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Pediatric Complete Atrioventricular Septal Defects Differential Diagnoses

  • Author: Michael D Pettersen, MD; Chief Editor: P Syamasundar Rao, MD  more...
 
Updated: Mar 02, 2016
 
 

Diagnostic Considerations

Important considerations

Early recognition/diagnosis of common atrioventricular canal is important. Common atrioventricular canal is often recognized early in the life of the patient, especially in an infant with Down syndrome, only because of abnormal precordial activity, a loud, single S2, or both.

Because data from most studies suggest that approximately 50% of children with Down syndrome have congenital heart disease and because the physical findings are often subtle, early referral of a patient with Down syndrome to a pediatric cardiologist is recommended.

Special concerns

Women who undergo successful repair of common atrioventricular canal should be able to tolerate pregnancy well if they are asymptomatic. If the patient has clinically significant residual atrioventricular valve insufficiency, a clinically significant residual ventricular septal defect (VSD), or poor ventricular function, the risks to both the mother and the fetus rise. The risk of a fetal congenital heart disease may be as high as 14% (range, 10-15%).

Data from some studies suggest that patients with congenital heart disease have substantially more stress in their lives than patients without chronic diseases. Even if this is true, findings suggest that children who have congenital heart disease have educational and occupational rates higher than those of age-matched control patients. Children with Down syndrome or other syndromes that affect cognitive function perform less well than other children.

Common atrioventricular canal defect is an endocardial cushion malformation resulting in an atrial septal defect, a VSD, and a common atrioventricular valve. Causes are multifactorial. Although the natural history is somewhat ominous, technologic advances over the past 20 years have greatly aided diagnosis and surgical correction of this complex malformation, yielding promising results.

Differential Diagnoses

 
 
Contributor Information and Disclosures
Author

Michael D Pettersen, MD Consulting Staff, Rocky Mountain Pediatric Cardiology, Pediatrix Medical Group

Michael D Pettersen, MD is a member of the following medical societies: American Society of Echocardiography

Disclosure: Received income in an amount equal to or greater than $250 from: Fuji Medical Imaging.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Alvin J Chin, MD Emeritus Professor of Pediatrics, University of Pennsylvania School of Medicine

Alvin J Chin, MD is a member of the following medical societies: American Association for the Advancement of Science, Society for Developmental Biology, American Heart Association

Disclosure: Nothing to disclose.

Chief Editor

P Syamasundar Rao, MD Professor of Pediatrics and Medicine, Division of Cardiology, Emeritus Chief of Pediatric Cardiology, University of Texas Medical School at Houston and Children's Memorial Hermann Hospital

P Syamasundar Rao, MD is a member of the following medical societies: American Academy of Pediatrics, American Pediatric Society, American College of Cardiology, American Heart Association, Society for Cardiovascular Angiography and Interventions, Society for Pediatric Research

Disclosure: Nothing to disclose.

Additional Contributors

Paul M Seib, MD Associate Professor of Pediatrics, University of Arkansas for Medical Sciences; Medical Director, Cardiac Catheterization Laboratory, Co-Medical Director, Cardiovascular Intensive Care Unit, Arkansas Children's Hospital

Paul M Seib, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, Arkansas Medical Society, International Society for Heart and Lung Transplantation, Society for Cardiovascular Angiography and Interventions

Disclosure: Nothing to disclose.

Acknowledgements

The authors and editors of Medscape Drugs & Diseases gratefully acknowledge the contributions of previous authors Michael McConnell, MD, and John Scheitler, MD, to the original writing and development of this article.

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Atrioventricular (A-V) valve leaflets viewed from the cardiac apex in normal valves (A) and in the Rastelli type A complete form of common A-V canal (B). In A, the normal tricuspid valve (TV) has anterior (AL), septal (SL), and posterior (PL) leaflets. A normal mitral valve (MV) has ALs and PLs.In B, the superior cushion–derived leaflet bridges the ventricular septum and attaches to the papillary muscle of the conus at its rightmost extent. A right superior leaflet (RSL) typically attaches to the papillary muscle of the conus and to the anterior papillary muscle of the right ventricle (RV), and a right lateral leaflet (RLL) attaches to the anterior papillary muscle of the RV and to the posterior papillary muscle of the RV. The inferior cushion–derived bridging leaflet is usually cleft, giving the appearance of a right inferior leaflet (RIL) and a left inferior leaflet (LIL).
Apical 4-chamber echocardiographic image demonstrating a complete atrioventricular septal defect. A large primum atrial septal defect, a large inlet ventricular septal defect, and a single common orifice atrioventricular valve are noted.
Apical 4-chamber echocardiographic image with color Doppler demonstrating moderately-severe insufficiency of the common atrioventricular valve.
Parasternal long axis echocardiographic image of a complete atrioventricular septal defect. A large inlet ventricular septal defect is seen. Accessory atrioventricular valve tissue is visualized within the left ventricular outflow tract.
Subcostal sagittal echocardiographic image demonstrating the common atrioventricular valve. The anterior bridging leaflet inserts onto the interventricular septum consistent with a Rastelli type A valve.
 
 
 
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