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Liver Diseases pp 219-229 | Cite as

Hepato- and Porto-pulmonary Hypertension

  • Ioan Tiberiu Nanea
  • Gabriela Silvia Gheorghe
Chapter

Abstract

Porto-pulmonary hypertension and hepato-pulmonary syndrome are infrequent complications of chronic hepatic diseases and portal hypertension but their prognostic role is important, limiting the survival of the patients. They are related to the portal hypertension but their mechanisms are different. Porto-pulmonary hypertension represents the association between portal hypertension and pulmonary arterial hypertension, increased pulmonary vascular resistance > 240 dyne∙s∙cm−5 or >3 u Wood, normal pulmonary wedge pressure and normal left ventricular end diastolic pressure. The occurrence of the porto-pulmonary hypertension is related to the hyperdynamic circulation due to the porto-systemic anastomoses. Hepato-pulmonary syndrome associates chronic liver disease or portal hypertension with increased alveolar- arterial gradient in O2 (P(A-a)O2 ≥ 15 mmHg or >20 mmHg for patients >65 years old while breathing ambient air), hypoxemia (PaO2 < 80 mmHg) and intrapulmonary vascular dilation proven by contrast-enhanced echocardiography or lung perfusion scanning. The diagnosis can be often confused with concomitant pulmonary or heart diseases. Both complications are difficult to deal with. In porto-pulmonary hypertenion the efficiency of the pulmonary vasodilatation treatment is uncertain. Moderate to severe porto-pulmonary hypertension is a contraindication for hepatic transplant. The hepatic transplant represents the elective treatment for hepato-pulmonary syndrome especially in patients in which hypoxemia is improved by the administration of 100% O2. 

Keywords

Pulmonary hypertension Liver cirrhosis Porto-pulmonary hypertension Porto-pulmonary syndrome Liver transplantation 

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Further Reading

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  2. EASL Clinical Practice Guidelines. Liver transplantation. J Hepatol. 2016;64(2):433–85.  https://doi.org/10.1016/j.jhep.2015.10.006.CrossRefGoogle Scholar
  3. Fritz JS, Fallon MB, Kawut SM. Pulmonary vascular complication of liver diseases. Am J Respir Crit Care Med. 2013;187(2):133–9.CrossRefGoogle Scholar
  4. Soulaidopoulos S, Cholongitas E, Giannakoulas G, et al. Review article: Update on current and emergent data on hepatopulmonary syndrome. World J Gastroenterol. 2018;24(12):1285–98.CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2020

Authors and Affiliations

  • Ioan Tiberiu Nanea
    • 1
  • Gabriela Silvia Gheorghe
    • 1
  1. 1.Carol Davila University of Medicine and PharmacyBucharestRomania

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