Pulmonary Atresia and VSD. IPCCC Code: 01.01.26, 12.31.05

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International Society for Nomenclature of

Paediatric and Congenital Heart Disease

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IPCCC Code: 01.01.26, 12.31.05
	AEPC Code:	Pulmonary atresia + VSD: Fallot type [with native pulmonary arteries present - 01.01.55], Waterston (ascending aorta-right pulmonary artery) anastomosis

	EACTS-STS Code:	Tetralogy of Fallot, Pulmonary atresia, [AP window (aortopulmonary window)-modifier, Acquired AP window (AP fistula), Surgically created, Surgically created central shunt - 10.20.81]. Alternative classification: Pulmonary atresia-VSD, Type A (Native central PA [PA proximal to the hilar bifurcation] present and No MAPCA(s)), Confluent RPA and LPA

Definition: NA

Common Synonyms: NA

This heart with tetralogy of Fallot has been tilted toward the right to demonstrate the tiny atretic pulmonary trunk (PT). The aorta is large and lies anterior and to the right of its normal position. There is an intact, stenotic Waterston shunt on the right lateral aspect of the ascending aorta. The superior caval vein (SCV) drains to the right atrium in the usual fashion and the tricuspid valve (TV) guards the inlet of the right ventricle (RV). There is right ventricular hypertrophy.

Contributor: Diane Spicer, BS

This simulated 5 chamber echocardiographic view demonstrates the overriding aorta and the perimembranous ventricular septal defect. The aortic valve (AV) lies above the interventricular septum (IVS), committed approximately 50% to the right ventricle and 50% to the left ventricle (LV) (red line). There are concordant atrioventricular connections and both the tricuspid (TV) and mitral (MV) valves are in fibrous continuity with the aortic valve at the roof of the ventricular septal defect. (RA-right atrium, LA-left atrium, LPA-left pulmonary artery)

Contributor: Diane Spicer, BS

AWG Certification: Pending