REOPENING OF PERSISTENT LEFT SUPERIOR CAVAL VEIN AFTER BIDIRECTIONAL CAVOPULMONARY CONNECTIONS

Persistent left superior vena cava (SVC) is a not uncommon finding in patients with congenital heart disease. This anatomical variant must b e recognised before doing a Glenn anastomosis, bidirectional cavopulmo nary connection or a Fontan-type procedure. Following these procedures , reopening of a left SVC leading to clinical cyanosis can occur. Five cases are described in whom persisting left SVCs were excluded before performing a bidirectional cavopulmonary connection or Fontan procedu re but (re-)opened after surgery, leading to cyanosis either by reduci ng effective pulmonary blood flow (bidirectional cavopulmonary connect ion) or by an obligatory right to left shunt (Fontan). These observati ons suggest that, embryologically, the lumen of the left SVC obliterat es rather than disappears. Balloon occlusion angiography of the innomi nate vein before cavopulmonary connections or Fontan procedures might improve detection of these collateral vessels.