Surgical Management
Optimal management of the two sub-sets remains nebulous.14-25,38 The subsets, nonetheless, are best segregated on the basis of right versus left isomerism. The markedly different features will post different surgical challenges. Overall surgical management has evolved with time. Moderately hypothermic cardiopulmonary bypass at 32°C, using cold blood cardioplegia, is now the most popular technique, replacing continuous cardiopulmonary bypass with low-flow perfusion, brief periods of deep hypothermic circulatory arrest, and deep hypothermic circulatory arrest with selective cerebral perfusion.14-25,38 Venous cannulation can be problematic in both subsets because of the systemic venous anomalies. Direct cannulation is preferred because of the likely complexity of any intraatrial repair. The intracardiac anomalies are more likely to be amenable to complete repair in those with left isomerism, with biventricular repairs reported in one-third to one-half of patients.14-25,38 Such repairs, nonetheless, often require complex atrial and ventricular baffle procedures.14-25,38 The complex malformations associated with right isomerism, in contrast, usually preclude biventricular repair.