Occurrence of hepatopulmonary syndrome in Budd-Chiari syndrome and the role of venous decompression.
OBJECTIVE
Hepatopulmonary syndrome (HPS) has been predominantly detected in cirrhotic patients and rarely in patients with noncirrhotic portal hypertension. The aim of this study was to determine the occurrence of HPS in patients with Budd-Chiari syndrome (only anecdotal reports available) and evaluate the role of venous decompression in its reversal.


METHODS
Twenty-nine consecutive cases of Budd-Chiari syndrome without primary cardiopulmonary disease were investigated by air contrast echocardiography and arterial blood gas analysis. Venous decompression (e.g., by balloon cavoplasty) was attempted when feasible.


RESULTS
Eight cases (27.6%) of HPS and 9 cases (31.0%) with positive contrast echocardiography but unimpaired oxygenation were detected. Duration of disease was longer (P = 0.026) among those with positive contrast echocardiography. Cavoplasty reversed 4 of 5 cases of HPS and 2 of 2 cases with positive contrast echocardiography alone. Venous decompression by drainage of amebic liver abscess (which was compressing hepatic venous outflow) also reversed 1 case of HPS. HPS was relieved by venous decompression in 5 of 6 cases.


CONCLUSIONS
HPS developed in a substantial fraction of our patients with Budd-Chiari syndrome, with positive contrast echocardiography occurring mainly in the benign, slowly progressing variety. Venous decompression showed promise in reversing such cases.
De
Binay K
BK
Department of Medicine, Institute of Post Graduate Medical Education and Research, Calcutta, India. binaykde@apexmail.com
Sen
Sambit
S
Biswas
Pranab K
PK
Mandal
Sanjay K
SK
Das
Debasish
D
Das
Utpalendu
U
Guru
Supriya
S
Bandyopadhyay
Kausik
K
D000328:Adult; D000800:Angioplasty, Balloon; D006502:Budd-Chiari Syndrome / Q000150:complications* / Q000000981:diagnostic imaging / Q000628:therapy*; D019299:Decompression, Surgical; D004452:Echocardiography; D005260:Female; D005500:Follow-Up Studies; D006503:Hepatic Veins; D020065:Hepatopulmonary Syndrome / Q000000981:diagnostic imaging / Q000209:etiology* / Q000628:therapy*; D006801:Humans; D008101:Liver Abscess, Amebic / Q000150:complications / Q000000981:diagnostic imaging / Q000628:therapy; D008297:Male; D014682:Vena Cava, Inferior