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Atrioventricular (AV) canal defect; Atrioventricular septal defect; AVSD
Endocardial cushion defect (ECD) is an abnormal heart condition in which the walls separating all four chambers of the heart are poorly formed or absent. It is a congenital heart disease, which means it is present from birth.
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 valves that separate the atria (top collecting chambers) from the ventricles (bottom pumping chambers).
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 of ECD may include:
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:
Surgery is needed to close the holes between the heart chambers, and to separate the common valve into distinct tricuspid and mitral valves. The timing of the surgery depends on the child's condition and the severity of the ECD. It can usually be done when the baby is 3 - 6 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.
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.
Children with congenital heart disease may need to take antibiotics before dental treatment. This helps prevent complications related to heart infections. Ask a cardiologist whether your child needs to take antibiotics.
ECD is associated with several genetic abnormalities. Couples with a family history of ECD may wish to seek genetic counseling before becoming pregnant.
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.
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