Pediatric Complete Atrioventricular Septal Defects Medication

  • Author: Michael D Pettersen, MD; Chief Editor: Steven R Neish, MD, SM   more...
 
Updated: Oct 12, 2011
 

Medication Summary

Medical treatment of complete atrioventricular septal defect (AVSD) is similar to treatment of any cardiac defect with volume overload. Digoxin is frequently used to decrease the heart rate and to increase inotropy, although little evidence (if any) suggests that it is effective in patients with congestive heart failure (CHF) due to left-to-right shunt lesions. Diuretics may decrease preload and ACE inhibitors decrease afterload. Care must be taken when administering ACE inhibitors to reproductive-age females, given their teratogenic effects. More recent, but limited, data suggest that the use of beta blockers in patients with left-to-right shunts who have CHF improves symptoms.[29]

The daily dosage of digoxin is approximately 5-10 mcg/kg/d. The diuretic used most frequently in the author's institution is furosemide 1-2 mg/kg/d. In children with clinical signs of CHF, 58% improved with enalapril. The mean maximal dose was 0.3 mg/kg/d. The most significant adverse effect observed was renal failure, particularly in young infants with large left-to-right shunts. Most of the older patients in the author's institution who need ACE inhibitors are treated with lisinopril because of its lower cost and long half-life. The dose generally is 0.5 mg/kg/d, but is individualized for each patient. Data about the efficacy of beta-blockers in patients with large left-to-right shunts is sparse. In small studies, beta-blockers appear to decrease renin levels and heart rates in infants with CHF due to left-to-right shunts.

Antibiotics for endocarditis prophylaxis are no longer recommended for most patients with congenital heart disease. Some significant exceptions are noted, including patients who have previously had endocarditis or patients within 6 months of their surgical repair. Current American Heart Association guidelines also recommend subacute bacterial endocarditis (SBE) prophylaxis for patients who have a complete repair and those who have a jet lesion aimed at a patch to impair the growth of endothelial cells on the patch.[30] This situation may occur in patients with atrioventricular septal defects and can only be discovered by the use of imaging modalities such as echocardiography.

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Inotropic agents

Class Summary

The agents provide symptomatic improvement for CHF. Positive inotropic agents increase the force of contraction of the myocardium and are used to treat acute and chronic CHF. Positive or negative chronotropic agents may also increase or decrease the heart rate, provide vasodilatation, or improve myocardial relaxation. These additional properties influence the choice of drug for specific circumstances.

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Digoxin (Lanoxicaps, Lanoxin)

 

Acts directly on cardiac muscle, increasing myocardial systolic contractions. Indirect actions increase carotid sinus nerve activity and enhance sympathetic withdrawal for any given increase in mean arterial pressure.

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Diuretic agents

Class Summary

These agents provide symptomatic improvement for CHF and promote the excretion of water and electrolytes by the kidneys. They are indicated to treat heart failure or hepatic, renal, or pulmonary disease when sodium and water retention has resulted in edema or ascites.

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Furosemide (Lasix)

 

Increases excretion of water by interfering with chloride-binding cotransport system, which inhibits sodium and chloride reabsorption in ascending loop of Henle and distal renal tubule.

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ACE inhibitors

Class Summary

These drugs are indicated for treatment of symptomatic CHF. ACE inhibitors are beneficial in all stages of chronic heart failure. Pharmacologic effects result in a decrease in systemic vascular resistance, reducing blood pressure, preload, and afterload.

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Captopril (Capoten)

 

Short-acting ACE inhibitor. Predominant action is suppressing the renin-angiotensin aldosterone system. Prevents conversion of angiotensin I to angiotensin II (potent vasoconstrictor), increasing levels of plasma renin and reducing aldosterone secretion.

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Enalapril (Vasotec)

 

Competitive ACE inhibitor. Reduces angiotensin II levels, decreasing aldosterone secretion.

