Pulmonary valve atresia without a ventricular septum defect

Infants born with pulmonary valve atresia and an intact ventricular septum need immediate medical attention in a medical center with experienced pediatric cardiologists and pediatric cardiovascular surgeons. Upon diagnosis, the infant will be admitted to an intensive care unit to stabilize his or her condition while subsequent treatment decisions are made. In the intensive care unit, oxygen will be administered using a ventilator to assist breathing. Medications will be administered to help the heart and lungs function as efficiently as possible and to keep the ductus arteriosus ductus arteriosus open in order to maintain blood flow to the lungs.

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Heart catheterization may be performed early on to evaluate any other heart defects as well as to evaluate an initial treatment. Heart catheterization usually will be performed to establish whether the foramen ovale foramen ovale or ductus arteriosus is still open, and to determine the amount and relative proportions of oxygen-rich and oxygen-poor blood circulating in the heart.

As an initial treatment, balloon atrial septostomy may be performed to increase the amount of oxygenated blood in the heart by creating an opening between the right and left atria (upper chambers of the heart). In this procedure, a special catheter with a balloon in the tip is guided through the foramen ovale and into the left atrium, where the balloon is inflated to open a connection between the atria.

Open-heart surgery is often required within the first week of life. The surgeon will either open the pulmonary valve, allowing blood flow to the lungs, or insert a conduit that includes a new valve to channel blood flow from the right ventricle to the lungs. Alternatively, the surgeon may replace the baby's closed valve with a prosthetic valve.

Following surgery, the baby's circulation may be normal. Patients with donor valves may need another heart valve replacement surgery within the next 10 years as the donor valve slowly wears out. In this situation, the surgeon will create an artificial connection (shunt) to divert blood from the aorta, which carries blood to the body, to the pulmonary artery, which delivers blood to the lungs for more oxygenation.

Additional surgery may be needed when the child is older, particularly in cases where the right ventricle is underdeveloped. The type of procedure depends upon the size of the right ventricle and the pulmonary artery. If they are normal in size and the right ventricle is able to pump blood, the goal of the open-heart procedure is to channel the blood flow through the heart in a normal pattern. If the right ventricle is small and unable to pump, doctors may perform an operation called the Fontan procedure, in which the right atrium is connected directly to the pulmonary artery to improve oxygenation of the blood.

A child who has pulmonary atresia requires intensive monitoring both before and after surgery. The child will also need lifelong monitoring by a congenital heart specialist, with regular blood tests, X-rays, and urine analysis.