Overview

What is pulmonary atresia in a child?

A congenital (existing at birth) cardiac condition known as pulmonary atresia causes the pulmonary valve to either not develop normally or to remain closed after birth. Blood cannot enter your lungs through your pulmonary artery and take up oxygen if your pulmonary valve isn't functioning. Instead, your body's blood circulates through low-oxygen areas.

Located on the right side of the heart, the pulmonary valve is one of the four valves in your body. The blood vessel that supplies blood to your lungs is located between your right ventricle and this structure. Normally, the pulmonary valve opens to allow blood to enter your pulmonary artery from your right ventricle.

How does pulmonary atresia affect the baby?

A baby with pulmonary atresia may not only lack a normal pulmonary valve but also exhibit the following symptoms:

• Blue lips, fingers, and toes caused by low oxygen levels in the blood.
• The tricuspid valve, which connects the right atrium and right ventricle, is undeveloped.
• A gap (the foramen ovale) that may remain open after birth rather than closing between the right and left atriums of their hearts. As a result, blood with low oxygen content can go from the right atrium to the left atrium and then through the left ventricle and into the aorta.

A dividing wall issue in the baby's heart is another possibility in cases of pulmonary valve atresia. Normally, this wall aids blood in traveling through the proper channels.

There is a solid wall between the two sides of your heart, known as the septum, and your heart has four chambers. Your lungs receive blood from the right side, which carries oxygen. The aorta, your heart's major artery, is the conduit via which the left half of your heart pumps blood that is rich in oxygen throughout your body.

Pulmonary atresia types-

• Ventricular septal defect combined with pulmonary atresia- Blood with and without oxygen can mix in this septum, a hole in the wall between the ventricles of the right and left brain.
• Unbroken ventricular septum accompanied by pulmonary atresia- Your heart has a complete (intact) wall between its left and right sides.

What symptoms of pulmonary atresia in a child?

Pulmonary atresia symptoms might include the following and are typically present in the first few hours or days of a baby's life:

• Rapid breathing.
• Bluish abrasion of the skin, especially the lips, fritters, and toes.
• Cold, pale, or glacial skin.
• Problems breathing.
• Fatigue or languor.

What causes pulmonary atresia in a child?

There is no known precise cause of pulmonary atresia. A fetus develops pulmonary valve atresia during the first eight weeks of pregnancy in the uterus.

What is the diagnosis and test for pulmonary atresia in a child?

Your healthcare practitioner will monitor the health of the fetus during your pregnancy with routine screenings. Fetal echocardiograms, which use safe sound waves, can be performed if an ultrasound reveals anything concerning. This also makes use of sound waves to magnify the fetal heart.

Your baby's doctor will examine them to see if there are any issues with their heart and lungs after delivery. When using a stethoscope to listen for cardiac murmurs, they will request tests that could involve:

The non-invasive pulse oximetry test determines the level of oxygen in your baby's blood.

• Chest X-ray: An imaging technique that takes a picture of your baby's insides from the outside using painless X-rays.
• Electrocardiogram (EKG or ECG)- An electrocardiogram, often known as an EKG or ECG, is a non-invasive, painless method of monitoring your baby's heart's electrical activity to determine whether or not their heart muscles are under any stress.
• Echocardiogram- A noninvasive, painless imaging technique is the echocardiogram. It creates a moving image of your baby's heart and heart valves using innocuous sound waves.
• Cardiac catheterization- An invasive technique called cardiac catheterization allows a medical professional to see your baby's heart. A tiny, flexible tube is inserted into a blood vessel in your baby's crotch. They transfer it to their heart's interior. To provide a clearer picture of your child's heart and valves, they might employ contrast dye.

What is the treatment for pulmonary atresia?

Children with atresia in their pulmonary valves typically require surgery to increase blood flow to the lungs.

How your child is affected by pulmonary atresia and their overall health will determine how best to treat them.

• Age
• Medical background

Brief medical interventions-

Medication and balloon atrial septostomy are two short-term therapies. For the majority of children, more surgery will most likely be required to increase lung blood flow.

