Episode 44: Navigating Pulmonary Arterial Hypertension Case

The goal is to learn , inspire and bring cardiopalm to the forefront of conversation . Thanks for joining me today and let's get after it . Hello , hello and welcome to today's episode of Talking All Things , Cardiopalm . I am your host , dr Rachel Barisi . Our episode today is sponsored by Jane , an all-in-one practice management software .

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I am still in scrubs and we're cheers-ing . Tonight I'm drinking jocoh juice from Limulus Brewery in Johnson County hazy IPA right up my alley , and it was one of those days . So we're going to be chatting , having a craft brew and talking all things Cardiopalm . So it was like one of those days .

I kind of started off a little bit wild and it was right off the bat it was getting to the shuttle on time . So I have two times that I can hit my shuttle and be on time , and that's 7.50 and 8 o'clock . And I pulled into the parking lot at 7.59 , and my 8 o'clock shuttle is already turning onto the main road . They come every 10 minutes .

Now normally I would just walk . You know I walk everywhere . I typically walk back to the parking lot but it's a 15-minute walk so I'm going to be arriving the same time that the shuttle . The next shuttle is coming around .

So started my day later than I expected today and then first patient out the gate , had a call-a-rapid response on , ended up having a possible seizure activity at the end of our session . So just like a little bit of a whirlwind of a day . But here we are Today . I actually wanted to switch gears .

I wanted to stick to doing a case , and we're going to talk about pulmonary arterial hypertension , which is something that I haven't talked about yet on this podcast . Pulmonary hypertension was one of those diagnoses that kind of came to my attention while I was in residency and the patients always seemed very severe .

Their desats were very significant and they had this weird presentation where they would be short of breath and their pulse ox would not show desaturation immediately . They'd have this delayed effect . So that kind of grabbed me an interest and I started diving in , and a colleague and mentee of mine actually presented at CSM in 2016 on this .

So pulmonary hypertension is considered a rare diagnosis and the title of our presentation was PAH . Not so Rare . It was cuter than that . I totally just fumbled it . Let me pull it . I have it actually up because I was queuing it up before I was going . Pulmonary hypertension redefining rare there you go .

You always need a little cutesy name at the CSM front , but pulmonary hypertension is one of those things that isn't really well understood and the research is changing . There's actually now a symposium that happens every few years . I think COVID messed up their timeline a bit but they meet pretty regularly as a world organization to discuss the diagnosis .

And what's interesting about pulmonary hypertension is that it's broken down into multiple groups and each group has its own identity and cause of pulmonary hypertension . And when I was seeing these patients at the VA they weren't on any specific pulmonary hypertension medications and so that also kind of piqued my interest in this whole situation .

And essentially my gentleman at the VA typically had pulmonary hypertension due to either significant lung disease or significant heart failure , and so that would be group three for the lung and group two for the heart as cause of pulmonary hypertension .

And when you have outside of group one cause of pulmonary hypertension , you're not actually on pulmonary hypertension specific meds , although that is starting to change a little bit . So group one is considered pulmonary arterial hypertension . It's like the quintessential version of pulmonary hypertension . It's a little less understood . There's a number of different causes .

Some is hereditable , some is idiopathic . They really just don't know . Drugs and toxins is kind of grouped into that as well , but they have very specific medication that can be utilized to help regulate pulmonary hypertension . So I had a patient recently and I'm just always like I got my , I got my PAH .

So I was like yes , he's on my schedule , let's do this , let's let's dig into his chart a little bit . So he was a 22 year old male , super young . He's diagnosed with PAH , group one , and it was actually written that way in the charts .

I was like just totally nerding out and like yes , we've labeled it correctly and no , like specific grouping , so group one is 1.1 , 1.2 , so on and so forth , and so that wasn't specified .

But he also had a rare congenital disease called Adams Oliver syndrome , and so I , to be fully transparent as I always am , I had no idea what that is , and so it is a rare congenital disease that can affect the limbs and toes and fingers , like cause webbing and such , but it also has the ability to affect the heart .

