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Alternative Names Return to topAtrioventricular (AV) canal defect; Atrioventricular septal defect; AVSD
Definition Return to top
Endocardial cushion defect (ECD) is an abnormal heart condition in which there is no separation between the chambers of the heart. Essentially, the middle part of the heart is missing. It is a congenital heart disease, which means it is present from birth.
Causes Return to top
Endocardial cushion defect occurs while a baby is still growing in the womb. The endocardial cushions are two areas of thickening that eventually develop into the wall (septum) that separates the four chambers of the heart. They also form the mitral and tricuspid valves.
The lack of separation between the two sides of the heart causes several problems:
There are two types of ECD:
ECD is strongly associated with Down syndrome. Several gene changes are also connected to ECD. However, the exact cause of ECD is unknown.
ECD may be associated with other congenital heart defects such as:
Symptoms Return to top
Symptoms of ECD may include:
Exams and Tests Return to top
Signs of ECD may include:
Children with partial ECD, who have only a small VSD and normal valves, may not have signs or symptoms of the disorder during childhood.
Tests to diagnose ECD include:
Treatment Return to top
Surgery is needed to close the holes between the heart chambers, and build new tricuspid and mitral valves. The timing of the surgery depends on your child's condition and the severity of the ECD, but it can usually be done when the baby is about 3 months old. Correcting an ECD may require more than one surgery.
Your doctor may prescribe medication before surgery if the ECD has made your baby very sick. The medicines will help the child gain weight and strength before surgery. Medications may include:
Surgery for a complete ECD should be done as early in the baby's first year of life as possible, before irreversible lung damage occurs. Babies with Down syndrome tend to develop lung disease earlier, and therefore early surgery is very important for these babies.
See also: Congenital heart defect corrective surgery
Outlook (Prognosis) Return to topHow well your baby does depends on the severity of the ECD, the child's overall health, and whether lung disease has already developed. Many children live normal, active lives after the ECD is corrected.
Possible Complications Return to top
Complications from ECD may include:
Certain complications of ECD surgery may not appear until the child is an adult. These include heart rhythm problems and a leaky mitral valve.
All children with congenital heart disease should take antibiotics before dental treatment. This helps prevent complications related to heart infections.
When to Contact a Medical Professional Return to topCall your health care provider if your child seems to tire easily, has trouble breathing, or has bluish skin or lips. You should also consult your health care provider if your baby is not growing or gaining weight.
Prevention Return to top
ECD is associated with several genetic abnormalities. Couples with a family history of ECD may wish to seek genetic counseling before becoming pregnant.
References Return to top
Park MK. Pediatric Cardiology for Practitioners, 5th ed. Philadelphia, Pa: Mosby Elsevier; 2008:181-189:chap 12.
Townsend Jr. CM, Beauchamp RD, Evers BM, et al. Sabiston Textbook of Surgery, 18th ed. Philadelphia, Pa: Saunders Elsevier; 2008:1760-1762.Update Date: 4/30/2008 Updated by: Mark A Fogel, MD, FACC, FAAP, Associate Professor of Pediatrics and Radiology, Director of Cardiac MR, The Children's Hospital of Philadelphia, Division of Cardiology, Philadelphia, PA. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.