Summative Diagnosis:
Cardiovascular phenotypes: Complex congenital heart defects associated with heterotaxy, dextrocardia, double outlet right ventricle (DORV), atrioventricular septal defect (AVSD), muscular ventricular septal defect (VSD), total anomalous pulmonary venous return (TAPVR), dual inferior vena cava (IVC), and ventricular myocardial non-compaction.

Noncardiovascular phenotype: Abnormal thoracic and abdominal organ situs anomalies, right-sided pancreas, asplenia, and gut malrotation.

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 Codes Code Description
110 Dextrocardia
600 Double outlet right ventricle
900 Totally anomalous pulmonary venous return
1100 Atrioventricular canal (endocardial cushion defect)
1320 Ventricular septal defect, muscular
1802 Excessive myocardial trabeculation or noncompaction
190 Heterotaxy Syndrome
2810 Inferior vena cava anomaly
3817 Abdominal situs ambiguous (abdominal heterotaxy)
4407 Intestinal malrotation
4771 Asplenia