Gooseneck deformity

Gooseneck deformity

Gooseneck deformity seen in

[A] Interrupted aortic arch
[B] Patent Ductus Atreriosus
[C] Atrioventricular Canal Defects
[D] Tetralogy of Fallot

Gooseneck deformity

Atrioventricular Canal Anatomy and Differences from Normal Hearts, a Systemic Review of Literatures – Scientific Figure on ResearchGate. Available from: https://www.researchgate.net/figure/Left-ventricular-angiograms-obtained-from-a-case-of-AVC-before-correction-the-A-P-view_fig1_311085782 [accessed 25 Jun, 2023]

Left ventricular angiograms obtained from a case of AVC before correction, the A-P view in end-diastole shows the typical gooseneck deformity arrow (A). Left ventricular angiograms obtained from a case of normal heart reveals normal left ventricular outflow tract (B). Ao aorla; PA = pulmnoniary artery (Culpepper et al 1978). The lower pictures are the same after adding a red line by us to demonstrate the difference.  

Normal – distance from the left AV valve annulus to the left ventricular apex = apex to the aortic annulus

“gooseneck deformity” – distance from the left AV valve annulus to the left ventricular apex < apex to the aortic annulus

The distance from the left AV valve annulus to the left ventricular apex is appreciably less than that from the apex to the aortic annulus, whereas normally the 2 distances are equal.

This inlet to outlet disproportion creates the characteristic “gooseneck deformity”

Gooseneck deformity

The presence of a large, single, oval-shaped AV annulus dislodges the aortic valve annulus from its normal “wedged” position between the annuli of the TV and MV

The anterior and superior displacement of the aortic valve by the common AVV makes the outlet dimension of the LV greater than the inlet dimension and elongates the left ventricular outflow tract (LVOT).

This anatomic abnormality is partially responsible for the characteristic “gooseneck deformity” initially described in the left ventriculogram but also appreciated in echocardiography.

ECG – Ashman-Phenomenon

Long cycle followed by short cycle and short cycle terminated by aberrant QRS complex

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