Pathology

Ventricles are inverted.

Aorta is anterior and to the left of the PA. Prefix L is because aorta is left.

Mirror image coronaries.

Associated anomalies
VSD in 80%.

PS in 50%. PS may be valvular or subvalvular and is usually seen in patients with VSD.

Systemic AV valve regurgitation (TR) is seen in 30%.

Dextrocardia in 50%.

WPW syndrome can occur.

Heart blocks can occur. First degree block occurs in 50%. Second degree block can progress to third degree block.

Third degree block occurs in 30%.

Clinical features

VSD + PS may cause cyanosis (like any VSD + PS).

VSD alone may cause heart failure (like any VSD).

Single S2.

Chest X-ray

Straight left cardiac border is formed by ascending aorta.

Echo

There is no aorto-tricuspid continuity.

PSAX- double circle is seen.

Treatment