Complete Atrioventricular Septal Defect (CAVSD) Guide
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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.
Key Takeaways
- • CAVSD is a congenital heart defect characterized by a large hole in the center of the heart and a single common valve.
- • Parents should monitor their baby for signs of heart failure before surgery, such as fast breathing, sweating, or difficulty feeding.
- • The surgical plan depends heavily on whether the baby's lower heart chambers are balanced or unbalanced.
- • Most children go on to live healthy, active lives after surgery but require lifelong cardiology monitoring for complications like leaky valves.
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:
- Understanding Your Child’s Diagnosis: Learn the basics of CAVSD, its strong link to Down syndrome, the symptoms to watch for in your baby, and how the condition is managed in the critical weeks before surgery.
- Balanced vs. Unbalanced Anatomy: Discover the most crucial factor in your child’s surgical plan. This page explains how the size of the heart’s lower chambers determines whether your child will have a two-ventricle repair or follow a single-ventricle pathway.
- Surgical Treatment and Timing: Find out when surgery typically happens, the different techniques surgeons use to rebuild the heart, and what to expect during your baby’s hospital stay in the cardiac intensive care unit.
- Long-Term Care and Outlook: Understand the importance of lifelong cardiology checkups, the common complications to monitor for (like leaky valves), and why the future is bright for most children with repaired CAVSD.
Frequently Asked Questions
What is a complete atrioventricular septal defect (CAVSD)?
What are the signs of heart failure in a baby with CAVSD?
What does it mean if my baby's CAVSD is balanced or unbalanced?
Should my baby get the RSV shot before CAVSD surgery?
Are children with CAVSD able to live normal lives after surgery?
Questions for Your Doctor
- • Based on my baby's echocardiogram, is their heart anatomy considered balanced or unbalanced?
- • What specific daily symptoms should I be monitoring to know if my baby's heart failure is worsening before surgery?
- • Do you recommend our child receive the RSV preventative shot (Synagis) while waiting for surgery?
- • How much experience does your center have in treating infants with CAVSD and my child's specific medical profile?
Questions for You
- • Have I noticed my baby sweating, breathing fast, or struggling to finish feeds?
- • Do I have a strong support system in place to help me navigate the emotional weight of this diagnosis?
- • Have I organized all of my baby's cardiology reports and echocardiogram results in one accessible place?
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References
- 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
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
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
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
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
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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