Evolution of management for Budd-Chiari syndrome: a team's view from 2564 patients.

Aim: Exploring the development of therapy for a formerly rare and life-threatening disorder, Budd-Chiari syndrome (BCS).

Methods: From 1981 to 2003, 2677 patients with BCS were admitted including 2546 cases that underwent intervention. Therapeutic means included 170 membranotomies, 181 cavoatrial shunts, 312 mesoatrial shunts including mesocavoatrial shunt, 67 mesojugular shunts, 232 radical resections, 1289 PTA, and 295 miscellaneous procedures.

Results: Patients treated in the first decade had an effective rate of 77.7%, patency for various approaches was approximately 60-90% at 7 years. In our early experience, 55.7% patients underwent shunts, 26.1% had the transcardiac membranotomy and only 0.8% the interventional modality. Recently this has shifted to 16.2%, 5.2%, and 55.6%, respectively (P < 0.01). In the early phase, 11.6% of patients were classified as stage I (less clinical severity), and 28.6% patients in stage IV (most severe). In contrast, recent patients, they were 56.1% and 4.8% (P < 0.01).

Conclusion: Chronologically, therapy has evolved from the relatively simple to the more complex (such as variety of shunts and radical correction) procedures, and finally, the interventional approaches as the main modality. However, for patients with type II (long segment occlusion in the inferior vena cava), major procedures maintain an essential role, especially the mesocavoatrial shunt. For those with type III (the HV lesions), shunts remain the procedures of choice. This increased use of the interventional methods is a reflection of greater clinical awareness and more frequent detection of much less severe cases. In stark contrast is declining numbers of advanced cases, signalling a landmark breakthrough in the management of BCS.