Hepatopulmonary Syndrome - Illness Script

HPS is a pulmonary disorder of chronic liver disease characterized by a triad of: 

1) chronic liver disease

2) pulmonary vascular dilatation & 

3) gas exchange abnormalities

PATHOPHYSIOLOGY:

 • Intrapulmonary capillary vasodilation leads to impaired arterial oxygenation through V/Q mismatch

 • Mismatch? Dilated vessels + absent tone = increased flow & longer distance for O2 to cross // overperfusion + decreased RBC transit time = increased Aa gradient

 • Intrapulmonary arteriovenous shunting causes mixed blood to pass through pleural and pulmonary a/v communications // a higher presence in the lower lung zones may be behind the mechanism of orthodeoxia

CLINICAL FEATURES:

 • Progressive dyspnea 

 • Platypnea - worsening dyspnea when moving from supiine to upright

 • Orthodeoxia - decrease in PaO ≥ 5% (or 4 mmHg) when moving from supine to upright

 • Stigmata of cirrhosis: jaundice, ascites, palmar erythema, spider angiomas, digital clubbing

DIAGNOSIS - TRIAD:

 • Liver disease &/or portal HTN

 • Aa gradient ≥15 mmHg (room air) - ABG

 • Pulmonary vascular dilatations - contrast-enhanced TTE with agitated saline - Microbubbles elude capture and reach the left heart

TREATMENT

 • No correlation between the presence or severity of HPS and the severity of liver disease (CPT. PELD. MELD)

 • In one large study, HPS mortality rate of ~12% 

 • No effective medical therapies other than supplemental oxygen

 • Liver transplant is the only definitive treatment. Marked improvement or resolution of syndrome in 85% post-transplant



By @Alert_Oriented3



#HPS #Hepatopulmonary #Syndrome #cirrhosis #diagnosis #hepatology