Ann Thorac Surg 1999;67:1494-1496
© 1999 The Society of Thoracic Surgeons
Case Reports
Intrahepatic venovenous shunting to an accessory hepatic vein after Fontan type operation
Naoki Yoshimura, MDa,
Masahiro Yamaguchi, MDa,
Yoshihiro Oshima, MDa,
Teruo Tei, MDb,
Kyoichi Ogawa, MDa
a Department of Cardiothoracic Surgery, Kobe Children’s Hospital, Kobe, Japan
b Department of Cardiology, Kobe Children’s Hospital, Kobe, Japan
Accepted for publication November 2, 1998.
Address reprint requests to Dr Yoshimura, Department of Cardiothoracic Surgery, Kobe Children’s Hospital, 1-1-1, Takakura-dai, Suma-ku, Kobe, 654-0081, Japan
e-mail: y-naoki{at}za2.so-net.ne.jp
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Abstract
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Because there are various types of systemic venous connections in patients with visceral heterotaxy, unanticipated abnormal systemic venous channels may pose additional problems after a Fontan type operation. We report a case of severe cyanosis caused by anomalous intrahepatic venovenous fistula to an accessory hepatic vein early after total cavopulmonary anastomosis.
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Introduction
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Total cavopulmonary anastomosis appears to have reduced the mortality of Fontan type operation, and its indications have been extended to more complex anomalies including visceral heterotaxy [1]. Because there are various types of systemic venous connections in patients with visceral heterotaxy [2, 3], unanticipated abnormal systemic venous channels may pose additional problems after the operation. We report a case of severe cyanosis resulting from anomalous intrahepatic venovenous shunting to an accessory hepatic vein early after total cavopulmonary anastomosis.
A 9-year-old boy with asplenia syndrome, univentricular heart, common atrioventricular valve, malposition of the great arteries, and supracardiac total anomalous pulmonary venous connection draining into the superior vena cava at its junction with the single atrium received a pulmonary artery banding at 26 days of age. He underwent a patch angioplasty of the connecting vein and superior vena cava at 1 year of age, and direct anastomosis of the common pulmonary venous channel to the common atrium at 3 years of age for pulmonary venous obstruction. Cardiac catheterization showed a mean pulmonary artery pressure of 15 mm Hg, and a morphological right ventricular ejection fraction of 56.5%. Echocardiography showed common atrioventricular valve incompetence caused by prolapsed leaflet. He underwent a total extracardiac cavopulmonary anastomosis with a 5-mm fenestration and semicircular annuloplasty of the common atrioventricular valve. He returned to the intensive care unit with oxygen saturation levels of 80% to 90%, and was extubated on the fourth postoperative day. The patient gradually experienced increasing cyanosis, and his oxygen saturation levels decreased to 60% to 70% 1 month after the operation. At cardiac catheterization, the systemic arterial oxygen saturation was 73% with marked desaturation at the atrial level. The mean pulmonary artery pressure was 13 mm Hg, and ejection fraction of the morphological right ventricle was reduced to 29.0%. Aortography revealed that multiple aortopulmonary collateral arteries arising from the descending aorta were distributed to the right upper lung. An angiogram of the superior vena cava demonstrated that contrast medium from the superior vena cava flowed retrogradely down the extracardiac conduit toward the inferior vena cava (Fig 1A). An inferior vena caval angiogram confirmed an accessory hepatic vein draining from the inferior vena cava through an anomalous intrahepatic venovenous fistula into the common atrium (Fig 1B). Liver ultrasonography showed marked dilatation of hepatic veins (Fig 2). Color Doppler imaging of the hepatic veins revealed that the blood flow in the left hepatic vein was directed toward the accessory hepatic vein through the anomalous venovenous fistula (Fig 2). After coil embolization of the persistent aortopulmonary collateral arteries, an emergent operation was performed. The heart was exposed through a left anterolateral thoracotomy, and the adhesion in the pericardium was carefully dissected. The surgical ligation of a large accessory hepatic vein draining to the left side and posteriorly into the common atrium, which was discovered during the operation, resulted in normalization of the oxygen saturation levels to 95% to 100%.
