been published due to the rarity of BCS. 3,4,5,11 We present the results of a clinical study of PTBA in the treatment of 101 patients with BCS.

Transcription

1 Feasibility and midterm outcomes of percutaneous transhepatic balloon angioplasty for symptomatic Budd-Chiari syndrome secondary to hepatic venous obstruction Tianxiao Li, MD, a Shuiting Zhai, MD, a Zhigang Pang, MD, b Xiuxian Ma, MD, c Huicun Cao, MD, a Weixing Bai, MD, a Ziliang Wang, MD, a and Wayne W. Zhang, MD, d Zhengzhou, Henan, Peoples Republic of China; and Seattle, Wash Objectives: This study evaluated the feasibility and outcomes of percutaneous transhepatic balloon angioplasty (PTBA) of the hepatic vein in the management of Budd-Chiari syndrome (BCS) secondary to hepatic venous outflow obstruction. Methods: From September 1996 to October 2008, 101 patients (52 males, 49 females) with BCS secondary to occlusion of the hepatic veins were prospectively treated using PTBA of the hepatic vein. Average age was 31.3 years (range, years). Nineteen had concurrent inferior vena cava (IVC) obstruction. All the patients presented with symptomatic portal hypertension. PTBA, with or without stenting, was performed after hepatovenography. Results: PTBA was successfully performed in 92 of the 101 patients. Sixty-eight patients underwent PTBA of right hepatic vein, followed by stent placement in two. PTBA was performed in 11 patients with left hepatic vein occlusion and in 13 patients with dominant accessory hepatic vein occlusion. The technical success rate was 92 of 101 (91%). Hepatic venous pressure was significantly decreased after balloon angioplasty/stenting (P <.01, paired t test). Symptoms were significantly improved in the 92 patients who had successful PTBA. Three patients had acute hepatic vein thrombosis during or after PTBA. Two patients sustained intraperitoneal bleeding from the transhepatic puncture track, and one had intrahepatic hematoma. Pulmonary embolism developed in one patient during the operation. All complications were managed nonoperatively. There were no perioperative deaths. Within 1 year, 74 of the 101 patients returned for follow-up, and 51 patients had follow-up at 2 years. The primary patency rates were 84% (62 of 74), 78% (58 of 74), and 76% (39 or 51) at 6, 12, and 24 months after PTBA, respectively. The secondary patency rates were 95% (70 of 74), 92% (68 of 74), and 84% (43 of 51) at 6, 12, and 24 months. Conclusions: PTBA of the hepatic vein is a safe and effective treatment of BCS. It is currently the most physiologic procedure, and the risk of postoperative encephalopathy is minimized because portal flow is not diverted. Midterm outcomes are satisfactory. Further investigation of the long-term outcomes is needed. (J Vasc Surg 2009;50: ) Budd-Chiari syndrome (BCS) is an uncommon condition characterized by hepatic venous outflow obstruction. 1,2 The obstruction may be caused by thrombosis, extrinsic compression, membranous webs, or postoperative complications after liver transplantation. Thrombus is the most frequent cause in Western countries, and membranous webs are more common in Asia. 3 Various treatments have been described, including anticoagulation, percutaneous balloon angioplasty with or without stenting, transjugular intrahepatic portosystemic shunt (TIPS), surgical shunts, and liver transplantation. However, only small series of patients treated with percutaneous transhepatic balloon angioplasty (PTBA) have From the Department of Interventional Radiology, People s Hospital of Henan Province, a Department of Surgery, Second Affiliated Hospital, Zhengzhou University, b and Department of Surgery, First Affiliated Hospital, Zhengzhou University, Zhengzhou c ; and Department of Surgery, Group Health Central Hospital, Seattle. d Competition of interest: none. Reprint requests: Tianxiao Li, MD, Department of Interventional Radiology, People s Hospital of Henan Province, Zhengzhou, Henan, People s Republic of China ( dr.litianxiao@yahoo.com.cn) /$36.00 Copyright 2009 by the Society for Vascular Surgery. doi: /j.jvs been published due to the rarity of BCS. 3,4,5,11 We present the results of a clinical study of PTBA in the treatment of 101 patients with BCS. MATERIAL AND METHODS From September 1996 to October 2008, 101 patients (52 