Pulmonary hypertension treatment

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About this video

This medical education video explains that treating pulmonary hypertension starts with finding and treating its underlying cause, which can be in the lungs, the heart, or elsewhere in the body. It covers lung-directed treatment, drugs that manage right and left heart symptoms such as diuretics and beta blockers, endothelin antagonists when no cause is found, and drastic options such as septostomy, clot removal, and transplant.

You'll be able to

  • explain why treating the underlying cause comes before drug treatment for pulmonary hypertension.
  • describe how right-sided and left-sided heart problems can contribute to pulmonary hypertension.
  • weigh the benefits and drawbacks of septostomy and transplant as last-line options.

Key words

Pulmonary hypertension
Elevated pressure in the pulmonary artery, which can be caused by many lung, heart, or systemic conditions.
Idiopathic
A term for a condition whose cause cannot be found after a full workup.
Endothelin antagonist
A drug that blocks endothelin, a chemical released by lung tissue that narrows the pulmonary vessels.
Septostomy
A procedure that cuts a hole between the right and left atria to relieve pressure on the right side of the heart.
Diuretic
A drug that makes the kidneys remove extra fluid, reducing blood volume and congestion.
Transcript

- [Voiceover] Talking about the treatment of pulmonary hypertension can be a little complicated just because it can be caused by so many other things. And in fact, our diagnosis is not over just having found with the catheter that the pressure's elevated in the pulmonary artery.

The workup is usually huge and extensive to find out why we have pulmonary hypertension. So our treatment really goes off of that. So you have to treat the underlying cause. And that really is the theme of this whole treatment video, is that we have to treat the underlying, whatever it is, that's causing the pulmonary hypertension in the first place, treat the underlying cause, okay.

So intuitively, we go to the lungs first. Pulmonary hypertension, what can happen in the lungs that lead to it. If we have asthma or COPD or chronic bronchitis, whatever it is, we'll try to reverse the underlying disease. So we might give patients drugs to decrease the hypoxia or the lack of oxygen, the lack of good ventilation in the lungs, so help with our pulmonary hypertension.

And like I promised before, I'll always mention in the lung videos that we have to stop smoking. Smoking itself can lead to pulmonary hypertension, not to mention the other things, like cancer or obstructive diseases that can happen in the lungs. Sometimes if we do the workup and we can't find out why we have pulmonary hypertension, we call it idiopathic or that it just over rises on its own.

In this case we might give patients what's called endothelin antagonists. So endothelin is literally the chemical that's released by the lung tissue to restrict the pulmonary vasculature. And sometimes that's necessary and it's good for us. But an overproduction would give us pathologic pulmonary hypertension.

And we want to give agonists or, I'm sorry, antagonists to stop that process. So this is going straight to the source. But we usually don't want to go there unless we can rule out other treatable causes of pulmonary hypertension in the lungs. So going outside the lungs, we're really entering a huge pool of possibilities of things that can cause our condition.

In general, if we have our heart and lungs like this and we think about the ways in which they're connected, it's the right side of the heart that pumps blood to the lungs, and then it's the left side that it is returned to. I'm not going to worry about which chamber right now.

But just think of the sidedness of it, right and left. So to treat problems on the right side... by the way, we're treating the symptoms as well as the causes. So a lot of symptoms on the right side is over volume, overloading of fluid. So we might give diuretics to have them pee out some of this extra volume, just decrease the blood volume, which will make them feel better symptoms-wise.

So we have less congestion going back there. We might do something called a beta blocker which decreases the heart rate, beta blocker. It plays on the beta receptors in the heart. And it decreases the rate, gives the right side a chance, give it some extra time to fill the lungs, decrease some of the workload on the heart so that it's not pushing against a wall so hard.

These same treatments could help the left side of the heart. Left side, remember, can cause pulmonary hypertension because it's not pumping out blood to the body like it's supposed to and it backs up into the lungs. So diuretics and beta blockers can help that too by decreasing the workload, giving this dysfunctional left heart a better chance to do its job.

But we can also give drugs that give the left side a little bit of a kick. So there's a whole big class of drugs called the inotropes or ionotropic drugs that bank the heart work harder so we can increase the motor power behind the left side, make it push out harder so we have less backup in the lungs and can alleviate the pulmonary hypertension or if we have a valve issue on the left side that's causing this ineffectiveness, we could repair the mitral valve because mitral valve dysfunction can lead to decreased left side work which leads to backup into lungs.

And then sometimes we can give people anticoagulate. And then sometimes we can give people anticoagulants because one thing that could cause really bad hypertension in the lungs is if you have little blood clots. A big blood clot in the lungs would kill you. But if you have lots of little ones, they're not enough to kill ya, but it would definitely create a lot of hypertension and blockage in the vasculature.

So anticoagulation could be for people who are prone to make these blood clots. Coagulation. And then of course you want to investigate why they're prone to coagulation in the first place and treat that. So our general idea, again, is just to show you that it literally could be anywhere in the body.

The problem could be in the liver. It could be in the feet. But the point is that we always come back to treating what we can treating the underlying cause. And a lot of times we're dealing with symptoms rather than causes because the cause can be hard to treat or it's global or we're dealing with a side effect of a drug that's necessary to treat something else.

So it's a little bit of a balance act. And sometimes we might need to get really drastic. And then the heart, surgically we can sometimes cut a hole between the top chambers, the right and left atria. The procedure of cutting this hole is called a septostomy. So sept for the septum and the wall between them.

And ostomy is a hole. All this does is alleviate some of that buildup pressure on the right side. So if we have a lot of backup in the right side, it's causing right heart failure and congestion and swelling, then cutting this hole allows some of it to go to the left side, which will lower the oxygen content in the left side.

But we are willing to sacrifice that to equalize the pressure a little bit and alleviate some of this right heart failure symptoms. And surgically, if we do get a lot of clots that we can't take care of with anticoagulation, there's an option to go in and physically remove the clots surgically.

And of course, you can't do this every week. This is kind of drastic. But if we think the culprit are these blood clots, then we can remove them and prevent them from coming back with drugs, then sometimes it's worth doing. And then at the end of the day we always have transplant as an option if all other therapies fail or we think we can switch out the lungs and prevent the same thing from happening, then we can always go to transplant as a last resort and give this person a new set of lungs.

So pulmonary hypertension, very complicated to treat. And the treatment depends largely on a good workup to find the underlying causes and playing with the drugs to control our symptoms and reverse the cause.

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