(A-Trio-Ven-Trick-U-Lar Sep-Tul Defects)
What is AV Canal defects
What are the signs & symptoms
How is it treated
What can a parent do
WHAT IS AV CANAL (See picture on page 2)
1. Atrioventricular Septal Defects are also known as Endocardial (N-doe-car-D-ul)
Cushion Defects.
a. The endocardial cushions are cells that produce the heart’s valves and septum
b. This is critical for the normal formation of the four (4) chambers of the heart
c. A problem in the development of the endocardial cushion cells may be associated
with atrioventricular septal defects.
2. It is a failure in the development of the middle part of the heart. The area that
divides the heart in half from top to bottom and divides it in half from side to side.
3. The structures in this area are the:
a. Lower part of the atrial septum—this is the wall that divides the 2 upper
chambers of the heart (the atria) into the right and left side. There is an
opening (hole) between the 2 atria
b. Upper part of the ventricular septum—this is the wall that divides the 2 lower
chambers of the heart (the ventricles) into the right and left side. There is an
opening (hole) between the 2 ventricles
c. The septum that separates the 2 valves (tricuspid & mitral) between the atria
and ventricles. The tricuspid valve is on the right side of the heart and the
mitral valve is on the left side of the heart. These valves allow blood to empty
from the top part of the heart into the bottom part of the heart.
4. These defects cause a common opening between all four (4) chambers of the heart;
it looks like a cross is missing from the middle of the heart. This common opening
allows mixing of oxygenated and non-oxygenated blood.
5. AV Septal defects are arranged in 1 or 2 types:
a. Complete—all of the defects noted above
b. Partial or Incomplete—part of the lower septum (wall) has formed and the 2
valves (tricuspid & mitral) are separated
c. Some infants with a partial (incomplete) or transitional type of AV Septal defect
may not be diagnosed until they are several months or even years old.
AV Septal Defect-1
AV Canal Defects
Mitral Valve
Tricuspid Valve
6. A Pediatric Cardiologist—a specialist in heart problems of babies and children—cares
for babies with AV Canal defects.
1. Murmur—This is the sound made as blood in the heart flows from the left ventricle
into the right ventricle and out of the pulmonary artery. It is heard when the
doctor, nurse practitioner and/or the nurse listens to your baby’s chest.
2. Cyanosis—This is the bluish coloring to the baby’s lips and skin.
3. Rapid/fast breathing (Tachypnea).
4. Work harder to breathe—have retractions.
5. Rapid/fast heart rate (Tachycardia).
6. Tires easily—even with everyday baby care, like feeding, handling, changing the
7. Poor weight gain and growth.
1. If the neonatologist notices any of the symptoms listed above and thinks the baby
has AV Septal Defects an echocardiogram may be ordered.
2. An echocardiogram is a painless ultrasound of the heart. It uses high frequency
sound waves to create a picture of the inside of your baby’s heart.
a. It also measures the blood flow through his heart
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b. The test will not hurt your baby
c. It is done at your baby’s bedside.
3. Infants with symptoms may be put on medicine. Some of the medicines we commonly
use are:
a. Lasix: a “diuretic” (“water medicine”), which means it helps the body get rid of
extra water. It does this by increasing the amount of urine your baby makes
b. Captopril: decreases shunting (unusual movement) of blood through the defects
in the heart [such as ventricular septal defect (VSD) or atrial septal defect
(ASD)] and treats high blood pressure
c. Digoxin: helps the heart pump stronger in a slower and more regular way and
treats children with heart failure.
4. While some infants may improve with the medicine, all infants with AV Septal
Defects will need corrective heart surgery.
a. Often, the infant can go home for a period of time before the surgery needs to
be done.
5. When your baby needs surgery, he will be transferred to either Duke University
Medical Center or The University Hospital at Chapel Hill for the surgery, if he/she
remains in the NICU.
a. You will be asked to sign a consent giving Cape Fear Valley Medical Center
permission to transfer your baby.
b. The doctors there will also talk with you about your baby’s surgery.
c. Sometimes surgery can be delayed until your baby is older.
6. If your baby is at home already, the Pediatric Cardiologist caring for your baby will
make arrangements for you and your baby to meet with the surgeon that will be
doing your baby’s heart surgery. He will make the arrangements for your baby to be
admitted to the hospital.
7. The surgery is done in the Operating Room and the baby is asleep for the surgery.
a. You will be asked to sign a consent (give permission) for the surgery.
8. Once your baby’s condition is stable and the doctors are happy with the baby’s
progress, the baby will be:
a. Returned to Cape Fear Valley Medical Center’s Neonatal Intensive Care Unit if
we sent the baby for the surgery
b. Discharged home if the baby was at home before his/her surgery.
9. Both the pediatric cardiologist and our neonatologist will talk with you about your
baby and what they recommend.
10. Please feel free to ask questions at any time.
1. An important part of the baby getting better is the parents’ love and concern.
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2. We encourage you to talk and sing to your baby, gently touch or rub them and hold
them close if tolerated by the baby. Talk with your baby’s nurse about this.
3. Your love and attention will go a long way in helping your little one on the road to
4. Once your baby returns to Cape Fear Valley Medical Center, keep up with your baby’s
progress each time you visit or call.
Reviewed/Revised: 01/06…..08/11
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Cardiac: Atrioventricular Septal Defects