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Semin Liver Dis 2000; Volume 20(Number 03): 391-396
DOI: 10.1055/s-2000-9392
Copyright © 2000 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA. Tel.: +1(212) 584-4662

DIAGNOSTIC PROBLEMS IN HEPATOLOGY

Portal Hypertension and Hepatopulmonary Syndrome in a Middle-Aged Man with Hepatitis B InfectionM. ISABEL. FIEL, THOMAS. D. SCHIANO1,2 , ARIEF. SURIAWINATA, SUKRU. EMRE2
  • 1Lillian and Henry M. Stratton-Hans Popper Department of Pathology; Division of Liver Diseases, Department of Medicine Mount Sinai School of Medicine of the City University of New York, New York
  • 2Recanati-Miller Transplant Institute, Mount Sinai School of Medicine of the City University of New York, New York
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Publication History

Publication Date:
31 December 2000 (online)

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CASE REPORT

A 55-year-old Turkish man was evaluated for orthotopic liver transplantation (OLT) because of advanced liver disease due to chronic hepatitis B viral infection (HBV), which he contracted in Turkey 35 years previously. Chest X-ray and echocardiogram were unremarkable at the time.

More recently, the patient suffered from incapacitating fatigue, generalized weakness, memory loss, pedal edema, and worsening shortness of breath. He was hospitalized several times due to hepatic encephalopathy. Physical examination demonstrated 4+ clubbing of the fingers, splenomegaly, peripheral edema, asterixis, and psychomotor retardation. He had proximal muscle wasting, but there was no jaundice or ascites. There was orthodeoxia and platypnea.

Laboratory testing demonstrated a total serum bilirubin of 2.8 mg/dL (nl = 1-1.5), prothrombin time of 16.2 seconds (nl = 10.3-13), and a serum albumin level of 2.6 g/dL (nl = 3.3-5.3). Four months earlier, the prothrombin time was 15.7 seconds and the albumin was 3.3 g/L. The alkaline phosphatase level was 104 U/L (nl = 32-115), ALT 32 U/L (nl = 2-50), AST 50 U/L (nl = 2-40). Serologic testing showed reactivity for HBsAg. Anti-HBe, anti-HBs, HBeAg, HBV-DNA, anti-HDV, and anti-HCV were all non-reactive. Autoantibodies were not detectable and serum iron indices were normal.

Chest x-ray showed a fine nodular reticular pattern in the lungs. Pulmonary function testing demonstrated a moderate restrictive defect with a diffusion capacity of 57%. Kveim test was inconclusive. Doppler echocardiogram showed left ventricular hypertrophy and, when agitated saline was infused, a right to left shunt was demonstrated consistent with pulmonary arteriovenous malformation and hepatopulmonary syndrome. CT scan of the chest demonstrated cardiomegaly and enlargement of both hila. The azygous vein was prominent with no lymphadenopathy noted. CT scan of the abdomen showed a small liver, estimated to be 700 mL in size with diffuse heterogenous echogenicity, and splenomegaly.

The clinical diagnosis was Child's C cirrhosis related to chronic hepatitis B, complicated by hepatopulmonary syndrome. The pulmonary disease was considered to be reversible with liver transplantation. Orthotopic liver transplantation was performed.

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