What causes Gooseneck sign in the heart on left ventricular angiography?
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The Gooseneck sign is caused by an atrioventricular septal defect (AVSD) AKA endocardial cushion defect, a congenital cardiac anomaly where there is deficient development of the atrial septum, ventricular septum, and atrioventricular valves. This results in a large communication among all four heart chambers with abnormal left-to-right and right-to-left shunting. Clinically, it is associated with conditions such as Down syndrome (trisomy 21), Edwards syndrome (trisomy 18), and asplenia syndrome.
Why is it called so?
It is named “Gooseneck sign” because on a left ventricular angiogram, the contour of the left ventricular outflow tract and the elongated, narrowed configuration of the left ventricular inflow tract resembles the curved neck of a goose.
Click here for an image. Reference: Pinterest
Pathophysiology
The sign develops due to the defect in the endocardial cushions leading to abnormal atrioventricular valve formation and a shortened or absent septum. The left ventricular catheter angiogram shows a characteristic lengthening and narrowing of the left ventricular inflow tract caused by the deficiency of the inferior atrial septum and the superior portion of the interventricular septum, producing the distinctive “Gooseneck sign” contour.
Alternative names: Endocardial cushion defect sign
Other associated named signs: None specific, but AVSD is associated with generalized cardiomegaly and pulmonary hypertension findings on imaging.
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