A 4 year old girl with an underlying diagnosis of atrioventricular discordance, double outlet right ventricle with pulmonary valvar atresia, and subpulmonary ventricular septal defect, was admitted for elective surgery. She had had surgery as a neonate to insert a right 5 mm Gore-tex modified Blalock Taussig shunt, and again at 3 years old for formation of a bidirectional Glenn anastomosis. Cardiac catheterisation showed mean pulmonary artery pressures of 7–10 mm Hg and she was admitted for completion of total cavopulmonary connection. Although this procedure was successful, the Blalock Taussig shunt previously inserted was surgically inaccessible due to adhesions and was therefore left patent.

Pleural effusions and tense ascites associated with poor urine output complicated the immediate postoperative period. The patient required inotropic support to a maximum of 0.36 μg/kg/min of noradrenaline (norepinephrine), 0.09 μg/kg/min of adrenaline (epinephrine), and 10 μg/kg/min of enoximone with 4 μg/kg/min …