conditions, the aim of this study was to explore and summarise the utilisation of eHealth in PAH.MethodWe searched PubMed, CINAHL and Embase for all studies reporting clinical trials on eHealth solutions for the management of PAH. No limitations in terms of study design or date of publication were imposed.Results18 studies (6 peer-reviewed journal papers and 12 conference papers) were identified. Seven studies addressed the accuracy, safety or reliability of eHealth technologies such as intra-arterial haemodynamic monitoring of the pulmonary artery pressure, self-administered 6-Minute walk test App, computerised step-pulse oximeter and ambulatory impedance cardiography. Two studies evaluated eHealth as part of the medical management and showed a reduction in hospitalisation rate.ConclusionsThe evidence of eHealth supporting the management of people with PAH is limited and only embraced through a few studies of small sample size and short-term duration. Given the proposed clinical benefits in heart failure, we postulate that the evaluation of eHealth for the clinical management of PAH is highly warranted.

Download Full-text

229: Clinical Benefits of Pharmacological Therapy in Exercise Induced Pulmonary Arterial Hypertension

The Journal of Heart and Lung Transplantation ◽

10.1016/j.healun.2008.11.859 ◽

2009 ◽

Vol 28 (2) ◽

pp. S146

Author(s):

M.H. Park ◽

G.V. Ramani ◽

W.J. Kop ◽

P.A. Uber ◽

M.R. Mehra

Keyword(s):

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Pharmacological Therapy ◽

Clinical Benefits ◽

Pulmonary Arterial ◽

Exercise Induced

Download Full-text

Information Experiences and Needs in Patients with Pulmonary Arterial Hypertension or Chronic Thromboembolic Pulmonary Hypertension

Nursing Research and Practice ◽

10.1155/2014/704094 ◽

2014 ◽

Vol 2014 ◽

pp. 1-8 ◽

Cited By ~ 2

Author(s):

Bodil Ivarsson ◽

Björn Ekmehag ◽

Trygve Sjöberg

Keyword(s):

Pulmonary Hypertension ◽

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Healthcare Professionals ◽

Healthcare Providers ◽

Chronic Thromboembolic Pulmonary Hypertension ◽

Psychosocial Problems ◽

Cost Effective ◽

Thromboembolic Pulmonary Hypertension ◽

Pulmonary Arterial

Background. Pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) are fatal, noncurable, but treatable diseases that strongly affect the patients.Objective. To describe patients’ experience of information relating to PAH or CTEPH.Methods. A qualitative method using content analysis was applied. Seventeen patients (thirteen women and four men) aged 28–73 years from a regional PAH centre were individually interviewed.Results. Three categories that describe patients’ experiences of information emerged: handling of information, struggling with feelings that also affect others, and vulnerability associated with uncertainty. The patients would have welcomed more information to relatives from the healthcare professionals. Shortcomings on communicating a prognosis were experienced. The mediated information and knowledge gave the patients insight into physical or psychosocial problems. Mutual exchange of information between patients and healthcare professionals were marred by different experiences of attitudes, behaviour, and ownership.Conclusions. In the future, healthcare organizations must struggle to achieve a holistic healthcare by making it more person-centred, and they must also promote cooperation between PAH centres and local healthcare providers. It is essential to determine the most appropriate and valuable path of information and communication and, thereby, the most cost-effective management of PAH or CTEPH.

Download Full-text

When to Start a Prostanoid in Pulmonary Arterial Hypertension Patients

European Cardiology Review ◽

10.15420/ecr.2012.8.3.204 ◽

2012 ◽

Vol 8 (3) ◽

pp. 204

Author(s):

Eva Goncalvesova ◽

Keyword(s):

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Oral Treatment ◽

Open Label ◽

Term Survival ◽

Treatment Delays ◽

First Line Therapy ◽

Clinical Benefits ◽

Pulmonary Arterial

Pulmonary arterial hypertension (PAH) is a progressive and debilitating disease with poor prognosis and limited therapeutic options. While currently available PAH treatments can provide substantial clinical benefits, they are not curative. The efficacy of prostanoid treatment in PAH is well-established in clinical trials, open-label and long-term observational studies, and prostanoid therapy is considered the most effective treatment for moderate to severe PAH. However, a growing body of data demonstrates that the majority of PAH patients are not treated with a prostanoid, even at the time of their death. The logistics of parenteral administration have limited prostanoid use in clinical practice in some centres; the lure of using a simple oral therapy can be compelling for both the physician and the patient. However, deterioration on oral therapies appears inevitable and therefore, there is a need to initiate early prostanoid therapy in eligible patients and to overcome barriers to their use. Oral treatment delays patient referral to specialist PAH centres, significantly affecting the prognosis. Recent studies have demonstrated that an aggressive early approach to treatment, utilising prostanoids as first-line therapy, either as monotherapy or in combination with oral therapies, results in excellent long-term survival.