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Lisinopril (Prinivil, Zestril)

 

Prevents conversion of angiotensin I to angiotensin II (potent vasoconstrictor), reducing aldosterone secretion.

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Contributor Information and Disclosures
Author

Michael D Pettersen, MD  Director of Echocardiography, Division of Cardiology, Children's Hospital of Michigan; Associate Professor of Pediatrics, Wayne State University School of Medicine

Michael D Pettersen, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, and American Society of Echocardiography

Disclosure: Nothing to disclose.

Specialty Editor Board

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.

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  Professor of Pediatrics, University of Pennsylvania School of Medicine; Attending Physician, Cardiology Division, Children's Hospital of Philadelphia

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

Disclosure: Nothing to disclose.

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.

Additional Contributors

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

References

  1. Van Praagh S, Vangi V, Sul JH, et al. Tricuspid atresia or severe stenosis with partial common atrioventricular canal: anatomic data, clinical profile and surgical considerations. J Am Coll Cardiol. Mar 15 1991;17(4):932-43. [Medline].

  2. Wenink AC, Zevallos JC. Developmental aspects of atrioventricular septal defects. Int J Cardiol. Jan 1988;18(1):65-78. [Medline].

  3. Moore KL, Persaud TVN. Before We are Born. 5th ed. Philidelphia, PA: WB Saunders; 1998.

  4. Wenink AC, Zevallos JC. Developmental aspects of atrioventricular septal defects. Int J Cardiol. Jan 1988;18(1):65-78. [Medline].

  5. Rastelli GC, Kirklin JW, Titus JL. Anatomic observations on complete form of persistent common atrioventricular canal with special reference to atrioventricular valves. Mayo Clin Proc. 1966;41:296-308.

  6. Bharati S, Lev M. Common Atrioventricular Orifice - Complete Type. In: The Pathology of Congenital Heart Disease: A Personal Experience with More Than 6,300 Congenitally Malformed Hearts. Armonk, NY: Futura Publinshing Company, Inc; 1996:553-586.

  7. Karl TR. Atrioventricular septal defect with tetralogy of Fallot or double-outlet right ventricle: surgical considerations. Semin Thorac Cardiovasc Surg. Jan 1997;9(1):26-34. [Medline].

  8. Hoffman JIE. Incidence of congenital heart disease: I. Postnatal incidence. Pediatr Cardiol. 1995;16:103-113.

  9. Rosenthal GL, Wilson PD, Permutt T, Boughman JA, Ferencz C. Birth weight and cardiovascular malformations: a population-based study. The Baltimore-Washington Infant Study. Am J Epidemiol. Jun 15 1991;133(12):1273-81. [Medline].

  10. Hoffman JIE. Incidence of congenital heart disease: II. Prenatal incidence. Pediatr Cardiol. 1995;155-165.

  11. Freeman SB, Taft LF, Dooley KJ, et al. Population-based study of congenital heart defects in Down syndrome. Am J Med Genet. Nov 16 1998;80(3):213-7. [Medline].

  12. Digilio MC, Marino B, Cicini MP, et al. Risk of congenital heart defects in relatives of patients with atrioventricular canal. Am J Dis Child. Dec 1993;147(12):1295-7. [Medline].

  13. Tweddell JS, Litwin SB, Berger S, et al. Twenty-year experience with repair of complete atrioventricular septal defects. Ann Thorac Surg. Aug 1996;62(2):419-24. [Medline].

  14. [Best Evidence] Miller A, Siffel C, Lu C, Riehle-Colarusso T, Frías JL, Correa A. Long-term survival of infants with atrioventricular septal defects. J Pediatr. Jun 2010;156(6):994-1000. [Medline].

  15. Bando K, Turrentine MW, Sun K, et al. Surgical management of complete atrioventricular septal defects. A twenty-year experience. J Thorac Cardiovasc Surg. Nov 1995;110(5):1543-52; discussion 1552-4. [Medline].

