eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiology

Atrioventricular Septal Defect, Unbalanced: Follow-up

Author: Mark A Law, MD, Fellow, Department of Pediatric Cardiology, Baylor College of Medicine,Texas Childrens Hospital
Coauthor(s): Ameeta Martin, MD, Clinical Associate Professor, Department of Pediatric Cardiology, University of Nebraska College of Medicine
Contributor Information and Disclosures

Updated: Aug 27, 2008

Follow-up

Further Inpatient Care

  • Admit for testing and surgical intervention.
  • Optimize management of congestive heart failure (CHF) before surgical repair and/or palliation.

Further Outpatient Care

  • Give careful attention to atrioventricular valve (AVV) function and development of left ventricular (LV) outflow obstruction on follow-up visits if a biventricular repair was accomplished.
  • If a single-ventricle repair was performed, follow-up care and complications occur as with other patients with a single ventricle. See Single Ventricle for a comprehensive list of postoperative complications and medical management.

Inpatient & Outpatient Medications

  • If a successful biventricular repair is accomplished, long-term cardiac medications are generally not needed after surgical convalescence.
  • In patients who underwent single-ventricle palliation, aspirin with or without warfarin (Coumadin) may be used to reduce the risk of thrombus formation within the Fontan circuit. Additionally, an ACE inhibitor may be prescribed to optimize ventricular function or for the treatment of AVV regurgitation. Antiarrhythmic medications are used as indicated for the treatment of specific arrhythmias.

Transfer

  • After diagnosis is made, or to ensure an accurate diagnosis, transfer the patient to a tertiary facility where pediatric cardiologists and/or cardiovascular surgeons are available.

Complications

  • Postoperative complications following biventricular repair include atrioventricular (AV) block, pulmonary hypertension, residual AVV regurgitation, AVV stenosis, and residual LV outflow tract obstruction.
  • Postoperative complications following univentricular repair include the following:
    • Pleural effusions, pericardial effusions, ascites
    • Atrial flutter and other atrial or, less commonly, ventricular arrhythmias
    • Pulmonary thromboembolism, stroke
    • Protein-losing enteropathy
    • Residual pulmonary branch stenosis
    • Formation of systemic venous collaterals resulting in a right-to-left shunt or the development of pulmonary arteriovenous fistulae
    • Low exercise capacity
    • Growth failure
    • Formation of systemic-to-pulmonary arterial collaterals that may result in a residual left-to-right shunt and excessive volume load on the systemic ventricle

Prognosis

  • Prognosis following biventricular repair is generally good. The operative mortality rate is generally less than 3%. Most patients remain asymptomatic with a normal functional status. Less than 10-15% of patients require reoperation for residual AVV insufficiency or LV outflow tract obstruction.13
  • Prognosis following univentricular repair is reasonable and improving as surgical techniques and medical management improve. However, the true long-term function of a single ventricle, especially a single right ventricle, remains unknown.
  • In 1983, Emanuel et al reported that 14% of offspring of mothers with AV septal defects have congenital heart disease.17

Miscellaneous

Medicolegal Pitfalls

  • Failure to recognize potential for early pulmonary vascular obstructive disease and, thus, failure to initiate surgical therapy accordingly
 


More on Atrioventricular Septal Defect, Unbalanced

Overview: Atrioventricular Septal Defect, Unbalanced
Differential Diagnoses & Workup: Atrioventricular Septal Defect, Unbalanced
Treatment & Medication: Atrioventricular Septal Defect, Unbalanced
Follow-up: Atrioventricular Septal Defect, Unbalanced
Multimedia: Atrioventricular Septal Defect, Unbalanced
References
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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. Berger TJ, Blackstone EH, Kirklin JW, et al. Survival and probability of cure without and with operation in complete atrioventricular canal. Ann Thorac Surg. Feb 1979;27(2):104-11. [Medline].

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

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

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

  7. 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. Dec 2007;50(6):379-82. [Medline].

  8. 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. Apr 2005;129(4):746-53. [Medline].

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

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

  11. 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.

  12. 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. Jul 1955;38(1):11-29. [Medline].

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

  14. 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. Dec 2007;84(6):2038-46; discussion 2038-46. [Medline].

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

  16. 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. Nov 2005;128(5):3537-44. [Medline].

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

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

  19. 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.

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

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

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Further Reading

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Keywords

unbalanced atrioventricular septal defect, AVSD, AV canal, AVC, unbalanced endocardial cushion defects, left ventricular–type septal defect, LV-type septal defect, left ventricular–type canal, LV-type canal, left ventricular–dominant AV septal defect, LV-dominant AV septal defect, left ventricular–dominant AV canal, LV-dominant AV canal, atrioventricular canal, atrioventricular septal defect, right ventricular–type septal defect, RV-type septal defect, right ventricular–type canal, RV-type canal, right ventricular–dominant AV septal defect, RV-dominant AV septal defect, right ventricular–dominant AV canal, RV-dominant AV canal, congestive heart failure, pulmonary artery banding, Down syndrome, tachypnea, failure to thrive, pulmonary outflow tract obstruction, coarctation of the aorta, trisomy 21

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Contributor Information and Disclosures

Author

Mark A Law, MD, Fellow, Department of Pediatric Cardiology, Baylor College of Medicine,Texas Childrens Hospital
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, and American Heart Association
Disclosure: Nothing to disclose.

Coauthor(s)

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.

Medical Editor

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, and Society for Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

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 College of Cardiology, American Heart Association, American Pediatric Society, American Society of Echocardiography, Society for Pediatric Research, Society of Pediatric Echocardiography, and Western Society for Pediatric Research
Disclosure: Nothing to disclose.

CME Editor

Gilbert Z Herzberg, MD, Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College; Consulting Staff, Department of Pediatrics, Sound Shore Medical Center
Gilbert Z Herzberg, MD is a member of the following medical societies: American Academy of Pediatrics
Disclosure: Nothing to disclose.

Chief Editor

Steven R Neish, MD, SM, Director of Pediatric Cardiology Fellowship Program, Associate Professor, Department of Pediatrics, Baylor College of Medicine
Steven R Neish, MD, SM is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, and American Heart Association
Disclosure: Nothing to disclose.

 
 
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