Sindrome epato-polmonare e ipertensione porto-polmonare. Inquadramento nosologico e considerazioni eziopatogenetiche

Translated title of the contribution: Hepatopulmonary syndrome and portopulmonary hypertension

Alessandro Milani, Maria Basso, Alessia Fiorini, Manuela Pardeo, Caterina Romano

Research output: Contribution to journalArticlepeer-review


The liver controls pulmonary vascular tone by releasing vasoactive substances. In severe liver failure, the imbalance between vasodilator and vasoconstrictive mediators may lead to alteration of the respiratory function, called as hepatopulmonary sindrome (HPS, when a significant decrease of the vascular pulmonary resistence occurs, with development of intrapulmonary vascular dilatations) and portopulmonary hypertension (PPH, when the vasoconstrictive prevalence, with an increase of the pulmonary vascular resistances). The clinical symptoms consist of various degree of dispnea and hypoxemia. An overt "cor pulmonale" syndrome with right-side heart failure may be present in the most severe forms of PPH. The alteration of pulmonary vascularization may be diagnosed by means of pulmonary angiography, contrast-enhanced echocardiography and perfusion lung nuclear scanning of the lungs. Both clinical syndromes respond poorly to medical treatment, the unique therapeutical possibilities being represented by orthotopic liver transplantation (or combined heart-lung-liver or lung-liver transplantation in patients with severe PPH).

Translated title of the contributionHepatopulmonary syndrome and portopulmonary hypertension
Original languageItalian
Pages (from-to)158-163
Number of pages6
JournalRecenti Progressi in Medicina
Issue number2
Publication statusPublished - Feb 2001

ASJC Scopus subject areas

  • Medicine(all)


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