Summative Diagnosis:
Cardiovascular defects: Heterotaxy with Double outlet right ventricle (DORV) with hypoplastic pulmonary artery, right atrial isomerism, right aortic arch (RAA), interrupted aortic arch type b (IAA), and aberrant right subclavian artery forming an incomplete vascular ring
Non-cardiovascular defects: Hypoplastic lung, hypoplastic kidney, thymus hypoplasia, cleft palate, missing pair of caudal sternal ribs, and central polydactyly

Phenotypic Similarity to Human Syndrome: Heterotaxy

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
0190 Heterotaxy syndrome
0600 Double outlet right ventricle
1252 Interrupted aortic arch type b
1770 Right atrial abnormality
2720 Right aortic arch
2731 Aberrant right subclavian artery
2760 Vascular ring
2966 Hypoplastic main pulmonary artery
4103 Polydactyly
4207 Hypoplastic lung
4876 Cleft palate