Polycystic liver disease, an atypical cause of cardiac tamponade

A 72-year-old female admitted with dyspnoea at rest, oedema and intolerance to lying down due to desaturation, tachypnoea and hypotension.

Chest X-ray showed elevation of the right hemidiaphragm and shift to the left of the cardiac silhouette (Fig. 1A).

Figure 1.

Collapse of the right heart due to hepatic cysts.

An urgent transthoracic echocardiogram was performed due to cardiac tamponade: in the apical four-chamber view, compression of the right atrium (Fig. 1B and C, red asterisk) due to an anechoic and partially septate infradiaphragmatic structure (yellow line) suggestive of a hepatic cyst (blue asterisk).

Investigations were completed with ultrasound (Fig. 1D) and chest and abdomen CT (Fig. 1E and F), in which multiple hepatic cysts were identified in relation to polycystic disease, the largest of them in the right lobe of the liver, 18 cm in diameter, elevating the diaphragm and compressing the right atrium.

Most patients with polycystic liver disease remain asymptomatic. Complications deriving from compression of neighbouring organs are uncommon, but can be severe depending on the location, and occur more frequently in females and in association with height-adjusted total liver volume >1,600 ml/m.¹

In this case, due to haemodynamic instability, urgent ultrasound-guided drainage of the giant cyst (1,500 ml of serous fluid) was chosen as bridge therapy to surgical fenestration.