males, 49 females) with symptomatic BCS due to hepatic vein occlusion, with or without inferior vena cava (IVC) obstruction, were treated with PTBA at the People s Hospital of Henan Province, China. This clinical study was approved by the Committee of Medical Affairs of People s Hospital of Henan Province. The medical records of the patients were retrospectively reviewed. The 101 patients presented with secondary portal hypertension for 3 to 156 months. Common clinical presentations are stomach bloating, abdominal pain, upper gastrointestinal bleeding, jaundice, hepatomegaly, and ascites (Table I). Patients were a mean age of 31.3 years (range, years). Of the 101 patients, 51 (50%) had occlusion in all three hepatic veins (right, middle, and left), 35 (35%) had disease in two veins, and 11 (11%) had single vein involvement. Thirteen (13%) had occlusion in dominant accessory he- 1079

2 1080 Li et al JOURNAL OF VASCULAR SURGERY November 2009 Table I. Comparison of clinical presentations before and after percutaneous transhepatic balloon angioplasty Presentation Pre-PTBA, No. (%) (N 101) Post-PTBA, No. (%) 6 mon 12 mon 24 mon (n 74) (n 74) (n 51) Stomach bloating 79 (78) 8 (11) 8 (11) 7 (14) Abdominal pain 34 (34) 4 (5) 3 (4) 2 (4) Upper GI bleeding 13 (13) Jaundice 11 (11) (2) Hepatomegaly 79 (78) 8 (11) 4 (5) 4 (8) Ascites 58 (57) 3 (4) 5 (7) 5 (10%) GI, Gastrointestinal; PTBA, percutaneous transhepatic balloon angioplasty. Fig 1. Percutaneous transhepatic venography demonstrates occlusion of the hepatic venous outflow. patic veins. Four patients (4%) had diffused stenosis or occlusion in all the hepatic veins and branches. Right hepatic vein obstruction was found 82% (83 of 101), followed by middle in 50% and left hepatic veins in 41%. In 19 patients, hepatic vein occlusion was combined with IVC stenosis. Procedure techniques Percutaneous transhepatic venography. Under fluoroscopic guidance, a 21-gauge Chiba needle with syringe is inserted through the eighth or ninth intercostals space on the midaxillary line. Negative pressure is applied on the syringe while the needle is slowly advanced in the direction toward the xiphoid. After blood return, a small amount of contrast is injected. When the entrance of right hepatic vein is confirmed, a inch guidewire is placed and the needle is removed. A 4F angiocatheter is then inserted into the right hepatic vein over the guidewire. Percutaneous Fig 2. A, Percutaneous transhepatic venography shows occlusion of the right hepatic vein. B, The occlusive lesion is successfully traversed under fluoroscopic guidance with roadmap. transhepatic venography is performed by injecting intravenous contrast through the catheter (Fig 1). The same technique is used for the left hepatic venography, but the puncture site is usually 2 to 3 cm below the xiphoid. Hepatic venous pressure is measured through the side port of the introducer sheath and recorded as cm H 2 O. Traversing obstruction. After hepatic venography, heparin is given intravenously at 50 IU/kg. A inch

3 JOURNAL OF VASCULAR SURGERY Volume 50, Number 5 Li et al 1081 Fig 3. A-D, The patient presented with symptomatic Budd-Chiari syndrome secondary to hepatic vein occlusion. Percutaneous transhepatic balloon angioplasty was performed and the right hepatic vein was widely patent after balloon angioplasty. exchange wire is then placed through the 4F angiocatheter, and a 7F introducer sheath is inserted over the wire after the angiocatheter is removed. To cross the occlusive lesion, we use a inch Glidewire (Boston Scientific, Natick, Mass) and a 4F Headhunter catheter (Cook, Bloomington, Ind). The Glidewire can easily pass through partial occlusive lesions. For the membranous or segmental obstructive lesions, we advance Headhunter catheter against the stump of the hepatic vein, then push and spin the Glidewire while advancing. If this technique fails, the stiff back end of the Glidewire can be used under the guidance of continuous fluoroscopy with roadmap. The stiff end of the glidewire should be advanced slowly and with 3 mm each movement. We successfully crossed 24 complete occlusive lesions in our group using this technique (Fig 2). Balloon angioplasty and stenting. After the obstruction is transversed, the Glidewire is switched to a stiff wire.

