Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

Pediatric Unbalanced Atrioventricular Septal Defects Clinical Presentation

  • Author: Mark A Law, MD; Chief Editor: Howard S Weber, MD, FSCAI  more...
 
Updated: Jan 04, 2016
 

History

Infants with unbalanced atrioventricular (AV) septal defects generally present in the first month of life with congestive heart failure (CHF) with tachypnea and failure to thrive due to pulmonary overcirculation, if no significant right-sided or left-sided obstruction is present.

If pulmonary outflow tract obstruction is present, infants may present with cyanosis or an audible murmur.

Occasionally, neonates may present in extremis with acidosis in the presence of ductal-dependent systemic circulation or cyanosis in the presence of ductal-dependent pulmonary circulation.

Patients with abdominal heterotaxy may present with situs inversus incidentally noted on routine chest radiography.

Next

Physical

Note the following:

  • Most children appear healthy, except the rare patient with features of Down syndrome.
  • This lesion is associated with various auscultatory findings, depending on the underlying physiology.
  • Murmurs of pulmonary stenosis, left ventricular (LV) outflow tract obstruction, or atrioventricular valve (AVV) regurgitation may be appreciated.[9]
  • Cyanosis may be present.
  • Reduced lower extremity pulses may suggest coarctation of the aorta, which may coexist with right ventricle (RV)–dominant atrioventricular canal (AVC).
Previous
Next

Causes

The genetic basis for this lesion has not been elucidated; however it can be associated with trisomy 21.

Unbalanced AV septal defect may be observed in patients with abdominal heterotaxy. The presence of complete AV septal defect is more than twice as frequent in patients with asplenia than in those with polysplenia.

Previous
 
 
Contributor Information and Disclosures
Author

Mark A Law, MD Associate Professor of Pediatrics, Fellowship Director of Pediatric Cardiology, Department of Pediatric Cardiology, University of Alabama School of Medicine

Mark A Law, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American College of Cardiology, American College of Physicians, American Heart Association

Disclosure: Nothing to disclose.

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.

Hugh D Allen, MD Professor, Department of Pediatrics, Division of Pediatric Cardiology and Department of Internal Medicine, Ohio State University College of Medicine

Hugh D Allen, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Echocardiography, Society for Pediatric Research, Society of Pediatric Echocardiography, Western Society for Pediatric Research, American College of Cardiology, American Heart Association, American Pediatric Society

Disclosure: Nothing to disclose.

Chief Editor

Howard S Weber, MD, FSCAI Professor of Pediatrics, Section of Pediatric Cardiology, Pennsylvania State University College of Medicine; Director of Interventional Pediatric Cardiology, Penn State Hershey Children's Hospital

Howard S Weber, MD, FSCAI is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, Society for Cardiovascular Angiography and Interventions

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

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

Ameeta Martin, MD Clinical Associate Professor, Department of Pediatric Cardiology, University of Nebraska College of Medicine

Ameeta Martin, MD is a member of the following medical societies: American College of Cardiology

Disclosure: Nothing to disclose.

References
  1. Anderson RH, Mccartney FJ, Shinebourne EA. Atrioventricular septal defects. Pediatr Cardiol. 1987. 1:571-609.

  2. VanPraagh R, Litovsky S. Pathology and embryology of common atrioventricular canal. Prog Pediatr Cardiol. 1999. 10:115-27.

  3. Beaton AZ, Pike JI, Stallings C, Donofrio MT. Predictors of repair and outcome in prenatally diagnosed atrioventricular septal defects. J Am Soc Echocardiogr. 2013 Feb. 26(2):208-16. [Medline].

  4. Daebritz S, del Nido PJ. Surgical management of common atrioventricular canal. Prog Pediatr Cardiol. 1999. 10:161-71.

  5. Emanuel R, Somerville J, Inns A, Withers R. Evidence of congenital heart disease in the offspring of parents with atrioventricular defects. Br Heart J. 1983 Feb. 49(2):144-7. [Medline].

  6. Berger TJ, Blackstone EH, Kirklin JW, et al. Survival and probability of cure without and with operation in complete atrioventricular canal. Ann Thorac Surg. 1979 Feb. 27(2):104-11. [Medline].

  7. Somerville J, Revel-Chion R, Van Der Cammen T. Atrioventricular canal defects - natural and unnatural history. Pediatr Cardiol. 1981. 404-416.

  8. Bull C, Rigby ML, Shinebourne EA. Should management of complete atrioventricular canal defect be influenced by coexistent Down syndrome?. Lancet. 1985 May 18. 1(8438):1147-9. [Medline].

  9. Vijarnsorn C, Khoo NS, Tham EB, Colen T, Rebeyka IM, Smallhorn JF. Increased common atrioventricular valve tenting is a risk factor for progression to severe regurgitation in patients with a single ventricle with unbalanced atrioventricular septal defect. J Thorac Cardiovasc Surg. 2014 Dec. 148 (6):2580-8. [Medline].

