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Related Experiment Videos

Complete atrioventricular canal.

Raffaele Calabrò1, Giuseppe Limongelli

  • 1Cardiologia pediatrica, Azienda Ospedaliera Monaldi, Via Bianchi Leonardo, 80131 Napoli, Italy. raffaele.calabro@unina2.it

Orphanet Journal of Rare Diseases
|May 26, 2006
PubMed
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Complete atrioventricular canal (CAVC) is a rare congenital heart defect affecting 2 in 10,000 births, often associated with Down syndrome. Early diagnosis and surgical intervention in infancy are crucial for managing this condition.

Area of Science:

  • Pediatric Cardiology
  • Congenital Heart Disease
  • Medical Genetics

Background:

  • Complete atrioventricular canal (CAVC), or complete atrioventricular septal defect, is a congenital heart malformation.
  • It involves an atrial septal defect, a common atrioventricular valve, and ventricular septum deficiency.
  • CAVC accounts for approximately 3% of all cardiac malformations.

Purpose of the Study:

  • To describe the characteristics, diagnosis, and management of complete atrioventricular canal.
  • To highlight the association between CAVC and Down syndrome.
  • To outline the timing and indications for surgical intervention.

Main Methods:

  • Diagnosis is suspected through electrocardiography and chest X-ray.
  • Echocardiography is used for confirmation and detailed anatomical assessment.

Related Experiment Videos

  • Cardiac catheterization is indicated for older patients with suspected irreversible pulmonary hypertension.

    • Main Results:

    • CAVC leads to significant shunting, causing right ventricular overload and pulmonary hypertension.
    • Symptoms typically manifest in infancy as congestive heart failure and failure to thrive.
    • Rastelli's classification delineates three types of CAVC based on valve morphology.

    Conclusions:

    • Early surgical repair, typically between 3-6 months of life, is essential.
    • Medical management serves as a temporary measure before surgery.
    • Delayed diagnosis can lead to irreversible pulmonary hypertension, precluding surgical options.