Fontan Procedure:

Any surgical operation that results in systemic flow of venous blood to the lungs without passing through a ventricle is known as the Fontan procedure. Fontan and Baudet presented a surgical method for tricuspid atresia correction in 1971, based on experimental and clinical data dating back to the 1940s.

The surgeon disconnects the inferior vena cava (IVC) from the heart and links it to the pulmonary artery via a conduit during the Fontan operation (tube). Makes a tiny hole in the conduit between the right atrium and the conduit. Some blood can still flow back to the heart through this hole (or fenestration).

Disease Overview:

Hypoplastic left heart syndrome (HLHS)

Hypoplastic left heart syndrome is a complicated and uncommon congenital cardiac condition (congenital). The left half of the heart is severely underdeveloped in this disease. The left side of your baby's heart can't adequately pump blood to the body if he or she is born with hypoplastic left heart syndrome.

Disease Signs and Symptoms:

Hypoplastic left heart syndrome causes babies to become very unwell soon after birth. The following are some of the signs and symptoms:

• Skin colour is grayish-blue (cyanosis)
• Breathing is difficult and rapid.
• Feeding problems
• Hands and feet are freezing.
• Pulse is weak.
• Being tired or inactive over an extended period of time
• Babies with hypoplastic left heart syndrome can fall into shock and die if the natural connections between the left and right sides of the heart (foramen ovale and ductus arteriosus) are allowed to shut in the first few days of life.
• Shock manifests itself in the following ways:
• Skin that is cool and clammy and might be pale or grey in colour.
• A fast and feeble pulse
• Breathing that is abnormally sluggish and shallow or very quick.
• Eyes that don't seem to be looking at you

Disease Causes:

When a baby's heart is still growing in the pregnancy, he or she has hypoplastic left heart syndrome. The reason behind this is unknown. If your family already has one kid with hypoplastic left heart syndrome, the chances of having another with the same issue are higher.

There are four chambers in a typical heart, two on the right and two on the left. When it comes to its most fundamental function, which is pumping blood throughout the body, the heart excels. The left and right sides of the heart perform separate functions.

The right side is in charge of transporting blood to the lungs. Oxygen enriches the blood in the lungs, which then flows to the left side of the heart. The left side of the heart pumps blood into the aorta, a major conduit that transports oxygen-rich blood throughout the body.

Disease Diagnosis:

Prior to birth

Even while still in the womb, a kid might be diagnosed with hypoplastic left heart syndrome. During the second trimester of pregnancy, your doctor may be able to detect the issue with a normal ultrasound exam.

Following the birth

If your infant exhibits grayish-blue skin or has problems breathing after birth, a doctor may suspect a cardiac issue, such as hypoplastic left heart syndrome. If your baby's doctor detects a heart murmur — an irregular sound generated by turbulent blood flow — when listening to the heart using a stethoscope, he or she may suspect a heart problem.

An echocardiography is commonly used to identify hypoplastic left heart syndrome. This test produces moving graphics on a video screen by using sound waves that reflect off your baby's heart.

The echocardiography will indicate a smaller than usual left ventricle and aorta if your kid has hypoplastic left heart syndrome. An abnormal cardiac valve may also be visible on an echocardiography.

This test can reveal blood flowing from the right ventricle into the aorta because it can track blood flow. An echocardiography can also detect other cardiac problems, such as an atrial septal defect.

Disease Treatment:

Several surgical techniques or a heart transplant are used to treat hypoplastic left heart syndrome. Your child's doctor will talk to you about treatment choices.

Doctors normally advocate delivery in a hospital with a heart surgery centre if the diagnosis is made before the baby is delivered.

Before surgery or transplant, your baby's doctor may suggest one or more of the following choices to assist manage your baby's condition:

Medication. The medicine helps to keep the ductus arteriosus open by widening the blood arteries.

Assist with breathing. If your infant is having problems breathing, a breathing machine (ventilator) may be needed to assist him or her acquire enough oxygen.

Intravenous fluids are a type of intravenous fluid that is injected into the Fluids will be given to your infant through a tube put into a vein.

Tube for feeding. A feeding tube might be used to feed your infant if he or she has difficulty feeding or tired while eating.

Atrial septostomy is a surgical procedure that removes the septum from the heart. If the foramen ovale shuts or is too small, this operation establishes or enlarges the opening between the heart's upper chambers (atria) to enable increased blood flow from the right atrium to the left atrium. An atrial septostomy may not be necessary if your baby already has an atrial septal defect.

Surgical treatments and other medical procedures

Hypoplastic left heart syndrome will very certainly necessitate many surgical operations for your youngster. These operations are performed by surgeons to construct distinct channels for oxygen-rich blood to reach the body and oxygen-poor blood to reach the lungs. There are three steps to the operation.

Norwood procedure is a method that is used to solve problems. Typically, this operation is performed during the first two weeks of your child's existence. This operation can be carried out in a variety of ways.

The aorta is reconstructed and connected to the heart's lower right chamber by surgeons (right ventricle). Surgeons install a tube (shunt) between the aorta and the arteries going to the lungs (pulmonary arteries), or a shunt between the right ventricle and the pulmonary arteries. The right ventricle may now pump blood to both the lungs and the rest of the body using this manner.

Glenn procedure in both directions. In most cases, this is the second operation. It is done when your child is between the ages of 3 and 6 months. Doctors disconnect the initial shunt from the pulmonary arteries and connect the pulmonary artery to one of the big veins that typically returns blood to the heart (the superior vena cava).

Fontan surgery is a treatment that involves removing a portion of This operation is often performed when your child is between the ages of 18 months and four years old. The surgeon builds a pathway for oxygen-poor blood to go directly into the pulmonary arteries from one of the blood channels that returns blood to the heart (the inferior vena cava). The blood is subsequently transported into the lungs through the pulmonary arteries.

A heart transplant is a procedure in which a person' A heart transplant is another surgical possibility. However, because the number of hearts available for transplantation is limited, this method is not employed as frequently. Your child will require drugs for the rest of his or her life to prevent the donor heart from being rejected.