Setting the Stage – What Are the Understudied PH Populations?

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Dr. Krasuski:
Thank you for joining us today. I'm Dr. Richard Krasuski from Duke University and our topic today is, "Addressing PH Subtypes, Raising Awareness of Understudied Populations." The learning objectives for this talk are to review the characteristics of PH patient groups that fall outside of the idiopathic category. We'll discuss the screening, diagnosis, and expedient referral of non-idiopathic patients to PH specialty centers from community generalists and specialty healthcare providers. We'll focus on understudied PH groups that require special diagnostic attention by all healthcare providers. And finally, we'll review treatment and management approaches to these understudied PH patients.

Setting the Stage, What Are the Understudied PH Populations? So where have prior studies of PAH been focused? The current classification scheme for pulmonary hypertension is comprised of five major groups. Historically, Group 1 PH, or PAH, has been the most often studied class of adult pulmonary hypertension. PH therapy trials have mainly enrolled white females in their 40s and 50s and have consistently excluded non-WHO Group 1 forms of PH. Initial clinical trials performed in newly diagnosed PAH and CTEPH patients were single-agent, placebo controlled, of short duration, and focused on changes in measures of exercise capacity and comprise of relatively small numbers of patients. Over the past decade, clinical trial designs for PH have evolved into much larger placebo controlled on background therapy and upfront combination therapy trials.

So here is the current clinical classification system of pulmonary hypertension. As you can see, there are five major groupings. Today, we're going to focus predominantly on connective tissue disease, congenital heart disease. And then, as you can see, these fall into Group 1, but they also cross over into Group 3 for connective tissue disease with restrictive lung disease. And finally, the congenital patients also fall into Group 5 under complex congenital heart disease and segmental pulmonary hypertension.

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