  16. McElhinney DB, Reddy VM, Silverman NH, Hanley FL. Accessory and anomalous atrioventricular valvar tissue causing outflow tract obstruction: surgical implications of a heterogeneous and complex problem. J Am Coll Cardiol. Nov 15 1998;32(6):1741-8. [Medline].

  17. Perloff JK. The Clinical Recognition of Congenital Heart Disease. Philadelphia, PA: WB Saunders; 1987:322-38.

  18. Sheffield VC, et al. Identification of a complex congenital heart defect susceptibility locus by using DNA pooling and shared segment analysis. Hum Molec Genet. 1997;6(1):117-121.

  19. Vannay A, Vasarhelyi B, Kornyei M, et al. Single-nucleotide polymorphisms of VEGF gene are associated with risk of congenital valvuloseptal heart defects. Am Heart J. Apr 2006;151(4):878-81. [Medline]. [Full Text].

  20. Cabrera A, Pastor E, Galdeano JM, et al. Cross-sectional echocardiography in the diagnosis of atrioventricular septal defect. Int J Cardiol. Jul 1990;28(1):19-23. [Medline].

  21. Chin AJ, Bierman FZ, Sanders SP, et al. Subxyphoid 2-dimensional echocardiographic identification of left ventricular papillary muscle anomalies in complete common atrioventricular canal. Am J Cardiol. Jun 1983;51(10):1695-9. [Medline].

  22. Silverman NH, Zuberbuhler JR, Anderson RH. Atrioventricular septal defects: cross-sectional echocardiographic and morphologic comparisons. Int J Cardiol. Dec 1986;13(3):309-31. [Medline].

  23. Del Pasqua A, Sanders SP, de Zorzi A, et al. Impact of three-dimensional echocardiography in complex congenital heart defect cases: the surgical view. Pediatr Cardiol. Apr 2009;30(3):293-300. [Medline].

  24. Takahashi K, Guerra V, Roman KS, Nii M, Redington A, Smallhorn JF. Three-dimensional echocardiography improves the understanding of the mechanisms and site of left atrioventricular valve regurgitation in atrioventricular septal defect. J Am Soc Echocardiogr. Dec 2006;19(12):1502-10. [Medline].

  25. Najm HK, Coles JG, Endo M, et al. Complete atrioventricular septal defects: results of repair, risk factors, and freedom from reoperation. Circulation. Nov 4 1997;96(9 Suppl):II-311-5. [Medline].

  26. Atz AM, Hawkins JA, Lu M, et al. Surgical management of complete atrioventricular septal defect: Associations with surgical technique, age, and trisomy 21. J Thorac Cardiovasc Surg. Jun 2011;141(6):1371-9. [Medline]. [Full Text].

  27. Kaza AK, Colan SD, Jaggers J, et al. Surgical interventions for atrioventricular septal defect subtypes: the pediatric heart network experience. Ann Thorac Surg. Oct 2011;92(4):1468-75. [Medline].

  28. Ten Harkel AD, Cromme-Dijkhuis AH, Heinerman BC, et al. Development of left atrioventricular valve regurgitation after correction of atrioventricular septal defect. Ann Thorac Surg. Feb 2005;79(2):607-12. [Medline].

  29. Buchhorn R, Hulpke-Wette M, Ruschewski W, et al. Effects of therapeutic beta blockade on myocardial function and cardiac remodelling in congenital cardiac disease. Cardiol Young. Feb 2003;13(1):36-43. [Medline].

  30. [Guideline] Wilson W, Taubert KA, Gewitz M, et al. Prevention of Infective Endocarditis. Guidelines From the American Heart Association. A Guideline From the American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee, Council on Cardiovascular Disease in the Young, and the Council on Clinical Cardiology, Council on Cardiovascular Surgery and Anesthesia, and the Quality of Care and Outcomes Research Interdisciplinary Working Group. Circulation. Apr 19 2007;[Medline].

