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Archives of Disease in Childhood logoLink to Archives of Disease in Childhood
. 1999 Aug;81(2):151–154. doi: 10.1136/adc.81.2.151

The effect of changing attitudes to Down's syndrome in the management of complete atrioventricular septal defects

K Amark 1, J Sunnegardh 1
PMCID: PMC1718042  PMID: 10490525

Abstract

OBJECTIVES—To describe the evaluation, decision making, and care of children with a complete atrioventricular septal defect (CAVSD).
STUDY DESIGN—Retrospective study of 136 consecutive cases from 1970 to 1996.
RESULTS—A total of 115 (85%) children had Down's syndrome. Denial of surgery without obvious medical reasons was more common in the early years, as was parental refusal of offered surgery and institutional care of the children. Improved results in later years encouraged surgical treatment for all these patients, but more liberal attitudes towards patients with Down's syndrome preceded the improved results. The use of echocardiography as a screening method for all newborns with Down's syndrome made it possible to plan for correction within the 1st months of life.
CONCLUSIONS—Changing attitudes in society and widespread use of echocardiography have significantly improved the management of children with a CAVSD and Down's syndrome.


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Selected References

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  1. Alexi-Meskishvili V., Ishino K., Dähnert I., Uhlemann F., Weng Y., Lange P. E., Hetzer R. Correction of complete atrioventricular septal defects with the double-patch technique and cleft closure. Ann Thorac Surg. 1996 Aug;62(2):519–525. [PubMed] [Google Scholar]
  2. Backer C. L., Mavroudis C., Alboliras E. T., Zales V. R. Repair of complete atrioventricular canal defects: results with the two-patch technique. Ann Thorac Surg. 1995 Sep;60(3):530–537. doi: 10.1016/0003-4975(95)00468-Z. [DOI] [PubMed] [Google Scholar]
  3. Bando K., Turrentine M. W., Sun K., Sharp T. G., Ensing G. J., Miller A. P., Kesler K. A., Binford R. S., Carlos G. N., Hurwitz R. A. Surgical management of complete atrioventricular septal defects. A twenty-year experience. J Thorac Cardiovasc Surg. 1995 Nov;110(5):1543–1554. doi: 10.1016/S0022-5223(95)70078-1. [DOI] [PubMed] [Google Scholar]
  4. Chin A. J., Keane J. F., Norwood W. I., Castaneda A. R. Repair of complete common atrioventricular canal in infancy. J Thorac Cardiovasc Surg. 1982 Sep;84(3):437–445. [PubMed] [Google Scholar]
  5. Di Carlo D., Marino B. Patient selection for repair of complete atrioventricular canal guided by echocardiography. J Am Coll Cardiol. 1995 Aug;26(2):574–575. doi: 10.1016/0735-1097(95)80038-i. [DOI] [PubMed] [Google Scholar]
  6. Greenwood R. D., Rosenthal A., Parisi L., Fyler D. C., Nadas A. S. Extracardiac abnormalities in infants with congenital heart disease. Pediatrics. 1975 Apr;55(4):485–492. [PubMed] [Google Scholar]
  7. Hagler D. J., Tajik A. J., Seward J. B., Mair D. D., Ritter D. G. Real-time wide-angle sector echocardiography: atrioventricular canal defects. Circulation. 1979 Jan;59(1):140–150. doi: 10.1161/01.cir.59.1.140. [DOI] [PubMed] [Google Scholar]
  8. Hanley F. L., Fenton K. N., Jonas R. A., Mayer J. E., Cook N. R., Wernovsky G., Castaneda A. R. Surgical repair of complete atrioventricular canal defects in infancy. Twenty-year trends. J Thorac Cardiovasc Surg. 1993 Sep;106(3):387–397. [PubMed] [Google Scholar]
  9. Julian-Reynier C., Aurran Y., Dumaret A., Maron A., Chabal F., Giraud F., Aymé S. Attitudes towards Down's syndrome: follow up of a cohort of 280 cases. J Med Genet. 1995 Aug;32(8):597–599. doi: 10.1136/jmg.32.8.597. [DOI] [PMC free article] [PubMed] [Google Scholar]
  10. Karlberg P., Taranger J., Engström I., Karlberg J., Landström T., Lichtenstein H., Lindstrom B., Svennberg-Redegren I. I. physical growth from birth to 16 years and longitudinal outcome of the study during the same age period. Acta Paediatr Scand Suppl. 1976;(258):7–76. doi: 10.1111/j.1651-2227.1976.tb14761.x. [DOI] [PubMed] [Google Scholar]
