Djordje ]ulafi},
Mirjana Peri{i},
Predrag Rebi}
Hepatopulmonary sidrome in Budd Chiary sy.
Address correspondence to:
Dr Djordje ]ulafi}
Mirijevski venac 49, 11160 Belgrade, Serbia
Tel. +381 11 342 8020
E-mail:dculafic@EUnet.yu
Gastroenterolo{ka sekcija SLD-
01752, 2003.
Liver and biliary tract
Jetra i bilijarni trakt
Clinic for Gastroenterology and Hepatology,
Institute for Digestive Diseases,
Clinical Center of Serbia, Belgrade, Serbia
ARCH GASTROENTEROHEPATOL 2003; 22 ( No 3 - 4 ): 57 - 61
Hepatopulmonary
syndrome as complication
of Budd-Chiari syndrome:
Case report
Hepatopulonalni sindrom kao komplikacija
Budd-Chiari sindroma: Prikaz slu~aja
(accepted December 15th, 2003)
Abstract
The patients with liver disease develop significant pulmonary vascular alterations, either intrapulmonary
vascular dilation leading to the hepatopulmonary syndrome or arteriolar vasoconstriction leading to porto-
pulmonary hypertension. A35-year old women was admitted to the Institute of Digestive Diseases, Clinical
Center of Serbia Belgrade due to dyspnea, central cyanosis, dull pains below the right costal arch, fatigue
and abdominal swelling. Duplex color Doppler ultrasonography showed the enlarged, congested liver with
the right lobe diameter of 185 mm. Lobus caudatus was hypertrophied, 130 x 110 mm in diameter, with
maximum flow velocity in drainage veins of 12 cm/sec. No blood flow was recorded in hepatic veins.
Portal vein was 13 mm in diameter, with hepatopetal blood flow, mean rate of 17 cm/sec. The spleen was
enlarged, with diameter of 170 x 55 mm, and large quantity of ascites was found in abdomen. Functional
and morphological examination established the diagnosis of hepatopulmonary syndrome. When room air
was breathed, the Pa,O2 value in a supine position was 7,30 kPa, while in a sitting position the Pa,O2 value
was 6,41 kPa. When 100% oxygen was breathed, the Pa,O2 value in supine position was 36,90 kPa, while
in the sitting position the Pa,O2 value was 23,60 kPa. Higher alveolo-arterial gradient, over 2 kPa, was con-
firmed in the supine position (3,73 kPa) and in the sitting position (4,53 kPa). The scanning of extratho-
racic organs showed that the radioisotope marker 99mTc-MAA skipped intrapulmonary circulation, being
accumulated in the brain and kidneys in order to confirm intrapulmonary arterio-venous shunts. Our case
indicated that both liver injury and portal hypertension contributed to development of hepatopulmonary
syndrome, which would significantly alter the quality of life and survival in affected patients.
Key Words:
Budd-Chiari syndrome,
hepatopulmonary syndrome.
Sa`etak
Pacijenti sa oboljenjem jetre razvijaju zna~ajne vaskularne promene u plu}ima, intrapulmonarna vaskular-
na dilatacija dovodi do nastanka hepatopulmonarnog sindroma dok artriolarna vazokonstrikcija dovodi do
portno-plu}ne hipertenzije. Pacijentkinja stara 35. godina primljena je na Institut za bolesti Digestivnog
sistema, Klini~kog Centra Srbije u Beogradu zbog dispnee, cijanoze centralnog tipa, tupih bolova ispod
desnog rebarnog luka, malaksalosti i oticanja trbuha. Dupleks kolor Doppler ultrasonografijom uo~ena je
uve}ana, kongestivna jetra sa promerom desnog lobusa 185 mm. Lobus kaudatus bio je hipertrofisan,
promera 130 x 110 mm, sa maksimalnim brzinom protoka krvi u drena`nim venama od 12 cm/sec. U
hepati~kim venama nije registrovan protok krvi. Portna vena bila je {irine 13 mm, sa hepatopetalnim pro-
tokom krvi, prose~ne brzine 17 cm/sec. Slezina je bila uve}ana, kraniokaudalnog promera 170 x 55 mm.
U abdomenu je bila prisutna velika koli~ina slobodne te~nosti. Na osnovu funkcionalnih i morfolo{kih
ispitivanja postavljena je dijagnoza hepatopulmonarnog sindroma. U uslovima udisanja sobnog vazduha
Pa,O2 iznosio je 7,30 kPa u le`e}em, dok je u sede}em polo`aju Pa,O2 iznosio 6,41 kPa. U situaciji kada
je udisan 100% kiseonik Pa,O2 iznosio je 36,90 kPa u le`e}em, dok je u sede}em polo`aju Pa,O2 iznosio
23,60 kPa. Povi{en alveolo-arterijalni gradijent, preko 2 kPa, potvr|en je u le`e}em (3,73 kPa) i sede}em
polo`aju (4,53 kPa). Skeniranjem ekstratorakalnih organa uo~ena je akumulacija radiofarmaka 99mTc-
MAA u mozgu i bubrezima, ~ime je potvr|eno intrapulmonaro artrio-vensko {antiranje. Na{ prikaz ukazu-
je da udru`enim delovanjem, o{te}enje jetre i portna hipertenzija, dovode do hepatopulmonarnog sindro-
ma, koji zna~ajno uti~e na kvalitet `ivota i du`inu pre`ivljavanja.
Klju~ne re~i:
Budd-Chiari sindrom,
hepatopulmonarni sindrom
