Budd-Chiari syndrome (BCS) is a series of syndromes resulting from portal hypertension and/or inferior vena cava hypertension caused by obstruction of blood flow in the hepatic veins and/or hepatic segment of the inferior vena cava. From January 1998 to July 2008, we performed more than 500 cases of endoluminal interventions for BCS, including 45 cases of segmental occlusive Bougainvillea syndrome with occlusion of the hepatic segment of the inferior vena cava for more than 3 cm, and achieved good results.
Clinical data
I. General information
The main clinical manifestations were abdominal distension in 36 cases, hepatosplenomegaly in 45 cases, varices in the thoracoabdominal wall in 42 cases, ascites in 25 cases, varices and hyperpigmentation in both lower limbs in 39 cases, chronic ulcers in 17 cases, history of vomiting blood and black stool in 12 cases, esophagogram showed that all 45 cases had All 45 cases had moderate to severe esophageal varices. All cases were confirmed by color Doppler ultrasound and inferior vena cava angiography, and in some cases magnetic resonance angiography was also performed. There were 44 cases of occlusion of the hepatic segment of the inferior vena cava, the length of the occluded segment was 3-8 cm, and 1 case of occlusion of the long segment of the inferior vena cava, the length of the occlusion was 18 cm. 4 cases had patency of all 3 hepatic veins, 32 cases had patency of at least 1 hepatic vein, and 9 cases had patency of all 3 main hepatic veins but the secondary hepatic veins.
Treatment methods
1.Inferior vena cava angiography
Seldinger technique is used to puncture the femoral vein to perform inferior vena cava angiography with 20ml/s of contrast, 40ml in total and 600-900PSI pressure. usually the occluded segment of inferior vena cava is located above or below T8 level, if it is located below T9 or even T10 level, it may be segmental occlusion. In this case, the right internal jugular vein can be punctured and a contrast catheter can be placed into the proximal end of the inferior vena cava for simultaneous imaging to clarify the extent and length of the occlusion.
2.Intracavitary treatment
The penetration of the occluded segment of the inferior vena cava is the key to endovenous treatment, which is usually done through the internal jugular vein, through the femoral vein or a combination of both. The transfemoral vein route is commonly used. Generally, the Amplatz rigid guidewire is fed under the guidance of a straight catheter and the inferior vena cava is penetrated to the right atrium with a rigid guidewire; when the lesion is tough, the Lunderquist rigid guidewire is used, which is more rigid. If there is still difficulty in penetration, Rups
After confirming the correct direction of puncture and following up with the cuff tube, the soft tip of the ultra-hard long exchange guidewire is introduced through the cuff tube to make the head end enter the superior vena cava or the internal jugular vein, and then the soft tip of the ultra-hard long exchange guidewire is introduced through the cuff tube with the appropriate diameter. A balloon dilation catheter is then used to dilate the diseased segment of the inferior vena cava. After dilatation, the inferior vena cava is confirmed to be patent by imaging and then delivered to an endovascular stent delivery device for implantation of a metal endoprosthesis. For more complicated BCS patients, we also used three-dimensional digital subtraction angiography (3D-DSA) technique to evaluate the inferior vena cava lesion from multiple angles and to find the best working angle for endoluminal treatment.
III. Statistical processing
SAS V8.0 software package was used for statistical analysis, and data were expressed as χ±s, and paired t-test was applied for measurement data.
RESULTS
I. Surgical results
Among the 45 cases in this group, two cases failed to penetrate the occluded segment of the inferior vena cava and were converted to atrial revascularization. one case had acute pericardial tamponade after rupture of the intrapericardial segment of the inferior vena cava following balloon dilation of the penetrated inferior vena cava lesion, and the rest cases had successful penetration and dilation of the lesioned segment of the inferior vena cava. one case of occlusion of the long segment of the inferior vena cava underwent only percutaneous transluminal angioplasty (percutaneous angioplasty). In one patient, the inferior vena cava lesion was dense and dense, so PTA was performed first to dilate the inferior vena cava to 12 mm, and then the inferior vena cava was dilated to 20 mm one month later and then a stent was implanted. In two patients, the distal part of the occluded inferior vena cava with fresh thrombus formation was first aspirated from the inferior vena cava, then the inferior vena cava was punctured and dilated to a diameter of 8-10 mm to restore the patency of the inferior vena cava. The inferior vena cava pressure decreased from (35.33±3.9)cmH2O before surgery to (9.49±2.0)cmH2O after surgery (t=43.68, P<0.01), and the difference was statistically significant. The original symptoms were significantly relieved 1 week after surgery, ascites disappeared, abdominal distension was reduced, the varicose veins of the thoracoabdominal wall collapsed, lower limb edema and varices were reduced, lower limb ulcers crusted, and esophageal varices were significantly reduced as seen on esophagogram.
The balloons used in this group were American Cordis, BARD and COOK products with diameters of 6~25 mm, and the stents were triple Z stents manufactured by Shenyang Yongtong Medical Equipment Co.
