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

Treatment

This condition is diagnosed early, and it requires a team ofexperts to coordinate the treatment. Treatment usually depends on the severityof the symptoms and family wishes. A specialist could connect the baby to theventilator to ease the breathing, and give some drugs to prevent ductusarteriosus and to help the heart to beat stronger.

Cardiac Catheterization

This procedure is used to assess the condition of the heartdefects. Doctor will insert a catheter into the arm or groins of the child andguide 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, toallow the blood to flow through the valves into the lungs.

Balloon Atrial Septostomy

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

Cardiac Surgery

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

Shunt and Stent Placement

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

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

Bidirectional Glenn and Fontan Procedures

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

Right Ventricular Outflow Tract Reconstruction

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

Additional Surgeries

Additional cardiac surgery might be needed in cases of notfully grown right ventricle. Some of the patients may need a valve repair orreplacement surgery at some point of their life.

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

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