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Ann Thorac Surg 2001;72:1641-1644
© 2001 The Society of Thoracic Surgeons

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Original article: cardiovascular

Thrombosis of intracardiac or extracardiac conduits after modified Fontan operation in patients with azygous continuation of the inferior vena cava

^a Division of Cardiovascular Surgery, Mayo Clinic, Rochester, Minnesota, USA
^b German Heart Institute, Berlin, Germany

Accepted for publication July 20, 2001.

* Address reprint requests to Dr Puga, Division of Cardiovascular Surgery, Mayo Clinic, 200 First St SW, Rochester, MN 55902, USA
e-mail: fpuga{at}mayo.edu

	Abstract

Top Abstract Introduction Patients and methods Results Comment References

 
Background. The Fontan procedure in patients with azygous continuation of the inferior vena cava, requires a cavo-pulmonary anastomosis, and deviation of the hepatic venous drainage to the pulmonary arteries using an intra- or extracardiac conduit.

Methods. We report thrombosis of two pericardial conduits and one Gore-Tex (W. L. Gore & Assoc, Flagstaff, AZ) graft used for deviation of hepatic venous blood to the pulmonary arterial tree in 3 patients aged 11, 24, and 28 years. Two of the conduits (pericardial) were intraatrial. The Gore-Tex graft was placed in an extracardiac position. The two pericardial conduits obstructed completely.

Results. One patient died at reoperation. In the 2nd patient, the conduit was excised and the hepatic veins were allowed to drain into the atrium. In the 3rd patient, partial thrombosis of the Gore-Tex conduit was noted 30 months after operation. The thrombus resolved with oral anticoagulation.

Conclusions. Conduits carrying only hepatic venous blood flow may have a higher risk of thrombosis. Anticoagulation or alternative methods of directing hepatic blood flow to the pulmonary circulation must be considered in these patients.

	Introduction

Top Abstract Introduction Patients and methods Results Comment References

 
Azygous continuation of the inferior vena cava (IVC) is a rare malformation frequently associated with the heterotaxia syndromes. Though there are many reports in the literature describing the anomaly, the experience with the surgical treatment of these patients is limited. Kawashima and colleagues [1] described a superior cava to pulmonary anastomosis to divert most of the systemic venous return to the pulmonary arterial confluence. The hepatic veins were allowed to continue to drain into the atrium resulting in an obligatory residual right-to-left shunt. The resulting arterial oxygen desaturation is relatively mild and tolerated well by patients. However, it has been reported that pulmonary arteriovenous malformations develop frequently in patients after the Kawashima operation resulting from lack of exposure of the pulmonary arterial bed to hepatic venous blood [2–5]. In an effort to prevent this complication, it is thought that diversion of the hepatic venous drainage to the pulmonary confluence is necessary in patients with azygous continuation of the inferior cava undergoing definitive palliation by the Kawashima procedure. In our practice, this has been accomplished by insertion of an intraatrial or extracardiac conduit. Intraatrial conduits have been used in our practice during Fontan procedures in patients with complex anomalies of the pulmonary and systemic venous drainage [6]. The use of an extracardiac conduit as a means for separating the systemic from the hepatic venous drainage during the Fontan procedure was first proposed by King and coworkers [6] and successfully used by Marcelletti and associates for diversion of the IVC blood to the pulmonary artery [7]. Rosenkranz and Murphy described diversion of the hepatic venous return to the pulmonary artery through an extracardiac Gore-Tex conduit (W. L. Gore & Associates, Flagstaff, AZ) [8].

	Patients and methods

Top Abstract Introduction Patients and methods Results Comment References

 
From 1972 to November 2000, 998 patients underwent a Fontan operation at the Mayo Clinic. An intracardiac conduit was used in 71 patients. Thrombosis of the intraatrial conduit occurred in 2 of these patients, both with azygous continuation of the inferior vena cava. By the year 2000, 40 patients have undergone an extracardiac Fontan procedure at the German Heart Center in Berlin. Conduit thrombosis occurred in 1 patient, also with azygous continuation of the inferior vena cava. Thus, in all 3 patients, the conduit only carried hepatic venous blood flow (Fig 1). In the 2 patients operated at the Mayo Clinic (aged 11 and 24 years), the conduit was made of autologous pericardium and placed inside a common atrium directing the hepatic venous effluent to the pulmonary arterial confluence. In the 3rd patient (28 years old), operated at the German Heart Institute, a 24-mm Gore-Tex conduit was placed in an extracardiac position. The 2 patients with thrombosed intraatrial autologous pericardial conduits were reoperated, while in the 3rd patient, partial thrombosis of the extracardiac conduit was treated successfully with permanent oral anticoagulation.

