Pulmonary atresia is a congenital heart defect that is usually diagnosed immediately after birth. In pulmonary atresia, the valve that allows blood from the heart to go to your or your baby's lungs (pulmonary valve) does not form properly. Instead of opening and closing so blood can travel from the heart to the lungs, a solid sheet of tissue is formed. Therefore, the blood does not travel through its normal way to take oxygen from the lungs. Instead, some blood travels to the lungs and lungs through the heart and other natural components in your arteries.

These passages are necessary as your baby develops in the womb, and they usually close immediately after birth. Children with pulmonary atresia often have a blue cast on their skin because they are not getting enough oxygen. Pulmonary atresia is a life-threatening condition. Procedures to correct your baby's heart condition and medications to help your baby's heart work more efficiently are the first steps in treating pulmonary atresia.

Symptoms of pulmonary atresia

Symptoms of pulmonary atresia usually appear in the first hours or days of the baby's life and may include:

• Fast breathing
• Blue on the skin, especially on the lips, fingers and toes.
• Cold, pale, or burning skin
• Respiratory problems
• Fatigue or tiredness
• Irritated
• Bad nutrition

Causes of pulmonary atresia

There is no cause for pulmonary atresia. To understand the problems caused by pulmonary atresia, it helps to know how the heart works.

How does the heart work

The heart is divided into four hollow chambers, two on the right and two on the left. In performing its basic function, pumping blood throughout the body, the heart uses its left and right sides for different tasks. The right side of the heart carries blood to the lungs through vessels called pulmonary arteries. In the lungs, the blood carries oxygen and returns to the left side of the heart through the pulmonary veins. Your baby sends blood through the aorta to the left side of the heart to supply oxygen to the body and the rest of the body.

Blood moves in one direction through your baby's heart through valves that open and close when the heart beats. The valve that allows your baby to draw oxygen from the heart and lungs to the lungs is called the pulmonary valve. In pulmonary atresia, the pulmonary valve does not develop properly, preventing it from opening. Blood does not flow from the right ventricle to the lungs.

Before birth, a malformed valve is not fatal because the placenta provides oxygen to your baby instead of the lungs. Blood entering the right side of your baby's heart passes through the hole (foramen ovale) between the upper chambers of your baby's heart, whereby oxygen-rich blood is pumped through the aorta to the rest of your baby's body. After birth, your baby's lungs need to supply her body with oxygen. In pulmonary atresia, without a functioning pulmonary valve, the blood must find another way to get to your baby's lungs.

Foremen's ovary usually closes immediately after birth, but remains open in pulmonary atresia. Newborns also have a temporary connection between the aorta and the pulmonary artery (ductus arteriosus). This route allows oxygen to travel through the lungs to the lungs, where your baby can take in oxygen to supply the body. The ductus arteriosus usually closes immediately after birth, but it can be left open with medication.

In some cases, there may be a second hole in the tissue that separates the main pumping chambers of your baby's heart, called a ventricular septal defect (VSD). VSD allows blood to pass through the right ventricle to the left ventricle. Children with pulmonary atresia and VSD often have additional abnormalities in the lungs and the arteries that carry blood to the lungs. Without VSD, the right ventricle receives less blood flow before birth and often does not fully develop. This is a condition known as pulmonary atresia with intact ventricular septum (PA / IVS).

Risk factors for pulmonary atresia

In most cases, the exact cause of a congenital heart defect such as pulmonary atresia is unknown. However, there are several factors that increase the risk of having a baby with congenital heart defects, including:

• Parents with congenital heart disease
• That delays the mother before conception
• Smoking before or during pregnancy
• A mother who does not adequately control her diabetes.
• The use of certain types of medications during pregnancy, such as some acne medications and blood pressure medications.

Diagnosis of pulmonary atresia

Tests to diagnose pulmonary atresia may include:

• Bone scan: X-rays show the size and shape of your baby's internal tissues, bones, and organs. This will help your pediatrician see the extent of your baby's pulmonary atresia.
• Electrocardiogram (ECG): In this test, sensor pads attached to wires (electrodes) measure the electrical impulses that your child's heart gives off. This test detects any abnormal heart rhythms (arrhythmia or dysrhythmia) and shows the tension of the heart muscle.
• Echocardiogram: In an echocardiogram, sound waves create detailed images of your child's heart. Your pediatrician will usually use an echocardiogram to diagnose pulmonary atresia. Before delivering your baby (fetal echocardiogram), your doctor can diagnose your baby's pulmonary atresia using an echocardiogram of your abdomen.
• Cardiac catheterization: In this test, your pediatrician will insert a thin, flexible tube (catheter) into the blood vessel in your baby's groin and guide your baby's heart using X-ray images. This test provides complete information about the structure of your heart. baby and his heart, pulmonary artery and aortic blood pressure and oxygen levels. Your pediatrician may inject a special dye into the catheter so that the arteries are visible under the X-ray.
  
