Description

Pulmonary Atresia (uh-TREE-zhuh) with ventricular septal defect, also called PA-VSD is a heart condition present from birth. That means that it is a congenital defect of the heart.

In pulmonary atresia, the valve between the heart and the lungs are not fully formed. This valve is called the pulmonic valve. The blood can't flow from the right lower chamber of the heart, called the right ventricle to the lungs. In PA-VSD, there is also a hole between the two pumping chambers of the heart.

Pulmonary atresia with ventricular septal defect is life threatening. A baby with pulmonary atresia finally, do not receive enough oxygen. One or more procedures or surgeries are needed to repair the heart.

Symptoms

The symptoms of pulmonary atresia with ventricular septal defect, also called PA-VSD, may appear at birth or shortly after. These may include:

  • Blue or gray skin. This change can be more easy or difficult to see depending on the color of the skin.
  • Rapid breathing or difficulty breathing.
  • Fatigue.
  • A bad diet.

When to see a doctor

Pulmonary Atresia with ventricular septal defect, also called PA-VSD, typically found during pregnancy or shortly after birth. If your baby has symptoms of this condition after the birth, contact a health professional immediately.

Causes

The cause of pulmonary atresia with ventricular septal defect, also called PA-VSD, is not clear. The majority of congenital heart defects occur during the first six weeks of pregnancy. The major blood vessels that run to and from the heart will also start to grow at this time. This is when a congenital heart defect, such as pulmonary atresia may occur.

In PA-VSD, pulmonary valve is not fully formed. There is also a hole in the heart called a ventricular septal defect. The hole allows blood to flow in and out of the right lower chamber of the heart. Some blood can also flow through a natural opening, called the ductus arteriosus. The ductus arteriosus usually closes shortly after birth. But medicines can keep it open.

In infants with pulmonary atresia, the lung of the arteries can be very small. Or may be missing. If the blood vessels are missing, the other ships of the way the body's main artery, called the aorta. These new blood vessels helps to send the blood to the lungs. Are the so-called major aortopulmonary collateral arteries, also called Mapcas.

Risk factors

It is not clear which increases the risk of pulmonary atresia with ventricular septal defect. Possible risk factors for congenital heart disease in general may include:

  • The habit of smoking. If you smoke, stop smoking. Smoking during pregnancy or exposure to cigarette smoke increases the risk of some congenital heart diseases.
  • The consumption of Alcohol. Drinking alcohol during pregnancy can increase the risk of heart disease in the baby.
  • Some medications. Some medications taken during pregnancy may increase the risk of congenital heart diseases. These include lithium (Lithobid) for bipolar disorder and isotretinoin (Claravis, Myorisan, others), which is used to treat acne. Talk with your health care team about all the medicines you take.
  • Genetics. Changes in certain genes can affect the way a baby's heart forms. For example, people with Down syndrome are often born with heart disease.
  • Diabetes. Having type 1 or type 2 diabetes during pregnancy can change the way a baby's heart forms. Diabetes that develops during pregnancy, it is called gestational diabetes. That usually does not increase a baby's risk of congenital heart disease.
  • Rubella, also called German measles. Having rubella during pregnancy can change the way a baby's heart forms. A blood test can be done before pregnancy to see if you are immune to rubella. If you're not, there is a vaccine available.

Diagnosis

Pulmonary Atresia with ventricular septal defect, also called PA-VSD, often is diagnosed during pregnancy or shortly after birth.

Tests

The tests that can be used to diagnose pulmonary atresia with ventricular septal defect include:

  • Pulse oximetry. For this simple test, a small sensor that clips in a hand or a foot. It checks the amount of oxygen in the blood.
  • X-ray of the chest. An x-ray of the chest shows the shape and size of the heart and lungs.
  • The echocardiogram. This is an ultrasound of the heart. The waves of sound, the images of the beating heart. An echocardiogram of the heart of a baby during pregnancy is called a fetal echocardiogram. You can diagnose pulmonary atresia.
  • An electrocardiogram, also called an ECG or EKG. This quick test shows how the heart is beating. Patches with sensors, called electrodes, stick in the chest and sometimes the arms or legs. The cables connect the patches to a computer, that prints or displays the results. An ECG can find an irregular heartbeat.
  • The cardiac catheterization. A doctor inserts a flexible tube called a catheter into a blood vessel, usually in the groin or wrist. Is guided into the heart. Contrast dye flows through the catheter into the arteries of the heart. The dye helps the arteries are shown more clearly in the images.
  • A ct scan of the heart, which is also called cardiac computed tomography. This test uses multiple X-rays to take images of the heart and blood vessels. It is a sample of the shape of the heart and lungs. A cardiac ct can help diagnose the major aortopulmonary collateral arteries, also called Mapcas. Knowing about the Mapcas is important for treatment planning.

Treatment

Infants with pulmonary atresia with ventricular septal defect, also called PA-VSD, needs immediate treatment. The treatment may include one or more surgeries or procedures.

Surgeries or other procedures

Infants with pulmonary atresia with PA-VSD, the needs of one or more surgeries or procedures to improve the flow of blood to the lungs. The treatment also helps the heart work better. The treatment depends on the structure of the pulmonary arteries, and if there are major aortopulmonary collateral arteries, also called Mapcas.

Surgeries or other procedures may include:

  • Catheter procedure. Thin, flexible tubes called catheters are used to examine the baby's heart and the blood supply to the lungs. Sometimes, a rigid tube called a stent is placed in the patent ductus arteriosus. This keeps the vessel open and allows blood to flow into the lungs.
  • Systemic to pulmonary artery shunt. This surgery may be necessary in the first days of life to send more blood to the lungs. The surgeon makes a connection, called a shunt, using a small tube. An example is the Blalock-Taussig shunt, also called the BT shunt.
  • Complete repair. This treatment can be done when a baby or after a bypass or stent is placed. During the complete repair, a surgeon closes the hole in the heart. The surgeon creates a pathway between the right lower chamber of the heart and the pulmonary artery.
  • Unifocalization. Sometimes, when most of the blood goes to the lungs is through Mapcas, the blood vessels need to be connected. This surgery is called unifocalization. The surgery may be done in stages. Can be done as part of a complete repair. Sometimes it is the first step towards an eventual complete repair.

Infants with pulmonary atresia with ventricular septal defect need regular check-ups by a doctor trained in heart disease present at birth. This type of doctor is called a congenital cardiologist.

Symptoms and treatment of Pulmonary atresia with ventricular septal defect