MPN Workshop of the Carolinas 2025 | The risk and management of pulmonary hypertension in patients with polycythemia vera

Polycythemia vera and all MPNs as a whole have been for a long time recognized to have an increased incidence of pulmonary hypertension. Pulmonary hypertension is, you know, you have two sides of the heart. The right side of the heart is really just there to pump blood through the lungs to get to the left side, which is the big meaty side that pumps blood through the rest of your body. And in pumping to the lungs, it’s kind of an easy thing to do. So the right side of the heart is not big and muscular. It’s kind of a wimpy side. And what happens in pulmonary hypertension, all of a sudden that wimpy side of the heart is made to do a lot more work, and over time, what happens is it fails. The right heart fails, and that’s associated with vastly shortened survival. 

So pulmonary hypertension is almost always something that needs to be treated and addressed. And although it’s been recognized that pulmonary hypertension is way more common in myeloproliferative neoplasms than expected for a population without these diseases, we’ve never really looked at what that means. Every other disease where there’s pulmonary hypertension, it’s always associated with a worse outcome. And so we looked at this, we’ve published a couple of papers on this now, but this most recent one was looking at a polycythemia vera group. And those people who had pulmonary hypertension had really vastly reduced overall survival. And interestingly, and this is something we didn’t really expect, but they also had shortened myelofibrosis-free survival, meaning that more of them progressed to myelofibrosis or progressed faster. 

And so that suggests that this is an important prognostic measure. It’s something that’s an echocardiogram that can be used to screen for pulmonary hypertension. So it’s non-invasive, and it is prognostic in that it is something that can be used to predict outcomes for patients. And I think that if you look at other diseases associated with increased risk of pulmonary hypertension, like sickle cell anemia and systemic sclerosis and others, there are built-in guidelines for screening because it matters. It’s easier to treat a little bit of something than a lot of something, and if you wait until a patient is symptomatic, you can improve a little bit, but in some ways, the horse is out of the barn. And so what we looked at is what could we predict clinical measures that might identify those people who should be screened? And so it turns out if you have polycythemia vera for more than about seven years, or if you’re over the age of 70, either one of them, not both, either one of them, you should definitely have an echocardiogram to screen for pulmonary hypertension. How often to screen? I mean, these are things I don’t have a clear answer on, but I tend to look more frequently than not because identifying pulmonary hypertension, there is a really broad literature that suggests these people should be referred to expert centers caring for pulmonary hypertension because outcomes are vastly improved. And so that’s what the purpose of screening is try to get them to see the cardiologist. 

So patients who are at risk for pulmonary hypertension should be referred to an expert center that treats pulmonary hypertension. There is a variety of different testing that the cardiologists do in addition to just confirming pulmonary hypertension, which really requires an invasive test. But even if you don’t want to take that step, the patient’s asymptomatic, they have an echo that suggests pulmonary hypertension. These are people, there are a number of measures that you can do, and it’s good to connect them with an expert in pulmonary hypertension so they can monitor them more closely, and when the time comes, if it comes, to do the invasive measures, because that’s where when they do a cardiac catheterization, looking at the right side of the heart, they can actually infuse drugs in to see if patients in real time are responding to the drugs. So it’s not just diagnostic, it’s therapeutic. So people come out of that with, yes, you have pulmonary hypertension, and yes, you respond to, for instance, nitrates. And now this is a way that can relieve the pressure on the right heart, prevent right heart failure, and prolong life. And so it’s important to get into that pathway as soon as possible.

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