Catheterization and Cardiovascular Diagnosis 27:21!i-219 (1992)
Budd-Chiari Syndrome Treated Successfully by Percutaneous Transluminal Balloon Angioplasty: Two Cases Followed-Up for 6 Years P. Chan, MD, C.P. Lee, MD, and Y.S. Lee, MD, FCCP, FICA We report 2 cases of Budd-Chiari syndrome that are due to complete membranous obstruction between the junction of the inferior vena cava and the right atrium. Both cases were treated successfully by King’s bioptome breakthrough followed by balloon dilatation of the membrane. The patients showed remarkable symptomatic improvement and promising hemodynamic and angiographic results immediatelyafter the procedure and 6 years later. We conclude that balloon angioplasty is a safe and effective method for treating this rare disease in selected cases. Q 1992 Wiley-uss, I ~ C . Key words: balloon angioplasty, King’s bioptome breakthrough,complete membranous obstruction
INTRODUCTION
Budd-Chiari syndrome is an uncommon disorder that is due to obstruction of the hepatic venous outflow. The lesion site may be in the hepatic vein(s), or in the hepatic or suprahepatic portion of the inferior vena cava. The inciting causes are various, including hematologic disorders (e.g, leukemia, polycythemia rubra verna, and sickle cell anemia), hepatocellular carcinoma, metastatic malignancy, liver abscess, subphrenic abscess, oral contraceptives, and pregnancy [ I]. Another rare cause is the presence of a membrane between the junction of the inferior vena cava (IVC) and the right atrium (RA), which is a type Ia obstruction according to Hirooka and Kimura’s classification [2]. Membranous obstruction of the IVC is suggested to be congenital and is the most clinically promising type of Budd-Chiari syndrome since it is potentially curable by surgery or balloon angioplasty [2,3]. This condition is common in Orientals and rare in Westerners [ 11. Before the introduction of balloon angioplasty [4,7], the only effective treatment for BuddChiari syndrome was surgery. Here we report 2 cases of complete membranous obstruction of the IVC (type Ia) that were treated by King’s bioptome breakthrough followed by balloon angioplasty. Both cases were followed up for 6 years and showed satisfactory results. CASE REPORTS Case 1
A 38-year-old man was admitted in April 1986 because of tarry stool passage and dizziness. The patient had had upper gastrointestinal bleeding twice in the pre0 1992 Wiley-Liss, Inc.
vious 5 years. Physical examination revealed hepatosplenomegaly and dilated tortuous veins on the abdominal wall. Laboratory data showed that his Hb concentration was 10 mg/dl and his serum albumin level was 3.2 g/dl. His other laboratory data were normal. Panendoscopy revealed grade IV esophageal varices and bleeding. Radionuclide imaging [8] showed hepatosplenomegaly and obstruction of the hepatic portion of the IVC. Cardiac catheterization and angiography were performed via the right femoral and right brachial veins. Simultaneous contrast injection in the RA and IVC showed that the membrane (about 2 mm thick) located at the supra hepatic position of the IVC completely obstructed the IVC (Fig. lA,B). The pressure gradient was 20 mmHg (IVC 24 mmHg; RA 4 mmHg). The site of the membrane was located with the aid of radiopaque markers. These radiopaque markers were used to guide the site of breakthrough in the membrane. The catheter in the right femoral vein was withdrawn, and a 145 cm 0.035 inch soft-tipped guide wire was left behind. Subsequently a 110 cm 7F sheath was inserted and accessed to the membrane. We employed King’s bioptome via the 110 cm sheath to bite a small hole in just one successful attempt; then we pushed the bioptome directly
From the Taipei Municipal Chung Hsiao Hospital and Chang Gung Medical Center, Taipei, Taiwan, Republic of China. Received January 15, 1992; revision accepted June 4, 1992. Address reprint requests to Dr. Paul Chan, Department of Cardiology, Taipei Municipal Chung Hsiao Hospital. Taipei, Taiwan, ROC.
216
Chan et al.
