Portopulmonary hypertension (PPHTN) is one of the most devastating consequences of end-stage liver cirrhosis. When a patient has moderate to severe PPHTN, his or her candiclature for liver transplantation is denied. Here we report a successful adult-to-adult living donor liver transplantation (LDLT) in a patient with moderate to severe PPHTN. The patient was a 58-yr-old female who was diagnosed with end-stage liver cirrhosis due to chronic hepatitis C. Preoperative evaluation revealed that the patient had moderate to severe PPHTN. Her mean pulmonary artery pressure (mPAP) was 35-47 mmHg without treatment. Continuous epoprostenol therapy was introduced to lower the mPAP. She underwent LDLT using an extended right hepatic lobe graft which was donated by her daughter. Prolonged artificial ventilation was necessary until postoperative day (POD) 25, after which her general condition gradually improved. By POD 72, she was in good condition and was allowed to leave the hospital. Currently, 1 yr after the operation, she visits the outpatient clinic regularly and enjoys a normal life. It should be noted, however, that the PPHTN markedly improved but did not completely resolve, as assessed by right heart catheterization 1 yr after successful LDLT.
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