Budd–Chiari syndrome complicating the surgical closure of patent foramen ovale in right minithoracotomy
Division of Radiology, 'Ospedali Riuniti Papardo-Piemonte' Hospital
Division of Cardiac Surgery, 'Ospedali Riuniti Papardo-Piemonte' Hospital
Author: Jamil Hajj-Chahine Department of cardio-thoracic Surgery, University Hospital of 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
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 . Furthermore,
the rate of restenosis ranges from 3 to 48%, with mild
improvement in case of stent implantation .
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  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
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
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.  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 . 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.  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 .
Late development of a Budd-Chiari syndrome after surgical closure of an atrial
septal defect has been reported . 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 .
Conflict of interest: none declared