View larger version (39K): [in this window] [in a new window]   Fig 1. Anatomy of anomalous systemic venous return and corrective procedures. (A) Azygous continuation of the inferior vena cava (IVC) and intracardial conduit connecting hepatic veins to the pulmonary artery. (B) Azygous and hemiazygous continuation of bilateral IVC and intracardiac conduit. (C) Hemiazygous continuation of the IVC and extracardiac conduit. (LPA = left pulmonary artery; LSVC = left superior vena cava; RPA = right pulmonary artery; RSVC = right superior vena cava.)

	Results

Top Abstract Introduction Patients and methods Results Comment References

Patient 2
An 11-year-old girl was seen at the Mayo Clinic with the diagnosis of univentricular heart, common atrium, left atrioventricular valve atresia, moderate insufficiency of the right atrioventricular valve, severe pulmonary stenosis, bilateral superior vena cava, azygous and hemiazygous continuation of two bilateral inferior venae cavae, and separate hepatic venous drainage into the common atrium. She had undergone a left Blalock-Taussig shunt done at another institution at 15 months of age. On October 10, 1986, she underwent a modified Fontan operation during which bilateral superior cava to pulmonary anastomoses were constructed, and an autologous pericardial conduit was placed inside the atrium diverting the hepatic venous effluent to the pulmonary arterial confluence (Fig 1B). The right atrioventricular valve was replaced with a 31-mm St. Jude prosthesis (St. Jude Medical, St. Paul, MN). The left subclavian artery was ligated. She was discharged on oral anticoagulation. She did well for 2 years. In 1988, she developed a protein-losing enteropathy and was treated medically with some success until 3 years later, when her condition worsened with progressive accumulation of ascitic fluid and edema of the lower abdominal wall and perineum. Two-dimensional echocardiography revealed total thrombosis of the pericardial conduit. The function of the valvular prosthesis was normal and ventricular function was preserved. On November 25, 1991, she underwent excision of the intracardiac conduit. The hepatic veins were completely obliterated, so that no lumen was apparent on initial inspection. Further dissection in the subdiaphragmatic area was done and the lumen was exposed. The veins were allowed to drain into the common atrium. The procedure was done under deep hypothermia and circulatory arrest. She recovered hemodynamically but suffered a severe neurologic ischemic injury. Computerized tomography demonstrated multiple bilateral brain infarcts. Her neurologic status deteriorated progressively, she developed multiple organ failure, and expired on the 5th postoperative day.

Patient 3
A 28-year-old man was evaluated at the German Heart Institute with the diagnosis of double outlet right ventricle, common atrium, left atrioventricular valve atresia, severe subvalvular, valvular, and supravalvular stenosis, hypoplastic main pulmonary artery bifurcation, bilateral superior venae cavae, hemiazygous continuation of the inferior vena cava to the left superior vena cava, and independent drainage of the hepatic veins into the inferior portion of the common atrium. The patient underwent a modified Fontan operation on March 20, 1996, with autologous pericardial patch enlargement of the pulmonary artery confluence, bilateral bidirectional superior cava to pulmonary artery anastomoses and insertion of a 24-mm Gore-Tex conduit in an extracardiac position between the hepatic veins and the right pulmonary artery (Fig 1C). A 5-mm fenestration was constructed between the extracardiac conduit and the atrium. The postoperative course was complicated by a low cardiac output state, fluid retention, and renal failure. Cardiac catheterization performed 3 weeks after the surgical procedure demonstrated wide patency of all anastomoses and no evidence of conduit thrombosis. The patient recovered slowly and was discharged from the hospital 41 days after the surgical procedure. He was prescribed aspirin. During the next 2 years, the patient experienced a decrease in exercise tolerance. Cardiac catheterization was performed on September 14, 1998, which revealed partial thrombosis of the Gore-Tex conduit. However, there was no measurable pressure gradient and no significant obstruction to hepatic venous drainage. The fenestration had closed spontaneously. Arterial saturation was 92% on room air. Oral anticoagulation with dicumarol was recommended. The last outpatient clinic visit was on January 20, 2000, 4 years after the surgical procedure. He was in NYHA functional class II. The left ventricular ejection fraction was 43%. There were no signs of conduit obstruction by magnetic resonance imaging or echocardiography. His arterial oxygen saturation was 90%.

