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.