Fig. 2. Balloon waist (arrows) at the obstruction site after the first dilatation, as shown by this anterioposterior view radiograph.
Fig. 1. A,B: Anteroposterior and lateral views of simultaneous angiiogram of IVC and RA show a thin membrane (arrows) obstructing IVC completely.
through the membrane from the IVC into the RA and the sheath was also inserted into the RA. After the 110 cm sheath was pushed from the IVC into the RA, the bioptome was withdrawn. Again a 145 cm 0.035 inch softtipped guide wire was inserted into the RA. The sheath was then withdrawn and exchanged for a 8F 20 mm Mansfield balloon catheter. The inflation pressure was about 4 atm, and one inflation was performed until the disappearance of the balloon waist (Fig. 2). A postangioplasty angiogram (Fig. 3A,B) showed a patent IVC. The process was smooth. The postoperative pressure gradient decreased to 4 mm Hg (IVC 14 mm Hg; RA 10 mm Hg). The patient’s symptoms were considerably alleviated, and the abdominal collaterals disappeared. Two years later the patient was admitted again for an evaluation. Hepatosplenomegaly and esophageal varices had disappeared. IVC angiography showed a patent IVC and its pressure was 9 mm Hg, RA 5 mm Hg. The patient was followed up regularly at the out-patient department until October 1991 when he was admitted to evaluate his lesion site with radionuclide imaging, the result of which was satisfactory.
Budd-Chiari Syndrome, Balloon Angioplasty
217
Case 2 A 53-year-old woman was admitted in October, 1985 because of intermittent abdominal pain for 5 years. Physical examination revealed hepatosplenomegaly , and venous engorgement was found on the abdominal wall; however, there was no evidence of edema or ascites. Laboratory data were normal. Both abdominal ultrasonography and radionuclide imaging showed obstruction of the hepatic portion of IVC. Cardiac catheterization and angiography revealed a thin membranous structure (Fig. 4) located at the junction between the IVC and the RA. The pressure gradient was 23 mm Hg (IVC 28 mm Hg; RA 5 mm Hg). Because a Mansfield balloon catheter was not available in Taiwan at that time, we therefore employed Miller’s atrioseptostomy catheter (Fig. 5 ) and the same procedure was performed smoothly. However the balloon of Miller’s catheter was smaller and could not stand high inflation pressure. The pressure gradient dropped unsatisfactorily to 14 mm Hg and the follow-up angiography immediately showed disappearnce of collateral circulation. The patient’s symptoms disappeared and she was discharged smoothly. She was admitted again 5 years later for a follow-up examination of the obstruction site. Cardiac catheterization and angiography showed a residual stenosis (Fig. 6) at the old lesion site but the pressure gradient was only 10 mm Hg. We used an 8F Mansfield balloon catheter to dilate the stenotic site (Fig. 7, 8). The postoperative gradient decreased to 2 mm Hg only (IVC 7 mm Hg; RA 5 mm Hg). The patient was quite well and has been followed up ever since then.
DISCUSSION
Before the introduction of successful balloon angioplasty in the treatment of Budd-Chiari syndrome by Equchi et al. in 1974 [4], the only effective treatment for this condition was surgery. However, early results were not quite satisfactory, as reported by Hirooka and Kimura. The average mortality was 30% after they had reviewed 70 cases [2]. In 1989, Chang at our center reported good results using transatrial membranotomy in 11 cases, with no mortality [9]. At our center, we are trying to compare the results of angioplasty with those of transatrial membranotomy. Since Eguchi’s report, more than 10 reports of balloon angioplasty have been presented, and the methods used include laser-assisted and Brockenbrough needle puncture of the membrane (4-7). Since the tip of the Brockenbrough needle is curved and the morphologic structure of the IVC is straight, it may puncture the wall Fig. 3. A,B: Anteroposterior and lateral views of postangio- of the IVC in inexperienced hands. Laser equipment is plas,tyangiograrn of IVC show patent IVC with contrast flowing very expensive and not easily available. In contrast, the into RA. King’s bioptome, which is used in endomyocardial bi-
Fig. 4. Anteroposterior view of IVC angiogram with simultaneous contrast injection from IVC and RA shows a thin membrane (arrows) obstructing IVC completely.
