Pulmonary Atresia with Intact Ventricular Septum

Pulmonary atresia with intact ventricular septum (PAIVS) is a rare congenital heart defect characterized by the complete blockage of the pulmonary valve, which connects the right ventricle to the pulmonary artery. This condition prevents blood from flowing from the right ventricle into the lungs for oxygenation.

Causes and Risk Factors

The exact cause of PAIVS is not fully understood, but it is believed to result from abnormal development of the heart during fetal growth. Certain genetic conditions, such as rubella infection during pregnancy, may increase the risk of congenital heart defects like PAIVS.

Symptoms

Infants with PAIVS often exhibit symptoms shortly after birth, including:

• Cyanosis (blue discoloration of the skin and mucous membranes)
• Shortness of breath or difficulty breathing
• Rapid heart rate
• Poor feeding and failure to gain weight
• Fatigue

Diagnosis

PAIVS is typically diagnosed using a combination of the following tests:

• Chest X-ray: To evaluate the size and shape of the heart and lungs
• Echocardiogram: An ultrasound test that uses sound waves to create images of the heart and its blood vessels
• Electrocardiogram (ECG or EKG): To assess the electrical activity of the heart
• Cardiac catheterization: A procedure that involves inserting a thin, flexible tube into an artery or vein to inject contrast dye and visualize the heart's blood vessels

Treatment

The primary goal of treatment for PAIVS is to restore blood flow to the lungs and improve oxygenation. Treatment options may include:

• Medications: To manage symptoms, such as prostaglandin E1 to keep the ductus arteriosus open and allow blood to bypass the blocked pulmonary valve
• Surgical procedures:
  • Blalock-Taussig shunt: A procedure that creates a connection between the subclavian artery and the pulmonary artery to increase blood flow to the lungs
  • Pulmonary valvotomy: A surgical procedure to open the blocked pulmonary valve
  • Right ventricular outflow tract reconstruction: Surgery to repair or replace the right ventricular outflow tract, including the pulmonary valve and artery
• Heart transplantation: In severe cases where other treatments are not effective, a heart transplant may be necessary

Prognosis

The prognosis for infants with PAIVS varies depending on the severity of the condition and the effectiveness of treatment. With prompt medical attention and surgical intervention, many children with PAIVS can lead active and normal lives.

Frequently Asked Questions (FAQs)

What is Pulmonary Atresia with Intact Ventricular Septum (PAIVS)?
A rare congenital heart defect where the pulmonary valve is blocked and the ventricular septum is intact.

What are the symptoms of PAIVS?
Cyanosis, shortness of breath, fatigue, and poor feeding in infants.

How is PAIVS diagnosed?
Echocardiogram, cardiac catheterization, and MRI or CT scans.

What is the treatment for PAIVS?
Surgery, balloon valvuloplasty, or transcatheter intervention to open the pulmonary valve.

Is PAIVS life-threatening?
Yes, if left untreated, it can lead to severe complications and death.

Can PAIVS be detected during pregnancy?
Yes, through fetal echocardiography.

What are the risks of PAIVS surgery?
Bleeding, infection, and cardiac complications.

Can children with PAIVS lead normal lives?
With successful treatment, many can lead active and normal lives.

Is PAIVS inherited?
Usually not, but can be associated with genetic syndromes.

What is the prognosis for PAIVS?
Variable, depending on severity and effectiveness of treatment.