Combined liver and (heart-)lung transplantation in liver transplant candidates with refractory portopulmonary hypertension.
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.
Pirenne
Jacques
J
Abdominal Transplant Surgery Department, University Hospital Leuven, Belgium. Jacques.Pirenne@uz.kuleuven.ac.be
Verleden
Geert
G
Nevens
Frederik
F
Delcroix
Marion
M
Van Raemdonck
Dirk
D
Meyns
Bart
B
Herijgers
Paul
P
Daenen
Willem
W
De Leyn
Paul
P
Aerts
Raymond
R
Coosemans
Willy
W
Decaluwe
Herbert
H
Koek
Gerrit
G
Vanhaecke
Johan
J
Schetz
Marie
M
Verhaegen
Marleen
M
Cicalese
Luca
L
Benedetti
Enrico
E
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
D007166:Immunosuppressive Agents