Fig. 5. Anteroposterior view of IVC angiogram shows faint contrast (arrows) flowing from IVC into RA. Balloon (6) of Miller’s atrioseptostomy catheter.
Fig. 6. Anteroposterior view of IVC angiogram taken before second dilatation shows residual stenosis (arrows).
Fig. 7. Balloon (6) waist made by residual stenosis.
Budd-Chiari Syndrome, Balloon Angioplasty
219
emphasize that the procedure should be performed cautiously, with exact localization of the obstruction site. In our experience, we consistently selected a 20 mm balloon, and one to two dilatations gave good results. Continuous monitoring of aortic pressure is also mandatory for early detection of IVC trauma in case of systolic pressure drop. Disappearance of the collateral circulation visualized by angiography , disappearance of abdominal collaterals, and remission of clinical symptoms all showed that this procedure was performed successfully. In conclusion, employing King’s bioptome in balloon angioplasty is another safe and effective alternative for treating complete membranous obstruction in BuddChiari syndrome, and the long-term survival of patients treated by this method can be quite promising.
REFERENCES
Fig. 8. Post-dilatationIVC angiogram shows relieving of residual stenosis (arrows).
opsy, is very popular and is available almost in every catheterization laboratory. Moreover, the bioptome is flexible and with its blunt tip it can be straightened in the sheath. Therefore it is not traumatic if the clip is opened until the tip has touched the membrane. However, one should still be careful not to mistakenly bite the wall of the IVC. The prognosis for this disease is usually poor. Most patients die of complications such as cirrhosis and portal hypertension within several months to years of diagnosis. All of the past reports in this area have given short-term results only. However, both the cases that we report have been followed up for 6 years and the subjects are still well. In 1989 Loya et al. [7] presented a similar case to those in our report. The major difference is the method used to pass through the membrane. We must
I . Mitchell MC, Biotnott JK, Kaufman, S, Cameron JL, Maddrey WC: Budd-Chiari syndrome: Etiology, diagnosis and management. Medicine (Baltimore) 61:199-217, 1982. 2. Hirooka M, Kimura, C: Membranous obstruction of the hepatic portion of inferior vena cava. Surgical correction and aetiological study. Arch Surg 100:656-663, 1970. 3. Lai GM, Liaw YF, Wu CS, Chang Chien CS, Chang CS: BuddChiari syndrome: A clinical study on the obstruction of hepatic portion of inferior vena cava. J Formosan Med Assoc 81:588-596, 1982. 4. Eguchi S, Takeuchi Y, Asano K: Successful balloon membranotomy for obstruction of the inferior vena cava. Surgery 76:837-840, 1974. 5. Sparano J , Chang J , Trasi S , Bonanno C: Treatment of Budd-Chiari syndrome with percutaneous transluminal angioplasty . Case report and review of literature. Am J Med 82:821-828, 1987. 6. Furui S, Yamauchi T, Obtomo K, Tsuchiya K, Makita Kohzoh. Takenake E: Hepatic inferior vena cava obstruction: Clinical results of treatment with percutaneous transluminal laser-assisted angioplasty. Radiology 166:673-677, 1988. 7. Loya YS, Sharma S, Amrapurkar DN, Desai HG: Complete membranous obstruction of inferior vena cava: Case treated by balloon dilatation. Cathet Cardiovasc Diagn 17:164-167, 1989. 8. Huang MJ, Liau YF, Tzen KY: Radionuclide venography in BuddChiari syndrome with intrahepatic vena-caval obstruction. J Nucl Med 26:145-148, 1985. 9. Chang CH, Lee MC, Shieh MJ, Chang JP, Lin PJ: Transatrial membranotomy for Budd-Chiari syndrome. Ann Thorac Surg 48: 409-412, 1989.
