Article

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

Department of Pediatric Cardiology, Oregon Health & Science University, Portland, OR, USA.
Pediatric Cardiology (impact factor: 1.3). 08/2010; 31(6):878-80. DOI:10.1007/s00246-010-9716-7 pp.878-80
Source: PubMed

ABSTRACT 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.

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Keywords

complex congenital heart disease
 
concurrent HLHS
 
heart syndrome
 
home ventilation
 
hospital discharge
 
large abdominal wall defects
 
large omphalocele
 
large omphaloceles
 
modified Norwood procedure
 
omphalocele
 
potential viability
 

Michael Hainstock