Pediatric Complete Atrioventricular Septal Defects Follow-up

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

Further Inpatient Care

Some children who have survived surgical repair of complete atrioventricular septal defect (AVSD) require prolonged hospitalization.

Causes are multifactorial but may include sepsis, pulmonary hypertension, residual left-to-right shunts through ventricular septal defects (VSDs), or significant atrioventricular valve insufficiency.

Associated noncardiac problems, including feeding difficulties, renal insufficiency, or pulmonary insufficiency, may require ongoing management and delayed discharge.

Next

Further Outpatient Care

As needed, outpatient care for nonsurgical patients with atrioventricular septal defect should focus on providing adequate nutrition and medications to lessen congestive heat failure (CHF) symptoms.

Outpatient care should prepare the child for surgical intervention at the age appropriate for the institution where the operation will be performed.

Postoperative outpatient care depends on any clinically significant residual problems.

Previous
Next

Inpatient & Outpatient Medications

Postoperative medications range from none to many of the medications used to treat CHF discussed in the Medication section.

Almost every child who has survived surgical repair of complete atrioventricular septal defect has some abnormality of an atrioventricular valve.

Antibiotic prophylaxis is recommended for patients during the first 6 months after complete repair and for patients who have a residual intracardiac shunt associated with prosthetic patch material.

Previous
Next

Transfer

Patients with complete atrioventricular septal defect should be transferred to an institution skilled in successfully treating patients with complex congenital heart disease.

Previous
Next

Complications

Postoperative complications include arrhythmias, low cardiac output, pulmonary hypertension, atrioventricular valve stenosis, and mitral insufficiency. Arrhythmias include heart block and junctional tachycardia; the latter usually subsides within 3-7 days after surgery. Postoperative left ventricular dysfunction may result in low cardiac output and even renal insufficiency. Inotropic drugs may be needed for several days after surgery.

In patients with pulmonary hypertension, sedation, paralysis, and mild hyperventilation with 100% oxygen may be required to prevent pulmonary hypertensive crisis and decreased right ventricular (RV) afterload. For pulmonary hypertension refractory to these measures, nitric oxide may be used to achieve pulmonary vasodilatation.

On occasion, patients may require long-term therapy, which might include phenoxybenzamine or calcium-channel–blocking drugs to manage an elevated pulmonary vascular resistance (PVR).

Severe mitral regurgitation may occur postoperatively and is ideally recognized on intraoperative transesophageal echocardiography (TEE). Residual mitral regurgitation is the most common indication for late reoperation after repair of complete atrioventricular septal defect. Approximately 5-10% of patients ultimately require mitral valve repair or replacment.[31, 32]

Anomalous attachment of atrioventricular valve tissue occasionally causes left ventricular (LV) outflow tract (LVOT) obstruction, in addition to the known tendency for patients with atrioventricular septal defect to have a small LVOT.[33] Such anomalous attachments may prevent complete relief of subaortic obstruction without mitral-valve replacement. Resection of some discrete obstructing tissue or, in some patients, a modified Konno procedure for tunnel-like LVOT obstruction or for obstruction caused by anomalous attachments of the mitral-valve apparatus may be performed. This procedure may complicate outflow-tract reconstruction and has had varied results.

A rare complication of the complete atrioventricular canal is subacute bacterial endocarditis. Successful repair during active endocarditis is reported.

Previous
Next

Prognosis

Without operation, the survival of patients with this lesion is poor. Death may occur during infancy secondary to heart failure or pneumonia. Death later in childhood results from progressive pulmonary vascular obstructive disease (PVOD). PVOD tends to develop more rapidly than in other congenital heart defects. Intimal fibrosis (Heath-Edwards grade 3 lesions) have been demonstrated to appear between age 6-12 months. Vascular dilation and plexiform lesions (Heath-Edward grade 4 lesions) may occur by age 1 year.[34] Evidence suggests that these changes develop earlier and progress more rapidly in infants with Down syndrome.

Risk factors for surgical and late mortality and morbidity are identified. Preoperative risk factors for mortality include small size, unbalanced ventricular size, New York Heart Association class IV, and severe atrioventricular valve insufficiency. The era of operation (before 1987), patient age at operation, presence of accessory atrioventricular valve orifices, and other congenital heart diseases also increase the surgical mortality risk. Down syndrome is surprisingly not an independent risk factor for morbidity and mortality and therefore should not limit intervention.

Infants can successfully undergo surgery, with a published mortality rate of 3.6% and with minimal long-term morbidity. Late survival is approximately 96%, and the reoperation rate is approximately 11%.

Published 10-year survival rates are 81-91%. Risk factors strongly associated with early death or the need for repeat operation include operation before 1987, postoperative pulmonary hypertensive crisis, immediate postoperative severe left atrioventricular valve regurgitation, and double-orifice left atrioventricular valve. In the past, death was significantly most common when complete atrioventricular septal defect with RV outflow tract obstruction was corrected in children younger than 5 years or weighing less than 15 kg. In the current era, most centers operate by age 6 months.

Previous
Next

Patient Education

For excellent patient education resources, visit eMedicine's Heart Center.

Previous
 
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].

 
Previous
Next
 
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.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
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.