Name of the Procedure:

Repair of Pulmonary Atresia with Ventricular Septal Defect (PA/VSD)

Summary

This procedure involves the surgical repair of a congenital heart defect known as pulmonary atresia with ventricular septal defect. Surgeons construct or replace a conduit that channels blood from the right or left ventricle to the pulmonary artery to ensure proper blood flow to the lungs.

Purpose

Pulmonary atresia with ventricular septal defect is a serious congenital heart disorder that prevents blood from properly flowing from the heart to the lungs. The goals of the procedure are to correct the heart’s structural defects, restore normal blood flow, and improve oxygen delivery throughout the body.

Indications

• Severe cyanosis (bluish skin due to lack of oxygen)
• Difficulty breathing or shortness of breath
• Failure to thrive or poor growth in infants
• Diagnosed congenital heart defect with compromised blood flow to the lungs
• Echocardiographic or cardiac catheterization findings indicating PA/VSD

Preparation

• Fasting for at least 8 hours prior to surgery
• Adjustments to medications (e.g., blood thinners)
• Preoperative imaging tests such as echocardiograms or MRIs
• Blood tests to assess overall health and readiness for surgery

Procedure Description

1. Anesthesia: General anesthesia is administered to ensure the patient is completely unconscious and pain-free during the procedure.
2. Incision: A midline incision is made along the sternum to access the heart.
3. Ventricular Septal Defect Repair: The ventricular septal defect (hole between the ventricles) is closed using a patch.
4. Conduit Placement: A conduit (synthetic or homograft) is constructed or replaced from the right or left ventricle to the pulmonary artery to facilitate proper blood flow.
5. Connection: The conduit is carefully connected to the pulmonary artery.
6. Closure: The heart is meticulously closed, and the sternum is wired back together.

Duration

The procedure typically takes between 4 to 6 hours.

Setting

This surgery is performed in a hospital's cardiac surgical unit.

Personnel

• Cardiothoracic surgeon
• Surgical nurses
• Anesthesiologist
• Perfusionist (for heart-lung machine operation)
• Pediatric cardiologist (if the patient is a child)

Risks and Complications

• Bleeding
• Infection
• Arrhythmias (irregular heartbeats)
• Blood clots
• Conduit obstruction or failure
• Long-term complications requiring additional surgeries

Benefits

• Improved oxygenation and overall cardiovascular function
• Relief from symptoms such as cyanosis and breathlessness
• Enhanced quality of life and normal growth in children

Recovery

• Intensive care unit (ICU) monitoring for the first few days
• Hospital stay of about 1 to 2 weeks
• Regular follow-up appointments with the cardiologist
• Activity restrictions for several weeks
• Gradual return to normal activities as directed by the healthcare team

Alternatives

• Palliative procedures (e.g., shunt operations) to improve blood flow temporarily
• Heart transplant in cases where repair is not feasible
• Pharmacological treatments to manage symptoms (though not corrective)

Patient Experience

• The patient will be under general anesthesia during the procedure, feeling no pain.
• Postoperative discomfort can be managed with medications.
• Some fatigue and soreness around the incision site are normal.
• Counseling and support are available for emotional and psychological adjustment following surgery.