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.
KeywordsPulmonary hypertension Liver cirrhosis Porto-pulmonary hypertension Porto-pulmonary syndrome Liver transplantation
- 2.The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS). 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2016;37:67–119.CrossRefGoogle Scholar
- 4.Bartolome SD, Krowka MJ. Pulmonary complications of intra-abdominal disease, part 13. In: Fishman’s pulmonary diseases and disorders. 5th ed. New York: McGraw-Hill; 2015. p. 2496-1508, ISBN: 978-1-25-958912-6.Google Scholar