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Pulmonary atresia with the intact ventricular system (PA/IVS) is a rare congenital heart condition present since the early stages of the baby development. It is manifested as abnormal or completely closed pulmonary valve. This condition causes the heart of a newborn to pump more blood to provide oxygen for the whole body.

Treatment

This condition is diagnosed early, and it requires a team of experts to coordinate the treatment. Treatment usually depends on the severity of the symptoms and family wishes. A specialist could connect the baby to the ventilator to ease the breathing, and give some drugs to prevent ductus arteriosus and to help the heart to beat stronger.

Cardiac Catheterization

This procedure is used to assess the condition of the heart defects. Doctor will insert a catheter into the arm or groins of the child and guide it to the heart using X rays.

Radiofrequency Ablation and Balloon Valvotomy

The specialist will insert a catheter to the child’s heart, and make a small hole in the valve and using the balloon open it even more, to allow the blood to flow through the valves into the lungs.

Balloon Atrial Septostomy

It’s similar to previous procedure, but the hole is made in the wall between right and left atria of the heart.

Cardiac Surgery

This type of surgery is usually performed at the first week of baby’s life. It mostly depends on the child’s condition and the size of the right ventricle.

Shunt and Stent Placement

A small tube (shunt) is surgically inserted into aorta and pulmonary artery, to provide the blood flow from the heart to the lungs.

Stent is also a tube, which may be inserted into connections between the atria of the heart or between the right ventricle and pulmonary artery.

Bidirectional Glenn and Fontan Procedures

This procedure is used to connect the blood vessels that carry the blood from the body with the blood vessels carrying blood to the lungs. That way, most of the blood flows directly to the lung.

Right Ventricular Outflow Tract Reconstruction

This surgical procedure is required in some children, to rebuild the right ventricular outflow tract.

Additional Surgeries

Additional cardiac surgery might be needed in cases of not fully grown right ventricle. Some of the patients may need a valve repair or replacement surgery at some point of their life.

After the surgery a child will need to be monitored by a heart specialist. Sometimes, PA/IVS patients may need antibiotics before the dental work and a surgery to prevent endocarditis.

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