Hepatopulmonary syndrome associated with cardiorespiratory disease
Section snippets
Study population
In our centre, during the last 3 years, we have identified five patients within a subset of 16 patients with HPS, as part of a more comprehensive study to fully evaluate pulmonary function and circulation in 83 patients with end-stage hepatic disease before and following liver transplantation, representing so far a prevalence of HPS of 19%. To be included, each patient had to meet all the criteria set out for HPS in reference 1, except for the absence of an intrinsic cardiopulmonary problem.
All
Case #1
A 54-year-old man presented with a 3-year history of progressive dyspnoea that had worsened over the previous 4 months. He was an ex-smoker of 37.5 packyears and had a 15-year history of alcohol abuse. He had been diagnosed with hepatic cirrhosis 5 years earlier on the basis of clinical, biologic, and echographic findings. He had not experienced gastrointestinal haemorrhage, hepatic encephalopathy or ascitic decompensation. He had also been diagnosed with chronic obstructive pulmonary disease
Discussion
A set of four diagnostic criteria for HPS has been proposed (1): presence of a chronic liver disease without intrinsic cardiopulmonary disease; normal chest radiograph or with nodular basal shadowing; gas exchange disturbances including an elevated AaPO2 with or without arterial hypoxaemia; and a positive CEE or, alternatively, demonstration of intravenous radiolabelled microspheres in extrapulmonary sites. Other additional features can be helpful to further establish the diagnosis of HPS, such
Acknowledgements
The authors wish to thank Professor Leo Fabbri, MD (Ferrara, Italy) for referring Case #2; and J. C. Paré, MD from the Institut de Malalties Cardiovasculars, P. Luburich, MD from the Servei de Radiodiagnòstic, and J. Ramírez, MD from the Departament d'Anatomía Patològica, all from our centre, for their generous contributions.
Supported by Grants from the Comissionat per a Universitats i Recerca (1997 SGR 00086) de la Generalitat de Catalunya and the Fondo de Investigación Sanitaria (FIS) 95/0752.
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