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PubMed This is a summary of 48 peer-reviewed journal articles Updated
Pediatric Cardiology

Complete Atrioventricular Septal Defect (CAVSD) Guide

At a Glance

Complete atrioventricular septal defect (CAVSD) is a treatable congenital heart condition involving a hole in the heart's center and a single common valve. Following corrective open-heart surgery, most children achieve successful repair and live healthy, active lives with ongoing cardiology care.

Learning that your baby has a heart condition can feel overwhelming, but a Complete Atrioventricular Septal Defect (CAVSD) is a well-understood condition [1]. Most children who undergo repair go on to live active, healthy lives [2].

CAVSD is a congenital (present at birth) heart defect where the center of the heart does not form correctly during the first few weeks of pregnancy [3][4]. This leaves a large hole between the upper and lower chambers, along with a single, large common valve instead of two separate valves [5]. Because of this, oxygen-rich and oxygen-poor blood mix, and the heart has to work much harder to pump blood to the body [6].

This resource guide is designed to help you understand your child’s diagnosis, prepare for the surgical journey, and know what to expect in the years ahead. While CAVSD is a complex diagnosis, it is highly treatable, and the exact path forward will depend on your child’s specific heart anatomy.

Navigating This Guide

To help you understand the diagnosis and the road ahead, we have broken down the information into four key areas:

01

Understanding Your Child's Diagnosis

Learn about Complete Atrioventricular Septal Defect (CAVSD) in infants. Understand symptoms, the Down syndrome link, and pre-surgery feeding and medical care.

02

Balanced vs. Unbalanced Anatomy

Learn the difference between balanced and unbalanced Complete Atrioventricular Septal Defect (CAVSD). Understand treatment paths and staged heart repairs.

03

Surgical Treatment and Timing

Learn what to expect during Complete Atrioventricular Septal Defect (CAVSD) surgery. Understand the best timing, patch techniques, and hospital recovery.

04

Long-Term Care and Outlook

Learn about long-term care after CAVSD repair surgery. Understand lifelong monitoring, endocarditis prevention, yearly checkups, and potential complications.

Common questions in this guide

What is a complete atrioventricular septal defect (CAVSD)?
CAVSD is a congenital heart condition where a baby is born with a large hole in the center of their heart and a single common valve instead of two separate ones. This causes oxygen-rich and oxygen-poor blood to mix, forcing the heart to work much harder.
What are the signs of heart failure in a baby with CAVSD?
Common signs that a baby's heart is struggling before surgery include sweating, breathing unusually fast, and having difficulty finishing their feeds. If you notice these symptoms worsening, contact your pediatric cardiology team.
What does it mean if my baby's CAVSD is balanced or unbalanced?
Balanced or unbalanced refers to the size of your baby's lower heart chambers (ventricles). This specific anatomy is crucial because it determines whether your child will have a two-ventricle repair or follow a single-ventricle pathway.
Should my baby get the RSV shot before CAVSD surgery?
Many doctors recommend the RSV preventative shot, called Synagis, to help protect vulnerable infants with heart conditions from severe respiratory infections while they wait for surgery. Discuss this option with your child's care team.
Are children with CAVSD able to live normal lives after surgery?
Yes, with successful surgical repair, most children with CAVSD go on to live active, healthy lives. However, they will require lifelong cardiology checkups to monitor for potential issues like leaky valves.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.Based on my baby's echocardiogram, is their heart anatomy considered balanced or unbalanced?
  2. 2.What specific daily symptoms should I be monitoring to know if my baby's heart failure is worsening before surgery?
  3. 3.Do you recommend our child receive the RSV preventative shot (Synagis) while waiting for surgery?
  4. 4.How much experience does your center have in treating infants with CAVSD and my child's specific medical profile?

Questions For You

Tap a prompt to share your answer — we'll use it plus this page's context to start a tailored conversation.

References

References (6)
  1. 1

    Impact of concomitant complex cardiac anatomy in nonsyndromic patients with complete atrioventricular septal defect.

    Ramgren JJ, Zindovic I, Nozohoor S, et al.

    The Journal of thoracic and cardiovascular surgery 2022; (163(4)):1437-1444 doi:10.1016/j.jtcvs.2021.08.039.

    PMID: 34503843
  2. 2

    Long-Term Outcome Up To 40 Years after Single Patch Repair of Complete Atrioventricular Septal Defect in Infancy or Childhood.

    Reynen S, Hövels-Gürich HH, Vazquez-Jimenez JF, et al.

    The Thoracic and cardiovascular surgeon 2021; (69(S 03)):e68-e75 doi:10.1055/s-0041-1740070.

    PMID: 34953470
  3. 3

    Optical coherence tomography for in vivo imaging of endocardial to mesenchymal transition during avian heart development.

    Courchaine K, Rugonyi S

    Biomedical optics express 2019; (10(11)):5989-5995 doi:10.1364/BOE.10.005989.

    PMID: 31799059
  4. 4

    Complete Atrioventricular Canal Defect in a Non-syndromic Adult Patient: An Unusual Presentation.

    Nabhan EM, Khoury SB, Bechara TE

    Cureus 2023; (15(8)):e43186 doi:10.7759/cureus.43186.

    PMID: 37692634
  5. 5

    Electro-vectorcardiographic demonstration of bifascicular block associated with ventricular preexcitation.

    Pérez-Riera AR, Barbosa-Barros R, Daminello-Raimundo R, et al.

    Annals of noninvasive electrocardiology : the official journal of the International Society for Holter and Noninvasive Electrocardiology, Inc 2019; (24(2)):e12550 doi:10.1111/anec.12550.

    PMID: 29673006
  6. 6

    Commentary: Repair of complete atrioventricular septal defect: The bar has been set.

    Overman DM

    The Journal of thoracic and cardiovascular surgery 2021; (161(6)):2154-2155 doi:10.1016/j.jtcvs.2020.10.008.

    PMID: 33198972

This guide provides educational information about complete atrioventricular septal defect (CAVSD). Always consult your child's pediatric cardiologist or cardiac surgeon for medical advice specific to your baby's unique anatomy.

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