And he also had an ASD and atrial septal defect , and so atrial septal defect is basically a hole between the right and left atria and ASD PFO are kind of synonymous , but not . The big difference between an ASD and a PFO is that a PFO , the flap doesn't close after birth , and an ASD is like an actual hole .

There is no flap , and so the problem with this is the size of that opening . And after birth that opening is supposed to close to prevent left side of the heart rushing into the right side of the heart . It's also to help prevent deoxygenated blood from the right side of the heart going to the left .

But as we know , across the board pressure gradients run everything . So pressure gradients run from high to low . So if you have this opening between your right and left atria , left side of the heart pressure is going to be greater than the right .

What you're going to end up with is increased blood volume pushing into the right atria , therefore the right ventricle , therefore the pulmonary artery and the lungs , and over time , if that ASD isn't corrected , will cause right side heart failure , increased pulmonary artery pressure . So I'm already like kind of sifting through this . Like which one came first ?

Was it the ASD that caused the right sided failure which led to the increased pulmonary artery pressure which is leading to pulmonary hypertension , or does he have true group one ? So the answer is I don't know , because no one's digging that deep into the chart , but this is where my brain goes in this moment .

And so he did have ASD closure but I think he had some issues with that closure . Like it's not complete . He has some like known bubbles on exam , so clearly there's still some volume switching from left to right . So that was like his big history on that part . But he's 22 years old , past medical history , like I said , pulmonary arterial hypertension .

He has this Adams Oliver syndrome . He had ASD with closure and he presented to the ED with shortness of breath and was found to have sat in the 70s on room air . Now this gentleman does not wear oxygen at baseline . He is a 22 year old male who is actively working as an IT person on a campus .

So no device , no history of falls , not very active based on our discussion , but able to safely walk across campus without oxygen without being short of breath . His mother actually made him go to the hospital .

He had his pulse ox on him and he was satting in the high 60s to low 70s and she actually came to pick him up and took him to the hospital and when he got to the hospital he was on seven liters and then had to be uptight rated to 10 liters high flow nasal cania . He was transferred to the ICU but stabilized pretty quickly .

In general he was noted to be fluid overloaded and he also had a viral panel run and he was positive for adenovirus . So he had a few things going on that could have caused this exacerbation . That's it . But they were also still potentially noting that the cause could have been an episode of binge drinking .

So he had gone out with his friends , had a night where he drank more than he normally does and then the next day he has this episode . So was it the drinking ? Is it the adenovirus ? Is it being fluid overload ? My guess is it's probably a combination of all three .

But they really pressed him quite a bit about the drinking , which doesn't seem to be his normal demeanor . But he is on some pretty specific meds related to pulmonary hypertension and these pulmonary hypertension meds have like very short half-life , they have like very significant burden and they're also super expensive .

So he is on remodulant , which is a sub-Q medication that is actually delivered via a mini-pump . He is also on two oral pulmonary hypertension specific meds , tadalafil and Ambriosentin . So he's on three pulmonary hypertension specific meds and you know most medications in general advise you not to drink . So I would be curious how much that interaction did cause it .

But I would like to note that his fluid overload was significant . His BNP on admission was 5,432 . So pretty significant overall and they started diuretting him . His shortness of breath improved and he was being down-tytrated pretty quickly . By the time I went to see him he was on two liters at rest .

Just another like little piece about pulmonary arterial hypertension or just pulmonary hypertension in general . In order to be diagnosed with pulmonary hypertension like an official diagnosis , you need a right heart cath . They will not give you that official diagnosis without a right heart cath .

The big differences between pulmonary arterial hypertension and the other groups of pulmonary hypertension have to do with not only the mean pulmonary artery pressure but pulmonary wood units and pulmonary artery wedge pressure .

So the quintessential pulmonary arterial hypertension definition , with confirmation of a right heart cath , is a mean pulmonary artery pressure of greater than 20 . And this number has changed . It went from 25 to 20 . I think we're staying at 20 right now , based on the last world meeting , and pulmonary vascular resistance greater than three wood units .

So they look at the amount of pulmonary vascular resistance in regards to an objective number .