4 1082 Li et al JOURNAL OF VASCULAR SURGERY November 2009 Table II. Comparison of free hepatic venous pressure before and after percutaneous transhepatic balloon angioplasty FHVP Veins Pre-PTBA Post-PTBA No. Mean SD Mean SD Hepatic vein Right Left Accessory Mean FHVP FVHP, Free hepatic venous pressure; PTBA, percutaneous transhepatic balloon angioplasty; SD, standard deviation. A balloon catheter is then inserted over the stiff wire. PTBA is performed till the waist disappears and remains for 2 minutes (Fig 3). Noncompliant balloons from 12 to 16 mm in diameter are used according to the sizes of hepatic veins. If a follow-up angiogram shows significant residual stenosis or recoiling after 15 minutes, a stent should be placed. Two patients in our group required stenting, and one mm Wallstent (Boston Scientific) was placed in each. Angioplasty is with a large balloon in patients with combined IVC stenosis. At the end of procedure, the introducer sheath is withdrawn into hepatic parenchyma. Gelfoam strips are pushed into the intrahepatic puncture track using inner dilator of the introducer sheath while the sheath is slowly pulled out. Patients were treated postoperatively with heparin drips and discharged home with oral warfarin for 6 months. RESULTS PTBA was successful in 92 of the 101 patients (91%). Venography demonstrated significant improvement of the hepatic venous outflow after balloon angioplasty. Free hepatic vein pressure (FHVP) was measured before and after PTBA. Hepatic venous pressures before and after PTBA were decreased in each patient. Mean pressure drops in this group were statistically significant after balloon angioplasty (Table II). Symptoms were significantly improved in the 92 patients who had successful PTBA. There were no perioperative deaths. Technique failure occurred in nine patients (9%). In four patients the long segmental occlusive lesions could not be crossed with different catheters and wires. The other four patients had diffused hepatic venous stenoses that were unsuitable for endovascular intervention due to lack of target veins. A large thrombus developed in one patient during the operation and the procedure was aborted. The nine patients with technical failure were referred to surgery for an open shunt. One patient sustained pulmonary embolization during balloon angioplasty and was successfully managed conservatively. Postoperatively, two patients had intraperitoneal bleeding and intrahepatic hematoma developed in one. Anticoagulation was temporarily held and bleeding stopped in all three patients without surgical intervention. P All of the patients in whom PTBA was successful underwent a baseline duplex ultrasound examination before discharge. Postoperative follow-up at 6, 12, and 24 months showed that primary patency rates were 84%, 78%, and 76%, respectively (Fig 4). In 12 patients (12%), redo PTBA was required for recurrent symptoms 24 months and was successful in all. The secondary patency rates were 95%, 92%, and 84% at 6, 12, and 24 months. Clinical presentations (Table I) and liver functions (Table III) were improved in most patients who had successful PTBA. Asymptomatic patients with hepatic vein restenosis after PTBA were managed with anticoagulation and follow-up ultrasound imaging. Univariate and multivariate analysis demonstrated that long segmental stenosis or occlusion is a risk factor for post-ptba hepatic venous restenosis. Sex, age, history of hepatitis, liver function, and clinical presentations at admission are not associated with the patency of the hepatic veins after PTBA. DISCUSSION BCS is a complex disorder characterized by hepatic venous outflow obstruction that may be located from small hepatic veins to the atriocaval junction. Obstruction is usually caused by thrombosis of hepatic veins, membranous webs within hepatic vein or IVC, or both, extrinsic compression from tumor or cyst, or postoperative complications after liver transplantation. 2,3,5-7 Thrombosis is the most frequent cause in Western countries, 3 while membranous webs is more common in Asia. 8 BCS may also be multifactorial in some patients. 9 Patients with BCS may present with hepatomegaly, right upper quadrant pain, ascites, or be asymptomatic. 