Summative Diagnosis:
Cardiovascular phenotypes: Double outlet right ventricle (DORV), atrioventricular septal defect (AVSD), ventricular septal defects (VSD)

Noncardiovascular phenotype: Cystic kidney, tubular dilations, hydroureter, and hydronephrosis

Phenotypic Similarity to Human Syndrome:
Nephronophthisis



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
600	Double outlet right ventricle
1300	Ventricular septal defect
1821	Hypoplastic right ventricle (subnormal cavity volume)
2050	Atrial septal defect
4502	Hydronephrosis
4508	Polycystic kidney disease