Arterial switch operation:⁶

The surgeon may need to correct the positions of the aorta and pulmonary artery if their positions are reversed. The vessels are then disconnected and later reconnected so that the new pulmonary artery (the vessel that used to be connected to the left ventricle but was surgically removed and reconnected to the right ventricle) receives deoxygenated blood from the right ventricle and takes it to the lungs, and the new aorta (the vessel that used to be connected to the right ventricle but is now post-surgery connected to the left ventricle) receives blood from the left ventricle and pumps oxygenated blood around the rest of the body. Then the hole between the ventricles is closed.

Balloon Atrial Septostomy:⁹

In this procedure, a balloon (at the end of a catheter) is placed in the foramen ovale to widen the hole between the two upper heart chambers. This hole increases the amount of blood available to the lungs, but it does allow deoxygenated and oxygenated blood to mix.

Balloon Valvotomy:¹⁰

A procedure designed to widen a narrowed valve. This is performed by inserting a catheter into a blood vessel and guiding it to the heart. The catheter has a balloon at its tip, which is inflated to enlarge the valve opening. Once the valve is widened, the balloon is deflated and removed.

Biventricular repair:^7,8

This consists mainly of helping increase blood flow within the aorta and pulmonary artery in addition to an arterial switch operation and double-root translocation (modified Nikaidoh operation), whereby the arteries are re-connected to their appropriate ventricles if necessary.

Blalock-Taussig shunt:

Typically the first operation, this procedure creates a pathway for blood to reach the lungs. The surgeon creates a connection between the first artery that branches off the aorta (referred to as the right subclavian artery) and the right pulmonary artery (the artery that typically goes from the heart to the lungs). Some of the blood traveling through the aorta towards the body will “shunt” through this connection and flow into the pulmonary artery, allowing the blood heading to the rest of the body to receive oxygen. However, the child will still be left with some degree of cyanosis due to the mixing of oxygen-poor and oxygen-rich blood. In some cases, this surgery is not necessary.

Fontan procedure:^3-5

This operation is typically performed when the patient is 18 to 36 months old and solves the blood mixing problem. The Glenn shunt is left in place and a second connection is made to direct blood from the inferior vena cava (the large blood vessel that goes from the lower body to the heart) to the right pulmonary artery. This allows deoxygenated blood to flow passively from the lower body into the right pulmonary artery to head straight to the lungs to be oxygenated. This connection varies based on the patient's anatomy. After this procedure, the blood with and without oxygen should finally stay separated from each other and the right ventricle is only in charge of pumping oxygenated blood to the body.

Glenn Procedure:^3,4

This is usually the second operation and is performed when the patient is around 4 to 12 months old. This replaces the previous Blalock-Taussig shunt with another connection between the superior vena cava (the large blood vessel that goes from the top of the body to the heart) to the right pulmonary artery. Then blood from the head and arms can passively flow into the pulmonary artery and proceed to the lungs to become oxygenated, without entering the right atria and ventricle. There will still be oxygen-poor and oxygen-rich blood mixing. This procedure works to reduce the workload on the single working ventricle and more efficiently pump oxygenated blood to the rest of the body.

Intraventricular Tunnel Repair:⁶

The surgeon constructs a tunnel within the heart to link the left ventricle to the aorta and places a patch to guide blood flow from the left ventricle to the aorta. This allows for oxygenated blood to be delivered to the systemic circulation without being mixed with deoxygenated blood in the right ventricle.
*Listen from 24:45 to 26 minutes

Maze procedure (EA):^1,2

If arrhythmias are severe, the surgeon may perform a maze procedure or ablation, both using scar tissue to block the heart signals that cause the irregular heartbeats. The surgeon does this by making small incisions in the upper chambers of the heart to create an intended rhythm of scar tissue. Because scar tissue does not conduct electricity, it blocks the arrhythmias. Heat or cold energy may be used to create scar tissue as well.

Norwood Procedure:

The Norwood procedure aims to create an unobstructed blood flow from the right ventricle to the systemic circulation (the blood going to the rest of the body). Because the left ventricle is underdeveloped and cannot deliver oxygen to the rest of the body, the surgeon will reconstruct the heart so that the right ventricle is pumping blood to the body instead. This involves creating a new “aorta” that goes from the right ventricle to the body, instead of from the left ventricle. Since the blood from the right ventricle is now being delivered to the rest of the body, a new method is needed by the body to get oxygen to the lungs to be re-oxygenated. This can be done via either the Blalock-Taussig shunt or the Sano shunt. At this time, the patent ductus arteriosus (the connection between the aorta and the pulmonary artery) is closed. This was kept open during the first few weeks of life before the surgeries in order to provide some oxygenated blood to the body. However, now that the right ventricle is delivering oxygenated blood to the body through a new, larger aorta, there is no need to shunt blood between this aorta and the pulmonary artery (especially as the B-T or Sano shunt is getting oxygenated blood to the right ventricle now).

Prostaglandin E medication:^{1, 2}

Prostaglandin E1 is given to keep the ductus arteriosus open (the ductus arteriosus is a short blood vessel that connects the fetal pulmonary artery to the aorta). Keeping this vessel open, which typically closes after birth, allows blood to flow from the pulmonary artery to the aorta and presents an alternate route for blood to receive oxygen.

Radiofrequency catheter ablation (EA):^1,2

This procedure treats arrhythmias. The doctor inserts one or more thin, flexible tubes called catheters into a blood vessel, usually in the groin. The doctor guides them to the heart. There are sensors on the topics of the catheters that use radiofrequency energy (heat) to damage small areas of the heart tissue. This will create scarring and therefore block the heart signals that cause arrhythmias.

Right Ventricular Outflow Reconstruction:^9,11

This operation is performed soon after birth to repair and/or reconstruct the pulmonary valve (RCH). The procedure involves improving blood flow from the right ventricle to the lungs, either by widening the existing pathway or by surgically placing a tube with a valve to create a new, functional connection to the pulmonary artery (a valved right ventricle to pulmonary artery conduit). A Blalock-Taussig (BT) shunt can also be used in this procedure to increase blood flow to the lungs

*Listen from 2:25 to 2:58

Stenting:⁹

A stent may also be put in place. The stent will create a connection from the aorta to the pulmonary artery by keeping the ductus arteriosus open. They keep the ductus arteriosus open by inserting a rigid tube in the natural connection between the aorta and pulmonary artery. By saving this opening, which usually closes after birth, blood can still travel to the lungs and become oxygenated.

Tricuspid valve repair (EA):^1,2

The surgeon will patch holes or tears in the valve flaps and remove extra tissue blocking the valve opening. This is an open-heart operation intended to fix any damaged tricuspid tissue. Another valve repair, known as the cone procedure, separates the heart muscle from the tissue that should form the tricuspid valve. With that tissue, the surgeon will create a working tricuspid valve. Sometimes, the valve might need to be repaired again or replaced in the future.

Tricuspid valve replacement (EA):^12,13

If the valve can't be repaired, valve replacement may be necessary. In the operation, the surgeon will remove the damaged valve and replace it with a biologic valve (made of human tissue) or one from a cow or pig. The surgeon will likely close any ASDs as well.