CardioExchange Archive

CardioExchange Editor John Ryan discusses the findings from his new Pulmonary Circulation paper on heterogeneity in pulmonary arterial hypertension (PAH) clinical practice, co-authored with Bradley Maron, MD, of Brigham and Women’s Hospital in Boston and Ghazwan Butrous, MD, of the University of Kent in the U.K. 

With the goal of identifying practice patterns, my colleagues and I performed a survey of more than 100 PAH providers worldwide, each with an average of 11 years of clinical experience taking care of patients with PAH.

Among the issues we identified was a lack of standardized care among PAH providers.

We found marked variability in choosing the first line agent with which to treat outpatient PAH (i.e., phospho-diesterase type 5 inhibitors or endothelin-receptor antagonist). Variability is common throughout healthcare, but when the medicines cost >$50,000 per year, we feel that there needs to be more agreement on which agents to choose and how they interact when you go from one medicine to another (currently unstudied). Perhaps of more concern is that, in the setting of cardiogenic shock from PAH, almost a third of providers were not choosing prostacyclins as first-line therapy — even though it is the only proven therapy.

The role of right heart catheterization (RHC) in PAH created controversy. When asked if RHC was a routine strategy to monitor the response to pulmonary vasodilator therapy in PAH patients, the mean score was 4.1 ± 2.0 (Likert scale of 1-7). This suggests considerable disagreement about the role that RHC actually has in following PAH patients. In contrast, there was considerable agreement about the role of echocardiogram in monitoring PAH patients, with a mean score of 6.1 (scale 1-7) — a practice that has not been validated.

The final highlight of our findings is that, when choosing an inotropic agent for these patients, 50% of providers would choose dobutamine as a first line agent — the other 50% would choose one of a variety of agents.

In summary, our survey found that (1) practice patterns among PAH providers vary considerably when it comes to diagnosis, monitoring, and treating patients with PAH and (2) there is no consensus in the treatment of patients with cardiogenic shock secondary to PAH; this is especially alarming because of the upwards of 50-60% mortality associated with cardiogenic shock from PAH.

Are you surprised by the lack of standardized care? What can be done to address this heterogeneity in practice?