Distinguishing Between Different Conditions Associated with Lung Disease in Patient with Liver Disease

 

Conditions to be distinguished (as listed by Rodriguez-Roisin and Krowka):

(1) hepatopulmonary syndrome

(2) porto-pulmonary hypertension

(3) cavo-pulmonary hypertension

(4) hereditary hemorrhagic telangiectasia (HHT, Rendu-Osler-Weber Syndrome)

 

Features of hepatopulmonary syndrome:

(1) may be associated with severe hypoxemia

(2) develops in a patient with a history of severe liver disease

(3) there is diffuse dilatation of the pulmonary vasculature with ventilation-perfusion mismatch

(4) breathing 100% oxygen will normalize hypoxemia

 

Features of porto-pulmonary hypertension:

(1) rarely associated with severe hypoxemia

(2) vasoconstriction in the lung results in obstruction to arterial blood flow

(3) endothelium and smooth muscle proliferate

(4) a plexogenic arteriopathy may develop

(5) in situ thrombosis may occur

(6) right heart failure develops as vascular resistance increases

(7) breathing 100% oxygen will normalize hypoxemia

 

Features of cavo-pulmonary hypertension:

(1) follows cavopulmonary anastomoses for congenital heart disease

(2) pulmonary arteriovenous malformations develop with diffuse and/or discrete vascular dilatation

(3) breathing 100% oxygen does not normalize hypoxemia

 

Features of HHT:

(1) hereditary with other affected family members

(2) may be associated with severe hypoxemia

(3) pulmonary vasculature shows dilatation which usually is discrete

(4) signs of HHT are seen in skin and other sites

(5) breathing 100% oxygen does not normalize hypoxemia