Author + information
- Received March 4, 1996
- Revision received May 30, 1996
- Accepted August 13, 1996
- Published online December 1, 1996.
- ↵⁎Address for correspondence: Dr. Tsung O. Cheng, The George Washington University Medical Center, 2150 Pennsylvania Avenue, NW, Washington, D.C. 20037.
Objectives This study sought to report the long-term result (up to 8 years) of percutaneous transluminal balloon angioplasty (PTBA) for Budd-Chiari syndrome (BCS) caused by membranous obstruction of the inferior vena cava (MOVC).
Background We previously reported on this nonoperative form of therapy in a smaller series of patients and found the short-term results to be excellent.
Methods We studied the long-term results of PTBA in the treatment of BCS caused by MOVC in 42 patients who underwent PTBA with the Inoue balloon catheter between June 1988 and February 1996. There were 28 men and 14 women with a mean age of 35.6 years (range 16 to 56). MOVC was incomplete in 27 patients and complete in 15. PTBA was successful in 38 patients (91%). The longest follow-up period was 8 years.
Results All 38 patients who successfully underwent PTBA showed marked symptomatic improvement. Immediately after PTBA, the diameter of the inferior vena cava at the MOVC increased from 1.7 ± 2 to 19.9 ± 3.5 mm (p<0.0001), the caval pressure below the MOVC decreased from 23.6 ± 8.5 to 12.0 ± 6.5 mm Hg (p<0.0001), and the enlarged liver size decreased from 6.5 ± 1.5 to 2.0 ± 1.5 cm below the right costal margin at the midclavicular line (p<0.0001). Over a follow-up period of up to 8 years (7 to 8 years in 4 patients, 5 to 7 years in 12, 3 to 5 years in 11, 2 to 3 years in 6 and <2 years in 9), MOVC returned in only 1 patient. This patient, our first, required a second PTBA 3 years later and a third 4.25 years after the second PTBA, in combination with stent placement for recurrence of stenosis.
Conclusions PTBA with the Inoue balloon catheter is an effective, safe and long-lasting alternative to surgical treatment of patients with BCS due to MOVC.
In the past the Budd-Chiari syndrome (BCS) was always treated surgically (1,2). The introduction of percutaneous transluminal balloon angioplasty (PTBA) offers a new therapeutic option (3). We (3,4) previously reported our experience with the use of the Inoue balloon catheter in treating BCS caused by membranous obstruction of the inferior vena cava (MOVC). In this report we address the long-term result (up to 8 years) of this form of treatment in 42 patients.
Patients. From June 1988 to February 1996, 42 patients with MOVC underwent PTBA with the Inoue balloon catheter. There were 28 men and 14 women, ranging in ages from 16 to 56 years (mean, 35.6). The duration of their symptoms ranged from 3 months to 12 years. Fifteen patients had complete and 27 had incomplete MOVC.
One patient had had a previous surgical finger dilation through a right atrial approach 6 months before PTBA. Another had undergone surgical sympathectomy 12 years before PTBA and had clinical recurrence of symptoms during the 5 years preceding PTBA.
Procedure. The details of PTBA of MOVC with the Inoue balloon catheter have been reported previously (3). Only a brief description of some key points of the technique will be presented here. For incomplete MOVC, a 0.025-in. (0.064-cm) special guide wire was inserted through the percutaneously inserted inferior vena cava catheter up to the right atrium, and the caval catheter was then removed over the special guide wire, which was left in situ in the right atrium. For complete MOVC, an 8F Mullins introducer set was advanced over a 0.032-in. (0.081-cm) guide wire into the inferior vena cava up to the level of obstruction. The guide wire was then exchanged for a straight transseptal needle; the latter was pushed carefully through the center of the MOVC under biplane fluoroscopy and pressure monitoring until the tip penetrated the MOVC and was advanced to above the caval entrance into the right atrium. The Mullins introducer set was then advanced over the transseptal needle into the right atrium. Finally the straight transseptal needle was exchanged for the special 0.025-in.guide wire and the Mullins introducer set removed. For the rest of the procedure the readers are referred to our earlier report (3).
- Abbreviations and Acronymns
- = Budd-Chiari syndrome
- = membranous obstruction of the inferior vena cava
- = percutaneous transluminal balloon angioplasty
In addition to intravenous administration of heparin, 4,000 U, during the procedure, all patients received continuous heparin infusions, 6,000 U/day, for 1 week and oral aspirin, 100 mg/day, for 3 months after PTBA.
Follow-up studies. All patients initially had a detailed history and physical examination as well as ultrasound studies, catheterization with pressure measurement and angiographic studies both before and after PTBA. Follow-up studies were carried out at regular intervals by questionnaires in all patients and by repeat clinical, esophagographic, two-dimensional ultrasonographic and angiographic examinations in most of our patients whenever feasible. The duration of follow-up was 7 to 8 years in 4 patients, 5 to 7 years in 12, 3 to 5 years in 11, 2 to 3 years in 6 and <2 years in 9.