  11. Källén B., Mastroiacovo P., Robert E. Major congenital malformations in Down syndrome. Am J Med Genet. 1996 Oct 16;65(2):160–166. doi: 10.1002/(SICI)1096-8628(19961016)65:2<160::AID-AJMG16>3.0.CO;2-O. [DOI] [PubMed] [Google Scholar]
  12. Marino B., Corno A., Carotti A., Pasquini L., Giannico S., Guccione P., Bevilacqua M., De Simone G., Marcelletti C. Pediatric cardiac surgery guided by echocardiography. Established indications and new trends. Scand J Thorac Cardiovasc Surg. 1990;24(3):197–201. doi: 10.3109/14017439009098069. [DOI] [PubMed] [Google Scholar]
  13. Newfeld E. A., Sher M., Paul M. H., Nikaidoh H. Pulmonary vascular disease in complete atrioventricular canal defect. Am J Cardiol. 1977 May 4;39(5):721–726. doi: 10.1016/s0002-9149(77)80135-5. [DOI] [PubMed] [Google Scholar]
  14. Santoro G., Marino B., Di Carlo D., Formigari R., Santoro G., Marcelletti C., Pasquini L. Patient selection for repair of complete atrioventricular canal guided by echocardiography. Eur J Cardiothorac Surg. 1996;10(6):439–442. doi: 10.1016/s1010-7940(96)80112-6. [DOI] [PubMed] [Google Scholar]
  15. Schneider D. S., Zahka K. G., Clark E. B., Neill C. A. Patterns of cardiac care in infants with Down syndrome. Am J Dis Child. 1989 Mar;143(3):363–365. doi: 10.1001/archpedi.1989.02150150121030. [DOI] [PubMed] [Google Scholar]
  16. Silverman N. H., Zuberbuhler J. R., Anderson R. H. Atrioventricular septal defects: cross-sectional echocardiographic and morphologic comparisons. Int J Cardiol. 1986 Dec;13(3):309–331. doi: 10.1016/0167-5273(86)90117-8. [DOI] [PubMed] [Google Scholar]
  17. Smallhorn J. F., Tommasini G., Anderson R. H., Macartney F. J. Assessment of atrioventricular septal defects by two dimensional echocardiography. Br Heart J. 1982 Feb;47(2):109–121. doi: 10.1136/hrt.47.2.109. [DOI] [PMC free article] [PubMed] [Google Scholar]
  18. Sondheimer H. M., Byrum C. J., Blackman M. S. Unequal cardiac care for children with Down's syndrome. Am J Dis Child. 1985 Jan;139(1):68–70. doi: 10.1001/archpedi.1985.02140030070032. [DOI] [PubMed] [Google Scholar]
  19. Studer M., Blackstone E. H., Kirklin J. W., Pacifico A. D., Soto B., Chung G. K., Kirklin J. K., Bargeron L. M., Jr Determinants of early and late results of repair of atrioventricular septal (canal) defects. J Thorac Cardiovasc Surg. 1982 Oct;84(4):523–542. [PubMed] [Google Scholar]
  20. WOOD P. The Eisenmenger syndrome or pulmonary hypertension with reversed central shunt. I. Br Med J. 1958 Sep 20;2(5098):701–709. doi: 10.1136/bmj.2.5098.701. [DOI] [PMC free article] [PubMed] [Google Scholar]
  21. Weintraub R. G., Brawn W. J., Venables A. W., Mee R. B. Two-patch repair of complete atrioventricular septal defect in the first year of life. Results and sequential assessment of atrioventricular valve function. J Thorac Cardiovasc Surg. 1990 Feb;99(2):320–326. [PubMed] [Google Scholar]
  22. Wilson L., Curtis A., Korenberg J. R., Schipper R. D., Allan L., Chenevix-Trench G., Stephenson A., Goodship J., Burn J. A large, dominant pedigree of atrioventricular septal defect (AVSD): exclusion from the Down syndrome critical region on chromosome 21. Am J Hum Genet. 1993 Dec;53(6):1262–1268. [PMC free article] [PubMed] [Google Scholar]
  23. Yamaki S., Yasui H., Kado H., Yonenaga K., Nakamura Y., Kikuchi T., Ajiki H., Tsunemoto M., Mohri H. Pulmonary vascular disease and operative indications in complete atrioventricular canal defect in early infancy. J Thorac Cardiovasc Surg. 1993 Sep;106(3):398–405. [PubMed] [Google Scholar]
  24. Zellers T. M., Zehr R., Weinstein E., Leonard S., Ring W. S., Nikaidoh H. 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. 1994 Nov 15;24(6):1565–1570. doi: 10.1016/0735-1097(94)90156-2. [DOI] [PubMed] [Google Scholar]

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