title: Combined liver and (heart-)lung transplantation in liver transplant candidates with refractory portopulmonary hypertension.
abstract: BACKGROUND
  Portopulmonary hypertension (PPHT) has a prevalence of 5-10% in liver transplantation (LiTx) candidates. Mild PPHT is reversible with LiTx, but more severe PPHT is a contraindication to LiTx given the high intraoperative mortality due to heart failure. Prostacyclin can reduce PPHT to a level at which LiTx can be performed. In patients refractory to that treatment, combined (heart-)lung-LiTx is the only life-saving option.
  
  
  METHODS
  We report two cases of (heart-)lung-LiTx in patients with refractory severe PPHT.
  
  
  RESULTS
  Patient 1, a 52-year-old female with viral cirrhosis and severe refractory PPHT, received a double-lung Tx followed by LiTx. After liver reperfusion, fatal heart failure occurred. Patient 2, a 42-year-old male with viral hepatitis and congenital liver fibrosis, also suffered from severe refractory PPHT. He successfully received an en bloc heart-lung Tx followed by LiTx. The rationale to replace the heart was an anticipated risk of intraoperative right heart failure after liver reperfusion and the technical ease of heart-lung versus double-lung Tx.
  
  
  CONCLUSIONS
  Severe refractory PPHT is a fatal condition seen as a contraindication to LiTx. This condition can be treated by replacing thoracal organs in addition to the liver. Additional evidence via development of a registry is required to further support application of liver-(heart-)lung Tx in patients with severe refractory PPHT.
authors.affiliation: Abdominal Transplant Surgery Department, University Hospital Leuven, Belgium. Jacques.Pirenne@uz.kuleuven.ac.be
authors.forename: Jacques
authors.initials: J
authors.lastname: Pirenne
authors.forename: Geert
authors.initials: G
authors.lastname: Verleden
authors.forename: Frederik
authors.initials: F
authors.lastname: Nevens
authors.forename: Marion
authors.initials: M
authors.lastname: Delcroix
authors.forename: Dirk
authors.initials: D
authors.lastname: Van Raemdonck
authors.forename: Bart
authors.initials: B
authors.lastname: Meyns
authors.forename: Paul
authors.initials: P
authors.lastname: Herijgers
authors.forename: Willem
authors.initials: W
authors.lastname: Daenen
authors.forename: Paul
authors.initials: P
authors.lastname: De Leyn
authors.forename: Raymond
authors.initials: R
authors.lastname: Aerts
authors.forename: Willy
authors.initials: W
authors.lastname: Coosemans
authors.forename: Herbert
authors.initials: H
authors.lastname: Decaluwe
authors.forename: Gerrit
authors.initials: G
authors.lastname: Koek
authors.forename: Johan
authors.initials: J
authors.lastname: Vanhaecke
authors.forename: Marie
authors.initials: M
authors.lastname: Schetz
authors.forename: Marleen
authors.initials: M
authors.lastname: Verhaegen
authors.forename: Luca
authors.initials: L
authors.lastname: Cicalese
authors.forename: Enrico
authors.initials: E
authors.lastname: Benedetti
mesh_terms: D000328:Adult; D005260:Female; D005500:Follow-Up Studies; D016041:Heart-Lung Transplantation; D006526:Hepatitis C / Q000601:surgery; D006801:Humans; D006976:Hypertension, Pulmonary / Q000601:surgery*; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D016031:Liver Transplantation; D008297:Male; D008875:Middle Aged; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D007166:Immunosuppressive Agents