Hepatopulmonary syndrome vs. Portopulmonary hypertension

Often confused as the same syndrome, Hepatopulmonary syndrome and Portopulmonary hypertension are two distinct disease processes. Both are characterized as pulmonary vascular abnormalities in the context of liver disease.

Hepatopulmonary Syndrome (HPS): triad of vasodilatation (Intrapulmonary vascular dilatations), abnormal oxygenation (an elevated alveolar-arterial oxygen gradient) and liver disease (any degree of liver disease). *LOOK FOR: digital clubbing, cyanosis and spider angiomas.

Portopulmonary hypertension (POPH): vascular obstruction secondary to portal hypertension (varices, splenomegaly, thrombocytopenia, portal vein abnormalities) with concomitant pulmonary arterial hypertension (mean pulmonary artery pressure >25 mmHg at rest, mean pulmonary capillary wedge pressure <15 mmHg, and pulmonary vascular resistance (PVR) >3 Wood units.

REFERENCES:

1. Porres-Aguilar M, Altamirano JT, Torre-Delgadillo A, Charlton MR, Duarte-Rojo A. Portopulmonary hypertension and hepatopulmonary syndrome: a clinician-oriented overview. Eur Respir Rev. 2012 Sep 1;21(125):223-33. doi: 10.1183/09059180.00007211.

2. Gupta S, Krowka M J. Hepatopulmonary syndrome. CMAJ Feb 2018, 190 (8) E223; DOI: 10.1503/cmaj.170253

Get Medical Pearls directly to your inbox every week!
Weekly posts with high yield medical knowledge, directly to your mailbox!
Dr. C Humphreys

Internal Medicine

Recent Posts

Obstructive sleep apnea & Polycythemia: Mechanism

Obstructive sleep apnea (OSA) is thought to lead to secondary polycythemia due to intermittent hypoxia,…

1 day ago

Pale Stool, Dark Urine & Biliary Obstruction: Mechanism

Catabolism of hemoglobin leads to the release of heme, which converts to biliverdin and then…

1 day ago

Why is Tongue Scalloping seen with Obstructive Sleep Apnea?

Tongue scalloping refers to the series of indentations along the sides of the tongue that…

1 month ago

Older Age and Obstructive Sleep Apnea (OSA): Mechanism

Older age is recognized as a risk factor for obstructive sleep apnea.[1] But why? Suspected…

7 months ago

How does Weight Affect Obstructive Sleep Apnea (OSA): Mechanism

Being overweight (BMI ≥ 25 kg/m2)/Obese (BMI ≥ 30 kg/m2) is one of the most…

7 months ago

Nocturia and OSA: Mechanism

Nocturia is a common symptom associated with obstructive sleep apnea. [1] Img Cred: Am J…

2 years ago

This website uses cookies.