Hepatopulmonary syndrome with progressive hypoxemia as an indication for liver transplantation: Case reports and literature review

Michael Joseph Krowka, Michael K. Porayko, David J. Plevak, S. Chris Pappas, Jeffrey L. Steers, Ruud A F Krom, Russell H. Wiesner

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Abstract

In the hepatopulmonary syndrome (HPS), a pulmonary vascular complication of liver disease, severe hypoxemia due to pulmonary vascular dilatation can be extremely debilitating. Determining whether patients with advanced liver disease and HPS should be considered for liver transplantation is difficult. We describe three patients with progressive and severe hypoxemia who underwent successful liver transplantation and had resolution of their arterial hypoxemia. In these patients, the progressive pulmonary deterioration accelerated the need and was considered an indication for liver transplantation rather than being considered an absolute or relative contraindication. In addition, we review the literature on 81 pediatric and adult patients with HPS who underwent liver transplantation and specifically highlight mortality, morbidity, syndrome resolution, and prognostic factors. Posttransplantation mortality (16%) was associated with the severity of hypoxemia (mean arterial oxygen tension [PaO2] in 68 survivors was 54.2 ± 13.2 mm Hg and in 13 nonsurvivors was 44.7 ± 7.7 mm Hg; P<0.03). Patients with a pretransplantation PaO2 of 50 mm Hg or lower had significantly more frequent mortality (30%) in comparison with those with a PaO2 greater than 50 mm Hg (4%; P<0.02). Pulmonary recommendations that address the severity of hypoxemia and candidacy for liver transplantation are discussed.

Original languageEnglish (US)
Pages (from-to)44-53
Number of pages10
JournalMayo Clinic Proceedings
Volume72
Issue number1
StatePublished - 1997

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Hepatopulmonary Syndrome
Liver Transplantation
Lung
Blood Vessels
Mortality
Liver Diseases
Survivors
Dilatation
Arterial Pressure
Hypoxia
Pediatrics
Oxygen
Morbidity

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Krowka, M. J., Porayko, M. K., Plevak, D. J., Chris Pappas, S., Steers, J. L., Krom, R. A. F., & Wiesner, R. H. (1997). Hepatopulmonary syndrome with progressive hypoxemia as an indication for liver transplantation: Case reports and literature review. Mayo Clinic Proceedings, 72(1), 44-53.

Hepatopulmonary syndrome with progressive hypoxemia as an indication for liver transplantation : Case reports and literature review. / Krowka, Michael Joseph; Porayko, Michael K.; Plevak, David J.; Chris Pappas, S.; Steers, Jeffrey L.; Krom, Ruud A F; Wiesner, Russell H.

In: Mayo Clinic Proceedings, Vol. 72, No. 1, 1997, p. 44-53.

Research output: Contribution to journalArticle

Krowka, MJ, Porayko, MK, Plevak, DJ, Chris Pappas, S, Steers, JL, Krom, RAF & Wiesner, RH 1997, 'Hepatopulmonary syndrome with progressive hypoxemia as an indication for liver transplantation: Case reports and literature review', Mayo Clinic Proceedings, vol. 72, no. 1, pp. 44-53.
Krowka, Michael Joseph ; Porayko, Michael K. ; Plevak, David J. ; Chris Pappas, S. ; Steers, Jeffrey L. ; Krom, Ruud A F ; Wiesner, Russell H. / Hepatopulmonary syndrome with progressive hypoxemia as an indication for liver transplantation : Case reports and literature review. In: Mayo Clinic Proceedings. 1997 ; Vol. 72, No. 1. pp. 44-53.
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