Summative Diagnosis:
Cardiovascular phenotype:
Cardiovascular phenotype: Heterotaxy with congenital heart disease (CHD): Double outlet right ventricle (DORV), atrioventricular (AVSD), common atrium, and ventricular septal defects (VSD), dual/bilateral inferior vena cava (IVC), coronary fistula, and ventricular non-compaction

Non-cardiac phenotype: Abnormal thoracic and abdominal organ situs anomalies, such as dextrogastria, left lung isomerism, liver isomerism, and asplenia


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
190 Heterotaxy syndrome
606 DORV + AVSD (AV canal)
1100 Atrioventricular canal (endocardial cushion defect)
1140 Common atrium
1300 Ventricular septal defect
1802 Excessive myocardial trabeculation or noncompaction
2230 Coronary fistula (arterio-venous or arterio-cameral)
2810 Inferior vena cava anomaly
3817 Abdominal situs ambiguous (abdominal heterotaxy)
4239 Left bronchial isomerism
4771 Asplenia
600 Double outlet right ventricle