Portopulmonary hypertension and hepatopulmonary syndrome

Marius M. Hoeper, Michael Joseph Krowka, Christian P. Strassburg

Research output: Contribution to journalArticle

354 Citations (Scopus)

Abstract

The clinically and pathophysiologically distinct entities of portopulmonary hypertension and hepatopulmonary syndrome occur in a substantial proportion of patients who have advanced liver disease of different causes. These disorders are notoriously underdiagnosed, but they have a substantial impact on survival and require focused treatment. Abnormal intrapulmonary vascular dilatation, the hallmark of hepatopulmonary syndrome, can cause profound hypoxaemia that can be very difficult to treat. By contrast, portopulmonary hypertension results from excessive pulmonary vasoconstriction and vascular remodelling that eventually leads to right-heart failure. Insights into the pathogeneses of these syndromes have led to novel therapeutic approaches. However, in severely affected patients, effective treatment remains a difficult task. In selected patients, liver transplantation represents the only treatment option, but the decision to do isolated liver transplantation is particularly challenging in patients who have severe pulmonary disease involvement. Data from several centres have contributed to provide criteria that allow improved prediction of which patients may, or may not, benefit from liver transplantation alone.

Original languageEnglish (US)
Pages (from-to)1461-1468
Number of pages8
JournalLancet
Volume363
Issue number9419
DOIs
StatePublished - May 1 2004

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Hepatopulmonary Syndrome
Hypertension
Liver Transplantation
Therapeutics
Vasoconstriction
Lung Diseases
Blood Vessels
Liver Diseases
Dilatation
Heart Failure
Lung
Survival

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Portopulmonary hypertension and hepatopulmonary syndrome. / Hoeper, Marius M.; Krowka, Michael Joseph; Strassburg, Christian P.

In: Lancet, Vol. 363, No. 9419, 01.05.2004, p. 1461-1468.

Research output: Contribution to journalArticle

Hoeper, Marius M. ; Krowka, Michael Joseph ; Strassburg, Christian P. / Portopulmonary hypertension and hepatopulmonary syndrome. In: Lancet. 2004 ; Vol. 363, No. 9419. pp. 1461-1468.
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