Aortic Stenosis and Aortic Atresia




Abstract


Aortic stenosis and atresia encompass a group of congenital heart diseases (CHDs) characterized by left outflow tract obstruction. It accounts for 3% of all CHDs. There is a 15% recurrence risk, one of the highest regarding CHD. It has a low association to chromosomal and extracardiac anomalies. It can progress to hypoplastic left heart syndrome. Fetal aortic valvuloplasty can be offered in selected cases in order to avoid hypoplastic left heart syndrome progression. Delivery should be planned in a tertiary referral center with cardiac surgery. Prognosis depends on the severity of the stenosis and left ventricle function at birth.




Keywords

congenital heart defect, left ventricle tract obstruction, aortic stenosis, aortic atresia

 




Introduction


Aortic stenosis (AS) affects 1.4 : 10,000 newborns. The spectrum of clinical manifestations varies widely according to the degree of valve stenosis.




Disease


Definition


AS and aortic atresia encompass a group of congenital heart diseases (CHDs) characterized by left ventricular outflow tract obstruction. Depending on the level of the defect, these are classified as supravalvular, valvular, or subaortic obstruction. Valvular stenosis is the most frequently found form in the fetus.


Prevalence and Epidemiology


AS accounts for 3% of all CHDs. There is a 15% recurrence risk, one of the highest among all forms of CHD.


Etiology and Pathophysiology


The etiology is multifactorial. Autosomal dominant and recessive inheritance forms have been reported. Subvalvular forms have been associated with hypertrophic myocardiopathy and maternal diabetes.


Aortic atresia is frequently associated with mitral atresia in the context of hypoplastic left heart syndrome (HLHS) ( Chapter 81 ). Isolated aortic atresia is uncommon. If present, it is characterized by fused or dysplastic valve leaflets, and the aortic valve can be bicuspid, monocuspid, or quadricuspid.


Clinical and ultrasound (US) manifestations depend on the severity of the obstruction and the functionality of the mitral valve.


Jul 7, 2019 | Posted by in OBSTETRICS & GYNAECOLOGY IMAGING | Comments Off on Aortic Stenosis and Aortic Atresia
Premium Wordpress Themes by UFO Themes