Budd–Chiari syndrome complicating the surgical closure of patent foramen ovale in right minithoracotomy

Interactive CardioVascular and Thoracic Surgery, Feb 2013

We present the case of a 45-year old female operated on for minimally invasive closure of patent foramen ovale, who suffered in the postoperative course of the Budd–Chiari syndrome caused by the thrombotic occlusion of the inferior vena cava. Medical treatment with oral anticoagulants and heparin was promptly established, avoiding a further increase of the thrombus that completely disappeared 3 months later.

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Budd–Chiari syndrome complicating the surgical closure of patent foramen ovale in right minithoracotomy

Fabrizio Ceresa 1 2 Fabrizio Sansone 1 2 Carmelo Anfuso 0 2 Francesco Patan 1 2 0 Division of Radiology, 'Ospedali Riuniti Papardo-Piemonte' Hospital , Messina, Italy 1 Division of Cardiac Surgery, 'Ospedali Riuniti Papardo-Piemonte' Hospital , Messina, Italy 2 Author: Jamil Hajj-Chahine Department of cardio-thoracic Surgery, University Hospital of Poitiers , Poitiers, France doi: 10.1093/icvts/ivs533 The Author 2012. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved We present the case of a 45-year old female operated on for minimally invasive closure of patent foramen ovale, who suffered in the postoperative course of the Budd-Chiari syndrome caused by the thrombotic occlusion of the inferior vena cava. Medical treatment with oral anticoagulants and heparin was promptly established, avoiding a further increase of the thrombus that completely disappeared 3 months later. Minimally invasive cardiac surgery Patent foramen ovale (PFO) - The minimally invasive approach for the surgical closure of patent foramen ovale (PFO) is performed through a right minithoracotomy. Cardiopulmonary bypass (CPB) is usually established between the right femoral vein (in addition to the right jugular vein) and the right femoral artery, and the incidence of surgical complications is negligible, except for the risk of groin injuries such as infection or lymphocele. We present the case of a young female operated on for minimally invasive PFO closure, complicated by a complete inferior vena cava (IVC) thrombosis, resulting in a BuddChiari syndrome. This serious complication was successfully managed by 3 months of medical treatment with anticoagulation, leading to a complete recovery of the IVC patency. A 45-year old female (58 kg 157 cm) affected by migraine and cryptogenic stroke, underwent minimally invasive PFO closure. No history of hypercoagulability was reported and the chemistry revealed just a trivial increase of the hepatic enzymes. As the standard protocol at our institution, the heparin infusion of 300 UI/kg was administered 3 min before the cannulation and the activation clotting time (ACT) was checked about 68 min later. ACT values >480 s were mandatory for the extracorporeal circulation. CPB was established between the right femoral vein/right jugular vein (connected by a Y-shaped derivation) and the right femoral artery, cannulated by a 20-Fr arterial cannula. After infusing 18 000 UI of heparin, achieving an ACT value of 515 s about 9 min later, a 22-Fr femoral venous cannula (Edwards Lifesciences, Irvine, CA, USA) was inserted into the right femoral vein up to the atrio-caval connection, under echocardiographic control. Moreover, a 17-Fr femoral artery cannula (Medtronic DLP, Minneapolis, MN, USA) was percutaneously inserted into the right internal jugular vein up to the atrio-caval connection. No complications during venous cannulation were reported. The 24-Fr and 17-Fr venous cannulae were routinely used at our institution in about 70 patients operated on for minimally invasive PFO closure and no cases of this complication have been reported so far. A right antero-lateral thoracotomy (6 cm) was performed at the fourth intercostal space (ICS), and the pericardium was opened in the forepart. Both of the caval veins were snared by vessels loops to obtain total CPB, avoiding air suction after the right atrium incision. The PFO was sutured by a 4/0 polypropylene running suture. Before suturing the right atrium, the vessel loop around the IVC was removed to check the patency of the orifice of the IVC into the right atrium. The aortic cross-clamp time was 20 min and the extracorporeal circulation was 38 min. ACT value 30 min after heparin administration was 553 s. Protamine sulphate (200 mg) was administered after removing the venous cannulae, and no further doses were necessary in the intensive care unit (ICU). Moreover, bleeding was trivial (about 200 ml in 24 h) and no pro-coagulant factors were administered postoperatively. The ICU stay was uneventful and the patient was discharged to the ward in postoperative day 1. Three days later, the onset of asthenia, dyspnoea, peripheral oedema, ascites and hepatomegaly associated with increased liver enzymes (AST 3460 IU/l, ALT 4850 IU/l, total bilirubin 1.8 mg/dl and direct bilirubin 0.6 mg/dl), required an echo evaluation, showing a partial thrombosis of the IVC close to the orifices of the hepatic veins. The Author 2012. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved. Figure 2: The images are obtained by steady-state free precession sequences, synchronized with beat heart. The first image (A) shows the thrombus causing the obstruction of the inferior vena cava (IVC) just below the conjunction with renal veins. After 2 weeks of anticoagulation therapy (B) the thrombus appears more inhomogeneous with initial signs of recanalization. Magnetic resonance angiography detected a stratified thrombus, causing a nearly complete occlusion of the IVC from the right common iliac vein to the hepatic segment (Figs. 1A and 2A), 2 cm away from the cavo-atrial connection, that was normal (Fig. 1C) as shown by the CT scanning. Moreover, the IVC near the renal segment was patent (Fig. 1C). Thus, the BuddChiari syndrome was confirmed. Medical treatment by intravenous heparin and warfarin (International Normalized Ratio 33.5) was promptly established, avoiding a further increase of the thrombus size, leading to a complete recovery of the hepatic function 1 week later. A complete assessment of the coagulation pattern was carried out and no abnormalities were detected. Three weeks later, magnetic resonance showed a complete recovery of the patency of the IVC near the orifices of the hepatic veins (Fig. 1B) and a partial thrombosis of the right common iliac vein up to the renal veins (Fig. 2B). The three-month follow-up was uneventful and the patient had no further symptoms related to the caval thrombosis. Thrombosis of the IVC is a very rare cause of the BuddChiari syndrome. Usually, the thrombosis depends on the hypercoagulable state or antiphospholipid syndrome, or more rarely depends on the veins stenosis after heart transplantation with the bicaval anastomosis technique [1, 2]. In this case of thrombosis of the IVC after closure of the PFO via a right thoracotomy on full CPB with peripheral cannulations, the cause may be most likely attributable to inadequate systemic heparinization or cannula malfunction. However, the ACT values were checked about 89 min after heparin administration and after 30 min of CPB: these values, ranging from 515 and 553 s, were adequate for a safe CPB. Moreover, we had no signs of cannula malfunctioning such as worsening of the venous drainage. Thus, the hypotheses of inadequate anticoagulation and cannula malfunctioning are not sufficiently supported. The gold standard of treatment for IVC thrombosis is the percutaneous balloon dilatation followed by stent placement, whenever thrombosis has a limited extension [1]. Furthermore, the rate of restenosis ranges from 3 to 48%, with mild improvement in case of stent implantation [34]. In case of widespread caval thrombosis, the surgical treatment should be preferred to obtain a complete resection of the thrombus, avoiding the risk of thrombo-embolic events [1] and recurrences. Obviously, the risks related to the CPB and deep hypothermic arrest are noteworthy. In this case, we have successfully treated the patient by a medical treatment. However, systemic and oral anticoagulation are by no means thrombolytic treatments and the resolution of the thrombus in this case was most probably spontaneous, even though the anticoagulation avoided a further increase of the thrombus size leading to a slow relief of the patients symptoms. In fact, the resolution of the thrombosis was probably related to the spontaneous fibrinolytic activity of the patient. In our opinion, standard treatments had a considerable risk of failure in this case because: (i) the thrombosis involved different segments of the IVC and the percutaneous approach was not enough; (ii) the surgical approach required a huge incision of the IVC (under deep hypothermic arrest) in order to remove the thrombus, and the risk of injuries of the vein and untreatable bleeding was considerable. Moreover, the origin of the thrombus remains unknown because the hypercoagulable state and the antiphospholipid syndrome were ruled out. To our knowledge, this is the first report of a near-complete thrombosis of the IVC complicating a minimally invasive PFO closure. In conclusion, we think that medical treatment in selected cases of BuddChiari syndrome due to IVC thrombosis may avoid a further increase of the size of the thrombus; moreover, the spontaneous fibrinolytic activity may lead to a complete recovery of the patency of the vein. Conflict of interest: none declared. In this interesting paper, Ceresa et al. [1] reported the case of a 45-year-old female patient successfully managed by heparin and oral anticoagulants for a Budd-Chiari syndrome, diagnosed three days after cardiac surgery. Only a handful of similar, but not identical cases have been described previously. However, we believe that there are some issues that need to be addressed. Despite advances in surgical care interventions, surgical bypass achieved by portocaval or mesentericocaval shunting is still associated with high rates of mortality and morbidity [2]. Less invasive techniques such as trans-jugular intrahepatic portosystemic shunting have yielded excellent results with a lower rate of procedurerelated mortality in patients with Budd-Chiari syndrome. Liver transplantation is an effective treatment for Budd-Chiari syndrome, however it is generally reserved for patients with fulminant hepatic failure or advanced cirrhosis after failure of shunts. Recanalizing of hepatic veins by means of balloon angioplasty and stent implantation is another minimally-invasive technique, but it carries a significant risk of massive pulmonary embolism. Han et al. [3] used a specially designed retrieval stent filter in eight consecutive patients with Budd-Chiari syndrome. This stent filter was shown to be safe and effective in all patients without incidental pulmonary embolism. Thrombolysis with streptokinase delivered systemically or locally is also another alternative but it is not a viable option after cardiac surgery [4]. Late development of a Budd-Chiari syndrome after surgical closure of an atrial septal defect has been reported [5]. In this case report, the patient suffered from symptoms related to the obstruction of the junction between the right atrium and the inferior vena cava 20 years after the primary surgery. This was due to the shrinkage of the surrounding tissue secondary to a calcified Teflon patch. This dreaded complication is known to occur after the Mustard procedure and after surgical correction of pulmonary vein stenosis [5]. Conflict of interest: none declared


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Fabrizio Ceresa, Fabrizio Sansone, Carmelo Anfuso, Francesco Patanè. Budd–Chiari syndrome complicating the surgical closure of patent foramen ovale in right minithoracotomy, Interactive CardioVascular and Thoracic Surgery, 2013, 214-216, DOI: 10.1093/icvts/ivs464