  31. Alsoufi B, Al-Halees Z, Khouqeer F, Canver CC, et al. Results of Left Atrioventricular Valve Reoperations Following Previous Repair of Atrioventricular Septal Defects. J Card Surg. Jun 15 2009;[Medline].

  32. Stulak JM, Burkhart HM, Dearani JA, et al. Reoperations after initial repair of complete atrioventricular septal defect. Ann Thorac Surg. Jun 2009;87(6):1872-7; discussion 1877-8. [Medline].

  33. Reeder GS, Danielson GK, Seward JB, et al. Fixed subaortic stenosis in atrioventricular canal defect: a Doppler echocardiographic study. J Am Coll Cardiol. Aug 1992;20(2):386-94. [Medline].

  34. Newfeld EA, Sher M, Paul MH, Nikaidoh H. Pulmonary vascular disease in complete atrioventricular canal defect. Am J Cardiol. May 4 1977;39(5):721-6. [Medline].

  35. Alexi-Meskishvili V, Ishino K, Dahnert I, et al. Correction of complete atrioventricular septal defects with the double-patch technique and cleft closure. Ann Thorac Surg. Aug 1996;62(2):519-24; discussion 524-5. [Medline].

  36. Backer CL, Mavroudis C, Alboliras ET, Zales VR. Repair of complete atrioventricular canal defects: results with the two- patch technique. Ann Thorac Surg. Sep 1995;60(3):530-7. [Medline].

  37. Capouya ER, Laks H, Drinkwater DC Jr, et al. Management of the left atrioventricular valve in the repair of complete atrioventricular septal defects. J Thorac Cardiovasc Surg. Jul 1992;104(1):196-201; discussion 201-3. [Medline].

  38. Cousineua AJ, Lauer RM, Pierpont ME, et al. Linkage analysis of autosomal dominant atrioventricular canal defects: exclusion of chromosome 21. Human Genetics. 1994;93(2):103-8. [Medline].

  39. DeLeon SY, Ilbawi MN, Wilson WR Jr, et al. Surgical options in subaortic stenosis associated with endocardial cushion defects. Ann Thorac Surg. Nov 1991;52(5):1076-82; discussion 1082-3. [Medline].

  40. Eisenberg LM, Markwald RR. Molecular regulation of atrioventricular valvuloseptal morphogenesis. Circ Res. Jul 1995;77(1):1-6. [Medline].

  41. Formigari R, Di Donato RM, Gargiulo G, et al. Better surgical prognosis for patients with complete atrioventricular septal defect and Down's syndrome. Ann Thorac Surg. Aug 2004;78(2):666-72; discussion 672. [Medline].

  42. Gembruch U, Knopfle G, Chatterjee et al. Prenatal diagnosis of atrioventricular canal malformations with up-to-date echocardiographic technology: report of 14 cases. Am Heart J. May 1991;121(5):1489-97. [Medline].

  43. 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].

  44. 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].

  45. Kosaki K, Curry CJ, Roeder E, Jones KL. Ritscher-Schinzel (3C) syndrome: documentation of the phenotype. Am J Med Genet. Feb 11 1997;68(4):421-7. [Medline].

  46. Kurnit DM, Aldridge JF, Matsuoka R, Matthysse S. Increased adhesiveness of trisomy 21 cells and atrioventricular canal malformations in Down syndrome: a stochastic model. Am J Med Genet. Feb 1985;20(2):385-99. [Medline].

  47. Lange A, Mankad P, Walayat M, et al. Transthoracic three-dimensional echocardiography in the preoperative assessment of atrioventricular septal defect morphology. Am J Cardiol. Mar 1 2000;85(5):630-5. [Medline].

  48. LeBlanc JG, Williams WG, Freedom RM, Trusler GA. Results of total correction in complete atrioventricular septal defects with congenital or surgically induced right ventricular outflow tract obstruction. Ann Thorac Surg. Apr 1986;41(4):387-91. [Medline].