Budd-Chiari Syndrome Treated Successfully by Percutaneous Transluminal Balloon Angioplasty: Two Cases Followed-Up for 6 Years P. Chan, MD, C.P. Lee, MD, and Y.S. Lee, MD, FCCP, FICA We report 2 cases of Budd-Chiari syndrome that are due to complete membranous obstruction between the junction of the inferior vena cava and the right atrium. Both cases were treated successfully by King’s bioptome breakthrough followed by balloon dilatation of the membrane. The patients showed remarkable symptomatic improvement and promising hemodynamic and angiographic results immediatelyafter the procedure and 6 years later. We conclude that balloon angioplasty is a safe and effective method for treating this rare disease in selected cases. Q 1992 Wiley-uss, I ~ C . Key words: balloon angioplasty, King’s bioptome breakthrough,complete membranous obstruction
INTRODUCTION
Budd-Chiari syndrome is an uncommon disorder that is due to obstruction of the hepatic venous outflow. The lesion site may be in the hepatic vein(s), or in the hepatic or suprahepatic portion of the inferior vena cava. The inciting causes are various, including hematologic disorders (e.g, leukemia, polycythemia rubra verna, and sickle cell anemia), hepatocellular carcinoma, metastatic malignancy, liver abscess, subphrenic abscess, oral contraceptives, and pregnancy [ I]. Another rare cause is the presence of a membrane between the junction of the inferior vena cava (IVC) and the right atrium (RA), which is a type Ia obstruction according to Hirooka and Kimura’s classification [2]. Membranous obstruction of the IVC is suggested to be congenital and is the most clinically promising type of Budd-Chiari syndrome since it is potentially curable by surgery or balloon angioplasty [2,3]. This condition is common in Orientals and rare in Westerners [ 11. Before the introduction of balloon angioplasty [4,7], the only effective treatment for BuddChiari syndrome was surgery. Here we report 2 cases of complete membranous obstruction of the IVC (type Ia) that were treated by King’s bioptome breakthrough followed by balloon angioplasty. Both cases were followed up for 6 years and showed satisfactory results. CASE REPORTS Case 1
A 38-year-old man was admitted in April 1986 because of tarry stool passage and dizziness. The patient had had upper gastrointestinal bleeding twice in the pre0 1992 Wiley-Liss, Inc.
vious 5 years. Physical examination revealed hepatosplenomegaly and dilated tortuous veins on the abdominal wall. Laboratory data showed that his Hb concentration was 10 mg/dl and his serum albumin level was 3.2 g/dl. His other laboratory data were normal. Panendoscopy revealed grade IV esophageal varices and bleeding. Radionuclide imaging [8] showed hepatosplenomegaly and obstruction of the hepatic portion of the IVC. Cardiac catheterization and angiography were performed via the right femoral and right brachial veins. Simultaneous contrast injection in the RA and IVC showed that the membrane (about 2 mm thick) located at the supra hepatic position of the IVC completely obstructed the IVC (Fig. lA,B). The pressure gradient was 20 mmHg (IVC 24 mmHg; RA 4 mmHg). The site of the membrane was located with the aid of radiopaque markers. These radiopaque markers were used to guide the site of breakthrough in the membrane. The catheter in the right femoral vein was withdrawn, and a 145 cm 0.035 inch soft-tipped guide wire was left behind. Subsequently a 110 cm 7F sheath was inserted and accessed to the membrane. We employed King’s bioptome via the 110 cm sheath to bite a small hole in just one successful attempt; then we pushed the bioptome directly
From the Taipei Municipal Chung Hsiao Hospital and Chang Gung Medical Center, Taipei, Taiwan, Republic of China. Received January 15, 1992; revision accepted June 4, 1992. Address reprint requests to Dr. Paul Chan, Department of Cardiology, Taipei Municipal Chung Hsiao Hospital. Taipei, Taiwan, ROC.
216
Chan et al.