Statistical analysis. All data are expressed as mean value ± SD. Comparisons were made by Student t test. Results were considered statistically significant if p<0.05.
Procedural success rate. PTBA with the Inoue balloon catheter was successful in 38 (91%) of 42 patients. The four failures were due to massive pulmonary embolism resulting in instantaneous death immediately after PTBA in one patient with unrecognized thrombosis of the inferior vena cava and were due to complete MOVC secondary to excessive thickness (5 to 8 mm) of the obstructive membrane in three patients who were successfully treated surgically by cavoatrial bypass grafting.
Hemodynamic findings. All patients who underwent successful PTBA showed significant hemodynamic improvement. The inferior vena cava pressure below the MOVC decreased from 23.6 ± 8.5 to 12.0 ± 6.5 mm Hg (p<0.0001). In one patient whose right atrial pressure was also measured, the pressure in the right atrium above the MOVC and in the inferior vena cava below the MOVC was -1 and 20 mm Hg, respectively, before PTBA and 1 and 7 mm Hg, respectively, after PTBA. Right atrial pressure was not measured in the other patients.
Angiographic findings. The thickness of the MOVC measured 1 to 5 mm. The diameter of the inferior vena cava at the site of the MOVC increased from 1.7 ± 2 to 19.9 ± 3.5 mm (p<0.0001). The infra-MOVC diameter of the inferior vena cava decreased from 40.0 ± 12.2 to 22.4 ± 8.4 mm after PTBA (p<0.0001). The venous collateral vessels that existed abundantly before PTBA disappeared after PTBA.
Follow-up evaluation. Clinical improvement was dramatic in all of the patients. Abdominal pain, nausea, vomiting, dyspnea on exertion and leg edema either disappeared or diminished significantly within 2 to 7 days after PTBA. The enlarged liver decreased markedly from 6.5 ± 1.5 cm below the right costal margin at the midclavicular line to 2.0 ± 1.5 cm after PTBA (p<0.0001). In patients with ascites, prompt diuresis occurred within 12 h after PTBA. Ultrasonography showed complete resorption or marked diminution of ascites within 3 weeks in most cases. Within 3 to 6 months, 28 (74%) of 38 patients returned to normal physical activities, which were very strenuous in most cases.
All 14 women in the study had either dysmenorrhea or amenorrhea before PTBA; 6, although married for many years, either were infertile or had repeated miscarriages before PTBA. After PTBA, two women became pregnant within 1 year and each gave birth to a healthy full-term baby.
All but 1 of the 38 patients showed continued improvement during a follow-up period of up to 8 years, as assessed both subjectively and objectively by esophagography, ultrasonography and angiography (Fig. 1). One patient, a 21-year old woman, the first patient in our series, remained asymptomatic for 31 months, then showed recurrence of symptoms due to restenosis (Fig. 2). After repeat PTBA she remained well for another 4.25 years, when restenosis again developed and a third PTBA was performed in combination with stent placement (Gianturco-Rosch Z stent, 25 mm in diameter, 5 cm in length) (Fig. 2). She remained well thereafter.
Definition of BCS. BCS, a relatively uncommon but devastating disorder, comprises a heterogeneous group of pathologic entities characterized by hepatic venous outflow obstruction and its attendant manifestations (5). The diversity of the causes and clinical presentations of BCS is reflected both in the variety of approaches to establish the diagnosis and in the several medical and surgical therapies available.
BCS may be classified as primary or secondary depending on its pathologic features. The primary type refers to congenital obstruction of the hepatic veins or the hepatic portion of the inferior vena cava. The secondary type, most common in Western countries, refers to obstruction of the same anatomic structures by a tumor or, more commonly, thrombus or thrombi in patients with some systemic diseases, usually my-eloproliferative disorders (6). Primary BCS is well known as MOVC, which is most common in Oriental countries and South Africa (3).
MOVC. MOVC is the most common cause of BCS worldwide (7). It accounts for -33% of the total number of cases reported in the world literature (8). MOVC is usually not difficult to diagnose, especially in countries where it is relatively common, such as Japan, China, South Africa and India. Clinical diagnosis is usually established on the basis of a constellation of typical symptoms and signs of portal hypertension and inferior vena cava obstruction. A two-dimensional ultrasonogram shows dilation of the inferior vena cava below the suprahepatic caval web and dilation of the hepatic veins (3). The latter finding is an important diagnostic feature differentiating MOVC from hepatic cirrhosis, in which hepatic veins are small, narrow and tortuous. Color and pulsed Doppler ultrasonograms of portal and hepatic veins show either absence or reversal of flow in the portal vein (3). Magnetic resonance imaging is also useful and has the added advantages of obtaining coronal and sagittal images, thus facilitating detection of the inferior vena cava web and evaluating the vessels without the need for contrast material (3). Furthermore, magnetic resonance imaging can also be used to distinguish slowly flowing blood from intraluminal thrombus by examining first- and second-echo images; slow flow usually causes an increase in relative signal density on a second-echo image, whereas thrombus shows either a lesser increase or a decrease in relative signal intensity (“paradoxic enhancement”) (3).