10 The natural history of BCS is poorly understood. Prognosis is usually dismal if the disease is left untreated. Owing to the rarity of BCS, no controlled prospective study has been performed. The management modalities that have been published include anticoagulation and treatment of underlying diseases, percutaneous transhepatic balloon angioplasty with or without stenting, TIPS, surgical shunts, and liver transplantation. 3,4,5,11 Medical treatment with anticoagulation, diuretic therapy, and periodic paracentesis is associated with poor oucomes. 12,13 Plessier et al 13 reported that 20% of the patients with BCS were successfully treated with conservative management alone. The use of a surgical portosystemic shunt in the treatment of BCS is debated because perioperative mortality has been documented up to 50%. 2,14,15 Therefore, open shunting procedures have been almost abandoned as an option for the treatment of BCS in Europe. 16 Liver transplantation should be reserved for patients with presentations of fulminant hepatic failure and end-stage chronic cirrhosis. 5 Percutaneous endovascular intervention has been increasing in the treatment of BCS in recent years. Restoration of good outflow in one of the three main hepatic veins is usually adequate to resolve BCS symptoms. 4,5,11 A retrospective review demonstrated that the mortality rates

5 JOURNAL OF VASCULAR SURGERY Volume 50, Number 5 Li et al Survival Function of Covariates 1.0 Cum Survival-Patency Time: Post-PTBA (Days) Variables in the Equation Step 1 95% CI for Exp(B) B SE Wald Df Sig. Exp(B) Lower Upper Fig 4. Cox Hazard modeling analysis shows the patency of hepatic veins after percutaneous transhepatic balloon angioplasty (PTBA). CI, Confidence interval; SE, standard error. Table III. Liver function changes (Child-Pugh) after percutaneous transhepatic balloon angioplasty Child-Pugh class Pre-PTBA Post-PTBA 6 mon 12 mon 24 mon A B C Patients, total PTBA, Percutaneous transhepatic balloon angioplasty. were 14% (3 of 21) in patients undergoing percutaneous intervention vs 44% (8 of 18) in the open shunting group. 17 However, reocclusion rates are higher after percutaneous endovascular procedures. 4,5 In 1993 the first report of TIPS for the treatment of BCS was published. 5 This procedure can effectively decompress the portal venous system and significantly improve the symptoms in patients with BCS. Currently, TIPS has been used as the first choice of BCS treatment in most insititutions. 18 Stent misplacement or migration during or after TIPS procedure is not uncommon, however. 8,19 Eapen et al 11 stated that TIPS was technically more difficult in BCS treatment, and reocclusion was more common due to the increased thrombotic tendency and longer shunt requirement in the BCS group than in other patients. Hepatic encephalopathy after TIPS is another major complication. Hepatic encephalopathy occurred in 17% of the BCS patients who underwent TIPS. 11 In the patients presenting with encephalopathy, the outcomes of TIPS were even worse. One of their three patients with encephalopathy at presentation died shortly after TIPS procedure. As the most physiologic technique restoring hepatic venous outflow, 4,11 percutaneous hepatic vein balloon angioplasty through the internal jugular vein, femoral vein, or the transhepatic approach has become more attractive. 3,4,5,11 PTBA of hepatic veins was successfully performed in 92 of our 101 patients, and the rates of postprocedural encephalopathy and death were zero. Our study also showed favorable midterm outcomes, with a primary patency of 76% and secondary patency of 84% at the 2-year follow-up. Using percutaneous hepatic vein revascularization as the first choice of BCS treatment has been recommended. 3,4,5,11 Most percutaneous intervention procedures are performed through transjugular or femoral approaches. Although a percutaneous transhepatic approach has been reported, 11,20 bleeding from the puncture track has been a major concern. A combined transjugular and percutaneous transhepatic technique avoids a large puncture track in the liver. 11 The procedure is more challenging, however, with prolonged radiation and operation time. To prevent bleeding from the puncture track after transhepatic intervention, we routinely insert Gelfoam strips through the introducer sheath while it is slowly being pulled out. The puncture track is sealed at the end of the procedures. Only 3.3% of our patients (3 of 92) had perihepatic hematoma or intraperitoneal bleeding after PTBA, and all were successfully managed conservatively.

