Diagnosing PAH Early is Difficult, But Critical

One of the biggest issues with pulmonary arterial hypertension (PAH) is that its symptoms are common with some of the prevalent pulmonary or cardiovascular disease in the country. But, it is a rare disease—physicians are sooner looking for an asthma diagnosis than they are PAH.

This is an issue that Gary Palmer, MD, MBA, Vice President of Medical Affairs for Actelion Pharmaceuticals and Victor Tapson, MD, Director of the Venous Thromboembolism & Pulmonary Vascular Disease Research Program at the Cedars-Sinai Medical Center, routinely see firsthand in patients. The chronic condition requires an early, strong therapeutic response—and therefore requires primary care physicians are ready to make the right call.

In an interview with MD Magazine® while at the 2018 CHEST Annual Meeting in San Antonio, TX, this week, Palmer and Tapson talked about outside elements that could possibly drive PAH, and their experience with patients who had delayed diagnoses.

MD Mag: What kind of non-clinical issues could drive pulmonary hypertension?

Tapson: Methamphetamine is certainly a thing that can cause PAH, we're learning. To me, it's undoubtedly a cause. You know, I saw it with a couple of my patients in the last few months. So, in terms of other environmental things, in terms of the smog inhalation, things like that aren't such big factors. I think, probably genetic factors are really important. Certain diseases like scleroderma.

MD Mag: How crucial is making the first diagnosis?

Palmer: You know, one of the sad things about this disease is it's often misdiagnosed for a long period of time. It's often missed as some sort of some asthma-like symptoms—you know, shortness of breath, maybe a little bit of heart failure. And often because it's a rare disease, it's not necessarily top of the list for most physicians to think about.

For primary care practitioners, maybe it is something that should be on their radar screen, where they've excluded things like asthma, heart failure—something that they need to bear in mind that perhaps this is pulmonary hypertension they’re missing and this patient should be referred for more specialized assessment to diagnose the disease.

Tapson: It's really important what Gary just mentioned, for primary care doctors to know: If someone tells you they had childhood asthma, you may automatically think the breathing symptoms have to do with asthma—even if they’re not wheezing at the time.

The delay in diagnosis is unbelievable. It can be over a year, year and a half, or even 2 years before your patient comes in. ‘Oh, you’re deconditioned, you need to lose weight. Oh, it's probably your asthma. Well, you're pregnant.’ One of the biggest disasters we see is a case where the patient is pregnant. We're told, well they're gaining weight, therefore they’re moving slower, that's why their heart’s rate is a little faster.

So I really think, the most important thing for primary care physicians to get is that if someone has unexplained dyspnea, could it be pulmonary hypertension? And a simple echo is a great screen to get you on track. And if you're not sure, refer the patient to a cardiologist or pulmonologist.

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