Expert Consensus Survey: Prostacyclin Pathway Agent Use in Pulmonary Arterial Hypertension - Pulmonology Advisor

Expert Consensus Survey: Prostacyclin Pathway Agent Use in Pulmonary Arterial Hypertension - Pulmonology Advisor
Cardiac MRI Could Be Useful in Pulmonary Arterial Hypertension Risk Stratification - Pulmonology Advisor
Deciding to Start a New Medication Can Be Overwhelming - Pulmonary Hypertension News

Posted: 05 Dec 2019 12:00 AM PST

A new consensus survey of expert opinions was published regarding the use of oral prostacyclin pathway agents in patients with various forms of pulmonary arterial hypertension (PAH) who received dual endothelin receptor antagonist (ERA)/phosphodiesterase type 5 inhibitor (PDE5i) therapy. The opinions were published in CHEST.

Researchers used MEDLINE to perform a systematic literature review of studies between 2008 and 2018 comprising adult patients with connective tissue disease (CTD)-associated PAH and function class (FC) 2 or 3 idiopathic, heritable, repaired congenital heart defect, or drug- or toxin-induced PAH (the latter was considered one etiologic grouping, IPAH+) who were treated with oral prostacyclin pathway agents. Pivotal trials for oral treprostinil and selexipag were also included if they focused on IPAH+ or CTD-associated PAH and FC 1 or 2 symptoms with background ERA/PDE5i therapy.

A series of clinical scenarios centered on 1 or the 3 etiologic groups (IPAH, CTD-associated PAH, and portopulmonary hypertension) were presented to the panelists for the development of the consensus. To create the consensus statements, the panel used the RAND/University of California Los Angeles Appropriateness Method, which incorporates the Delphi method and the nominal group technique.

A total of 458 case scenarios were accepted by Delphi 2. Based on the available data, the panelists agreed that selexipag may be considered for patients with IPAH+ and low- or intermediate-risk hemodynamic parameters who receive dual oral ERA/PDE5i therapy. This was specific to patients in FC 2 who had not been hospitalized for PAH in the past 6 months but had moderate to severe right ventricular dysfunction.

The panelists also agreed that intravenous or subcutaneous prostacyclin should be the treatment of choice in patients with IPAH+ or CTD-associated PAH who receive dual oral therapies and have high-risk hemodynamic parameters. Overall, a total of 14 consensus statements on the use of oral prostacyclin pathway agents that included 13 clinical scenarios where selexipag may be considered were discussed by the panelists.

A limitation of the survey included the paucity of evidence (only 5 clinical trials) regarding the use of selexipag and oral treprostinil.

Because of these limitations, the authors concluded that the "expert opinions must be validated with rigorous prospective studies, and this document may serve as a template for future investigations."

Disclosure: This clinical trial was supported by Actelion Pharmaceuticals. Several study authors declared affiliations with the pharmaceutical industry. Please see the original reference for a full list of authors' disclosures.

Reference

McLaughlin VV, Channick R, De Marco T, et al. Results of an expert consensus survey on the treatment of pulmonary arterial hypertension with oral prostacyclin pathway agents [published online November 16, 2019]. CHEST. doi:10.1016/j.chest.2019.10.043

Topics:

Pulmonary Arterial Hypertension Pulmonary Hypertension

Cardiac MRI Could Be Useful in Pulmonary Arterial Hypertension Risk Stratification - Pulmonology Advisor

Posted: 10 Dec 2019 12:00 AM PST

Cardiac magnetic resonance imaging (MRI) is useful for risk stratification in patients with pulmonary arterial hypertension (PAH), according to study results published in the American Journal of Respiratory and Critical Care Medicine.¹

Individual risk stratification is used to determine the therapeutic strategy for patients with PAH. This risk assessment integrates pulmonary hemodynamics, symptoms, functional capacity, laboratory values, and echocardiographic parameters to classify patients as low, intermediate, or high risk. Cardiac MRI is considered to be the gold standard for evaluating right ventricular (RV) volumes and function, and is recommended for the follow-up of patients with PH, but has not been integrated into risk stratification.