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Fig 1. (A) Angiogram of the superior vena cava demonstrating that contrast medium from the superior vena cava (SVC) flowed retrogradely down the extracardiac conduit toward the inferior vena cava (IVC). (PA = pulmonary artery.) (B) Angiogram of the inferior vena cava demonstrating that contrast medium streams through the left hepatic vein (lt HV) opacifying a nest of intrahepatic venovenous fistulas to the accessory hepatic vein (HV) draining to the common atrium.
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Fig 2. Liver ultrasonography showing marked dilatation of hepatic veins (left). Color Doppler imaging showing the direction of blood flow in the hepatic veins. Blood flow in the left hepatic vein (lt HV) was directed toward the accessory hepatic vein (HV) through an anomalous venovenous (V-V) fistula (right).
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Comment
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During the early postoperative phase of a Fontan type operation, high systemic venous pressure is necessary for adequate transpulmonary blood flow. This high systemic venous pressure may lead to generalized edema, effusions, and hepatic venous congestion. Owing to this physiology, any residual venous connection that may short-circuit systemic venous return into the pulmonary venous atrium creates a potentially progressive right-to-left shunt resulting in profound progressive cyanosis [4–6]. In this report, we describe a rare case of a patient with visceral heterotaxy who experienced profound cyanosis after fenestrated total cavopulmonary anastomosis resulting from an accessory hepatic vein draining to the common atrium. Although some of the hepatic veins drain separately from the inferior vena cava into the common atrium in some patients with visceral heterotaxy [2, 3], an accessory hepatic vein draining from the left side of the liver to the common atrium is a very rare anatomic disorder [4, 5]. An accessory hepatic vein draining into the common atrium is of no hemodynamic significance preoperatively, but after the Fontan type operation, the pressure gradient between the inferior vena cava and the pulmonary venous atrium may stimulate the formation of an intrahepatic venovenous fistula [4, 5]. This is the main reason for difficulty in detecting the accessory hepatic vein preoperatively.
Indeed, despite appropriate investigation the accessory hepatic vein may be difficult to detect before and during the operation, and it becomes hemodynamically significant only after the Fontan type operation. The development of intrahepatic venovenous fistula is diagnosed reliably by angiography in the inferior vena caval portion of the cavopulmonary anastomosis with special emphasis on the liver circulation. Surgical intervention or transcatheter occlusion should be considered to resolve the cyanosis. We performed surgical intervention because transcatheter occlusion of the accessory hepatic vein often resulted in failure in published reports [4, 5]. Surgical correction of this acquired right-to-left shunt can be accomplished with relative ease and safety.
In summary, the presence of intrahepatic venovenous fistula resulting from accessory hepatic vein to the pulmonary venous atrium should be considered in a patient who shows progressive cyanosis after a Fontan type operation, and surgical intervention should lead to prompt resolution of cyanosis.
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Addendum
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The patient died of progressive ventricular dysfunction 5 months after the operation. Postmortem examination showed the accessory hepatic vein draining to the common atrium was completely ligated.
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References
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Gentles T.L., Mayer J.E., Jr, Gauvreau K., et al. Fontan operation in five hundred consecutive patients: factors influencing early and late outcome. J Thorac Cardiovasc Surg 1998;114:376-391.[Abstract/Free Full Text]
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Uemura H., Ho S.Y., Devine W.A., Kilpatrick L.L., Anderson R.H. Atrial appendages and venoatrial connections in hearts from patients with visceral heterotaxy. Ann Thorac Surg 1995;60:561-569.[Abstract/Free Full Text]
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Rubino M., Van Praagh S., Kadoba K., Pessotto R., Van Praagh R. Systemic and pulmonary venous connections in visceral heterotaxy with asplenia: diagnostic and surgical considerations based on seventy-two autopsied cases. J Thorac Cardiovasc Surg 1995;110:641-650.[Abstract/Free Full Text]
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Fernandez-Martorell P., Sklansky M.S., Lucas V.W., Kashani I.A., Cocalis M.W., Jamieson S.W. Accessory hepatic vein to pulmonary venous atrium as a cause of cyanosis after the Fontan operation. Am J Cardiol 1996;77:1386-1387.[Medline]
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Gatzoulis M.A., Shinebourne E.A., Redington A.N., Rigby M.L., Ho S.Y., Shore D.F. Increasing cyanosis early after cavopulmonary connection caused by abnormal systemic venous channels. Br Heart J 1995;73:182-186.[Abstract/Free Full Text]
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Abstract
Introduction
