eMedicine Specialties > Cardiology > Congenital Heart Disease in the Adult

Endocardial Cushion Defects: Follow-up

Author: Mary C Mancini, MD, PhD, Professor, Department of Surgery, Louisiana State University Health Sciences Center
Coauthor(s): Henry G Hanley, MD, Chief of Cardiology Section, Freedman Memorial Cardiology; Professor, Department of Medicine, Louisiana State University Health Sciences Center
Contributor Information and Disclosures

Updated: Oct 16, 2008

Follow-up

Further Inpatient Care

Postoperative recovery requires 5-10 days of hospitalization, depending upon the condition of the child prior to surgery and whether palliative or complete correction is undertaken. With palliation (ie, pulmonary artery banding), the presurgical condition of volume overload still must be regulated. With complete correction, recovery generally is uneventful.

Further Outpatient Care

Continued observation is needed with regularly scheduled echocardiography in order to assess the integrity of the AV valvular reconstruction. This area is prone to development of valvular insufficiency that may require further intervention as the child grows older.

Inpatient & Outpatient Medications

Digitalis: This agent provides myocardial support during the postoperative period and can be discontinued after 2-3 years.

Diuretics: Generally, furosemide is prescribed for several months after repair in order to correct volume overload; it is discontinued once euvolemia is reached.

Deterrence/Prevention

The current limited knowledge of the genetic abnormalities that predispose to the formation of the endocardial cushion defect can be expanded greatly with current advances in the Human Genome Project. As the knowledge base expands, prenatal detection and possibly treatment may be possible in the future.

Complications

Since synthetic material is used to repair the atrial and ventricular septal defect, the child is at risk of infection. Other potential complications include complete heart block, ventricular arrhythmia, and AV valve stenosis and/or insufficiency.

Prognosis

The long-term results of surgical correction for this malformation depend upon the degree of preoperative pulmonary vascular disease and upon the amount of residual AV valve regurgitation. If the pulmonary vasculature is protected and the amount of valvular regurgitation is reduced substantially, prognosis is good. When severe pulmonary vascular disease is present preoperatively, morbidity and mortality rates are high. Complete heart block and arrhythmias may occur after correction, and their incidence increases with age. As the patient grows older, mitral valve replacement may be needed.

The surgical mortality rate in patients with partial endocardial cushion defects is 0-6%, while that for the complete defect ranges from 3-10%.

Patient Education

Parents must be instructed to ensure that antibiotic prophylaxis for dental procedures is instituted for the child. Good dental hygiene for the child is imperative.

Miscellaneous

Medicolegal Pitfalls

A candid discussion must take place with the parents of these children addressing all the potential operative complications with their implications for the child in the future. The issue of future mitral valve replacement should be covered with them before the initial operative intervention. The risk of endocarditis, cardiac arrhythmia, and long-term pulmonary complications must be covered.

 


More on Endocardial Cushion Defects

Overview: Endocardial Cushion Defects
Differential Diagnoses & Workup: Endocardial Cushion Defects
Treatment & Medication: Endocardial Cushion Defects
Follow-up: Endocardial Cushion Defects
Multimedia: Endocardial Cushion Defects
References

References

  1. Person AD, Klewer SE, Runyan RB. Cell biology of cardiac cushion development. Int Rev Cytol. 2005;243:287-335. [Medline].

  2. Cooper RS. Endocardial cushion defects: embryology, anatomy and pathophysiology. Adv Cardiol. 2004;41:118-26. [Medline].

  3. Tardif JC, Schwartz SL, Vannan MA, et al. Clinical usefulness of multiplane transesophageal echocardiography: comparison to biplanar imaging. Am Heart J. Jul 1994;128(1):156-66. [Medline].

  4. Weyman AE, Wann LS, Caldwell RL, et al. Negative contrast echocardiography: a new method for detecting left-to-right shunts. Circulation. Mar 1979;59(3):498-505. [Medline].

  5. Williams RG, Rudd M. Echocardiographic features of endocardial cushion defects. Circulation. Mar 1974;49(3):418-22. [Medline].

  6. Holmvang G, Palacios IF, Vlahakes GJ, et al. Imaging and sizing of atrial septal defects by magnetic resonance. Circulation. Dec 15 1995;92(12):3473-80. [Medline].

  7. Jacobstein MD, Fletcher BD, Goldstein S, Riemenschneider TA. Evaluation of atrioventricular septal defect by magnetic resonance imaging. Am J Cardiol. Apr 15 1985;55(9):1158-61. [Medline].

  8. Hanley FL, Fenton KN, Jonas RA, et al. Surgical repair of complete atrioventricular canal defects in infancy. Twenty-year trends. J Thorac Cardiovasc Surg. Sep 1993;106(3):387-94; discussion 394-7. [Medline].

  9. Prêtre R, Dave H, Kadner A, Bettex D, Turina MI. Direct closure of the septum primum in atrioventricular canal defects. J Thorac Cardiovasc Surg. Jun 2004;127(6):1678-81. [Medline].

  10. Silverman N, Levitsky S, Fisher E, et al. Efficacy of pulmonary artery banding in infants with complete atrioventricular canal. Circulation. Sep 1983;68(3 Pt 2):II148-53. [Medline].

