Summative Diagnosis:
Cardiac phenotypes: Double outlet right ventricle (DORV) with subaortic ventricle septal defect (VSD), aortic arch hypoplasia/interrupted aortic arch (IAA), aortic stenosis/preductal coarctation, and atrioventricular septal defect (AVSD), coronary fistula
Noncardiac phenotypes: Kidney anomalies with hydronephrosis, hydrureter, and small glomerular cysts

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
0600 Double outlet right ventricle
0606 DORV + AVSD (AV canal)
1250 Interrupted aortic arch
1430 Aortic stenosis - supravalvar
2230 Coronary fistula (arterio-venous or arterio-cameral)
2704 Aortic arch hypoplasia
3950 {S,D,D}
4502 Hydronephrosis