Summative Diagnosis:
Cardiovascular phenotypes: Complex congenital heart disease associated with heterotaxy with right isomerism. Phenotypes observed include dextrocardia/mesocardia, double outlet right ventricle (A,L,L) with anterior placement of the aorta (DORV, Taussig-Bing subtype), right atrial isomerism, unbalanced atrioventricular septal defect (AVSD), double outlet primitive single ventricle, hypoplastic left ventricle (LV).

Noncardiovascular phenotype: Visceral organ situs defects including dextrogastria, right lung isomerism, intestinal malrotation, asplenia. Also observed are hypoplastic thymus, right side diaphragmatic hernia, malaligned sternal vertebra, and craniofacial defects including anencephaly, acrania, agnathia, microstima, microcephaly, low set ears, proboscis, cylcopia, and anophthalmia.

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
110	Dextrocardia
200	Single ventricle
102	Levocardia
1140	Common atrium
140	Mesocardia
1811	Hypoplastic left ventricle (subnormal cavity volume)
190	Heterotaxy Syndrome
1910	Asplenia Syndrome (Right Isomerism)
2700	Abnormal aortic arch
2720	Right aortic arch
2731	Aberrant right subclavian artery
2760	Vascular ring
2810	Inferior vena cava anomaly
3817	Abdominal situs ambiguous (abdominal heterotaxy)
4100	Skeletal, skin, muscle anomaly
4134	Skull anomaly, congenital
4201	Diaphragmatic hernia
4240	Right bronchial isomerism
4332	Anencephaly
4407	Intestinal malrotation
4864	Anophthalmia
4906	Non-cardiac abnormality
4907	Non-cardiac thoracic abnormality
606	DORV + AVSD (AV canal)
610	DORV, Taussig bing