Atrioventricular Septal Defect (AVSD)

Atrioventricular septal defect (AVSD)

Major etiologies of AVSD

1. Chromosomal abnormalities

  • Trisomy 21 (Down syndrome)
  • Most common etiology overall
  • Present in ~40–50% of fetuses with AVSD
  • Especially complete AVSD
  • Often associated with:
    • Duodenal atresia (double bubble)
    • Mild ventriculomegaly
    • Short long bones
    • Echogenic bowel
    • Increased NT in first trimester

2. Syndromic and genetic causes

  • Ellis–van Creveld Syndrome
    • Short limbs
    • Postaxial polydactyly
    • Narrow thorax
  • Noonan Syndrome
  • CHARGE Syndrome
  • Heterotaxy Syndrome
    • Especially left atrial isomerism
    • Often with interrupted IVC
    • Abnormal cardiac situs
  • Smith–Lemli–Opitz Syndrome

3. Sporadic developmental cause

Occurs due to abnormal development of:

  • Endocardial cushions

This is the fundamental embryologic basis of AVSD:

  • Failure of fusion of superior and inferior endocardial cushions
  • Leads to:
    • Common AV valve
    • Septal defects at atrial and ventricular level

Most isolated AVSDs without syndromes fall into this group.

4. Maternal/environmental factors

These are weaker associations but relevant:

  • Maternal diabetes
  • Maternal obesity
  • Retinoic acid exposure
  • Poorly controlled phenylketonuria

Types of AVSD

Complete AVSD

  • Strongest association with Trisomy 21
  • Often detected prenatally
  • More likely syndromic

Partial AVSD

  • May be isolated
  • Lower chromosomal risk than complete AVSD

Transitional AVSD

  • Intermediate features
  • Genetic risk depends on associated findings

Important combined associations

When you see AVSD + double bubble, think strongly of:

  • Trisomy 21

Prenatal risk estimates to remember

  • AVSD → Trisomy 21 risk ~40–50%
  • AVSD + additional anomalies → Risk increases significantly
  • Isolated AVSD → Still ~30–40% chromosomal risk