  

Treatment of pulmonary atresia

Your child will need emergency medical help once symptoms of pulmonary atresia develop. The choice of surgeries or procedures depends on the severity of your child's condition.

Drugs

An intravenous drug called prostaglandin prevents the closure of the natural connection (ductus arteriosus) between the pulmonary artery and the aorta. This is not a permanent solution, but it will give your doctor more time to decide what type of surgery or procedure is best for your child.

Catheterization procedures

In some cases, it can be repaired through a long, thin tube (catheter) that is inserted into a large vein in your baby's groin and connected to the heart. These policies include:

• Balloon atrial septostomy: A balloon can be used to expand the natural hole (foramen ovale) in the wall between the two upper chambers of the heart. This hole usually closes immediately after birth. Expanding this increases the amount of blood available to travel to the blood vessels.
  
• Stent placement: Your pediatrician can place a stent tube at the natural connection between the aorta and the pulmonary artery (ductus arteriosus). This opening usually closes immediately after birth. Keeping it open allows blood to travel to the lungs.
  

Heart surgery

The surgical repair needed depends on the size of your child's right ventricle and pulmonary artery. Babies with pulmonary atresia often require heart operations over time. Some examples:

• Maneuvers: Creating a shunt (shunt) of the main blood vessels leading from the heart (aorta) to the pulmonary arteries allows adequate blood flow to the lungs. However, babies usually get over this shunt within a few months.
  
• Glenn's approach: In this surgery, one of the large veins that normally returns blood to the heart is connected directly to the pulmonary artery. Another large vein continues to supply blood to the right side of the heart, which sends it through a surgically repaired pulmonary valve. This helps the right ventricle grow.
  
• Fonton approach: If the right ventricle is too small to be useful, surgeons can create a pathway using the Fontaine procedure, which, if not most, allows blood to flow directly into the pulmonary artery.
  
• Heart transplant: In some cases, the heart is too damaged to repair and a heart transplant may be necessary.
  

Future treatments

Pulmonary atresia is likely to be repaired one day with fetal surgery during pregnancy. A recent small study has shown good results, but more research is needed.

Lifestyle and home remedies

Here are some tips for caring for your child after you return home from the hospital:

• Make regular subsequent appointments with your pediatrician: Your baby will need regular appointments with a doctor trained in congenital heart conditions. These appointments should continue even as your child grows older. Ask your pediatrician how often to see your child.
  
• Help your child stay active: Encourage regular play and activities that your child can tolerate, with plenty of opportunities for rest and sleep. Being active can help keep your child's heart healthy. As your child grows, talk with a cardiologist about what activities are best for him.
  
• Keep up with routine well-child care: Standard vaccinations are encouraged for children with congenital heart defects, as well as vaccines against the flu, pneumonia and respiratory syncytial virus infections.
  
• Preventive antibiotics: Your child's cardiologist may recommend that your child take preventive antibiotics before certain dental and other procedures to prevent bacteria from entering the bloodstream and infecting the inner lining of the heart (infective endocarditis). Practicing good oral hygiene — brushing and flossing teeth, getting regular dental checkups — is another good way of preventing infection.
  

Complications

• Without treatment, pulmonary atresia is almost always fatal. Even after surgical repairs, you should carefully monitor your child's health for any changes that may indicate a problem.
  
• People with structural heart problems such as pulmonary atresia have a higher risk of developing endocarditis than the general population. Infective endocarditis is an inflammation of the valves and lining of the heart caused by a bacterial infection.
  
• Even after treatment, people born with pulmonary atresia appear to be at higher risk for certain heart problems, such as abnormal heart rhythms (arrhythmia) and heart failure in adults.
  

Prevention of

pulmonary atresia

Since the exact cause of pulmonary atresia is unknown, it may not be possible to prevent it. However, there are some things you can do to lower your baby's risk of congenital heart defects, including:

• Manage chronic medical conditions: If you have diabetes, controlling your blood sugar can lower your risk of heart disease. If you have other chronic conditions, such as high blood pressure or epilepsy, and you need medications, talk with your doctor about the risks and benefits of these medications.
  
• Do not smoke: Smoking cigarettes during pregnancy increases the risk of heart defects in your baby.
  
• Maintain a healthy weight: If you're obese, you have a higher risk of having a baby with a congenital heart defect.
  
• Get a German measles (rubella) vaccine: If you develop German measles during pregnancy, it may affect your baby's heart development. Being vaccinated before you try to conceive likely eliminates this risk. However, no link has been shown between rubella and the development of pulmonary atresia.