Hepatopulmonary syndrome associated with cardiorespiratory disease

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Abstract

Background/Aims: Hepatopulmonary syndrome is defined as a clinical triad including chronic liver disease, abnormal pulmonary gas exchange resulting ultimately in profound arterial hypoxaemia, and evidence of intrapulmonary vascular dilatations. We report five patients with liver cirrhosis diagnosed with hepatopulmonary syndrome whohad associated chronic obstructive or restrictive respiratory diseases.

Methods: Clinical, radiographic and constrast-enhanced echocardiographic findings, and systemic and pulmonary haemodynamic and gas exchange, including ventilation-perfusion distributions, measurements were assessed in all five patients.

Results: Echocardiography was consistent with the presence of intrapulmonary vasodilation without intracardiac abnormalities, and high resolution computed tomographic scan features were compatible with clinical (3 cases) or histopathological diagnoses (2 cases) of associated respiratory disorders. The most common prominent functional findings were moderate to severe arterial hypoxaemia, caused by moderately to severely increased intrapulmonary shunting and/or mild to moderate low ventilation-perfusion areas, and hypocarbia along with an increased cardiac output and a low pulmonary artery pressure and vascular resistance.

Conclusions: These functional characteristics, classically reported in the setting of clinically stable, uncomplicated hepatopulmonary syndrome, conform to a distinctively unique, chronic gas exchange pattern. Equally important, these pulmonary haemodynamicgas exchange hallmarks are not influenced by the coexistence of chronic cardiorespiratory disease states. These data may have clinical relevance for elective indication of hepatic transplantation in patients with life-threatening hepatopulmonary syndrome.

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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