Hepatopulmonary Syndrome

Perplexed

Step 1: View clinicals

A 48 year old man presents with progressively worsening dyspnea for 3 months, which is worse when sitting up. There is no history of fever, cough or chest pain and he is otherwise asymptomatic. He was diagnosed with alcoholic cirrhosis 5 years ago, following which he stopped drinking. An upper GI endoscopy performed 6 months ago showed grade II esophageal varices, which were banded. A chest x-ray and ECG are performed and found to be normal.


Step 2: Order all relevant investigations

Full Blood Count

WBC/DC: 4,100/mm3 N: 70% L: 25% Hb: 14.3 g/dL Hct (PCV): 42% Platelets: 80,000/mm3

Arterial Blood Gases

PaO2 (supine): 71 mmHg PaO2 (sitting up): 58 mmHg

Contrast CT Chest

The pulmonary parenchyma and bronchial tree appear normal. No pleural effusions are present. Multiple dilated peripheral pulmonary arterioles are noted.

Echocardiography

The heart is structurally normal, with no evidence of intra-cardiac shunting. Contrast enhancement with agitated saline shows microbubble opacification of the left atrium 4 cardiac cycles after that of the right atrium.


Step 3: Select appropriate management

Antibiotics
Interferon-Alpha
Steroids
Liver transplantation


Score: ★★☆