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 School 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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10.1097/00007890-200201150-00025

Cite this

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 -