Partial atrioventricular septal defect

Orpha code: 1330OMIM code:

Definition

A rare congenital cardiac malformation that is a variant of an atrioventricular septal defect (AVSD) with an interatrial communication (ostium primum defect) just above the common atrioventricular (AV) valve, no interventricular communication just below the atrioventricular valve, a common atrioventricular junction but separate right and left atrioventricular valvar orifices, and a three-leaflet, left-sided component of the common atrioventricular valve (''cleft''). Shunting is restricted to the atrial level because of fusion of the leaflets of the common AV valve with the crest of the ventricular septum.

Disease data
Classification

Morphological anomaly

Synonyms
PAVC
Partial AVSD
Partial atrioventricular canal defect
PAVC
Partial AVSD
Partial atrioventricular canal defect
ORPHA code
1330
OMIM code
-
ICD10 code
Q21.2
ICD11 code
-

No additional description.

Orphanet - interntowa baza danych dotyczących rzadkich chorób i sierochych leków. ©INSERM 1999 - Dostępna na stronie www.orphanet.pl