	Comment

Top Abstract Introduction Patients and methods Results Comment References

 
The importance of hepatic blood flow for prevention or reversal of pulmonary arteriovenous malformation has been reported previously [5–8]. Recently, Uemura and colleagues [9] reported the presence of pulmonary arteriovenous fistulae in 3 patients with azygous continuation of the inferior vena cava who had undergone total cavo-pulmonary anastomosis prior to 12 years of age. They described marked progression of the right lung fistulae in 2 patients in whom the hepatic venous blood flow was diverted to the left lung. The authors emphasized the importance of exposing both lungs to hepatic venous blood. Kawashima and associates reported that pulmonary arteriovenous fistulae develop only rarely in older patients [10]. Arteriovenous fistulae did not develop in any of 14 patients who underwent the Kawashima operation at age 12 years or older [10, 11]. In 1 of our patients (aged 32 years) the thrombosed intraatrial conduit was removed and the hepatic veins were allowed to drain again into the atrium. This patient has not developed pulmonary arteriovenous fistulae during the 9 years that have elapsed since the surgical conversion. Kawashima has speculated that in the older patients the "putative substance" in hepatic venous blood could be transported to the lung through well-developed systemic-to-pulmonary arterial collaterals [11]. It has also been suggested in the previous reports from the Mayo Clinic that use of intracardiac conduits is not indicated in the case of interruption of the IVC with independent hepatic venous drainage to the atrium, since thrombosis may occur because of the relative low flow in the conduit from hepatic veins [12, 13]. Thus, it is not unreasonable to believe that diversion of the hepatic veins to the pulmonary circulation may not be necessary in patients undergoing the Kawashima procedure after the age of 12 years. However, in younger children, the hepatic venous blood flow should be diverted to the pulmonary arterial circulation. This can be accomplished by one of the following methods: (1) an intracardiac tunnel procedure when allowed by the pulmonary venous drainage; (2) an intracardiac conduit; or (3) an extracardiac conduit. Perhaps, a smaller diameter conduit should be used in these patients to prevent stagnation of blood predisposing to thrombosis.

The tunnel technique may not be suitable in patients with anomalies of the pulmonary venous drainage which can interfere with successful intraatrial separation of the systemic from the pulmonary venous blood flows. The use of conduits, either intracardiac or extracardiac, obviate the need of tunneling and has excellent results in patients with normal inferior caval drainage. Indeed, long-term patency of these conduits continues to be excellent regardless of the material used—Gore-Tex, homograft tissue, or autologous pericardium. The use of these conduits in patients with azygous continuation of the IVC may seem to be a reasonable extrapolation. However, there is one crucial difference. Namely, the blood flow through the conduit in these patients is significantly less than that of the normal inferior vena caval drainage as is true for most Fontan patients. Thus, the risk of conduit thrombosis may be significantly higher due to the lower blood flow.

The issue of anticoagulation therapy after Fontan procedure remains controversial [14]. Oral anticoagulation with dicumarol did not prevent conduit thrombosis at least in 1 of our patients. Nevertheless, it seems reasonable to recommend oral anticoagulation in patients with azygous continuation of the inferior vena cava. Further data are needed to either confirm or refute this opinion.

Because of low blood flow from hepatic veins, placement of the artificial conduit or homograft should be avoided especially when the conduit has already been thrombosed once. Thus, it would be reasonable to search for alternative ways of diverting hepatic blood flow to the pulmonary circulation in patients with hepatic vein thrombosis.

Alternative techniques to direct the hepatic venous blood flow to the pulmonary circulation include: (1) anastomosis of the hepatic veins to the dilated azygous veins using a cuff of the atrial tissue [15]; and (2) a circumferential atrial tunnel from the origin of hepatic veins to superior vena cava by means of a flap of right atrial free wall [16–18]. These techniques remain unproven.

In conclusion, we believe that hepatic vein–pulmonary artery conduit use in patients with azygous continuation of the inferior vena cava may have an increased risk of thrombosis. Chronic oral anticoagulation may be necessary in these patients. Alternative surgical methods that avoid the use of hepatic vein-to-pulmonary artery conduit deserve consideration and further study.

	References

Top Abstract Introduction Patients and methods Results Comment References

 

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