Editor's and Guest Editor's Memo

In this, the second of two issues on “Guidelines Gaps,” we are pleased to provide additional insightful articles and commentary on the challenges of following guidelines for the management of pulmonary arterial hypertension (PAH) and its heterogeneous group of underlying disorders at a crucial time in the evolution of medical treatments. We present these in the context of the sections of the European Society of Cardiology and European Respiratory Society's guidelines 2015 treatment algorithm (as presented in Figure 2 on page 16 of this publication).

As we stated in the previous issue, challenges remain—and will remain—regarding optimal treatment of patients with this rare disease. Not only are we adding to our armamentarium of new drugs, but daily we are gaining knowledge from our patients about the effects of different routes of administration, mono- versus combination therapy, and quality-of-life considerations as we try to balance the risks and benefits of newly-available tools to add to evidence in treating this unique population.

We urge you, the clinician reader, to consider these two issues together as a source of more knowledge for you to apply as you optimize management for your patients. In addition to the actual guideline documents that have been offered by various organizations as means of guidance to practitioners, we offer the experience and perspective of leading clinicians in these two issues. We urge you to consider the challenges of guideline writing as described by Dr James Klinger in the previous issue along with topics including calcium channel blocker therapy, the state of monotherapy, and introduction of newer agents as you read in this second issue about combination and infusion prostacyclin therapies offered by Drs. Schilz and Myung Park. Plus, Drs. Nicole Ruopp and Harrison Farber present the gaps and controversies that factor into the uncertainties still remaining despite great gains in this field. As a wrap-up to the two issues, we offer a transcript of a lively discussion among Drs. Burger, Robert Bourge, Richard Channick, and Srinivas Murali, moderated by Dr Schilz, that touches on a multitude of experiences gained in treating PAH patients over more than two decades including risk assessment, treatment strategies, goal setting, escalation of therapy, and application of guidelines. We hope you will find these two issues to be thought-provoking and useful.