  11. Studer M, Blackstone EH, Kirklin JW, et al. Determinants of early and late results of repair of atrioventricular septal (canal) defects. J Thorac Cardiovasc Surg. Oct 1982;84(4):523-42. [Medline].

  12. Carmi R, Boughman JA, Ferencz C. Endocardial cushion defect: further studies of "isolated" versus "syndromic" occurrence. Am J Med Genet. Jun 1 1992;43(3):569-75. [Medline].

  13. Chin AJ, Keane JF, Norwood WI, Castaneda AR. Repair of complete common atrioventricular canal in infancy. J Thorac Cardiovasc Surg. Sep 1982;84(3):437-45. [Medline].

  14. Cohen MS, Spray TL. Surgical management of unbalanced atrioventricular canal defect. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2005;135-44. [Medline].

  15. Digilio MC, Giannotti A, Marino B, Dallapiccola B. Atrioventricular canal and 8p- syndrome. Am J Med Genet. Sep 1 1993;47(3):437-8. [Medline].

  16. Ebels T, Ho SY, Anderson RH, et al. The surgical anatomy of the left ventricular outflow tract in atrioventricular septal defect. Ann Thorac Surg. May 1986;41(5):483-8. [Medline].

  17. Fisher EA, Doshi M, DuBrow IW, et al. Effect of palliative and corrective surgery on ventricular volumes in complete atrioventricular canal. Pediatr Cardiol. Jul-Sep 1984;5(3):159-65. [Medline].

  18. Korenberg JR, Kawashima H, Pulst SM, et al. Molecular definition of a region of chromosome 21 that causes features of the Down syndrome phenotype. Am J Hum Genet. Aug 1990;47(2):236-46. [Medline].

  19. Neufeld EA, Sher M, Paul, MH. Pulmonary vascular disease in complete atrioventricular canal defect. Am J Cardiology. 1977;39:721-726.

  20. Soto B, Bargeron LM, Pacifico AD, et al. Angiography of atrioventricular canal defects. Am J Cardiol. Sep 1981;48(3):492-9. [Medline].

  21. Van Mierop LH, Alley Rd, Kausel HW, Stranahan A. The anatomy and embryology of endocardial cushion defects. J Thorac Cardiovasc Surg. Jan 1962;43:71-83. [Medline].

  22. Vida VL, Barnoya J, Larrazabal LA, Gaitan G, de Maria Garcia F, Castañeda AR. Congenital cardiac disease in children with Down's syndrome in Guatemala. Cardiol Young. Jun 2005;15(3):286-90. [Medline].

  23. Wakai CS, Edwards JE. Pathologic study of persistent common atrioventricular canal. Am Heart J. Nov 1958;56(5):779-94. [Medline].

Further Reading

Keywords

endocardial cushion defects, atrioventricular septal defects, atrioventricular canal defects, AV septal defects, canalis atrioventricularis communis, persistent atrioventricular ostium, abnormal development of endocardial cushions, heart failure, pulmonary vascular disease, congestive heart failure, CHF, ostium primum atrial septal defect, minimal insufficiency of the left AV valve, atrial arrhythmia, trisomy 21, Down syndrome

Contributor Information and Disclosures

Author

Mary C Mancini, MD, PhD, Professor, Department of Surgery, Louisiana State University Health Sciences Center
Mary C Mancini, MD, PhD is a member of the following medical societies: American Heart Association, American Medical Association, American Thoracic Society, Association for Academic Surgery, Association for Surgical Education, International College of Surgeons, International Society for Heart and Lung Transplantation, New York Academy of Sciences, Phi Beta Kappa, and Southern Thoracic Surgical Association
Disclosure: Nothing to disclose.

Coauthor(s)

Henry G Hanley, MD, Chief of Cardiology Section, Freedman Memorial Cardiology; Professor, Department of Medicine, Louisiana State University Health Sciences Center
Henry G Hanley, MD is a member of the following medical societies: American College of Cardiology, American College of Physicians, American Heart Association, and Society of Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.

Medical Editor

Park W Willis IV, MD, Sarah Graham Distinguished Professor of Medicine and Pediatrics, University of North Carolina at Chapel Hill School of Medicine
Park W Willis IV, MD is a member of the following medical societies: American Society of Echocardiography
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Frank M Sheridan, MD, Cardiology, Providence Everett Medical Center
Frank M Sheridan, MD is a member of the following medical societies: American College of Cardiology, American Heart Association, and Society for Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.

CME Editor

Amer Suleman, MD, Consultant in Electrophysiology and Cardiovascular Medicine, Department of Internal Medicine, Division of Cardiology, Medical City Dallas Hospital
Amer Suleman, MD is a member of the following medical societies: American College of Physicians, American Heart Association, American Institute of Stress, American Society of Hypertension, Federation of American Societies for Experimental Biology, Royal Society of Medicine, and Society of Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.

Chief Editor

Park W Willis IV, MD, Sarah Graham Distinguished Professor of Medicine and Pediatrics, University of North Carolina at Chapel Hill School of Medicine
Park W Willis IV, MD is a member of the following medical societies: American Society of Echocardiography
Disclosure: Nothing to disclose.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.