So the big pathophysiologic piece of pulmonary hypertension is that you have increased pressure in the lungs and the reason why you have increased pressure in the lungs is because the vessels within the lungs themselves are going through changes , which include vasoconstriction and thickening , and so that creates a whole slew of issues , including increased pressure within the

lungs , which leads to an increase in pulmonary artery pressure . But what makes it specific to group one is that the pulmonary vascular resistance is high , and then they also look at pulmonary artery wedge pressure and in the PAH group should be less than 15 . And essentially what this is showing you is that the left heart is not the cause of it .

So if you can think back to the components of a right heart cath , the really cool part about a right heart cath is that it gives you direct measurements of everything on the right side of the heart and gives an indirect measurement of the left side of the heart , and it does that by literally creating a balloon effect inside that pulmonary artery and then it

gives a pulmonary artery wedge pressure which helps determine if the left side of the heart is involved . So it basically gives you a mirror image of the left atria pressure because it sits over top of it . So when it's less than 15 millimeters of mercury pulmonary artery wedge pressure , it's telling you that the left heart is not involved or not .

The problem For group two , where left heart disease is the cause of pulmonary hypertension , you do have an increased pulmonary artery wedge pressure , greater than 15 . And so that's part of like the defining characteristics of it . So 22 year old male on two liters of oxygen on approach had been down-tied , traded .

I think the highest he was on in the ICU was 15 liters high flow nasal cannula . His goal SPO2 , is 92 , greater than 92% .

And then just some more background information that right heart cath was performed in August , which is the same time that he was placed on remodulant , which is a sub-Q pulmonary hypertension specific medication , and so a lot , like I mentioned before , a lot of the pulmonary hypertension meds are very potent and also have a very short half-life , so they take a little

time to get to dose . So he started his remodulant mini pump in August and was being gradually increased on dosage until probably a few weeks prior to his admission . So he was finally just on his full dose . That was recommended . And then he had this episode . But it had to be a ramp up period .

You can't just kind of go in at full dose and just from like long-term perspective it has about a four hour half-life .

So whenever working with this patient population , if it is a sub-Q infusion , if that timing is important , so they have a four hour span typically you want to definitely check in and just kind of see where you're at somewhere in that timeframe . So it's a longer half-life than some of the other medications like Flolan or Veletri , but it's still a short half-life .

So the sub-Q infusions with the mini pump are a little bit easier to deal with because they can be placed for weeks and months and their primary side effect is local erythema and pain . And he actually had a lot of pain at the site because he just had a site change . So just something to pay attention to .

It was enough that he was receiving pain medication for it and was potentially going to decline because it was that uncomfortable . Other than that , he was very motivated to work with PT . I had asked lots of questions about pulmonary rehab or cardiac rehab . He had never been offered that .

So we spent a lot of time discussing potential benefits of either or and I think this is just a conversation point of if you have someone with pulmonary hypertension , should they go into pulmonary rehab or should they go to cardiac rehab . In theory they could qualify for either .

One of the big things that I like about pulmonary rehab over cardiac in this patient population is the focus on breathing exercises , and that is a huge component to treatment of patients with pulmonary hypertension .

So just being able to pace and pair breath with movement and understand oxygen saturation and titration and the whole bit is a very important piece that I think can sometimes get overlooked . So he had never been taught breathing exercise . He had never been to pulmonary or cardiac rehab . I mean he is 22 years old .

From a stature perspective he was a very low BMI I don't think I wrote it down , but he was a small statured guy , very low muscle mass . He looked cyanotic visually , even when he was satting okay . So just like things that were on observation , he moved fine . He went from supine to sit independently .

I didn't have to worry about him , I didn't have to guard him . It wasn't a physical assist . But going from supine to sit did create some shortness of breath and some dizziness . So we did all of the things . I did a full chest exam on him . We assessed vitals throughout .

We maintained oxygen at 2.5 liters at rest and then up-tied traded to , I believe 3 liters during ambulation . I'm just going to go back up towards my note because I had some findings on exam for him . Yeah , so he had decreased lateral chest wall movement . He had apical breathing at his primary breathing pattern with positive accessory muscle use .

