Thread by @ebtapper

portopulmonary hypertension (PPH) and how I found hepatology:
a #cirrhosis #tweetorial

I had no clue what I was going to do in med school

And 1st year...it sucked. Soul-crushing memorization
Didnt feel like the kind of meaningful learning I experienced in college
Then I had the opportunity to shadow a bad-ass transplant anesthesiologist.
She changed my life

I was introduced to a pt w/#cirrhosis & PPH.
She was diagnosed after being screened (see criteria Fig1)
PPH was controlled (see tx options Fig 2)
But she had a rocky time periOp.
Desperate, my mentor started imatinib based on a recent case report (Fig3)
She did better!(Fig 4)

So we wrote a case report.
In it, we detailed our center’s experience w/PPH
This meant I reviewed 1652 charts. These patient’s stories - why they needed transplant, what happened, how they coped, even their medlists&TTEs…they taught me so very much.
Also, PPH=not good(Fig1)

More importantly:
I learned how a great doctor fights for her patients
I saw a liver transplant fix a lung disease!
I learned how through transplant the liver is an organ of second chances and I saw a patient thrive with that gift.
I got hooked on hepatology

Back to PPH.

Recall from the hepatopulmonary syndrome #tweetorial:
we are thinking PPH if dyspneic without hypoxia

https://twitter.com/ebtapper/status/1085617206891819008

But we are also screening for it in transplant candidates because it is so morbid

Key PPH Facts
1. Not rare, not common. 5% of transplant candidates.(Fig 1)
2. Need right❤️cath 4 dx, but est.RVSP 38mmHg by TTE~100% sens(Fig2)
3. 5-yr survival 14% w/o therapy, better with tx, best with transplant(Fig3)
4. Waitlist survival related to PVR and MELD(Fig4)

PPH pathogenesis
1. Unclear
2. Higher in women based on cohort study(Fig1)
3. Genetic studies highlight estrogen signaling and cell growth regulators (Fig2)
4. Higher levels of Endothelin-1 (potent vasoconstrictor) and IL-6(Fig3)

Portopulmonary Treatment pearls:
1. Beta-blockers not great idea in PPH (poor exercise capacity)(Fig1)
2. IV treprostinil associated with gram negative sepsis (Fig2)
3. Imatinib failed a rigorous trial, not recommended (Fig3)
4. great treatment review tandfonline.com/doi/pdf/10.158…

So that concludes this brief #cirrhosis #tweetorial on portopulmonary hypertension. I hope you enjoyed it.
✔️Hepatology is the best
✔️Chart reviews are a great way to learn about medicine
✔️PPH uncommon but very morbid. TTE to screen, right❤️cath to diagnose, MPAP>25mmHg,PVR>240