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Management Anomalies of Caval Veins and Hepatic Veins As highlighted, most anomalies of course and connection remain clinically silent and require no treatment On the other hand, anomalies of drainage necessitate redirection of the systemic venous blood to reach the lungs for oxygenation, as opposed to the left atrium.27 In most instances, this can be achieved by surgical redirection of the anomalous vein or veins In some cases, with alternative sites of drainage of blood, this can be achieved by percutaneous closure of the anomalous channel if an optimal alternative route for drainage of blood is patent.94–97 Rarely, in some cases of totally anomalous systemic venous connection, the right atrium and right ventricle are too small to permit complete correction As an alternative, a bidirectional Glenn shunt can be constructed, along with an atrial septectomy to improve oxygen saturation.28 In one 13-yearold girl reported by us, the right ventricle grew to achieve normal dimensions following such a protocol, highlighting the significance of preload in the growth of the cardiac chambers (see Fig 27.17 B–C).98 Acquired anomalous of drainage of the systemic veins require treatment of the underlying pathology Systemic-to-pulmonary venous connections also may resolve with the treatment of thrombosis of right superior caval vein Nonetheless, in some patients with large or persistent collateral channels, it may be necessary to perform closure by percutaneous implantation of devices or surgical ligation Portosystemic Shunts Portosystemic shunts, both intrahepatic due to problems with the venous duct and extrahepatic (Abernethy malformation), improve with closure of the abnormal shunt However, this mandates prior assessment of the adequacy of the intrahepatic portal venous system In patients with an absent or a diminutive intrahepatic portal venous system, liver transplantation remains the only option.80,88,89 Annotated References Anderson RH, Brown NA, Moorman AF Development and structures of the venous pole of the heart Dev Dyn 2006;235:2–9 The review discusses recent findings concerning the embryologic origin of the systemic venous sinus of the right atrium and its relationship to the developing pulmonary vein Cochrane AD, Marath A, Mee RB Can a dilated coronary sinus produce left ventricular inflow obstruction? An unrecognized entity Ann Thor Surg.1994;58:1114–1116 Agnoletti G, Annechino F, Preda L, Borghi A Persistence of the left superior caval vein: can it potentiate obstructive lesions of the left ventricle? Cardiol Young 1999;9:285–290 Two investigations pointing to the role of the persistent left superior caval vein in producing an obstruction to the inlet of the morphologically left ventricle Debich DE, Devine WA, Anderson RH Polysplenia with normally structured hearts Am J Cardiol 1990;65:1274–1275 Bartram U, Fischer G, Kramer HH Congenitally interrupted inferior vena cava without other findings of the heterotaxy syndrome: report of five cases and characterization of a rare entity Pediatr Dev Pathol 2008;11:266–273 Initial observations confirming the presence of interruption of inferior caval vein without isomerism of atrial appendages Gupta SK, Juneja R, Anderson RH, Gulati GS, Devagourou V Clarifying the anatomy and physiology of totally anomalous systemic venous connection Ann Pediatr Cardiol 2017;10:269–277 Gupta SK, Shetkar SS, Ramakrishnan S, Kothari SS Saline contrast echocardiography in the era of multimodality imaging—importance of “bubbling it right.” Echocardiography 2015;32:1707–1709 Two reviews emphasize the need of differentiating anomalous connection from anomalous drainage They also provide an algorithmic approach for the use of saline contrast echocardiography in the diagnosis of anomalous systemic venous connections Knauth A, McCarthy KP, Webb S, et al Interatrial communication through the mouth of the coronary sinus Cardiol Young 2002;12:364– 372 This study not only extended the observations
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