He was able to increase his lateral costal movement with tactile cues and we did lateral costal movement with overpressure on exhalation and so he really did great with improving his ability to control that and that also related or ended up decreasing his accessory muscle use .

We talked a lot about after we did some parrug breathing and we did parrug breathing with bilateral upper extremity movement . I had asked if he's ever done any sort of like diaphragmatic breathing or resistance training with diaphragm .

So we talked about potentially looking into something like the breather for respiratory muscle training , because the research with respiratory muscle training and pulmonary hypertension has a lot of benefit .

He also , if I remember correctly I'm looking back in my note yes , with sniff , the sniff technique , he was able to activate his diaphragm but had pretty decreased excursion .

On the first attempt it was all upper chest and no diaphragm excursion , and so we had to , instead of doing three sniffs consecutively , we had to break it down and go one at a time until we had good excursion and then we were able to combine those breathing exercises .

The one you know which breathing exercise I'm coming with you inspiratory holds was the game changer on his sats . As soon as we did inspiratory holds , sats popped right up . So we had a lot of assessment kind of throughout .

Initially his sats were around 93% sitting edge of bed and then as soon as we added breathing activities and inspiratory holds he was actually sitting at 99% for a period of time . As soon as we incorporated upright activity in standing , heart rate and SPO2 dropped a bit . So his heart rate increased to 112 and his SPO2 dropped to 96% .

So preemptively I did up-to-tie trade him to three for ambulation . He was able to ambulate one lap in total but we did it via intervals . So we did three intervals , monitoring heart rate and sats . Anytime his sats would drop to 93 , 92% . That was our cue to stop and rest .

He wanted to kind of barrel through and just go and so we made sure that this was an education point . Right , rushing is only going to drop those sats quicker . Heart rate increase is gonna make you quit quicker .

So we did a lot of education as we were ambulating , on pacing , monitoring SPO2 and gauging signs and symptoms without the objective numbers in front of us . So he was able to tolerate three bouts of about a hundred feet each time and his stopping point was right around that 117 , 120 beats per minute and same thing .

He had one drop to 88% on his third bout , which recovered pretty quickly with a sitting rest break and inspiratory holds . His blood pressure across the board was pretty flat and also a little bit soft , so he was sitting like 96 over 57 . So his maps were like mid 60s pretty much throughout .

He did have a slight increase post-ambulation , which is actually a good response in the world of aerobic response . He didn't have any drops . What else do we whoops ? Sorry , what else do we have with this gentleman ? He was a fun one , very willing to perform all breathing exercises , very engaged in understanding how he could improve upon his breathing .

We talked a lot about endurance training via intervals , the importance of strength training in this patient population and then the importance of breathing exercises , which he had never done before .

So he was really fun to work with and it's always really exciting to see the things that you are teaching right in front of you working in real time and then also making impact on that patient . So the trifecta is really . The trifecta really came about because of pulmonary hypertension . It's kind of the bread and butter to improvement .

Breathing exercises , aerobic training typically via interval training first , and strengthening those three pillars are super important to pretty much most cardio pulmonary patients , but especially pulmonary arterial hypertension . I'm just checking his chart to see if there's anything else . Let's talk a little bit about his meds .

So , like I said before , he was on remodulin via a mini pump , so sub-Q infusion . That pump can last for weeks to months . Overall it has a longer half-life , around four hours , so a little bit longer timeframe than flowlin or volitri , which is very short half-life . It's three to five minutes .

So if you have a patient who has pulmonary arterial hypertension and they're on flowlin or volitri via IV , that IV needs to be checked before working with physical therapy .

If the infusion is almost complete , don't start your session because the half-life is about three to five minutes and it can be life-threatening to have a abrupt stop and obviously then not restarting that infusion pretty immediately .

