Successful treatment of severe hepatopulmonary syndrome with a sequential use of TIPS placement and liver transplantation

Hepatopulmonary syndrome (HPS) is a complication of portal hypertension (PH) defined by the presence of liver disease, abnormal pulmonary gas exchange and evidence of intrapulmonary vascular dilatations (IPVD) producing a right to left intrapulmonary shunt. Liver transplantation (LT) is the treatment of choice; however, severe hypoxemia may contraindicate LT. The use of transjugular intrahepatic portosystemic shunts (TIPS) could be effective in HPS, although available data is limited. Aim: To report a clinical case of severe HPS treated sequentially with TIPS and LT. Case report: A 46 year old female cirrhotic patient presented with rapidly progressive dyspnea, hypoxemia (PaO2 60 mmHg, SaO(2) 92%) and increased alveolar-arterial oxygen gradient (A-a) (46 mmHg). She also had orthodeoxia (SaO(2) 87% in sitting position, but 91% in a prone position). A CT scan and pulmonary angiography were normal. Spirometric assessment showed a mild restrictive pattern and a desaturation was observed in a six-minute walking test. Contrast-enhanced echocardiography (CEE) showed intrapulmonary shunting. A HPS was diagnosed and liver transplantation was disregarded due to severe hypoxemia. The patient underwent TIPS placement. After four weeks, a significant improvement of dyspnea and a complete remission of orthodeoxia were seen. One year later, the patient was successfully transplanted. Interestingly, six months after LT, and in the absence of dyspnea, a new CEE showed persistent passing of bubbles to the left cavities. Comments/Conclusion: Persistent right-to-left shunt after TIPS placement and liver transplantation in spite of the improvement of pulmonary function tests suggests long-term persistence of structural changes in the pulmonary vascular tree after liver transplantation. Because of lack of data, it is not possible to recommend the routine use of TIPS as a part of the conventional management of HPS. However, in patients with severe hypoxemia TIPS placement can reasonably be used as a bridge towards transplantation.