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

Jacques Pirenne; Geert Verleden; Frederik Nevens; Marion Delcroix; Dirk Van Raemdonck; Bart Meyns; Paul Herijgers; Willem Daenen; Paul De Leyn; Raymond Aerts; Willy Coosemans; Herbert Decaluwe; Gerrit Koek; Johan Vanhaecke; Marie Schetz; Marleen Verhaegen; Luca Cicalese; Enrico Benedetti

doi:10.1097/00007890-200201150-00025

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

Jacques Pirenne, Geert Verleden, Frederik Nevens, Marion Delcroix, Dirk Van Raemdonck, Bart Meyns, Paul Herijgers, Willem Daenen, Paul De Leyn, Raymond Aerts, Willy Coosemans, Herbert Decaluwe, Gerrit Koek, Johan Vanhaecke, Marie Schetz, Marleen Verhaegen, Luca Cicalese, Enrico Benedetti

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

46 Scopus citations

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 (US)
Pages (from-to)	140-142
Number of pages	3
Journal	Transplantation
Volume	73
Issue number	1
DOIs	https://doi.org/10.1097/00007890-200201150-00025
State	Published - Jan 15 2002
Externally published	Yes

ASJC Scopus subject areas

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, G., 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), 140-142. https://doi.org/10.1097/00007890-200201150-00025

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, 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. 140-142. https://doi.org/10.1097/00007890-200201150-00025

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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 = "Jacques Pirenne and Geert Verleden and Frederik Nevens and Marion Delcroix and {Van Raemdonck}, Dirk and Bart Meyns and Paul Herijgers and Willem Daenen and {De Leyn}, Paul and Raymond Aerts and Willy Coosemans and Herbert Decaluwe and Gerrit Koek and Johan Vanhaecke and Marie Schetz and Marleen Verhaegen and Luca Cicalese and Enrico Benedetti",

year = "2002",

month = jan,

day = "15",

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

language = "English (US)",

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pages = "140--142",

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

AU - Pirenne, Jacques

AU - Verleden, Geert

AU - Nevens, Frederik

AU - Delcroix, Marion

AU - Van Raemdonck, Dirk

AU - Meyns, Bart

AU - Herijgers, Paul

AU - Daenen, Willem

AU - De Leyn, Paul

AU - Aerts, Raymond

AU - Coosemans, Willy

AU - Decaluwe, Herbert

AU - Koek, Gerrit

AU - Vanhaecke, Johan

AU - Schetz, Marie

AU - Verhaegen, Marleen

AU - Cicalese, Luca

AU - Benedetti, Enrico

PY - 2002/1/15

Y1 - 2002/1/15

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.

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EP - 142

JO - Transplantation

JF - Transplantation

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ER -

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

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