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Semin Liver Dis 2006; 26(3): 265-272
DOI: 10.1055/s-2006-947294
Copyright © 2006 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA.

Evolving Dilemmas and Management of Portopulmonary Hypertension

Michael J. Krowka1
  • 1Division of Gastroenterology and Hepatology, Mayo Clinic College of Medicine, Rochester, Minnesota
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Publication History

Publication Date:
19 July 2006 (online)

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ABSTRACT

Portopulmonary hypertension (POPH) is an uncommon but serious pulmonary vascular consequence of portal hypertension. Untreated, POPH results in right heart failure and death, regardless of the severity of portal hypertension. The existence of moderate to severe POPH has posed higher risk for orthotopic liver transplantation (OLT). New and evolving medical approaches may change the natural history of this disorder. Such approaches include administration of prostacyclins, endothelin receptor antagonists, and phosphodiesterase inhibitors. Administration may involve 24-hour continuous infusions, periodic inhaled, and oral routes. Liver transplantation in patients with moderate to severe POPH is problematic. Aside from patients being denied OLT specifically because of POPH, intraoperative death has occurred and unacceptable mortality during the transplant hospitalization has been reported. Finally, guidelines are evolving concerning which patients will benefit from the combination of evolving medical therapies and OLT. Whether the natural history of POPH can be dramatically changed remains an optimistic conjecture.

KEYWORDS

Liver transplantation - echocardiography - pulmonary hypertension

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Michael J KrowkaM.D. 

Professor of Medicine, Vice-Chair, Division of Pulmonary and Critical Care, Division of Gastroenterology and Hepatology, Mayo Clinic College of Medicine

200 First Street SW, Rochester, MN 55905