Q22.6: Hypoplastic right heart syndrome

Your heart did not develop properly before you were born. This means that the right lower chamber (ventricle) of your heart is much smaller than normal.

The heart pumps blood through the body. Inside the heart are 4 chambers. There are 2 heart atria and 2 ventricles. Both heart atria and both ventricles are separated from each other by a partition. Major, important blood vessels branch out of the heart. The main artery (aorta), originating from the left lower chamber (ventricle) of the heart, supplies the whole body with oxygen-rich blood. The right ventricle pumps oxygen-poor blood through the pulmonary artery to the lungs, where the blood is then enriched with oxygen.

The blood cannot flow properly through your right ventricle. This is because the entrance or exit to the right ventricle has become very narrow or even blocked. Your left ventricle is therefore pumping most of the blood both into the body and into the lungs. This blood is a mixture of oxygen-poor and oxygen-rich blood.

This heart defect often results in an inadequate amount of oxygen flowing into the body. This makes the skin appear bluish in color. You may also have labored breathing. When the heart does not pump enough blood, fluid can build up in the abdomen or legs. The symptoms may begin shortly after birth.