Pulmonary hypertension in hereditary haemorrhagic telangiectasia

Figure 1 Characteristic echocardiogram of a patient with pulmonary hypertension. A: Apical 4-chamber view showing dilatation of the right ventricle; B: Apical 4-chamber view with Doppler signal (continuous wave) showing an increased right ventricular- right atrial pressure gradient (4.4 m/s); C: Subcostal view showing dilatation of the inferior caval vein corresponding with an increased pressure in the right atrium; D: Parasternal short axis view showing flattening of the interventricular septum (D-sign) and dilatation of the right ventricle.

Figure 2 Hepatic arteriovenous malformations. Computed tomography with contrast in arterial fase showing extensive filling of the hepatic veins (arrows), and diffuse hepatic arteriovenous malformations (asterix).

Figure 3 Pulmonary arteriovenous malformations. A: Computed tomography of the chest with large pulmonary arteriovenous malformation (PAVM) in the lower lobe of the right lung; B: Pulmonary angiogram of the PAVM in the same patient; C: Selective pulmonary angiogram of the PAVM in the apex of the lower lobe of the right lung; D: Repeat angiogram after transcatheter embolisation of the PAVM with a vascular plug.