Case report
Accessory hepatic vein to pulmonary venous atrium as a cause of cyanosis after the Fontan operation

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Abstract

The presence of an accessory hepatic vein to the pulmonary venous atrium should be considered in the setting of progressive cyanosis following the Fontan procedure. An inferior vena caval angiogram with “levophase” should demonstrate it. Surgical intervention or trans-catheter occlusion should lead to prompt resolution of cyanosis.

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Cited by (30)

  • Case of the Season: Atresia of the Coronary Sinus Ostium With Persistent Left Vitelline Vein Connecting to the IVC Resulting in a Left-to-Right Shunt

    2020, Seminars in Roentgenology
    Citation Excerpt :

    Other cases of persistent left vitelline vein have been described,4,5 typically found incidentally as they do not create a left to right shunt in isolation- the coronary sinus and hepatic veins, even when connected by the persistent left vitelline vein, ultimately both drain to the right atrium. Cases of patients with functionally single ventricle circulation requiring Fontan palliation have developed similar shunts from the pulmonary venous (left) atrium to the hepatic veins,6,7 presumably by growth of veno-venous collaterals along the remnants of the embryologic vitelline venous connections. However the combination of a persistent left vitelline vein, atresia of the coronary sinus ostium, and unroofing of the coronary sinus resulting in a left to right shunt has not been previously described.

  • Systemic to pulmonary venous collaterals in adults with single ventricle physiology after cavopulmonary palliation

    2015, International Journal of Cardiology
    Citation Excerpt :

    Subsequently, it is hypothesized that this pressure gradient leads to development of collateral venous connections between the higher pressure SVC and low pressure IVC or pulmonary veins, serving as a “pop-off” from a high pressure to a low pressure circuit [7,8]. In addition to this well-established “pathway,” many patients will develop numerous collateral venous channels after complete cavopulmonary palliation with the Fontan operation where both the SVC and IVC return is directed to the pulmonary artery [9–12]. These venous collaterals allow deoxygenated systemic venous blood to “bypass” the lungs and therefore result in progressive cyanosis and decreased functional capacity.

  • An accessory left hepatic vein draining into the atrium separately: A rare unique finding during liver retrieval and a challenge for reconstruction of the cava prior to implantation

    2014, Transplantation Proceedings
    Citation Excerpt :

    Computed tomography (CT) angiography has a major role in identifying such anomalies. Martorell et al [4] described an accessory hepatic vein to pulmonary venous atrium as the cause of persistent cyanosis after the fontan fenestration operation. Yoshii et al [5] described a case of accessory hepatic vein complicating extracardiac total cavopulmonary connection.

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Dr. Rothman's address is: Division of Pediatric Cardiology, 8445, UCSD Medical Center, 200 West Arbor Drive, San Diego, California 92103.

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