Portopulmonary Hypertension in Compensated Cirrhosis: When Haemoptysis Tells the Story

English:

Portopulmonary hypertension (PoPH) is a rare but severe pulmonary vascular complication of portal hypertension (PHT), typically associated with advanced liver disease. We report the case of a 54-year-old male with compensated liver cirrhosis secondary to chronic hepatitis C, who presented with repeated moderate-volume haemoptysis. His medical history included a splenectomy performed 18 years before, without previous cardiopulmonary symptoms. A comprehensive diagnostic assessment, including contrast-enhanced thoracoabdominal computed tomography (CT), transthoracic echocardiography (TTE), right heart catheterisation (RHC), and pulmonary function testing (PFT), demonstrated that haemodynamic and clinical parameters are consistent with PoPH, characterised by pre-capillary pulmonary hypertension (PH) in the context of PHT. High-resolution CT with vascular reconstructions revealed extensive portosystemic collateral circulation, including dilated paraumbilical, perigastric and perioesophageal veins. Bronchoscopy showed a floating blood clot in the right lower lobe bronchus without evidence of endobronchial lesions. Laboratory and imaging investigations ruled out other common causes of haemoptysis such as infection, malignancy, coagulopathy or thromboembolism. These findings supported the hypothesis of portopulmonary collateral vessels as a possible source of haemoptysis. The patient had preserved liver function (Child-Pugh class A) and stable haemodynamics, allowing the initiation of oral sildenafil therapy, which was followed by complete cessation of bleeding. This case illustrates the diagnostic challenges of PoPH with atypical presentations such as haemoptysis and highlights the value of advanced imaging in detecting vascular abnormalities. It also raises awareness of a possible association between prior splenectomy and delayed PoPH onset. Early detection and targeted therapy, in the context of a multidisciplinary approach, are essential for improving outcomes in this underdiagnosed but life-threatening condition.