Hepatopulmonary Syndrome in a Nigerian Man with Decompensated Alcoholic Liver Cirrhosis

Abstract

Hepatopulmonary syndrome is a rare complication of liver disease and portal hypertension. It has not been previously described in medical literature in Nigeria. We report this case of a 64-year-old man who presented with a 3 months’ history of recurrent breathlessness, platypnoea and leg swelling. He had been diagnosed with liver cirrhosis and had variceal band ligation in 2018, and had been managed for 2 episodes of hepatic encephalopathy prior to referral. Physical examination showed he was in respiratory distress, centrally cyanosed, had grade 4 digital clubbing, tachypnoea, platypnoea, orthodeoxia and bibasal coarse crepitations He also had a non-tender hepatomegaly, splenomegaly and ascites with no leg edema. Investigations showed negative screening for hepatitis B and C, elevated AST and GGT, conjugated hyperbilirubinemia, hypoalbuminemia, negative COVID-19 test, polycythemia, thrombocytopenia, prolonged INR and coarse enlarged liver with irregular margins on ultrasound. Gastroscopy revealed Grade 3 oesophageal varices, Forrest 3 pre-pyloric ulcer and portal hypertensive gastropathy. Spirometry demonstrated a restrictive pattern of airflow limitation.  Contrast echocardiography demonstrated intrapulmonary shunt. Alpha-1- anti-trypsin was not deficient. The peripheral cyanosis resolved with oxygen therapy. He was initially commenced on pentoxyfylline but terminated therapy due to side effects. The relevant literature was reviewed. Liver transplantation is the only effective therapy for HPS and prompt recognition and referral for transplant is key. We therefore present the first documented and published case of hepatopulmonary syndrome in Nigeria and the West African sub-region.