30's F - s/p MVR several months ago. Home on RVAD. Course co | Figure 1

%27%3e%3cg%20id=%27Group-14%27%20transform=%27translate(13.000000,%20103.176124)%27%3e%3cg%20id=%27Group-Copy-5%27%20transform=%27translate(0.000000,%20182.676124)%27%3e%3crect%20id=%27Rectangle-8%27%20fill=%27%23E2E0DE%27%20x=%270%27%20y=%270%27%20width=%2736%27%20height=%2736%27%20rx=%2718%27%3e%3c/rect%3e%3cpath%20d=%27M28,27.6503435%20C28,22.127496%2023.5228474,17.6503435%2018,17.6503435%20C12.4771525,17.6503435%208,22.127496%208,27.6503435%20M18,16.3472505%20C20.8165136,16.3472505%2023.099749,14.0640151%2023.099749,11.2475015%20C23.099749,8.43098783%2020.8165136,6.1477524%2018,6.1477524%20C15.1834863,6.1477524%2012.9002509,8.43098783%2012.9002509,11.2475015%20C12.9002509,14.0640151%2015.1834863,16.3472505%2018,16.3472505%20Z%27%20id=%27Combined-Shape%27%20fill=%27%23FFFFFF%27%20fill-rule=%27nonzero%27%3e%3c/path%3e%3c/g%3e%3c/g%3e%3c/g%3e%3c/g%3e%3c/svg%3e)

Deleted account

30's F - s/p MVR several months ago. Home on RVAD. Course complicated by multiple PE's. Now on INO, Flolan, Thoratec. Not a candidate for heart-lung transplant. Yes, that systolic PAP is correct.

Relevant Case Information

Review Question

Do endothelin receptor antagonists increase how much a person is capable of exercising (exercise capacity), improve symptoms, or reduce death in people with pulmonary arterial hypertension (PAH)?

Background

Pulmonary arterial hypertension is a devastating disease characterised by an increase in pulmonary vascular resistance which leads to right heart failure and ultimately death. Endothelin receptor antagonists are a class of strong vasodilators (medications that open (dilate) blood vessels) capable of stopping the process of cell division, which could dilate and result in a favourable pulmonary arterial structural alteration.

Study Characteristics

We reviewed the evidence from randomised studies (studies in which people are assigned to one of two or more treatment groups using a random method). After a thorough search and assessment of the medical literature, we identified 17 studies with a total of 3322 participants for inclusion in the review. A vast majority of the participants had PAH without known cause (idiopathic). The evidence is current to November 2020.

Key Results

Endothelin receptor antagonists probably increase exercise capacity, improve World Health Organization functional class (a measurement of how severe a person's pulmonary hypertension symptoms are), and may improve death rates and symptoms in people with PAH; however, they may also increase the risk of liver damage, although this was rare. The question of the effects of endothelin receptor antagonists on PAH has now likely been answered.

Certainty of the Evidence

Overall, the evidence presented is of moderate certainty due to the high occurrence of missing data.