A 27-year-old man with unrepaired tetralogy of Fallot (TOF), major aortopulmonary collateral arteries, Eisenmenger syndrome. and cirrhosis presented with recurrent episodes of haemoptysis. Computed tomography of the chest demonstrated pulmonary atresia with a ventricular septal defect (VSD), systemic-to-pulmonary collateral vessels and a left upper-lobe abnormality concerning pulmonary haemorrhage.

Echocardiography showed a left ventricular ejection fraction of 55–60%, moderately increased right ventricular wall thickness, moderate aortic regurgitation and an overriding aorta. Pulmonary artery systolic pressure was severely elevated, while the VSD demonstrated unrestricted right-to-left shunting. Tricuspid regurgitation velocity was 4.3 m/s, corresponding to an estimated right ventricular systolic pressure of 85 mmHg.

Right heart catheterisation was performed before initiating pulmonary arterial hypertension (PAH)-directed therapy. Mean pulmonary artery pressure was 68 mmHg, confirming severe pulmonary hypertension. The assessment also demonstrated preserved left ventricular function, a VSD and aortic insufficiency, with aortography suggesting a patent ductus arteriosus.

Given severe PAH associated with Eisenmenger physiology, recurrent haemoptysis, and coexisting cirrhosis, an endothelin receptor antagonist was considered. The choice of agent required particular consideration of its hepatic safety profile.