TIPS for veno-occlusive disease: Is the contraindication real?
Authors: Marco Senzolo, Evangelos Cholongitas, David Patch, Andrew Kenneth Burroughs
Journal: Hepatology (2005)

To the Editor: The recent review on clinical practice guidelines for transjugular intrahepatic portosystemic shunt (TIPS) did not recommend TIPS for veno-occlusive disease (VOD)1 because TIPS has not been shown to change prognosis in patients with VOD after bone marrow transplantation (BMT). However, it was implied that VOD is only seen after BMT, whereas it can be seen in other settings in which TIPS may offer a potentially useful treatment. To date, 26 VOD patients treated with TIPS, have survived, 20% of the total2-11 (Table 1). All but 2 (with previous liver transplantation) underwent BMT. The most common causes of death, which usually occur within 1 to 3 weeks following diagnosis, were multiorgan failure (MOF), sepsis, and hemorrhage, all due to underlying hematological disease. The remainder of the deaths was due to portal hypertension and renal insufficiency complicating VOD. An issue not touched upon by the reviewer is that the reported failure of TIPS to change prognosis may be due to a misdiagnosis of VOD. Sometimes it is difficult to distinguish it from cholangitis lenta or acute graft-versus-host disease (GVHD), and so in some patients specific therapy for VOD may be delayed. The interval between the onset of VOD and TIPS placement is only mentioned in only a few reports and could influence the success of TIPS. If MOF is already present, patients are probably being treated too late. Conversely, in the only patient who survived in one series,2 the TIPS was placed 180 days after VOD diagnosis; recovery probably would have occurred in any case. Earlier intervention may be worthwhile, but it needs formal assessment. A further issue is that when VOD is complicated by direct parenchymal injury (e.g., due to cyclophosphamide), TIPS may also be contraindicated, as liver dysfunction would not be expected to improve with decompression of the hepatic outflow. Clinical VOD after liver transplantation is reported in about 2% of cases in the largest series. It seems to correlate with severe steroid resistant rejection and/or azathioprine therapy. Severe VOD in this setting does not improve with defibrotide, and retransplantation has been performed. In liver transplant patients, when VOD is diagnosed there is usually no other organ failure present (with the exception of some patients having renal dysfunction/failure). Although 70% of patients will recover spontaneously, the remainder comprise severe forms characterized by severe ascites, impaired sodium excretion, renal insufficiency, and a hepatic venous pressure gradient (HVPG) > 20 mmHg. This group of patients may benefit from hepatic decompression, providing the timing of the procedure is early in the course. If defibrotide is used, its failure could be a criterion to attempt TIPS. Thus, we believe it is important to explore the role of TIPS as an option for patients with progressive VOD without other forms of concomitant liver injury who do not respond to medical therapy. This should be before other organ failure occurs, and especially after liver transplantation, as no other therapeutic option is available except retransplantation. Marco Senzolo*, Evangelos Cholongitas*, David Patch*, Andrew Kenneth Burroughs*, * Liver Transplantation and Hepatobiliary Unit, Royal Free Hospital, London, United Kingdom.