Name of the Procedure:

Repair of Transposition of the Great Arteries with Ventricular Septal Defect and Subpulmonary Stenosis (Without Surgical Enlargement of Ventricular Septal Defect)

Summary

This surgical procedure aims to correct a congenital heart defect where the major arteries are switched. It involves repairing the swapped arteries, associated hole in the heart, and narrowing below the pulmonary valve, and does so without enlarging the hole.

Purpose

The procedure addresses congenital heart defects known as transposition of the great arteries (TGA) with a ventricular septal defect (VSD) and subpulmonary stenosis. The goal is to restore normal blood flow and improve oxygen delivery throughout the body, ultimately ensuring that the heart functions properly.

Indications

• Cyanotic symptoms (bluish discoloration of the skin due to low oxygen)
• Difficulty breathing
• Poor growth and development in infants
• Diagnosis of TGA with VSD and subpulmonary stenosis confirmed by echocardiography or cardiac MRI

Preparation

• Fasting for a specific period before surgery
• Reviewing current medications and adjusting them as necessary
• Diagnostic tests including echocardiogram, chest X-ray, and possibly cardiac catheterization
• Preoperative blood tests and assessment by an anesthesiologist

Procedure Description

1. The patient is placed under general anesthesia.
2. An incision is made in the chest to access the heart.
3. The heart is temporarily stopped and connected to a heart-lung machine.
4. The arteries (aorta and pulmonary artery) are re-positioned (arterial switch).
5. The ventricular septal defect is repaired by closing the hole with a patch.
6. Any narrowing below the pulmonary valve (subpulmonary stenosis) is relieved without needing to enlarge the VSD.
7. The heart is then restarted, and the chest incision is closed.

Duration

The procedure typically takes between 4 to 6 hours, depending on the complexity.

Setting

This surgery is performed in a hospital, specifically in an operating room equipped for pediatric cardiac surgeries.

Personnel

• Pediatric cardiac surgeon
• Anesthesiologist
• Surgical nurses
• Perfusionist (operates the heart-lung machine)
• Pediatric cardiologist (may assist intraoperatively with echocardiography)

Risks and Complications

• Common risks: bleeding, infection, arrhythmias
• Rare risks: stroke, damage to heart or surrounding structures, need for additional surgeries
• Complications: heart block, residual VSD, issues with coronary arteries

Benefits

• Improved oxygenation and blood flow
• Enhanced growth and development
• Normalized heart function
• Majority of patients can expect improved quality of life shortly after recovery

Recovery

• Initial ICU stay for monitoring (typically 1-2 weeks)
• Gradual activity resumption over several weeks
• Follow-up visits with the pediatric cardiologist
• Medications to support heart function and prevent clot formation

Alternatives

• Medical management to stabilize symptoms
• Cardiac catheterization techniques for less invasive interventions
• Palliative care approaches if surgery is not an option
• Each alternative has its pros and cons, including less definitive correction of the defect or limited effectiveness over the long term compared to surgery.

Patient Experience

• During: The patient will be under general anesthesia and will not feel pain during the procedure.
• After: Discomfort at the incision site, managed with pain relief medications.
• Recovery involves a stay in the ICU followed by gradual return to normal activities, guided by healthcare providers.