Thread by @ebtapper

Hepatopulmonary Syndrome - or is Portopulmonary hypertension? What’s the diff (brief #tweetorial)
Pulm complications of #cirrhosis can be devastating. This month on rounds, I was reminded that these conditions are confusing.
#meded

Step 1:
Recognize when a pulmonary complication could be present or clinically important (4 transplant)
This is how they looked when first described:
1. Portopulmonary: Dyspnea without hypoxia (Fig 1)
2. HPS: Hypoxia (Fig 2)

Step 2:
TTE (Fig 3)

Ordering "agitated saline" echocardiography will always be funny

TTE▶️most sensitive way 2 dx HPS(Fig1)
The bubbles dont go right▶️left immediately (like with septal defects) but do after a few heart beats
Bonus: do not rely on pulse ox!(Fig2)
Screen or go 2 TTE on by symptoms

But what's the deal with hepatopulmonary sydrome?

1.Lung vasculature gets funky. i.e. diffuse/proliferating spider nevi(Fig1)
2.Shunt. V/Q mismatch 🔼in dependent areas (Fig2)
3.🔽hypoxic pulm vasconstriction(Fig3)
4.Orthodeoxia! Sitting up🔼shunt physiology,🔽PaO2 (Fig4)

Can anyone tell me why hepatopulmonary syndrome develops in the first place?

Hepatopulmonary syndrome gets better with:
1. Transplant (often) (Fig 1). We even prioritize HPS for transplant. Hence outcomes for non-transplant
2. Not much else else. Some have tried garlic (true story), pentoxyfiline, and embolizing the shunts. You might try one some day too.

To summarize:
✔️Hypoxia --> Hepatopulmonary
✔️Transpulmonary Shunt --> Hepatopulmonary --> Bubble Echo
✔️Orthodeoxia --> #Cirrhosis is easily the wildest condition in all of medicine

I hope you enjoyed this brief #tweetorial on hepatopulmonary syndrome. I will return shortly to discuss portopulmonary hypertension and how a patient with that condition convinced me to pursue hepatology.