  10. Levine JC, Geva T. Echocardiographic assessment of common atrioventricular canal. Prog Pediatr Cardiol. 1999. 10:137-151.

  11. Jegatheeswaran A, Pizarro C, Caldarone CA, Cohen MS, Baffa JM, Gremmels DB, et al. Echocardiographic definition and surgical decision-making in unbalanced atrioventricular septal defect: a Congenital Heart Surgeons' Society multiinstitutional study. Circulation. 2010 Sep 14. 122(11 Suppl):S209-15. [Medline].

  12. Cohen MS, Jegatheeswaran A, Baffa JM, Gremmels DB, Overman DM, Caldarone CA, et al. Echocardiographic Features Defining Right Dominant Unbalanced Atrioventricular Septal Defect: A Multi-institutional Congenital Heart Surgeons' Society Study. Circ Cardiovasc Imaging. 2013 Jul 1. 6(4):508-13. [Medline].

  13. Prakash A, Lacro RV, Sleeper LA, Minich LL, Colan SD, McCrindle B, et al. Challenges in Echocardiographic Assessment of Mitral Regurgitation in Children After Repair of Atrioventricular Septal Defect. Pediatr Cardiol. 2011 Sep 10. [Medline].

  14. Toh N, Kanzaki H, Nakatani S, Kohyama K, Ohara T, Kim J. Partial atrioventricular septal defect assessed by real-time three-dimensional echocardiography: a case report. J Cardiol. 2007 Dec. 50(6):379-82. [Medline].

  15. Barrea C, Levasseur S, Roman K, Nii M, Coles JG, Williams WG. Three-dimensional echocardiography improves the understanding of left atrioventricular valve morphology and function in atrioventricular septal defects undergoing patch augmentation. J Thorac Cardiovasc Surg. 2005 Apr. 129(4):746-53. [Medline].

  16. van Son JA, Phoon CK, Silverman NH, Haas GS. Predicting feasibility of biventricular repair of right-dominant unbalanced atrioventricular canal. Ann Thorac Surg. 1997 Jun. 63(6):1657-63. [Medline].

  17. Cohen MS, Jacobs ML, Weinberg PM, Rychik J. Morphometric analysis of unbalanced common atrioventricular canal using two-dimensional echocardiography. J Am Coll Cardiol. 1996 Oct. 28(4):1017-23. [Medline].

  18. Foker JE, Berry J, Steinberger J. Ventricular growth stimulation to achieve two-ventricle repair in unbalanced common atrioventricular canal. Prog Pediatr Cardiol. 1999. 10:173-86.

  19. Lillehei CW, Cohen M, Warden HE, Varco RL. The direct-vision intracardiac correction of congenital anomalies by controlled cross circulation; results in thirty-two patients with ventricular septal defects, tetralogy of Fallot, and atrioventricularis communis defects. Surgery. 1955 Jul. 38(1):11-29. [Medline].

  20. Backer CL, Stewart RD, Bailliard F, Kelle AM, Webb CL, Mavroudis C. Complete atrioventricular canal: comparison of modified single-patch technique with two-patch technique. Ann Thorac Surg. 2007 Dec. 84(6):2038-46; discussion 2038-46. [Medline].

  21. Drinkwater DC, Laks H. Unbalanced atrioventricular septal defects. Semin Thorac Cardiovasc Surg. 1997 Jan. 9(1):21-5. [Medline].

  22. Journois D, Baufreton C, Mauriat P, et al. Effects of inhaled nitric oxide administration on early postoperative mortality in patients operated for correction of atrioventricular canal defects. Chest. 2005 Nov. 128(5):3537-44. [Medline].

  23. Apfel HD, Gersony WM. Clinical evaluation, medical management and outcome of atrioventricular canal defects. Prog Pediatr Cardiol. 1999. 10:129-36.

  24. Bricker J, McNamara D, Garson A. Defects of the atrial septum including the atrioventricular canal. In: Science and Practice of Pediatric Cardiology. Lippincott Williams & Wilkins. 1990:1036-1051.

  25. Kirklin JW, Barratt-Boyes BG. Atrioventricular canal defect. Cardiac Surgery. 2nd ed. Churchill Livingstone Inc; 1993. 693-747.

  26. Nadas AS. Endocardial cushion defects. Flyer DC, ed. Nadas' Pediatric Cardiology. Hanley & Belfus Inc; 1992. 577-86.

Previous
Next
 
Echocardiogram image revealing a left ventricular dominant atrioventricular (AV) canal defect.
Catheterization in a patient with a left ventricular (LV)–dominant atrioventricular (AV) canal defect. The catheter is positioned in the pulmonary artery demonstrating pulmonary artery band and branch pulmonary arteries.
ECG of a 3-month-old female with a left ventricular (LV)–dominant atrioventricular (AV) canal. The ECG reveals left axis deviation with an initial counterclockwise frontal loop.
Echocardiogram clip demonstrating common atrioventricular (AV) valve regurgitation in a patient with a left-ventricular (LV)–dominant AV canal defect.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.