Fig. 2. Balloon waist (arrows) at the obstruction site after the first dilatation, as shown by this anterioposterior view radiograph.
Fig. 1. A,B: Anteroposterior and lateral views of simultaneous angiiogram of IVC and RA show a thin membrane (arrows) obstructing IVC completely.
through the membrane from the IVC into the RA and the sheath was also inserted into the RA. After the 110 cm sheath was pushed from the IVC into the RA, the bioptome was withdrawn. Again a 145 cm 0.035 inch softtipped guide wire was inserted into the RA. The sheath was then withdrawn and exchanged for a 8F 20 mm Mansfield balloon catheter. The inflation pressure was about 4 atm, and one inflation was performed until the disappearance of the balloon waist (Fig. 2). A postangioplasty angiogram (Fig. 3A,B) showed a patent IVC. The process was smooth. The postoperative pressure gradient decreased to 4 mm Hg (IVC 14 mm Hg; RA 10 mm Hg). The patient’s symptoms were considerably alleviated, and the abdominal collaterals disappeared. Two years later the patient was admitted again for an evaluation. Hepatosplenomegaly and esophageal varices had disappeared. IVC angiography showed a patent IVC and its pressure was 9 mm Hg, RA 5 mm Hg. The patient was followed up regularly at the out-patient department until October 1991 when he was admitted to evaluate his lesion site with radionuclide imaging, the result of which was satisfactory.
Budd-Chiari Syndrome, Balloon Angioplasty
217
Case 2 A 53-year-old woman was admitted in October, 1985 because of intermittent abdominal pain for 5 years. Physical examination revealed hepatosplenomegaly , and venous engorgement was found on the abdominal wall; however, there was no evidence of edema or ascites. Laboratory data were normal. Both abdominal ultrasonography and radionuclide imaging showed obstruction of the hepatic portion of IVC. Cardiac catheterization and angiography revealed a thin membranous structure (Fig. 4) located at the junction between the IVC and the RA. The pressure gradient was 23 mm Hg (IVC 28 mm Hg; RA 5 mm Hg). Because a Mansfield balloon catheter was not available in Taiwan at that time, we therefore employed Miller’s atrioseptostomy catheter (Fig. 5 ) and the same procedure was performed smoothly. However the balloon of Miller’s catheter was smaller and could not stand high inflation pressure. The pressure gradient dropped unsatisfactorily to 14 mm Hg and the follow-up angiography immediately showed disappearnce of collateral circulation. The patient’s symptoms disappeared and she was discharged smoothly. She was admitted again 5 years later for a follow-up examination of the obstruction site. Cardiac catheterization and angiography showed a residual stenosis (Fig. 6) at the old lesion site but the pressure gradient was only 10 mm Hg. We used an 8F Mansfield balloon catheter to dilate the stenotic site (Fig. 7, 8). The postoperative gradient decreased to 2 mm Hg only (IVC 7 mm Hg; RA 5 mm Hg). The patient was quite well and has been followed up ever since then.
DISCUSSION
Before the introduction of successful balloon angioplasty in the treatment of Budd-Chiari syndrome by Equchi et al. in 1974 [4], the only effective treatment for this condition was surgery. However, early results were not quite satisfactory, as reported by Hirooka and Kimura. The average mortality was 30% after they had reviewed 70 cases [2]. In 1989, Chang at our center reported good results using transatrial membranotomy in 11 cases, with no mortality [9]. At our center, we are trying to compare the results of angioplasty with those of transatrial membranotomy. Since Eguchi’s report, more than 10 reports of balloon angioplasty have been presented, and the methods used include laser-assisted and Brockenbrough needle puncture of the membrane (4-7). Since the tip of the Brockenbrough needle is curved and the morphologic structure of the IVC is straight, it may puncture the wall Fig. 3. A,B: Anteroposterior and lateral views of postangio- of the IVC in inexperienced hands. Laser equipment is plas,tyangiograrn of IVC show patent IVC with contrast flowing very expensive and not easily available. In contrast, the into RA. King’s bioptome, which is used in endomyocardial bi-
Fig. 4. Anteroposterior view of IVC angiogram with simultaneous contrast injection from IVC and RA shows a thin membrane (arrows) obstructing IVC completely.
