Fig. 18.1
18.1.2 Unroofed Coronary Sinus Syndrome
18.1.3 Right Superior Caval Vein to the Left Atrium
18.1.4 Inferior Caval Vein into the Left Atrium
18.1.5 Indirect Inferior Caval Vein Connections
18.2 Surgical Management of Systemic Venous Route Abnormalities
Venous route abnormalities generally do not require surgical attention because the connections are physiologic, unless they are associated with cardiac abnormalities that require cardiopulmonary bypass to repair. Under these circumstances, the choice of venous drainage strategies becomes paramount for a successful cardiopulmonary bypass interval. The most common systemic venous route abnormality is the left SCV that drains unobstructed into the coronary sinus. In the majority of cases, it is wise to insert a venous catheter into this vein in preparation for cardiopulmonary bypass, to ensure proper drainage and avoid cerebral venous hypertension. Care must be taken to identify and preserve the phrenic nerve, which lies on the left side of the left SCV. Snugger control of this structure is best achieved in the intrapericardial portion of the left SCV to avoid injury to the phrenic nerve. Other challenging anatomic variations of systemic venous route return are the conditions of azygos or hemiazygos continuation of the ICV. Cannulation of this structure can be problematic. We have found that the best drainage technique is achieved with a large, straight catheter, the tip of which is advanced slightly (2 mm) into the orifice of the azygos or hemiazygos vein. The other nearby tributaries will be drained effectively in this manner, and the SCV snugger can be comfortably applied to ensure complete and unencumbered drainage. In general, hepatic veins that connect from below the diaphragm to the right atrium can be directly cannulated with small, angled venous cannulas. Care must be taken not to advance the catheters too far; doing so may result in poor drainage because the hepatic vein tributaries are closely aligned. In rare instances, the hepatic veins are large in number, small in caliber, and present a difficult cannulation dilemma. Under these circumstances, the surgeon may elect to institute cardiopulmonary bypass with a single-catheter technique and establish deep hypothermic circulatory arrest (DHCA) for the repair. This is an excellent option if individual cannulation of the hepatic veins involves increased risks.