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

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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 languageEnglish
Pages (from-to)140-2
JournalTransplantation
Volume73
Issue number1
DOIs
Publication statusPublished - 1 Jan 2002

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