Summative Diagnosis:
Cardiovascular Phenotype: Complex congenital heart defects associated with heterotaxy such as dextroversion with transposition of the great arteries (TGA), atrioventricular septal defect (AVSD), common atrium, right aortic arch (RAA), inferior vena cava (IVC) anomalies such as dual IVC, superior-inferior ventricles, total anomalous pulmonary venous return (TAPVR), aberrant left subclavian artery, and bilateral patent ductus arteriosus (PDA) forming vascular ring. This may be associated with mesocardia and dextrocardia.
Noncardiovascular Phenotype: Abnormal thoracic situs anomalies including dextrogastria, right bronchial isomerism, and inverted liver lobation. Also observed was cleft palate and hypoplastic thymus

Fyler Codes
The Fyler code developed by The Boston Children's Heart Foundation in Boston Children's Hospital provides a hierarchical clinical diagnosis of congenital cardiovascular defects and other disorders. These codes are used to delineate pathology in the mutant mouse models that parallel human disease and can be cross referenced to the International Pediatric and Congenital Cardiac Code (IPCCC) (http://www.ipccc.net/).

Fyler Code ID Code Description
0102 Levocardia
0110 Dextrocardia
0140 Mesocardia
0184 Superior-inferior ventricles (upstairs-downstairs ventricles)
0190 Heterotaxy syndrome
0700 D-loop transposition of the great arteries
1100 Atrioventricular canal (endocardial cushion defect)
1140 Common atrium
2700 Abnormal aortic arch
2720 Right aortic arch
2730 Aberrant left subclavian artery
2760 Vascular ring
2810 Inferior vena cava anomaly
3817 Abdominal situs ambiguous (abdominal heterotaxy)
4240 Right bronchial isomerism
4876 Cleft palate
4906 Non-cardiac abnormality
4907 Non-cardiac thoracic abnormality