Download Full-text

Pulmonary arterial hypertension: the burden of disease and impact on quality of life

European Respiratory Review ◽

10.1183/16000617.0063-2015 ◽

2015 ◽

Vol 24 (138) ◽

pp. 621-629 ◽

Cited By ~ 55

Author(s):

Marion Delcroix ◽

Luke Howard

Keyword(s):

Quality Of Life ◽

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Support Groups ◽

Multidisciplinary Care ◽

Holistic Treatment ◽

Patient Health ◽

Related Quality ◽

Pulmonary Arterial

Pulmonary arterial hypertension (PAH) is a debilitating disease that pervades all aspects of a patient's daily life. It is also increasingly acknowledged that the burden of PAH extends to older patients and carers. Until recently, the adverse effect of disease symptoms on the physical, emotional and social factors governing patient health-related quality of life (HRQoL) remained largely unrecognised. With a shift in therapeutic objectives to longer term improvements and HRQoL benefits, clinical trials now frequently include HRQoL measures as study end-points. Most HRQoL instruments used in patients with PAH are generic or non-disease-specific questionnaires and therefore may not accurately capture PAH disease burden. New PAH-specific HRQoL instruments currently undergoing validation include emPHasis-10 and Pulmonary Arterial Hypertension-Symptoms and Impact (PAH-SYMPACT; Actelion Pharmaceuticals Ltd, Allschwil, Switzerland). Using various HRQoL measures, pharmacological therapies have been shown to improve HRQoL in patients with PAH. Patients also derive HRQoL benefits from nonpharmacological strategies, which include the emotional support provided by multidisciplinary care and support groups that is fundamental to patient wellbeing. Looking to the future, validated PAH-specific HRQoL instruments together with dedicated guidelines and procedures are essential to support the translation of HRQoL scores to the clinic, thus enabling a holistic treatment approach to the management of patients with PAH.

Download Full-text

My life with pulmonary arterial hypertension: a patient perspective

European Heart Journal Supplements ◽

10.1093/eurheartj/suz208 ◽

2019 ◽

Vol 21 (Supplement_K) ◽

pp. K54-K59

Author(s):

Pisana Ferrari ◽

Hall Skåra

Keyword(s):

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Healthcare Professionals ◽

Treatment Guidelines ◽

Guideline Development ◽

Treatment Strategies ◽

Patient Treatment ◽

Dietary Recommendations ◽

Patient Representatives ◽

Pulmonary Arterial

Abstract In recent years, there has been an increase in therapeutic options and treatment strategies for pulmonary arterial hypertension (PAH). However, patients still report delays in receiving a diagnosis, which is a significant burden associated with the disease, and which shows a general lack of disease awareness. This review has been written by two PAH patients to describe the patient experience and explore the ways in which patients are increasingly being given a voice in developing approaches to treatment. As patients with PAH are living longer, it is important that they work with healthcare professionals to develop treatment strategies that improve and maintain quality of life. Healthcare professionals should consider a holistic approach to disease management, including dietary recommendations, individually adapted exercise, and options for counselling where available, alongside therapeutic treatments. The experiences of patients with PAH are important not just for individual patient treatment but should also be considered in clinical trial design and guideline development. Patient representatives and patient associations can play an influential role in improving the treatment and management of PAH. In this review, we use our experiences as patient representatives to describe the current situation of patients with PAH from first experiencing symptoms to receiving treatment, using two patient cases as examples. We also discuss the role of patient advocacy in improving PAH care and future roles for patient associations and patient representatives in the design of clinical trials and development of new treatment guidelines.

Download Full-text

Treprostinil in the treatment of pulmonary arterial hypertension

Future Cardiology ◽

10.2217/fca-2020-0021 ◽

2020 ◽

Cited By ~ 1

Author(s):

Jeremy Feldman ◽

Naomi Habib ◽

Jade Fann ◽

Johh J Radosevich

Keyword(s):

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Infusion Pump ◽

High Morbidity ◽

Routes Of Administration ◽

The Past ◽

Technological Advances ◽

Recent Developments ◽

Pulmonary Arterial ◽

Routes Of Drug Administration

Despite progress over the past 30 years, pulmonary arterial hypertension remains a condition with high morbidity and mortality. Pharmacological and technological advances have shifted the approach to treating pulmonary arterial hypertension. Recent developments revolve heavily around novel routes of drug administration and delivery. In 2009, inhaled treprostinil was approved followed by oral treprostinil in 2013 providing patients with more convenient routes of administration compared with the parenteral alternatives. We are on the cusp of having the first fully implantable infusion pump for continuous intravenous treprostinil delivery. In 2019, generic treprostinil was approved, making the medication much more affordable for patients. In this review, we discuss in detail the recent developments surrounding both traditional and novel treprostinil products.

Download Full-text