Distinguishing pulmonary arterial hypertension in patients is a difficult early diagnosis. But seeking out a combination of unexplained symptoms is a good first step.
Unfortunately, many of the symptoms of pulmonary arterial hypertension (PAH) are almost too common—shortness of breath, fatigue, dyspnea—for a primary care physician to make an initial diagnosis of the rare disease. That’s why Gary Palmer, MD, MBA, and Victor Tapson, MD, advise physicians look for a combination of these symptoms.
In an interview with MD Magazine® while attending the 2018 CHEST Annual Meeting in San Antonio, TX, the respective Vice President of Medical Affairs for Actelion Pharmaceuticals and Director of the Venous Thromboembolism & Pulmonary Vascular Disease Research Program at the Cedars-Sinai Medical Center advocated for improved diagnosis rates. That starts with thorough screenings that could lead to echocardiograms.
MD Mag: Can we improve how we diagnose PAH?
Tapson: I think we need to a better job. And I think the main thing, again, is suspecting the diagnosis. Once it's on your list, then you'll do something about it. If it doesn't even pop in your brain—and it's a fairly rare disease in primary care practice—but then once the it pops inside your brain, you've got a chance. If you think, ‘Well maybe it's heart failure,’ at least you get an echo and you'll figure out what's going on. I think we’re still under-testing out there in the primary care world—even in the pulmonary-cardiology world, we’re under-testing.
So I think we need to do a better job at suspecting it and going to get at least that initial test. Now an echocardiogram is not the final solution, but it’s a great pivotal test that gets you on the way and can make you now strongly suspect the diagnosis and move on if necessary to a right heart catheterization, which is essential in starting therapy. You’ve got to have that right heart catheterization before we can initiate therapy.
Palmer: And that's part of the difficulty with this disease, as well. There's no simple test that you can just run in a doctor's office to diagnose this disease. They need specialized testing. Right heart catheterization is not necessarily a difficult thing to do, but it's invasive and it requires specialized physicians to do it. And I think that's also part of why many of these cases are missed for a long period of time, and have often declined to significantly before they’re ultimately diagnosed and put on therapy.
MD Mag: What is your message to primary care physicians?
Tapson: I think it can't be said enough: I have so much respect for primary care doctors because they have so much they have to know, and so many different presentations they have to see and deal with. But with unexplained dyspnea, think about pulmonary hypertension. If you think about it, and maybe move on to do an echo, you may help move this diagnostic front up much, much sooner than it normally takes place.
Palmer: I’d like to echo that, as well. This is a rare disease, it's a rare condition. But it's something that with this combination of symptoms of shortness of breath, lack of energy, and fatigue, when physicians have excluded many of the other causes, it needs to be something that's on their radar screen, and that they need to at least include in their diagnosis scheme to make sure the patients don't have.
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