Pulmonary atresia

Diagnosis

There are many tests available for pulmonary atresia, including:

• Chest X-ray. This procedure allows the medical team to see the extent of pulmonary atresia as it shows the size and shape of the heart and lungs.
• Electrocardiogram (ECG or EKG). This test helps detect heart arrhythmia and abnormal heart muscle stress. The doctor places electrodes on the chest, the arms or legs to monitor the cardiac electricity, which are connected to a computer to show results.
• Echocardiogram. This main method to diagnose pulmonary atresia creates pictures of a moving heart using sound waves. A doctor might do a fetal echocardiogram, which is an echocardiogram done on a pregnant woman, to detect pulmonary atresia in a baby in the womb.
• Cardiac catheterization. In this procedure, a catheter is inserted into a blood vessel of the arm or groin before being guided to an artery of the heart. Contrast material is also used to make X-ray images of the heart arteries in a higher quality.

Treatment

Babies who are having symptoms of pulmonary atresia are required to seek immediate medical care. A doctor will choose a proper treatment option, including surgery, based on each baby’s medical condition.

Medications

A doctor sometimes gives medications to prevent the ductus arteriosus from closing to allow blood to flow from the aorta to the pulmonary artery. This way of treatment for pulmonary atresia, despite being temporary, allows the medical team to decide which kind of surgery is the most suitable for each infant patient.

Surgery or other procedures

In certain cases, pulmonary atresia is treated using a catheter inserted into a large vein in the groin and guides it to the heart. There are many procedures that use a catheter to treat the disease, including:

• Balloon atrial septostomy. This procedure aims to widen the natural hole (foramen ovale) of the wall that separates the upper two chambers with a balloon. Although this hole naturally closes after a baby is born, keeping this hole widened allows more blood to flow to the lungs.
• Stent placement. A stent is placed in the ductus arteriosus, which is the natural connection between the aorta and pulmonary artery, which naturally closes after a baby is born, keeping it widened also allows more blood to flow to the lungs.

In babies, if pulmonary atresia persists, they might be required to undergo multiple cardiac surgeries.

There are many surgery options. The best treatment will be chosen based on the size of the right ventricle and pulmonary artery.

• Shunting. A doctor makes a bypass or shunt to connect the aorta which delivers blood out of the heart with the pulmonary arteries. This allows more blood to flow to the lungs. This method might remain effective for just a few months among babies as they will quickly grow too big for the bypass.
• Glenn procedure. A doctor uses this surgery to directly connect the pulmonary artery to one of the large veins responsible for making blood flow back to the heart. The right side of the heart will still receive blood from another large vein. Then the blood is pumped through the surgically repaired pulmonary valve, which helps enlarge the right ventricle.
• Fontan procedure. This surgery is recommended for those with a small right ventricle. A doctor will make an artificial channel that directs almost or all blood that flows to the heart to go to the pulmonary artery.
• Heart transplant. A doctor might recommend a heart transplant to those with cardiac deterioration that cannot be treated.