Hepatopulmonary syndrome – commonly unrecognized complication in liver cirrhosis

Abstract

Cilj: Hepatopulmonalni sindrom (HPS) kao plućnu komplikaciju uznapredovale bolesti jetre karakterizira trijas: jetrena bolest, arterijska hipoksemija i dilatacija plućnog krvožilja. Prevalencija iznosi i do 47 %, no u kliničkoj praksi rijetko se prepoznaje i dijagnosti cira, stoga je cilj ovoga prikaza upozoriti na često postojanje HPS-a u bolesnika s cirozom jetre i respiratornom simptomatologijom, te istaknuti dijagnosti čke postupke i adekvatno liječenje. Prikaz slučaja: Bolesnik je 54-godišnjak s cirozom jetre (Child-Pugh C 10) i dispnejom. Kliničkim pregledom (dispneja) i laboratorijskim nalazima (arterijska hipoksemija, ortodeoksija) postavljena je sumnja na HPS. Primjenom kontrastne ehokardiografi ije, višeslojne računalne tomografije (MSCT) pluća i CT plućne angiografije, potvrđena je radna dijagnoza. Rasprava i zaključak: U bolesnika s cirozom jetre i respiratornim tegobama treba posumnjati na HPS i potvrditi ga odgovarajućim dijagnostičkim metodama. Dijagnostika ovog sindroma obuhvaća detaljnu anamnezu i fizikalni pregled, analizu plinova u arterijskoj krvi, preglednu snimku grudnih organa, kontrastnu ehokardiografi ju, MSCT pluća, scinti grafi ju makroagregati ma albumina obilježenih tehnecijem i, u dvojbenim slučajevima, plućnu angiografiju. Provedena su brojna istraživanja o učinkovitosti različitih lijekova, ali niti jedan se nije pokazao dovoljno učinkovitim u liječenju ovog sindroma. Jedina potencijalno kurati vna metoda danas je ortotopna transplantacija jetre (OTJ).Aim: Hepatopulmonary syndrome (HPS), a pulmonary complicati on of liver cirrhosis, is the triad of liver disease, arterial hypoxemia and intrapulmonary vascular dilatation. Prevalence may be up to 47%, but in clinical practi ce it is rarely recognized and diagnosed. We present the case of HPS in a pati ent with liver cirrhosis and respiratory simptomatology and emphasise the importance of early recogniti on of HPS because of timely and adequate therapy approach. Case report: The pati ent was a 54-year-old male with liver cirrhosis (Child Pugh C 10) and dyspnea. Clinically (dyspnea) and biochemically (arterial hypoxemia, ortodeoxia) our presumpti ve diagnosis was HPS. Early diagnosis was established by applying contrast echocardiography, lung MSCT and CT pulmonary angiography. Discussion and conclusion: It is necessary to recognize HPS as a complication in liver cirrhosis and to verify it by using proper diagnostic methods. Diagnostics of HPS include detailed anamnesis and physical examination, blood gas analysis, chest x-ray, contrast echocardiography, lung MSCT, lung perfusion scinti graphy (99m-Tc macro aggregated albumin lung perfusion scan) and CT pulmonary angiography in doubtful cases. Various trials evaluating drug effi ciency have been performed, but no medicati on has been found suitable for HPS treatment. Orthotopic liver transplantati on (OLT) is the only potential curative method