Paediatric Cardiology

Atrial Septal Defect

An atrial septal defect (ASD) is an opening or hole in the wall that separates the two upper chambers of the heart. This wall is called the atrial septum. The hole causes oxygen-rich blood to leak from the left side of the heart to the right side. This causes extra work for the right side of the heart, since more blood than necessary is flowing through the right ventricle to the lungs. If the ASD is small enough, it can be closed with a special device. The procedure is done in the heart catheterization lab.

What happens during the closure procedure?

The procedure is performed while your child is under a general anaesthetic. This means that your child will be asleep during the procedure. Not every ASD can be closed with heart catheterization. Therefore, we first need to measure the ASD to make sure it can be closed with a device in the catheterization lab.

When your child is asleep, we will do a test called a transesophageal echocardiogram. “Echocardiogram” ,”Transesophageal” means we do the ultrasound with a small probe that is placed in your child’s esophagus, the tube that connects the mouth to the stomach. This test will measure the size of the hole and help place the closure device.

If the test shows that the hole is too big to close with the device, we will wake up your child and send him or her to the recovery room. Your child’s cardiologist will discuss the next steps with you and your child.
If the hole is small enough and in the right position, we will go on with the catheterization.

During the catheterization, the doctor puts a catheter with a small deflated balloon on the tip through the blood vessel to the hole. The balloon is inflated to measure the size of the hole again. If the hole can be closed with the device, the doctor puts the closure device inside the catheter and places the device into the hole. Once the device is in place, the doctor takes out the catheter and covers the cut on your child’s leg with a bandage.

Coarctation of the aorta repair

Coarctation of the aorta is a narrowing of the aorta, the main blood vessel carrying oxygen-rich blood from the left ventricle of the heart to all of the organs of the body.Coarctation occurs most commonly in a short segment of the aorta just beyond where the arteries to the head and arms take off, as the aorta arches inferiorly toward the chest and abdomen. This portion of the aorta is called the “juxta ductal” aorta, or the part near where the ductus arteriosus attaches. It is also called the aortic isthmus.

The ductus arteriosus is a blood vessel that is normally present in a fetus and has special tissue in its wall that causes it to close in the first hours or days of life. Coarctation may be caused by the presence of extra ductal tissue extending into the adjacent aorta which results in aortic narrowing as the ductal tissue contracts.

In babies with coarctation, the aortic arch may also be small (hypoplastic). Coarctation may also occur with other cardiac defects, typically involving the left side of the heart. The defects most commonly seen with coarctation are bicuspid aortic valve and ventricular septal defect. Coarctation may also be seen as a part of more complex single ventricle heart defects. Coarctation of the aorta is common in some patients with chromosomal abnormalities, such as Turner’s syndrome.

In the presence of a coarctation, the left ventricle has to work harder, since it must generate a higher pressure than normal to force blood through the narrow segment of aorta to the lower part of the body. If the narrowing is very severe, the ventricle may not be strong enough to perform this extra work, resulting in congestive heart failure or inadequate blood flow to the organs of the body.

Patent Ductus Arteriosus (PDA) & Repair/ Closure

PDA is a heart problem that is frequently noted in the first few weeks or months after birth. It is characterized by the persistence of a normal fetal connection between the aorta and the pulmonary artery which allows oxygen-rich (red) blood that should go to the body to recirculate through the lungs. All babies are born with this connection between the aorta and the pulmonary artery. While your baby was developing in the uterus, it was not necessary for blood to circulate through the lungs because oxygen was provided through the placenta. During pregnancy, a connection was necessary to allow oxygen-rich (red) blood to bypass your baby’s lungs and proceed into the body. This normal connection that all babies have is called a ductus arteriosus.

At birth, the placenta is removed when the umbilical cord is cut. Your baby’s lungs must now provide oxygen to his or her body. As your baby takes the first breath, the blood vessels in the lungs open up, and blood begins to flow through them to pick up oxygen. At this point, the ductus arteriosus is not needed to bypass the lungs. Under normal circumstances, within the first few days after birth, the ductus arteriosus closes and blood no longer passes through it.

In some babies, however, the ductus arteriosus remains open (patent) and the condition now becomes known as patent ductus arteriosus (PDA). The opening between the aorta and the pulmonary artery allows oxygen-rich (red) blood to recirculate into the lungs. Patent ductus arteriosus occurs twice as often in girls as in boys.

PDA Repair or Closure

The majority of children and some infants with PDA are candidates for repair in the cardiac cath lab. The goal is to repair the PDA before the lungs become diseased from too much blood flow and pressure and to restore an efficient pattern of blood flow. Surgical repair is also indicated if one of the previously mentioned conservative treatments have not been successful.

Repair is usually indicated in infants younger than 6 months of age who have large defects that are causing symptoms, such as poor weight gain and rapid breathing. For infants who do not exhibit symptoms, the repair may often be delayed until after 6 to 12 months of age. Your child’s cardiologist will recommend when the repair should be performed. Transcatheter coil closure of the PDA is frequently performed first if possible because it is minimally invasive. Children need to be at least 5 kg to be considered for transcatheter closure. Thus, premature infants, because of their small size, are not candidates for this procedure, and require surgical closure of the PDA. Your child’s PDA may be repaired surgically in the operating room.

The surgical repair, also called PDA ligation, is performed under general anaesthesia. The procedure involves closing the open PDA with stitches or clips in order to prevent the surplus blood from entering your child’s lungs.

Tetralogy of Fallot (TOF)

Tetralogy of Fallot (TOF), a conotruncal abnormality, is a constellation of four findings: an aorta that overrides the right ventricular outflow tract; right ventricular outflow obstruction; a large subaortic VSD; and hypertrophy of the right ventricle . The frequent coexistence of an ASD can make for a pentalogy. Occasionally, patients with unrepaired TOF only present in adulthood because of a remarkable balance between the pulmonic obstruction and the VSD, which limits cyanosis.

Early palliation with a systemic-to-arterial shunt (e.g., Blalock-Taussig), which connects the subclavian and pulmonary arteries , facilitates growth of the pulmonary arteries and is a precursor to definitive surgical repair in the young child. Definitive repair often involves complete removal of the pulmonic valve and therefore results in wide open pulmonic regurgitation. Although the repair is tolerated for several years, the right ventricle eventually succumbs to volume overload and progressively increases in size.

Ventricular Septal Defect (VSD) & Closure/ Repair

A ventricular septal defect (VSD) is an opening or hole in the wall that separates the two lower chambers of the heart. This wall is called the ventricular septum. The hole causes oxygen-rich blood to leak from the left side of the heart to the right side. This causes extra work for the right side of the heart, since more blood than necessary is flowing through the right ventricle to the lungs. The hole is usually closed with surgery. However, in certain situations, your child’s cardiologist and surgeon may think it is best to close the hole with a special device. This procedure is done in the heart catheterization lab.

What happens during the closure procedure?

The procedure is performed while your child is under a general anaesthetic. This means that your child will be asleep during the procedure. Not every VSD can be closed with heart catheterization. Therefore, we first need to measure the VSD to make sure it can be closed with a device in the catheterization lab. When your child is asleep, we will do a test called a transesophageal echocardiogram. “Echocardiogram” means a heart ultrasound. “Transesophageal” means we do the ultrasound with a small probe that is placed in your child’s esophagus, the tube that connects the mouth to the stomach. This test will measure the size of the hole and help place the closure device.

If the test shows that the hole is too big to close with the device, we will wake up your child and send him or her to the recovery room. Your child’s cardiologist will discuss the next steps with you and your child.
If the hole is small enough and in the right position, we will go on with the catheterization.

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