Combined liver and (heart-)lung transplantation in liver transplant candidates with refractory portopulmonary hypertension

J. Pirenne; G. Verleden; F. Nevens; M. Delcroix; D. van Raemdonck; B. Meyns; P. Herijgers; W. Daenen; P. de Leyn; R. Aerts; W. Coosemans; H. Decaluwe; G.H. Koek; J. Vanhaecke; M. Schetz; M. Verhaegen; L. Cicalese; E. Benedetti

doi:10.1097/00007890-200201150-00025

Combined liver and (heart-)lung transplantation in liver transplant candidates with refractory portopulmonary hypertension

J. Pirenne^*
, G. Verleden
, F. Nevens
, M. Delcroix
, D. van Raemdonck
, B. Meyns
, P. Herijgers
, W. Daenen
, P. de Leyn
, R. Aerts
, W. Coosemans
, H. Decaluwe
, G.H. Koek
, J. Vanhaecke
, M. Schetz
, M. Verhaegen
, L. Cicalese
, E. Benedetti

^*Corresponding author for this work

NUTRIM - Institute of Nutrition and Translational Research in Metabolism

Research output: Contribution to journal › Article › Academic › peer-review

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. CONCLUSION: 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.

Original language	English
Pages (from-to)	140-2
Journal	Transplantation
Volume	73
Issue number	1
DOIs	https://doi.org/10.1097/00007890-200201150-00025
Publication status	Published - 1 Jan 2002

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Pirenne, J., Verleden, G., Nevens, F., Delcroix, M., van Raemdonck, D., Meyns, B., Herijgers, P., Daenen, W., de Leyn, P., Aerts, R., Coosemans, W., Decaluwe, H., Koek, G. H., Vanhaecke, J., Schetz, M., Verhaegen, M., Cicalese, L., & Benedetti, E. (2002). Combined liver and (heart-)lung transplantation in liver transplant candidates with refractory portopulmonary hypertension. Transplantation, 73(1), 140-2. https://doi.org/10.1097/00007890-200201150-00025

@article{09572fd036bc40fa887ed1c22868d06e,

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. CONCLUSION: 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.",

author = "J. Pirenne and G. Verleden and F. Nevens and M. Delcroix and \{van Raemdonck\}, D. and B. Meyns and P. Herijgers and W. Daenen and \{de Leyn\}, P. and R. Aerts and W. Coosemans and H. Decaluwe and G.H. Koek and J. Vanhaecke and M. Schetz and M. Verhaegen and L. Cicalese and E. Benedetti",

year = "2002",

month = jan,

day = "1",

doi = "10.1097/00007890-200201150-00025",

language = "English",

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journal = "Transplantation",

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Pirenne, J, Verleden, G, Nevens, F, Delcroix, M, van Raemdonck, D, Meyns, B, Herijgers, P, Daenen, W, de Leyn, P, Aerts, R, Coosemans, W, Decaluwe, H, Koek, GH, Vanhaecke, J, Schetz, M, Verhaegen, M, Cicalese, L & Benedetti, E 2002, 'Combined liver and (heart-)lung transplantation in liver transplant candidates with refractory portopulmonary hypertension', Transplantation, vol. 73, no. 1, pp. 140-2. https://doi.org/10.1097/00007890-200201150-00025

TY - JOUR

T1 - Combined liver and (heart-)lung transplantation in liver transplant candidates with refractory portopulmonary hypertension

AU - Pirenne, J.

AU - Verleden, G.

AU - Nevens, F.

AU - Delcroix, M.

AU - van Raemdonck, D.

AU - Meyns, B.

AU - Herijgers, P.

AU - Daenen, W.

AU - de Leyn, P.

AU - Aerts, R.

AU - Coosemans, W.

AU - Decaluwe, H.

AU - Koek, G.H.

AU - Vanhaecke, J.

AU - Schetz, M.

AU - Verhaegen, M.

AU - Cicalese, L.

AU - Benedetti, E.

PY - 2002/1/1

Y1 - 2002/1/1

N2 - 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. CONCLUSION: 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.

AB - 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. CONCLUSION: 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.

U2 - 10.1097/00007890-200201150-00025

DO - 10.1097/00007890-200201150-00025

M3 - Article

SN - 0041-1337

VL - 73

SP - 140

EP - 142

JO - Transplantation

JF - Transplantation

IS - 1

ER -

Combined liver and (heart-)lung transplantation in liver transplant candidates with refractory portopulmonary hypertension

Abstract

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