Fig. 5. Anteroposterior view of IVC angiogram shows faint contrast (arrows) flowing from IVC into RA. Balloon (6) of Miller’s atrioseptostomy catheter.
Fig. 6. Anteroposterior view of IVC angiogram taken before second dilatation shows residual stenosis (arrows).
Fig. 7. Balloon (6) waist made by residual stenosis.
Budd-Chiari Syndrome, Balloon Angioplasty
219
emphasize that the procedure should be performed cautiously, with exact localization of the obstruction site. In our experience, we consistently selected a 20 mm balloon, and one to two dilatations gave good results. Continuous monitoring of aortic pressure is also mandatory for early detection of IVC trauma in case of systolic pressure drop. Disappearance of the collateral circulation visualized by angiography , disappearance of abdominal collaterals, and remission of clinical symptoms all showed that this procedure was performed successfully. In conclusion, employing King’s bioptome in balloon angioplasty is another safe and effective alternative for treating complete membranous obstruction in BuddChiari syndrome, and the long-term survival of patients treated by this method can be quite promising.
REFERENCES
Fig. 8. Post-dilatationIVC angiogram shows relieving of residual stenosis (arrows).
opsy, is very popular and is available almost in every catheterization laboratory. Moreover, the bioptome is flexible and with its blunt tip it can be straightened in the sheath. Therefore it is not traumatic if the clip is opened until the tip has touched the membrane. However, one should still be careful not to mistakenly bite the wall of the IVC. The prognosis for this disease is usually poor. Most patients die of complications such as cirrhosis and portal hypertension within several months to years of diagnosis. All of the past reports in this area have given short-term results only. However, both the cases that we report have been followed up for 6 years and the subjects are still well. In 1989 Loya et al. [7] presented a similar case to those in our report. The major difference is the method used to pass through the membrane. We must
I . Mitchell MC, Biotnott JK, Kaufman, S, Cameron JL, Maddrey WC: Budd-Chiari syndrome: Etiology, diagnosis and management. Medicine (Baltimore) 61:199-217, 1982. 2. Hirooka M, Kimura, C: Membranous obstruction of the hepatic portion of inferior vena cava. Surgical correction and aetiological study. Arch Surg 100:656-663, 1970. 3. Lai GM, Liaw YF, Wu CS, Chang Chien CS, Chang CS: BuddChiari syndrome: A clinical study on the obstruction of hepatic portion of inferior vena cava. J Formosan Med Assoc 81:588-596, 1982. 4. Eguchi S, Takeuchi Y, Asano K: Successful balloon membranotomy for obstruction of the inferior vena cava. Surgery 76:837-840, 1974. 5. Sparano J , Chang J , Trasi S , Bonanno C: Treatment of Budd-Chiari syndrome with percutaneous transluminal angioplasty . Case report and review of literature. Am J Med 82:821-828, 1987. 6. Furui S, Yamauchi T, Obtomo K, Tsuchiya K, Makita Kohzoh. Takenake E: Hepatic inferior vena cava obstruction: Clinical results of treatment with percutaneous transluminal laser-assisted angioplasty. Radiology 166:673-677, 1988. 7. Loya YS, Sharma S, Amrapurkar DN, Desai HG: Complete membranous obstruction of inferior vena cava: Case treated by balloon dilatation. Cathet Cardiovasc Diagn 17:164-167, 1989. 8. Huang MJ, Liau YF, Tzen KY: Radionuclide venography in BuddChiari syndrome with intrahepatic vena-caval obstruction. J Nucl Med 26:145-148, 1985. 9. Chang CH, Lee MC, Shieh MJ, Chang JP, Lin PJ: Transatrial membranotomy for Budd-Chiari syndrome. Ann Thorac Surg 48: 409-412, 1989.
