Congenital Heart Defects - HLHS



1. Hypoplastic ascending aorta and aortic arch.
2. Hypoplastic left ventricle.
3. Large patent ductus arteriosus supplying the only source of blood flow to the body.
4. Atrial septal defect allowing blood returning from lungs to reach the single ventricle.

Hypoplastic Left Heart Syndrome (HLHS) is one of the most complex cardiac defects seen in the newborn and remains probably the most challenging to manage of all congenital heart defects. It is one of a group of cardiac anomalies that can be grouped together under the description "single ventricle" defects.

In a child with Hypoplastic Left Heart Syndrome, all of the structures on the left side of the heart (the side which receives oxygen-rich blood from the lungs and pumps it out to the body) are severely underdeveloped.
5. The PDA has been ligated and the hypoplastic aorta is opened longitudinally from the descending aorta to near its origin at the heart.
6. The main pulmonary artery has been divided and the distal end closed.


7. A Gore-tex™ modified Blalock-Taussig shunt from the innominate artery to the right pulmonary artery supplies blood flow to the lungs.
8. The aorta has been reconstructed using the proximal main pulmonary artery, the hypoplastic aorta, and a patch so that the single right ventricle now ejects primarily to the body.