POSTOPERATIVE MANAGEMENT OF SEVERE RIGHT VENTRICULAR DYSFUNCTION FOLLOWING ONE-AND-A-HALF VENTRICULAR REPAIR IN A CHILD WITH DOWN SYNDROME AND ATRIOVENTRICULAR CANAL DEFECT WITH EBSTEIN’S ANOMALY: A CASE REPORT

Dhananj Shivganesh B. R.*, Ankur Dogra, Kanika Prashar

ABSTRACT

Down syndrome is strongly associated with atrioventricular canal defects (AVCD), with up to 40% of affected children presenting with this lesion. The coexistence of Ebstein’s anomaly is rare but significantly complicates surgical repair due to the high risk of postoperative RV dysfunction.[2,3] One-and-a-half ventricular repair (1.5VR), achieved by combining intracardiac repair with a BD Glenn, provides an effective strategy for unloading the dysfunctional RV.[1–4] However, postoperative management remains challenging and is often the key determinant of outcome. Here, we present a case where vigilant intensive care interventions, including inotropic support, fluid optimisation, and airway protection, resulted in a favourable recovery.

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