Tricuspid Atresia (TA)

The word "Atresia" means "no opening." In tricuspid atresia, the valve is missing between the upper-right chamber (the right atrium) and the lower-right chamber (the right ventricle).

Normally, the tricuspid valve allows blood to pass from the right atrium to the right ventricle. The right ventricle then pumps blood into the lungs to pick up oxygen before it flows back into the left side of the heart. But with tricuspid atresia, blood cannot reach the lungs or the left side of the heart unless other defects are present.

Defects that often happen along with Tricuspid Atresia include:

* Atrial Septal Defect (ASD)

* Ventricular Septal Defect (VSD)

* Patent Ductus Arteriosus (PDA)

These defects usually allow some of the blood to flow through the lungs, but oxygen-poor blood still gets pumped into the body along with the oxygen-rich blood. The oxygen-poor blood makes the fingers, toes, and lips appear blue. This condition is called Cyanosis.

SYMPTOMS OF TRICUSPID ATRESIA

Symptoms are noted shortly after birth. The following are the most common symptoms of Tricuspid Atresia. However, each child may experience symptoms differently. Symptoms may include:

* Blue color of the skin, lips, and nail beds

* Rapid breathing

* Labored breathing

* Rapid heart rate

* Cool, clammy skin

DIAGNOSIS

* CHEST X-RAY – a test that uses radiation to take pictures of structures inside the chest.

* ELECTROCARDIOGRAM(ECG, EKG) – a test that records the heart's activity by measuring electrical currents through the heart muscle; it may show signs of heart strain or enlargement.

* ECHOCARDIOGRAM – a test that uses high-frequency sound waves (ultrasound) to examine the size, shape, and motion of the heart.

* CARDIAC CATHETERIZATION – an x-ray of the heart's circulation that is done after injection of a contrast dye.

TREATMENT FOR TRICUSPID ATRESIA

The patient will most likely be admitted to the intensive care unit (ICU) or special care nursery once symptoms are noted. Initially, the patient will be placed on oxygen, and possibly even on a ventilator, to assist his/her breathing. Intravenous (IV) medications may be given to help the heart and lungs function more efficiently.

CARDIAC CATHETERIZATION procedure,which is mostly used as a diagnostic procedure, can as well be used as a initial treatment procedure for some heart defects.

As a part of the Cardiac Catheterization, a procedure called a BALLOON ATRIAL SEPTOSTOMY may be performed to improve mixing oxygen-rich (red) blood and oxygen-poor (blue) blood.

An intravenous (IV) medication called Prostaglandin E1 is given to keep the ductus arteriosus from closing.

The various operations that can be done are:

* BLALOCK-TAUSSIG SHUNT:

This first operation creates a pathway for blood to reach the lungs. A connection is made between the first artery that branches off the aorta (called the Right Subclavian Artery) and the right pulmonary artery. Some of the blood traveling through the aorta towards the body will "shunt" through this connection and flow into the pulmonary artery to receive oxygen. However, the child will still have some degree of cyanosis since oxygen-poor (blue) blood from the right atrium and oxygen-rich (red) blood from the left side of the heart mix and flow through the aorta to the body.

* GLENN SHUNT:

This is the second operation, often performed at about 4 to 12 months of age, replaces the Blalock-Taussig shunt with another connection to the pulmonary artery. In this operation, the Blalock-Taussig shunt is removed, and the superior vena cava (the large vein that brings oxygen-poor blood from the head and arms back to the heart) is connected to the right pulmonary artery. Blood from the head and arms passively flows into the pulmonary artery and proceeds to the lungs to receive oxygen. However, oxygen-poor (blue) blood returning to the heart from the lower body through the inferior vena cava will still mix with oxygen-rich (red) blood in the left heart and travel to the body, so the child will remain mildly cyanotic. This operation helps create some of the connections necessary for the final operation, the Fontan procedure.

* FONTAN PROCEDURE:

This operation is often performed at about 18 to 36 months of age, and allows all the oxygen-poor (blue) blood returning to the heart to flow into the pulmonary artery, greatly improving the oxygenation of the blood. The Glenn shunt, connecting the superior vena cava to the right atrium, is left in place. A second connection is made directing blood from the inferior vena cava to the right pulmonary artery.