• Medication- Alprostadil is a medication given intravenously (IV) to newborns diagnosed with pulmonary atresia. The ductus arteriosus, which joins the aorta to the pulmonary artery, is prevented by this medication from beginning to seal as it usually would in the hours or days following delivery. This must remain open in order for your baby's pulmonary arteries to get oxygen-poor blood from the aorta. Following that, it travels to their lungs to absorb oxygen.
• Balloon atrial septostomy- A cardiac catheterization can be used by your child's doctor to widen the septum (the wall that separates the right and left atrium). Your baby's lungs receive better blood flow as a result. Through a blood vessel and the foramen ovale, they place a catheter into your child's heart. The catheter's tip balloon is inflated. In order to create an opening in the atrial septum, they swiftly withdraw the catheter. After that, blood with low oxygen content might move from the right atrium to the left, entering the left ventricle and aorta.

Which surgical procedures are performed?

The kind of pulmonary atresia repair surgery your child requires will depend on a number of variables, such as:

• Their pulmonary artery and right ventricle sizes.
• The blood-pushing capacity of their right ventricle.

Surgery to treat a ventricular septal defect and pulmonary atresia

The right ventricle may normally pump blood to your baby's lungs and is fully grown. During surgery-

• Closing the ventricular septal defect in your child.
• Putting a donor artery and valve in the space between the pulmonary artery and your child's right ventricle. This permits blood to enter their pulmonary artery and reach their lungs through their right ventricle.

Surgery for pulmonary atresia without a ventricular septal defect-

The right ventricle of your infant is typically underdeveloped in situations like this. In the first few years of their existence, they require three different surgeries. Blood with low oxygen content is redirected to the patient's pulmonary artery and lungs during certain open heart surgeries:

The first procedure entails inserting a shunt or tube straight into your baby's pulmonary artery from the aorta or one of its branches. This makes sure that during the first few months of life, their lungs receive enough blood.

Your child will have a bi-directional Glenn or hemi-Fontan treatment between the ages of 4 and 8 months. The shunt inserted during the initial procedure is taken out by a medical professional. The right pulmonary artery and the superior vena cava, a large vein that transports deoxygenated blood from the upper body to the heart, are then connected. This permits blood to get straight to your child's lungs and pulmonary artery from their superior vena cava.

Your child will undergo the Fontan procedure, which is their third and last operation, between the ages of three and six. The inferior vena cava, which is the vein that supplies the heart with deoxygenated blood from the lower body, is connected to the pulmonary artery by the surgeon during this procedure.

What should I do if my child has pulmonary atresia?

Common pulmonary atresia complications include:

• Growth delays.
• Irregular cardiac rhythms.
• Cardiac arrest.
• Illness of the liver.

After surgical repair, it is crucial to keep an eye out for any problems. Since fontan circulation is abnormal, your child will require regular monitoring for the rest of their lives. People who have Fontan circulation may eventually experience liver malfunction, irregular heart rhythms, exhaustion, abdominal and leg edema, and difficulties breathing. A heart transplant is necessary for certain people.

What is the pulmonary atresia survival rate?

The survival rate at age 1 is 50%, and at age 10, it is 8%, if pulmonary atresia with a ventricular septal defect is not surgically corrected. Without surgery, most people do not survive past their 30s.

Living with

When should my child visit a physician?

Your child's pediatric cardiologist will schedule follow-up appointments on a regular basis. Two to four weeks after leaving the hospital, this begins. As they get older, some kids might require more heart catheterizations, operations, or specialized drugs.

A cardiologist must provide lifelong care for individuals with pulmonary atresia. At least once every six months, they might have appointments.

Long-term issues could consist of:

• Irregular heartbeats.
• Heart breakdown.
• The pulmonary artery narrowing.

Follow-up care on a regular basis might lessen or even eliminate these issues.

Why Tender Palm Super-Speciality Hospital for Pediatric Pulmonary Atresia?

Tender Palm Hospital has the most experienced team of Pediatric Cardiologists, Pediatric Cardiac Surgeons, and diagnostics with the latest and International standard infection control measures in Lucknow, India. The Pediatric Cardiac Science Centre team has decades of experience in successfully treating Pediatric Pulmonary Atresia.

To seek an expert consultation for Pediatric Pulmonary Atresia in Lucknow, India

Call us at +91-9076972161
Email at care@tenderpalm.com

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