IS THE DIAGNOSIS OF IDIOPATHIC NON-CIRRHOTIC PORTAL HYPERTENSION BE CONSIDERED IN PATIENTS WITH PRIMARY ANTIBODY DEFICIENCIES?

Introduction: TIPS confers a survival advantage for treating both variceal haemorrhage[1] and refractory ascites [2].Following audit of our TIPS service from 2004-2009, we redefined a multidisciplinary approach to improve technical and clinical outcome from 2009-2012.Methods: Consecutive patients undergoing attempted TIPS from 2004-2009 were identified and demographics, disease aetiology, synthetic function, technical and clinical success defined.We subsequently instituted the following changes to our TIPS service: 1. an elective TIPS anaesthetist list; 2. use of VIATORR ® PTFE-covered stents; 3. two interventional radiologists per procedure.Outcomes were re-analysed from 2009-2012.Results: From 2004-2009, 36 patients (7.2 cases/year) were identified (63.8% male; mean (SD) age 49.8 (±14.7)years; Child's A 22%/B 61%/C 17%; aetiology ALD 50%, NASH 17%, mixed aetiology 8%, PBC 6%).Indications for TIPS were variceal haemorrhage (68.2%), refractory ascites (27.3%) and portal hypertensive gastropathy (2.8%).TIPS technical success was 22/36 (61.1%).In those with successful TIPS, clinical success (resolution of bleeding or ascites) occurred in 13/22 (59.1%).Early complications occurred in 6/22 (27.2%) and 12-month survival was 9/20 (45%) (mean follow up of surviving patients 32 (±19.8)months).From 2009-2012, 64 patients (21.3 cases/year) underwent TIPS (69% male; mean (SD) age 53 (±12) years; Child's A 13%/B 44%/C 44%; aetiology ALD 61%, NASH 9%, HCV/ALD 8%).Indications were variceal haemorrhage (68.8%), ascites (29.7%) and hydrothorax (1.6%).TIPS technical success was 62/64 (96.8%, p < 0.0001) with clinical success in 53/60 (88.3%, p = 0.009).10/62 (16.1%) had early complications (4 encephalopathy; 2 infections (1 resulting in death); 2 pulmonary oedema; 2 failure to control bleeding).A further 12/59 (20.3%) developed late encephalopathy.12 month survival increased to 29/44 (65.9%, p = 0.17) (mean follow up of surviving patients 18 (±11.6)months).Conclusions: We have demonstrated that technical and clinical outcomes, in patients undergoing TIPS, can be improved by establishing a coherent multidisciplinary approach including dedicated hepatology assessment, combined with adequate interventional radiology and anaesthetic support.This co-ordinated approach is essential to deliver an effective service at a time when demand for TIPS is likely to increase[1].