What is Aortic Atresia?

Aortic Atresia
Single outlet ventriculo-arterial connections with functionally univentricular heart. (A) Aortic atresia with intact ventricular septum (single pulmonary outlet). Four chamber echocardiographic cut of the heart showing the severe hypoplasia of the left cardiac chambers. Note the tiny inlet portion of the left ventricle. This heart is only suitable for Norwood univentricular repair. (B) Pulmonary atresia with intact ventricular septum (single aortic outlet) with hypoplastic right ventricle. The size of the right ventricle is such that this heart may be suitable for one and half ventricle repair.The new concept of univentricular heart. Frescura C, Thiene G - Frontiers in pediatrics (2014). Not Altered. CC.

Aortic atresia is a rare congenital heart defect where the connection of the left ventricle to the aorta is blocked, affecting the flow of blood from the left ventricle to the body. 

What is the Pathology of Aortic Atresia?

Aortic atresia pathology is characterized by congenital fusion of aortic cusps that can be subvalvular, valvular, or supra-valvular in location. 

How does Aortic Atresia Present?

Aortic atresia presents as a murmur, cyanosis, rapid or difficulty breathing, irritability, low energy, and clammy skin. 

How is Aortic Atresia Diagnosed?

Aortic atresia diagnosis is based on chest x-ray, 2D echo, electrocardiogram, cardiac catheterization and MRI. 

How is Aortic Atresia Treated?

Aortic atresia treatment involves giving IV prostaglandin to keep the ductus arteriosus open for an alternative blood flow. Cardiac catheterization and balloon atrial septostomy may also be done.

What is the Prognosis of Aortic Atresia?

Aortic atresia prognosis is poor with incidence of more than 8 in 1000 live births, a serious defect that can lead to mortality without immediate intervention.