Portopulmonary Hypertension
Presence of pulmonary ...

Portopulmonary Hypertension

Presence of pulmonary arterial hypertension in a patient with portal hypertension

No other obvious causes (chronic lung disease, left heart disease, thromboembolism)

1) SCREEN - Transthoracic echocardiogram

 • Right ventricular systolic pressure (RVSP) - Estimates pulmonary artery pressure via tricuspid regurgitant velocity using Bernoulli principle.  No consensus on definite cutoff - Previous studies suggested >50mmHg correlated with moderate (or worse) PPHTN

 • Peak tricuspid regurgitation velocity (TRV) -  Cutoff > 2.8 m/s

 • Other signs: Right ventricular size, Right atrial pressure, Pulmonary artery diameter, Flattening of ventricular septum

2) DIAGNOSE - Right heart catheterization Needed for definitive diagnosis

Diagnostic criteria: mPAP > 25mmHg, PVR > 3 Woods units, PA wedge pressure < 15mmHg


Endothelin receptor antagonists:

 - Mechanism: competitive antagonism of endothelin receptors, blocking the vasoconstricting effect of endothelin

 - Effect: improves functional class, exercise capacity, and cardiopulmonary hemodynamics

Phosphodiesterase-5 inhibitors:

 - Mechanism: facilitates the vasodilatory effects of nitric oxide through reduced cGMP metabolism

 - Effect: improves functional class and cardiopulmonary hemodynamics

Prostacyclin analogues:

 - Mechanism: causes vasodilation and reduced vascular smooth muscle proliferation

 - Effect: shown to improve cardiopulmonary hemodynamics

Soluble guanylate cylase stimulators:

 - Mechanism: sensitizes soluble guanylate cyclase (sGC) to nitric oxide (NO) and directly stimualtes sGC receptors independent of NO

 - Effect: PATENT-1 study showed improved functional status and hemodynamics

Liver transplantation: Consider in those who respond to medical therapy

Lizzie Aby, MD @LizzieAbyMD

#Portopulmonary #Hypertension #diagnosis #management #hepatology
Contributed by

Dr. Michael Chew
GrepMed Gastroenterology-Hepatology Editor, Academic Hospitalist UC Davis Medical Center Internal Medicine, Yale Gastroenterology Fellow '22

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