The combination of Hepato-pulmonary syndrome and porto-pulmonary hypertension is very rare in patients with cirrhosis. This case is presented with Hepato-pulmonary syndrome with ascites and Hepato-splenomegaly and evidence of pulmonary hypertension which may be due to the underlying liver disease and/ or the increased blood flow through the arterial-venous fistula that the patient has for end-stage renal disease treatment with hemo-dialysis. The treatment of both POPH and HPS when co-exist in the same patient is challenging. These patients usually, have multiple co-morbidities and are not candidates for liver transplantation. Even though, liver transplantation can cure both syndromes but is contra-indicated in patients with high POPH. Whether combined lung and liver transplantation can be used in patients with high POPH is a matter for discussion. More studies are needed to tackle the co-existence of both syndromes in patients with portal hypertension.
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