Differentiation between membranous obstruction and thrombosis of inferior vena cava. Thrombus formation is not an uncommon complication of MOVC, occurring in 28.6% (6 of 21) of patients (3). It is essential to exclude thrombosis of the inferior vena cava before performing PTBA. The only death in our series was in a patient with unrecognized thrombosis below the web in the inferior vena cava who died from massive pulmonary embolism immediately after the procedure (3). In addition to magnetic resonance imaging, inferior vena cavography may offer some clue to the existence of a thrombus because of differing shades of contrast density at the site of the obstruction in the inferior vena cava (3). Thrombotic occlusion of the inferior vena cava is an absolute contraindication to PTBA.
PTBA versus operation. Although hepatic venous outflow obstruction may be circumvented by different forms of operative decompression or shunting procedures (2,9) and liver transplantation (1), surgical treatment of MOVC is far from ideal. First, placing a synthetic graft in the venous system to bypass the inferior vena cava obstruction is a difficult procedure (10). Second, the graft is also prone to occlusion despite long-term anticoagulant therapy, possibly because the liver may fall back on the graft and obstruct the flow (10). Third, synthetic grafts in patients with hepatic venous outflow obstruction (who may have an underlying clotting tendency) are predisposed to a high rate of occlusion (5). Last, but not the least, an 8-year patient survival rate of 100% in our series compares very favorably with a cumulative 5-year survival rate of 59% in the Johns Hopkins series of BCS as reported by Klein et al (2).
Advantages of the Inoue balloon catheter. Because of our extensive experience with the Inoue balloon catheter in percutaneous balloon mitral valvuloplasty (11), we chose this catheter for management of MOVC. A single Inoue balloon is large enough for the inferior vena cava. The diameter of the inferior vena cava is usually 20 to 22 mm in adults. Using single Inoue balloon catheter with a balloon inflation diameter of 21.6 ± 1.9 mm (range 18 to 24) and a total number of 2.6 ± 0.7 inflations (range 1 to 4), we obtained very satisfactory immediate and long-term results. A balloon/inferior vena cava diameter ratio of 1:1 to 1.1:1 seems to be optimal for MOVC dilation. The Inoue balloon catheter is ideally suited for dilating the MOVC, because the waist of the pillow-shaped balloon tends to “lock” in the obstructing membrane once the inflated distal portion of the balloon is pulled against the MOVC and as the proximal portion of the balloon is beingprogressively inflated. Other advantages of the Inoue balloon catheter include 1) lower inflation pressure (1 to 2 atm); 2) shorter duration of inflation (∼3 s); 3) stronger rubber-nylon micromeshed balloon than polyethylene balloon; and 4) need for only one single-balloon catheter with adjustable balloon sizes depending on the inflation volume of the saline-diluted Renografin-76 mixture used.
PTBA versus stenting. Restenosis after PTBA for MOVC can be treated either with redilation or with stents. The limited number worldwide of published cases of stent use in MOVC (12–18) should make one proceed with caution. Furthermore, the placement of stents not only adds cost to the procedure but may interfere with orthotopic liver transplantation, which may ultimately be indicated in patients with severe fibrosis or end-stage liver dysfunction (1).
The development of restenosis in only one patient in our series after PTBA without stent placement is certainly most encouraging. We therefore believe that most cases of MOVC can be managed by PTBA without stenting. We propose that the use of a stent be considered only under the following circumstances: 1) restenosis after repeat PTBA; 2) long segmental obstruction of the inferior vena cava; or 3) enlarged caudate lobe of the liver, hepatoma or other abdominal tumor that may impinge on the inferior vena cava.
Only one patient in our series had restenosis. She was the first patient in our series, and in her procedure we used a balloon whose diameter was only 18 mm, which was clearly suboptimal. In subsequent patients we used larger balloon diameters (20 to 25 mm; average 23 mm). Our first patient underwent repeat PTBA when restenosis developed 3 years after the initial procedure, and she remained asymptomatic for another 4.25 years, when restenosis again developed. She underwent a third PTBA combined with placement of a stent, and she remained well afterward.
Conclusions. BCS is a rare condition, and treatment guidelines have generally been based on anecdotal reports of small number of patients. However, the recent report (10) that BCS was present in nearly 10% of patients treated surgically for portal hypertension indicated that it may be less rare than previously thought. Despite the wide geographic variability in the etiology and manifestations of BCS, it has been said (19) that, Kipling to the contrary, East and West do meet in the modern management of BCS. Major advances in diagnosis and treatment of BCS over the past decade plus the encouraging long-term results of PTBA in our patients with MOVC should prompt continuation of the recent resurgence of interest in this condition (7,20–23).
- Received March 4, 1996.
- Revision received May 30, 1996.
- Accepted August 13, 1996.
- American College of Cardiology
- Cheng TO,
- Yang X,
- Chen C.
- Wang ZG,
- Wang SH,
- Wu JD
- Kaul U,
- Agarwal R,
- Jain P,
- Wasir HS.
- Mahmoud AEA,
- Mendoza A,
- Meshikhes AN