This week Dr. Kartika presented an interesting case of a patient with Etoh cirrhosis who developed progressive dyspnea on exertion secondary to hepatopulmonary syndrome (HPS).
1. Liver disease
2. Pulmonary vascular dilation
3. Hypoxemia with increased A-a gradient
Liver dysfunction → Release or failure to clear circulating vasodilators such as Nitric Oxide (NO) → inability to constrict pulmonary capillaries leading to relative high perfusion per ventilating unit (i.e. V-Q mismatch).
Mechanism of hypoxemia in HPS
TTE Findings in HPS:
Take Home Points:
1) Consider HPS in patients with cirrhosis and elevated A-a gradient
2) Diagnosis can be aided by TTE showing R—>L shunt with bubble study after 3 beats
3) TIPS controversial for treatment of HPS and in some circumstances can exacerbate intrapulmonary shunt.
N Engl J Med 2008; 358:2378-2387