Pulmonary atresia with ventricular septal defect (PA/VSD) is a congenital heart defect in which the pulmonary valve is absent and there is a hole in the wall (septum) between the two lower chambers of the heart (ventricles). This results in oxygen-poor (blue) blood from the right ventricle being pumped out to the body instead of to the lungs. The oxygen-rich (red) blood from the left ventricle is unable to reach the lungs, so the baby is not able to get enough oxygen.

The symptoms of Pulmonary atresia with ventricular septal defect can vary depending on the severity of the defect. Common symptoms include:

- Cyanosis (blue discoloration of the skin due to lack of oxygen)
- Rapid breathing
- Poor feeding
- Poor weight gain
- Sweating
- Heart murmur
- Difficulty breathing
- Fatigue
- Poor exercise tolerance
- Swelling of the legs, feet, and abdomen

Pulmonary atresia with ventricular septal defect (PA/VSD) is a congenital heart defect that occurs when the pulmonary valve does not form properly and there is a hole in the wall (septum) between the two lower chambers of the heart (ventricles). The exact cause of PA/VSD is unknown, but it is believed to be due to a combination of genetic and environmental factors. Possible causes include:

• Genetic mutations: Certain genetic mutations can cause the pulmonary valve to not form properly, leading to PA/VSD.

• Environmental factors: Exposure to certain environmental toxins, such as alcohol, drugs, and certain medications, during pregnancy can increase the risk of PA/VSD.

• Chromosomal abnormalities: Certain chromosomal abnormalities, such as Down syndrome, can increase the risk

1. Surgery: The primary treatment for pulmonary atresia with ventricular septal defect is surgery. The goal of the surgery is to create a pathway for blood to flow from the right ventricle to the pulmonary artery. This is usually done by connecting the right ventricle to the pulmonary artery with a conduit.

2. Medications: Medications may be prescribed to help manage symptoms and reduce the risk of complications. These may include diuretics to reduce fluid buildup in the lungs, and medications to reduce the risk of infection.

3. Catheterization: Catheterization may be used to open up the pulmonary artery and allow blood to flow to the lungs. This procedure is usually done in combination with surgery.

4. Transplant: In some cases, a heart transplant may be necessary to treat pulmonary atresia

1. Maternal diabetes
2. Maternal rubella infection
3. Maternal use of certain medications during pregnancy
4. Genetic factors
5. Low birth weight
6. Premature birth
7. Congenital heart defects in other family members

There is no cure for pulmonary atresia with ventricular septal defect (PA/VSD). Treatment typically involves medications to help manage symptoms and surgery to repair the defect. Medications may include diuretics to reduce fluid buildup in the lungs, beta-blockers to reduce the workload on the heart, and ACE inhibitors to reduce blood pressure. Surgery may involve patching the hole in the septum, or implanting a device to help the heart pump more efficiently.