Téma: Overview works

The hepatopulmonary syndrome

Martin Zima

The hepatopulmonary syndrome is characterized by the concomitant presence of hepatic dysfunction (chronic or acute), and/or portal hypertension, intrapulmonary vascular dilatation and impaired arterial oxygenation in the absence of other cardiopulmonary disease. The term hepatopulmonary syndrome was suggested by Kennedy and Knudson in 1977, who described a patient in whom exertional dyspnea developed 4 years after portocaval shunting for complications of alcoholic cirrhosis. The pathophysiology of hepatopulmonary syndrome ist stil unknown. The most accepted hypothesis proposes a defect in the synthesis and metabolism of pulmonary vasoactive substances by the impaired liver. On the basis of their effects capillary and precapillary vascular dilatations and/or arteriovenous shunts develops with consecutive oxygen desaturation. Pulmonary angiography is helpful in delineating pulmonary vascular abnormalities. The hepatopulmonary syndrome type I („minimal or advanced“) is characterized by a diffuse pattern that responds well to 100% oxygen. Type II is chracterized by focal arteriovenous malformations, and have a poorer response to 100% oxygen.Various therapeutic options have either failed or produced minimal improvement. Supplemental oxygen therapy (2–4 l/min) may provide symtomatic relief, but liver transplantation is the most promising therapy for hepatopulmonary syndrome type I and vascular embolization for type II.

Ročník 2004  Témy časopisu Internal medicine 9 / 2004

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MUDr. Viera Fedelešová, CSc.
prof. MUDr Martin Haluzík, CSc.
prof. MUDr. Štefan Hrušovský, CSc. Dr.SVS.
prof. MUDr. Rudolf Hyrdel, CSc.
doc. MUDr. Oľga Jurkovičová, CSc.
doc. MUDr. Zdenko Killinger, PhD.
doc. MUDr. Soňa Kiňová, PhD.
prof. MUDr. Peter Mitro, PhD.
doc. MUDr. Viliam Mojto, CSc., MHA
prof. MUDr. Karel Pacák, DrSc.
prof. MUDr. Juraj Payer, CSc., FRCP
doc. MUDr. Ján Podoba, CSc.
prof. MUDr. Igor Riečanský, DrSc.
doc. MUDr. Ján Staško, PhD.
doc. MUDr. Mária Szántová, PhD.
prof. MUDr. Ivan Tkáč, PhD.

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1. Shaheen NJ, Crosby NA, Bozymski EM, et al. Is there publication bias in the reporting cancer risk in Barrett ́ esophagus? Gastroenterology 2000; 119: 333-338.
2. Stenestrand U, Wallentin L. Swedish Register of Cardiac Intensive Care (RIKS-HIA): Early statin treatment following acute myocardial infarction and 1-year survival. JAMA 2001; 285(4): 430-436.
3. LIPID Study Group. Prevention of cardiovascular events and death with pravastatin in patients with coronary heart disease and a broad range of initial cholesterol levels. N Engl J Med 1998; 339: 1349-1357.
4. Jurkovičová O, Spitzerová H, Cagáň S. Komorové arytmie a náhla srdcová smrť pri akútnom infarkte myokardu. Bratisl Lek Listy 1997; 98: 379-389.
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d. endothelial growth factor


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