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There are two main groups of CHD: cyanotic congenital heart disease and acyanotic congenital heart disease.

Cyanotic (low oxygen in the blood) congenital heart disease

Cyanotic congenital heart disease involves heart defects that reduce the amount of oxygen delivered to the rest of the body. This is sometimes called a critical congenital heart defect. Babies born with cyanotic congenital heart disease usually have low levels of oxygen and need surgery. Examples include:

  • Left heart obstructive lesions: This type of defect reduces blood flow between the heart and the rest of the body (systemic blood flow). Examples include hypoplastic left heart syndrome (when the heart is too small on the left side) and interrupted aortic arch (aorta is incomplete).
  • Right heart obstructive lesions: This type of defect reduces blood flow between the heart and lungs (pulmonary blood flow). Examples include tetralogy of Fallot (a group of four defects), pulmonary atresia and tricuspid atresia (valves don’t develop correctly).
  • Mixing lesions: With this type of defect, the body mixes systemic and pulmonary blood flow. One example is transposition of the great arteries when the two main arteries leaving the heart are reversed. Another is truncus arteriosus, when the heart has only one main artery, instead of two, to carry blood to the body.

Acyanotic (blood oxygen level is acceptable) congenital heart disease

Acyanotic congenital heart disease involves a defect that causes blood to pump through the body abnormally. For example:

  • Hole in the heart: One of the heart’s walls can have an abnormal opening. Depending on the location of the hole, this may be called atrial septal defect, atrioventricular canal, patent ductus arteriosus or ventricular septal defect.
  • Problem with the aorta: The aorta is the main artery that carries blood away from your heart to the rest of the body. It can be too narrow (aortic coarctation). Or the aortic valve (which opens and closes to regulate blood flow) may be restricted in opening and or a more common defect with only two flaps instead of three (called bicuspid aortic valve).
  • Problem with the pulmonary artery: The pulmonary artery carries blood from the right side of the heart to the lungs to get oxygen. If this artery is too narrow, it’s called pulmonary artery stenosis.


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