II. Follow-up results
Thirty-five cases in this group were followed up, with a follow-up rate of 77.8% (35/45), and the follow-up period ranged from 3 to 46 months, with an average of 28.6 months. The main means of follow-up was color Doppler ultrasound and/or inferior vena cava angiography. Except for one case in which intra-stent thrombosis was found in the angiography 15 months after surgery and was converted to atrial artificial vessel diversion, there was no stent displacement or hepatic vein obstruction in other patients, and all lower limb ulcers healed. The acute pericardial tamponade cases were discharged 1 month after surgical repair of the inferior vena cava, with no discomfort other than intercostal neuralgia and disappearance of the original symptoms at 6 months of follow-up. There was no pulmonary embolism or death in this group.
DISCUSSION
I. Key points of intracavitary treatment for segmental occlusive BCS
Segmental occlusive BCS used to be treated by atrial artificial vessel diversion. However, this procedure is very traumatic and difficult for many patients to accept, and it is prone to intravascular thrombosis. With the development of interventional radiology, the efficacy of endoluminal intervention for septal BCS has been confirmed, but endoluminal treatment for segmental occlusive BCS, especially for patients with long segmental inferior vena cava occlusion, has certain difficulties and potential risks. In order to improve the success rate of endoluminal treatment and avoid complications, inferior vena cava capnography is feasible to understand the length of the inferior vena cava lesion and to observe the misalignment of the proximal and distal vessels of the occlusion. If the femoral vein approach is difficult to puncture the inferior vena cava lesion, it can be replaced by the internal jugular vein approach, which often improves the success rate of puncture. After 2007, the author also used 3D-DSA technique to accurately assess the morphology of the inferior vena cava lesion in more complex BCS patients. 3D-DSA rotational acquisition images can show the anatomy of the inferior vena cava from different angles, allowing the overlapping vessels to expand, facilitating the detection of collateral vessels originating from the occluded end and It can clearly show the opening position and spatial relationship, and facilitate the search for the best working angle for puncturing the inferior vena cava lesion. For lesions with mechanization, fibrosis, and calcification, it is often difficult to fully dilate. In this case, a small balloon should be used first to gradually increase the pressure and repeatedly expand it several times to make it fully open. In one of our patients, the lesioned segment of the inferior vena cava was dense, and during the dilation process, the inferior vena cava was first dilated to 12 mm in diameter to restore the patency of the inferior vena cava, and then further balloon dilation and stenting were performed one month later with good results, avoiding possible complications such as rupture of the inferior vena cava. For patients with fresh thrombosis in the distal part of the inferior vena cava lesion, catheter aspiration and thrombolysis can be followed by puncture dilation of the lesion and stent implantation. In our group, two patients with fresh thrombosis were treated with catheter aspiration, and then the lesioned inferior vena cava was punctured and dilated to a diameter of 8-10 mm and then thrombolized with a thrombolytic catheter to obtain better results.
Opening of hepatic vein
Regardless of the left, right, middle hepatic vein or collateral hepatic vein, as long as one of them is well developed and patent, only the inferior vena cava needs to be opened. If the three main hepatic veins are not open, only one of the well-developed hepatic veins should be opened to get better clinical results. This is because in hepatic vein occlusion, there is a wide range of traffic between the collateral hepatic veins and the left, middle and right hepatic veins. In this group of cases, at least one hepatic vein or parahepatic vein is open, so there is no separate hepatic vein opening.
How to prevent and manage the complications of pericardial tamponade
Pericardial tamponade is a serious complication of intracavitary treatment. Pericardial tamponade usually does not occur after the catheter penetrates the pericardium or atrium as long as it is not dilated. Therefore, it is important to make sure that the catheter is located in the center of the lesion when puncturing, and to prevent it from entering the collateral vessels or deviating to the lateral wall, and the position of the catheter should be observed in the frontal and lateral positions respectively. During and after balloon dilation, we should pay attention to the physiological response of the patient and changes in vital signs, and then we should take an image to understand whether there is contrast spillage and pericardial effusion after balloon dilation. Do not implant the stent easily until it is clear. If there is pericardial tamponade, resuscitation should be organized quickly. In one of our patients, hemorrhage of the intrapericardial segment of the inferior vena cava occurred during balloon dilation, resulting in pericardial tamponade, which may be related to the toughness of the patient’s lesion and the large diameter of the balloon (25 mm) we used.
Once the pericardial tamponade was identified, the patient should be transported to the operating room immediately. At the same time, pericardiocentesis is performed to aspirate the blood accumulated in the pericardium, and the extracted blood is returned to the body through the internal jugular vein for temporary “extracorporeal circulation” to temporarily relieve the pressure in the pericardial cavity and prevent hemorrhagic shock from occurring due to excessive blood loss, so as to buy time for resuscitation.
In conclusion, the intracavitary treatment of segmental occlusive Buerger syndrome is difficult and potentially dangerous, but with gentle and meticulous intraoperative procedures, the application of 2D-DSA and 3D-DSA techniques, preoperative and intraoperative knowledge of the structure of the inferior vena cava lesion, and reasonable use of interventional equipment, better near- and mid-term results can be achieved.