IPCCC: 06.06.01
 

AEPC Derived Term:    

AVSD: isolated atrial component (primum ASD)(partial) (06.06.01) 

EACTS-STS Derived Term:    

ASD, Primum (06.06.01)

ICD10 Derived Term:    

Definition:  A congenital cardiac malformation that is a variant of an atrioventricular septal defect (atrioventricular canal) with an interatrial communication just above the atrioventricular valve, no interventricular communication just below the atrioventricular valve, separate right and left atrioventricular valvar orifices, and varying degrees of malformation of the left sided component of the common atrioventricular valve. The bridging leaflets of the common atrioventricular valve are bound down to the crest of the scooped out ventricular septum so that the potential for shunting through the atrioventricular septal defect is possible only at the atrial level and not at the ventricular level.

Common Synonyms:  Incomplete atrioventricular septal defect with isolated atrial component; Incomplete atrioventricular canal defect with isolated atrial component; Ostium primum atrial septal defect

Discussion: The subcostal long axis views of both the right (A060601-13a) and left (A060601-13b) sides of the heart show a common atrioventricular junction with separate valvar orifices, this also known as an ‘ostium primum’ defect. The superior and inferior bridging leaflets are attached by a connecting tongue of valve tissue, creating the separate right and left valvar orifices. The valve tissue is adherent along the entire length of the ventricular septum. There are concordant atrioventricular and ventriculo-arterial connections. The characteristic ‘scooping out’ (red dots) of the ventricular septum is easily appreciated and shunting would occur entirely at the atrial level. The lower edge of the atrial septum is illustrated with yellow dots. The upper portion (oval fossa or fossa ovalis) of the atrial septum is virtually intact with a tiny probe patent defect. (A-aorta, CS-coronary sinus, LAA-left atrial appendage, LV-left ventricle, OF-oval fossa, PV-pulmonary valve, PVV-pulmonary vein, RPA-right pulmonary artery, RV-right ventricle, SCV-superior caval vein)

Modality: Anatomic specimen

Orientation: Right subcostal long axis

Description: In this view, the common atrioventricular valve is clearly separated from the pulmonary valve (PV) by a muscular infundibulum.

Contributor: Diane Spicer, BS

Institution: The Congenital Heart Institute of Florida (CHIF)

Image Label: A060601-13a

Source of Image: Van Mierop Archive, University of Florida, Gainesville, Florida

Image Certification: 5 August 2011

AWG Rating:

Modality: Anatomic specimen

Orientation: Left subcostal long axis

Description: In this instance the zone of apposition (white arrow) is distant from the ventricular septal crest where as in most other specimens with the same defect, the zone of apposition lies adjacent to the ventricular septal crest. The upper portion of the atrial septum is well formed with a tiny septal defect (red arrow).

Contributor: Diane Spicer, BS

Institution: The Congenital Heart Institute of Florida (CHIF)

Image Label: A060601-13b

Source of Image: Van Mierop Archive, University of Florida, Gainesville, Florida

Image Certification: 5 August 2011

AWG Rating:

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