Hypoplastic Left Heart Syndrome

  • The left side of the heart is very poorly formed and cannot support the main circulation (round the body). The left ventricle and aorta are abnormally small (hypoplastic). This is amongst the most severe forms of heart defect.

    Affected infants usually become severely symptomatic soon after birth (as the ductus closes). This is one of the most serious cardiac malformations and leads to death in the newborn period in almost all affected babies, unless surgery or Heart Transplantation can be offered.


    Surgery consists of a 'Norwood' operation. This involves connecting the origin of the pulmonary artery to the aorta, to allow the right ventricle to pump blood to the main circulation and a 'Shunt' operation. The atrial septum is removed to allow blood to pass freely from the left atrium to the right side of the heart. The 'Shunt' involves insertion of a tiny piece of artificial tube (made from Goretex) between the right arm artery and the right pulmonary artery, to maintain blood flow to the lungs. Another option is to use a Goretex "Conduit" from RV to PA, instead of a "Shunt".


    Other surgery is carried out later in childhood and may involve several operations. The second operation is usually performed at about three months and is a "Bi-directional Cavo-pulomonary Shunt" (BCPS). This is an intermediate stage towards the final operation. It involves channeling blood from the SVC to the pulmonary arteries.


    The final operation is called the 'Fontan Operation', which is carried out at three to four years (or later). This leads to blue blood (low in oxygen) being channelled through the lungs, without any 'pump' driving it. The right ventricle then pumps the red blood (high in oxygen), round the body. This operation makes the child 'Pink' - but does not correct the original problem. Not all affected children need to have a Fontan operation. If they are well, with less drastic surgery, they may not have this 'Final' big operation. Some children may be unsuitable for a Fontan operation and alternative forms of treatment may be offered.