6 1084 Li et al JOURNAL OF VASCULAR SURGERY November 2009 CONCLUSIONS PTBA is currently the most physiologic modality for the treatment of BCS. The technical success rate of PTBA is 90%. Post-PTBA hepatic encephalopathy is minimized because there is no diversion of portal flow compared with shunt procedures such as TIPS. Packing of the transhepatic puncture track with Gelfoam strips may effectively prevent postoperative bleeding. Our experience demonstrated that PTBA is effective and safe, with satisfactory midterm outcomes in the treatment of BCS. Long-term follow-up is needed. AUTHOR CONTRIBUTIONS Conception and design: TL, WZ Analysis and interpretation: TL, SZ, XM, HC, WZ Data collection: TL, SZ, ZP, XM, HC, WB, ZW Writing the article: TL, ZP, WZ Critical revision of the article: TL, ZP, WZ Final approval of the article: TL, WZ Statistical analysis: TL Obtained funding: Overall responsibility: TL,WZ REFERENCES 1. Wang ZG, Zhang FJ, Yi MQ, Qiang LX. Evolution of management for Budd-Chiari syndrome: a team s view from 2564 patients. ANZ J Surg 2005;75: Langlet P, Valla D. Is surgical portosystemic shunt the treatment of choice in Budd-Chiari syndrome? Acta Gastroenterol Belg 2002;65: Hoekstra J, Janssen HLA. Vascular liver disorders (I): diagnosis, treatment and prognosis of Budd-Chiari syndrome. Netherl J Med 2008;66: Beckett D, Olliff S. Interventional radiology in the management of Budd Chiari syndrome. Cardiovasc Intervent Radiol 2008;31: Horton JD, San Miguel FL, Ortiz JA. Budd-Chiari syndrome: illustrated review of current management. Liver Int 2008;28: Navarro F, Le Moine MC, Favre JM, Belghiti J, Cherqui D, Adam R, et al. Specific vascular complications of orthotopic liver transplantation with preservation of the retrohepatic vena cava: review of 1316 cases. Transplantation 1999;68: Parrilla P, Sánchez-Bueno F, Figueras J, Jaurrieta E, Mir J, Margarit C, et al. Analysis of the complications of the piggy-back technique in 1,112 liver transplants. Transplantation 1999;67: Klein AS. Management of Budd-Chiari syndrome. Liver Transpl 2006; 12(suppl 2):S Denninger MH, Chait Y, Casadevall N, Hillaire S, Guillin MC, Bezeaud A, et al. Cause of portal or hepatic venous thrombosis in adults: the role of multiple concurrent factors. Hepatology 2000;31: Valla DC. The diagnosis and management of the Budd-Chiari syndrome: consensus and controversies. Hepatology 2003;38: Eapen CE, Velissaris D, Heydtmann M, Gunson B, Olliff S, Elias E. Favourable medium term outcome following hepatic vein recanalisation and/or transjugular intrahepatic portosystemic shunt for Budd Chiari syndrome. Gut 2006;55: McCarthy PM, van Heerden JA, Adson MA, Schafer LW, Wiesner RH. The Budd-Chiari syndrome. Medical and surgical management of 30 patients. Arch Surg 1985;120: Plessier A, Sibert A, Consigny Y, Hakime A, Zappa M, Denninger MH, et al. Aiming at minimal invasiveness as a therapeutic strategy for Budd-Chiari syndrome. Hepatology 2006;44: Bachet JB, Condat B, Hagege H, Plessier A, Consigny Y, Belghiti J, et al. Long-term portosystemic shunt patency as a determinant of outcome in Budd-Chiari syndrome. J Hepatol 2007;46: Perello A, Garcia-Pagan JC, Gilatert R, Suárez Y, Moitinho E, Cervantes F, et al. TIPS is a useful long-term derivative therapy for patients with Budd-Chiari syndrome uncontrolled by medical therapy. Hepatology 2002;35: Murad SD, Plessier A, Hernandez-Guerra M, Primignani M, Elias E, Bahr M, et al. A Prospective follow-up study on 163 patients with Budd-Chiari syndrome: Results from the European network for vascular disorders of the liver (EN-VIE). J Hepatol 2007;46:S Fisher NC, McCafferty I, Dolapci M, Wali M, Buckels JA, Olliff SP, et al. Managing Budd-Chiari syndrome: a retrospective review of percutaneous hepatic vein angioplasty and surgical shunting. Gut 44: Olliff SP. Transjugular intrahepatic portosystemic shunt in the management of Budd-Chiari syndrome. Eu J Gastroenterol Hepatol 2006;18: Clavien PA, Selzner M, Tuttle-Newhall JE, Harland RC, Suhocki P. Liver transplantation complicated by misplaced TIPS in the portal vein. Ann Surg 1998;227: Zhang CQ, Fu LN, Xu L, Zhang GQ, Jia T, Liu JY, et al. Long-term effect of stent placement in 115 patients with Budd-Chiari syndrome. World J Gastroenterol 2003;9: Submitted Apr 5, 2009; accepted Jun 9, 2009.