Hypoplastic left heart syndrome associated with large omphalocele and hypoplastic left bronchus.

We present a patient with the combination of hypoplastic left heart syndrome (HLHS), large omphalocele, and hypoplastic left bronchus who underwent a modified Norwood procedure and was successfully discharged home. The child required tracheostomy with home ventilation and we elected to delay the primary repair of the omphalocele. To our knowledge, this is the first reported case with this combination of anomalies surviving till hospital discharge. Our experience demonstrates the potential viability of patients with concurrent HLHS, large abdominal wall defects, and airway anomalies and may additionally advocate for delayed repair of large omphaloceles in patients with complex congenital heart disease.