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Endocardial cushion defect


Alternative Names:

Atrioventricular (AV) canal defect; Atrioventricular septal defect; AVSD

Symptoms:

Symptoms of ECD may include:

  • Baby tires easily
  • Bluish skin color (the lips may also be blue)
  • Failure to gain weight and grow
  • Frequent pneumonia
  • Lack of appetite
  • Pale skin (pallor)
  • Rapid breathing
  • Rapid heartbeat
  • Sweating
  • Swollen legs or abdomen (rare in children)
  • Trouble breathing, especially during feeding
Signs and tests:

Signs of ECD may include:

  • An abnormal electrocardiogram (ECG)
  • An enlarged heart
  • Heart murmur

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:

  • Ultrasound of the heart (echocardiogram) to see blood flow
  • An electrocardiogram (ECG), which measures the electrical activity in the heart
  • Chest x-ray, which may show an enlarged heart
  • Magnetic resonance imaging (MRI) of the heart, which provides a detailed image of the heart through the use of powerful magnets
  • Cardiac catheterization (in some cases), a procedure in which a thin tube (catheter) is placed into the heart to see blood flow and take accurate measurements of blood pressure and oxygen levels
Treatment:

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:

  • Diuretics (water pills)
  • Drugs that make the heart contract more forcefully (inotropic agents), such as digoxin

Surgery for a complete ECD should be done in the baby's first year of life, 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.

Expectations (prognosis):
How 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.
Complications:

Complications from ECD may include:

  • Congestive heart failure
  • Death
  • Eisenmenger syndrome
  • High blood pressure in the lungs
  • Irreversible damage to the lungs

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.

Calling your health care provider:
Call 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:

ECD is associated with several genetic abnormalities. Couples with a family history of ECD may wish to seek genetic counseling before becoming pregnant.

References:

Scholz TD, Reinking BE. Congenital heart disease. In: Gleason CA, Devaskar S, eds. Avery's Diseases of the Newborn. 9th ed. Philadelphia, Pa: Saunders Elsevier; 2011:chap 55.


Review Date: 6/5/2012
Reviewed By: Kurt R. Schumacher, MD, Pediatric Cardiology, University of Michigan Congenital Heart Center, Ann Arbor, MI. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M. Health Solutions, Ebix, Inc.

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed physician should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. Copyright 2002 A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.

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