Tricuspid Atresia

Dr. Ira Shah
Tricuspid Atresia - Patient Education
What is tricuspid atresia?
The heart consists of 4 chambers - the right and left atrium (These are the chambers that receive the blood - the right atrium receives impure blood from the rest of the body and left atrium receives pure blood from the lungs) and the right and left ventricles (These are the chambers that pump the blood from the heart - right ventricle receives impure blood from right atrium and sends it to the lungs and left ventricle receives pure blood from left atrium and sends it to the body). Right atrium is separated from the right ventricle by a valve called as tricuspid valve. Left atrium is separated from the left ventricle by a valve called as mitral valve. The function of the valve is to allow blood to flow from each atrium to their respective ventricle. In tricuspid atresia, there is no tricuspid valve. Thus no blood can flow from right atrium to the right ventricle. Thus the right ventricle is small and not fully developed. The impure blood then that reaches the right atrium flows through a hole between the right atrium and left atrium (atrial septal defect) to the left atrium and then to the left ventricle. Thus the left ventricle receives both pure and impure blood. This blood then is sent to the body and certain amount reaches the right ventricle by a hole between the two ventricles (Ventricular Septal Defect) and then to the lungs and from there after it is oxygenated it goes back to the left atrium.

What are the symptoms of tricuspid atresia?
Since there is mixing of both pure and impure blood in the heart and that blood causes into the body, the child may appear blue. Some children may have too much blood flowing into the lungs through the hole in the ventricles and this could lead to breathlessness. Since the heart has to function more to supply adequate oxygen to the body, there may be heart failure and growth retardation.

How is tricuspid atresia diagnosed?
The diagnosis is made by echocardiography.

What are the other problems associated with tricuspid atresia?
About 15-20% of patients with tricuspid atresia may have other heart defects. Sometimes pulmonary valve (valve that regulates flow from right ventricle to the lungs) may be absent and volume of blood reaching the lungs may be low and thus less blood will get oxygenated leading to increased bluish colour of skin. Some patients have problems of the arteries.

What is the treatment of tricuspid atresia?
The treatment consists of surgical repair. Without surgery, the patient rarely survives to adulthood. In case of absence of pulmonary valve may require a shunt to increase blood flow to the lungs. In this, a connection is made from the arteries such as subclavian artery or aorta to the pulmonary artery to increase oxygenation of blood and decrease bluishness.

In patients where more amount of blood is reaching the lungs, then pulmonary artery is banded to decrease blow flow to the lungs and to decrease workload of the heart. Corrective surgery in form of Fontan surgery is done when the child is older (after 4 years of age). This consists of connecting right atrium to the pulmonary artery and the hole in the atrium and ventricle is closed. Children with tricuspid atresia require lifelong follow up by a cardiologist.

Tricuspid Atresia Tricuspid Atresia 08/01/2015
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