Pulmonary atresia-intact ventricular septum syndrome is a rare congenital heart defect in which the pulmonary valve is absent and the ventricular septum is intact. This means that the right ventricle is unable to pump blood to the lungs, and the oxygen-poor blood from the right atrium is instead directed to the left atrium and left ventricle. This results in a lack of oxygenated blood being circulated throughout the body. Treatment typically involves a series of surgeries to create a pathway for oxygenated blood to reach the lungs.

The symptoms of Pulmonary atresia-intact ventricular septum syndrome vary depending on the severity of the condition. Common symptoms include:

• Cyanosis (blue skin color)

• Rapid breathing

• Poor feeding

• Poor weight gain

• Sweating

• Poor exercise tolerance

• Heart murmur

• Abnormal heart sounds

• Abnormal chest X-ray

• Abnormal echocardiogram

• Abnormal electrocardiogram (ECG)

• Abnormal cardiac catheterization results

Pulmonary atresia-intact ventricular septum syndrome is a rare congenital heart defect that occurs when the pulmonary valve does not form properly and the ventricular septum (the wall between the two lower chambers of the heart) remains intact. The exact cause of this condition is unknown, but it is believed to be due to a combination of genetic and environmental factors. Possible causes include:

• Chromosomal abnormalities
• Maternal diabetes
• Maternal drug use
• Maternal infections
• Exposure to certain medications or toxins
• Abnormal development of the heart during fetal development

1. Surgery: The primary treatment for Pulmonary Atresia-Intact Ventricular Septum Syndrome is a series of surgeries. The first surgery is a Blalock-Taussig shunt, which is a procedure that creates a connection between the aorta and the pulmonary artery to increase blood flow to the lungs. The second surgery is a Glenn shunt, which is a procedure that creates a connection between the superior vena cava and the pulmonary artery to increase blood flow to the lungs. The third surgery is a Fontan procedure, which is a procedure that creates a connection between the inferior vena cava and the pulmonary artery to increase blood flow to the lungs.

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

1. Maternal diabetes
2. Maternal rubella infection
3. Maternal use of certain medications during pregnancy
4. Family history of congenital heart defects
5. Chromosomal abnormalities
6. Exposure to certain environmental toxins
7. Low birth weight
8. Premature birth

There is no cure for Pulmonary Atresia-Intact Ventricular Septum Syndrome. Treatment focuses on managing the symptoms and complications of the condition. Medications may be used to help manage the symptoms, such as diuretics to reduce fluid buildup in the lungs, and medications to help control the heart rate. Surgery may be necessary to improve blood flow to the lungs, and to repair or replace the heart valves.