Hepatopulmonary Syndrome

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).

Triad of:

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

Hepatopulmonary System Image.JPG

Diagnostic Criteria:

HPS diagnostic criteria.JPG

TTE Findings in HPS:

HPS TTE findings.JPG

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