  49. Leversha AM, Wilson NJ, Clarkson PM, et al. Efficacy and dosage of enalapril in congenital and acquired heart disease. Arch Dis Child. Jan 1994;70(1):35-9. [Medline].

  50. Maslen CL. Molecular genetics of atrioventricular septal defects. Curr Opin Cardiol. May 2004;19(3):205-10. [Medline].

  51. Miyamura H, Eguchi S, Watanabe H, et al. Total circular annuloplasty with absorbable suture for the repair of left atrioventricular valve regurgitation in atrioventricular septal defect. J Thorac Cardiovasc Surg. Jun 1994;107(6):1428-31. [Medline].

  52. Rizzoli G, Mazzucco A, Maizza F, et al. Does Down syndrome affect prognosis of surgically managed atrioventricular canal defects?. J Thorac Cardiovasc Surg. Oct 1992;104(4):945-53. [Medline].

  53. Roach RM, Tandon R, Moller JH, Edwards JE. Ebstein's anomaly of the tricuspid valve in persistent common atrioventricular canal. Am J Cardiol. Feb 1 1984;53(4):640-2. [Medline].

  54. Rosenthal GL, Wilson PD, Permutt T, et al. Birth weight and cardiovascular malformations: a population-based study. The Baltimore-Washington Infant Study. Am J Epidemiol. Jun 15 1991;133(12):1273-81. [Medline].

  55. Ross DA, Nanton M, Gillis DA, Murphy DA. Atrioventricular canal defects: results of repair in the current era. J Card Surg. Sep 1991;6(3):367-72. [Medline].

  56. Sakamoto K, Galletti L, Touchot A, et al. Two-stage correction of transposition of great arteries with complete atrioventricular canal. Ann Thorac Surg. Jan 1998;65(1):250-2. [Medline].

  57. Sigfusson G, Ettedgui JA, Silverman NH, Anderson RH. Is a cleft in the anterior leaflet of an otherwise normal mitral valve an atrioventricular canal malformation?. J Am Coll Cardiol. Aug 1995;26(2):508-15. [Medline].

  58. Sim EK, Black MD, Smallhorn J, et al. Surgical repair of complete atrioventricular septal canal defects with absent posterior leaflet. Ann Thorac Surg. Nov 1995;60(5):1399-400. [Medline].

  59. Tedziagolska M. Two-patch repair of atrioventricular canal. Ann Thorac Surg. May 1996;61(5):1589-90. [Medline].

  60. Tennant SN, Hammon JW Jr, Bender HW Jr, et al. Familial clustering of atrioventricular canal defects. Am Heart J. Jul 1984;108(1):175-7. [Medline].

  61. Torfs CP, Christianson RE. Anomalies in Down syndrome individuals in a large population-based registry. Am J Med Genet. Jun 5 1998;77(5):431-8. [Medline].

  62. van Son JA, Kinzel P, Mohr FW. Repair of Ebstein's anomaly associated with partial atrioventricular canal. J Card Surg. Nov-Dec 1997;12(6):434-6. [Medline].

  63. van Son JA, Van Praagh R, Falk V, Mohr FW. Pericardial patch augmentation of the tissue-deficient mitral valve in common atrioventricular canal. J Thorac Cardiovasc Surg. Oct 1996;112(4):1117-9. [Medline].

  64. Yamaki S, Yasui H, Kado H, et al. Pulmonary vascular disease and operative indications in complete atrioventricular canal defect in early infancy. J Thorac Cardiovasc Surg. Sep 1993;106(3):398-405. [Medline].

  65. Zellers TM, Zehr R, Weinstein E, et al. Two-dimensional and Doppler echocardiography alone can adequately define preoperative anatomy and hemodynamic status before repair of complete atrioventricular septal defect in infants < 1 year old. J Am Coll Cardiol. Nov 15 1994;24(6):1565-70. [Medline].

 
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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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