Summative Diagnosis:
Cardiovascular phenotype: Dextrocardia and congenital heart disease associated with situs inversus totalis and heterotaxy, such as double outlet right ventricle (DORV), ventricular septal defects (VSD), right aortic arch (RAA), dual inferior vena cava (IVC)
Noncardiovascular phenotype: Abnormal thoracic and abdominal organ situs anomalies, such as dextrogastria, pulmonary isomerism, and malaligned sternal vertebra. Airway cilia were dyskinetic, slow, or immotile

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
0100 Situs inversus totalis
0110 Dextrocardia
0190 Heterotaxy syndrome
0602 DORV, ventricular defect committed to aorta
1300 Ventricular septal defect
1310 Ventricular septal defect, membranous
1320 Ventricular septal defect, muscular
2700 Abnormal aortic arch
2720 Right aortic arch
2810 Inferior vena cava anomaly
3804 Congenital heart disease
3817 Abdominal situs ambiguous (abdominal heterotaxy)
3974 {I,L,I}
4100 Skeletal, skin, muscle anomaly
4238 Bronchial isomerism
4851 Kartagener syndrome (siewart syndrome)(primary ciliary dyskinesia)