title: Successful treatment of severe hepatopulmonary syndrome with a sequential use of TIPS placement and liver transplantation.
abstract: 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.
  
  
  OBJECTIVE
  To report a clinical case of severe HPS treated sequentially with TIPS and LT.
  
  
  METHODS
  A 46 year old female cirrhotic patient presented with rapidly progressive dyspnea, hypoxemia (PaO2 60 mmHg, SaO2 92%) and increased alveolar-arterial oxygen gradient (A-a) (46 mmHg). She also had orthodeoxia (SaO2 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.
  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.
authors.affiliation: Department of Gastroenterology, Escuela de Medicina, Pontificia Universidad Católica de Chile, Santiago, Chile.
authors.forename: Carlos
authors.initials: C
authors.lastname: Benítez
authors.forename: Marco
authors.initials: M
authors.lastname: Arrese
authors.forename: Jorge
authors.initials: J
authors.lastname: Jorquera
authors.forename: Iván
authors.initials: I
authors.lastname: Godoy
authors.forename: Andrea
authors.initials: A
authors.lastname: Contreras
authors.forename: Soledad
authors.initials: S
authors.lastname: Loyola
authors.forename: Pilar
authors.initials: P
authors.lastname: Domínguez
authors.forename: Nicolás
authors.initials: N
authors.lastname: Jarufe
authors.forename: Jorge
authors.initials: J
authors.lastname: Martínez
authors.forename: Rosa María
authors.initials: RM
authors.lastname: Pérez-Ayuso
mesh_terms: D004417:Dyspnea / Q000209:etiology; D004452:Echocardiography; D005080:Exercise Test; D005260:Female; D020065:Hepatopulmonary Syndrome / Q000175:diagnosis / Q000209:etiology / Q000601:surgery*; D006801:Humans; D000860:Hypoxia / Q000209:etiology; D008103:Liver Cirrhosis / Q000150:complications* / Q000601:surgery; D016031:Liver Transplantation; D008875:Middle Aged; D019168:Portasystemic Shunt, Transjugular Intrahepatic; D012720:Severity of Illness Index; D013147:Spirometry; D016896:Treatment Outcome