Table 1.

Composition of the aggregate cardiac defect groups according to the clinical nomenclature and administrative nomenclature.

Aggregate cardiac defect group	Clinical nomenclature†	Administrative nomenclature‡
Tetralogy of Fallot	Tetralogy of Fallot	745.2 – Tetralogy of Fallot
	Tetralogy of Fallot with absent pulmonary valve
	Tetralogy of Fallot with atrioventricular septal defect
	Double outlet right ventricle, tetralogy of Fallot type
	Pulmonary atresia with ventricular septal defect§
	Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral artery(ies)
Transposition of the great arteries	Transposition with intact ventricular septum	745.10 – Transposition of the great arteries
	Transposition with intact ventricular spetum and left ventricular outflow tract obstruction	745.11 – Double outlet right ventricle
	Transposition with ventricular septal defect	745.19 – Other transposition
	Transposition with ventricular septal defect and left ventricular outflow tract obstruction
	Transposition, not otherwise specified
	Double outlet right ventricle, transposition-type
Hypoplastic left heart syndrome	Hypoplastic left heart syndrome	746.7 – Hypoplastic left heart syndrome
	Hypoplastic left heart syndrome with ventricular septal defect

^†“The Clinical nomenclature” is derived from the International Congenital Heart Surgery Nomenclature and Database Project of The European Association for Cardio-Thoracic Surgery and The Society of Thoracic Surgeons.

^‡The “Administrative nomenclature” is derived from the International Classification of Diseases, Ninth Revision, Clinical Modification diagnosis codes.

^§For the clinical nomenclature, hearts with discordant ventriculo-arterial connections, pulmonary atresia, and ventricular septal defect are grouped with Transposition of the great arteries, not with Tetralogy of Fallot.