Using a referral center registry, Lewis and colleagues carefully analyzed cardiac MRI data in a large PAH patient population at baseline and, for a proportion of the patients, at the first follow-up.² They were able to identify patients at low, intermediate, and high risk for 1-year mortality based on RV ejection fraction, and patients at low and high risk were identified using RV end systolic volume index percent predicted and left ventricular (LV) end diastolic volume index.¹ Cardiac MRI is probably the best possible way to determine RV adaptation and maladaptation in PH through its holistic assessment of RV and LV volumes and function.

This work will hopefully introduce cardiac MRI parameters into the risk stratification of PAH.¹ The authors concluded that, "Additional parameters such as SV/[end systolic volume], RV mass, measures of RV deformation such as feature-tracking strain, 4D-flow in the right ventricle including assessment of vorticity, late enhancement, and T1 and T2 mapping, in combination with right atrial phase assessment with strain imaging, are promising tools to be explored in future in terms of prognosis and perhaps risk stratification."

References

1. Richter MJ, Tello K. Against the odds: risk stratification with cardiac MRI in pulmonary arterial hypertension [published on November 14, 2019]. Am J Respir Crit Care Med. doi:10.1164/rccm.201910-2069ED

2. Lewis RA, Johns CS, Cogliano M, et al. Identification of cardiac MRI thresholds for risk stratification in pulmonary arterial hypertension [published online October 24, 2019]. Am J Respir Crit Care Med. doi:10.1164/rccm.201909-1771OC

Topics:

Pulmonary Arterial Hypertension Pulmonary Hypertension

Deciding to Start a New Medication Can Be Overwhelming - Pulmonary Hypertension News

Posted: 13 Dec 2019 12:00 AM PST

"Don't Google it. Don't Google it."

This is the internal dialogue I was having as my pulmonologist gave me a new prescription. I was told about the potential benefits and the possible side effects before I left the office. And while I appreciate that there is a pill to help ease the stress on my heart and lungs, the unknown is scary.

Starting a new medication is physically and mentally overwhelming. My body has to push through side effects and symptoms. I experience anxiety and worry about what is a normal reaction and what needs to be reported to the doctor. I have to remind myself of the potential benefits to push through.

I avoid Googling side effects and symptoms. I like to tune in to how my body is feeling without having my judgment skewed by others' experiences. I often have increased fatigue, headaches, and nausea when starting a new medication. If the symptoms persist — or start to interfere with my everyday life — reaching out to my doctor helps ease my concerns. More often than not, I am assured that I am having a normal reaction.

Sometimes, unusual side effects or impaired physical abilities are a result of taking a new medication in combination with an existing one. I like to consult a pharmacist and ask about interactions between medications.

Starting a new medication also affects my mental health. Before I begin, my anxiety increases. I wonder whether the medication is going to work. I worry about what will happen if the medication isn't as therapeutic as the doctors expect.

It's a gamble. Nobody knows how I will respond to it or whether the reaction will be positive. The only way to find out is to begin taking it. For me, that is the most difficult step.

I can't control how my body reacts to a new treatment. When my anxiety gets the best of me, it helps to remind myself that it is my decision to continue to take a medication. I can choose to tolerate the side effects and symptoms, or I can let my doctor know when I have had enough. Recognizing that I have choices makes it less overwhelming.

***

Note: Pulmonary Hypertension News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website. The opinions expressed in this column are not those of Pulmonary Hypertension News or its parent company, BioNews Services, and are intended to spark discussion about issues pertaining to pulmonary hypertension.

I am a 27 year old from the smallest state in the US, Rhode Island. I manage multiple chronic conditions, some are visible illnesses thanks to my oxygen I carry around, but most are invisible illnesses. I hope my posts "Recharged and Rewired" will show those reading that just because I need oxygen charged daily and my body is wired a little differently, doesn't mean I can't be the best version of myself every day!

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