So always check with your nursing staff about medications with this patient population , especially if they're on pulmonary hypertension-specific meds , now the medications that typically are utilized when they're not group one are oral medications , and one of the most common oral meds used for pulmonary hypertension outside of group one is sildenafil or todalafil , and so

sildenafil is basically a derivative of what was created for Viagra so an unofficial , I guess , story , because I don't know all of the facts on this , but basically when Viagra was supposed to be a cardiac medication , for my understanding , and its job was to help vasodilate , and when that medication was utilized in male patients , they ended up with the ability to

create an erection , so then it was marketed towards that diagnosis , but sildenafil is basically Viagra . Very similar compounds . I don't know how different they are in their chemical makeup , but they are very similar . This is important for a few reasons .

The biggest reason is if you have a patient who's on sildenafil and also takes Viagra or any of the other erectile dysfunction meds , if they use their Viagra , they cannot take their sildenafil because then they're gonna be double dosing and they're gonna bottom out their blood pressure . So super important . I've had that happen in the cardiac rehab world .

Patient came in , his blood pressure was like nothing over nothing and I was like probing for what's happening here , what's different , and it literally took quite some time for him to state that he used Viagra last night and nothing to be ashamed of . But that can really bottom you out and end up in a medical emergency .

So that can be an important education point for your patients . But he was on sildenafil , which is basically another version of sildenafil and it's a pulmonary I'm sorry a phosphodiesterase five inhibitor and so basically it's helping that piece of vasoconstriction within the lungs . What else was he on ?

He was also on he was on one more , oh , ambrosentan , which is actually another medication specific to group one , and this one is an endothelian receptor antagonist and again same thing , very specific to the pathophys of pulmonary arterial hypertension .

So there are a number of things that happen with pulmonary arterial hypertension that basically cause the changes within the small pulmonary vessels in the lungs and the precursor of all of those things is basically vasoconstriction and vessel changing .

So there is endothelial dysfunction that occurs and so that leads to decrease in prostacycline as well as decrease in nitric oxide . Both of those things are potent vasodilators . So if you decrease both of them then you're going to increase vasoconstriction and also creates platelet aggregation and it increases endothelian one , which is another potent vasoconstrictor .

So basically everything that's happening on the underlying pathophys of pulmonary hypertension causes pulmonary vasoconstriction of the vessels within the lungs , which then leads to more increased pulmonary artery pressure , which leads to right ventricular failure .

And then one other piece of this is that hypoxia is a big component of pulmonary hypertension and hypoxia causes vasoconstriction in the lungs to help accommodate the VQ mismatch and essentially it makes it worse . Okay so , this patient population . Another , just like clinical tip is that you want to oxygenate this patient population . You want to keep them above goal .

So check what their goal SPO2 is . Commonality is usually greater than 90% or potentially greater than 92% , pending your physician , but really just depends on their goals . There are times you'll see patients with goals . In the 80s I've had a number of conversations with cardiologists .

I had one guy at the VA that I just truly had such a great working relationship with , easy to talk to and just very willing to educate , and he was very pro . Oxygenate these patients , because when you push them into hypoxia you essentially just worsen the condition and create more pulmonary vasoconstriction and you're just kind of fighting yourself .

Okay , last thing , because I didn't say it before so I'm gonna backtrack he did have a right heart cath done . It was actually done . In August I don't know if I said this out loud His pulmonary artery pressures were 105 over 50 , which is a mean of 67 .

So he was well above that 20 marker and his pulmonary wedge pressure was 8 , which is less than 15 , which indicates that it's not related to left side heart issues . So just a little background there . Hopefully I didn't jump around too much .

I had some pulled information from his chart and just pieces of my note kind on the side here that I was trying to piece together for you and I was hoping to give a little bit more pathophys this time around , so hopefully that was beneficial for you .

Alright , if you have any questions , please reach out to me on Instagram , at all things Cardio Palm , or reach out on text . I will drop my number in the show notes and , if you enjoyed this , if this was helpful for you , if this was something that you are seeing in your hospital setting , reach out . Love to chat about it .

I think that's all I got for you today . I hope you have a wonderful evening and whatever you have to do , I'll get after it . You , you , you , you , you , you , you , you , you , you , you , you , you , you , you , you , you , you , you , you , you , you , you , you , you , you , you , you , you , you , you , you , you , you , you , you , you you .