Skip to main content

Abstract

Atrial septal defect (ASD) is a communication between both atria that allows blood shunting across the defect. It’s one of the common congenital defects with 6–10% as a single defect and in 30% associated with complex lesions [1]. There are several types based on the site of the defect at the interatrial septum (IAS); Fig. 2.1 demonstrates the anatomical location of the different types. Secundum ASD represents the most common type with a defect in the septum primum. Primum ASD is due to a defect in the endocardial cushions and accounts for a partial atrioventricular (AV) canal defect. Sinus venosus ASD is due to a defect in the atrial septum at the site of infolding of the vena cava; it could be superior at the superior vena cava (SVC) and is usually accompanied by partial anomalous pulmonary venous drainage with the right upper pulmonary veins entering into the right atrium at the site of the defect. Less common is inferior sinus venosus ASD at the site of entrance of the inferior vena cava (IVC). The least common type is coronary sinus defect where it opens into the left atrium [1].

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 84.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 109.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Attie F, Rosas M, Granados N, Buendia A, Zabal C, Calderon J. Anatomical closure for secundum atrial septal defect in patients aged over 40 years. A randomised clinical trial. JACC. 2001;38(7):2035–42.

    Article  CAS  PubMed  Google Scholar 

  2. Brochu M-C, Baril J-F, Dore A, Juneau M, De Guise P, Mercier L-A. Improvement in exercise capacity in asymptomatic and mildly symptomatic adults after atrial septal defect percutaneous closure. Circulation. 2002;106:1821–6.

    Article  PubMed  Google Scholar 

  3. Du ZD, Hijazi ZM, Kleinman CS, Silverman NH, Larntz K, Investigators Amplatzer. Comparison between transcatheter and surgical closure of secundum atrial septal defect in children and adults: results of a multicenter non-randomized trial. J Am Coll Cardiol. 2002;39:1836–44.

    Article  PubMed  Google Scholar 

  4. Gatzoulis MA, Freeman MA, Siu SC, Webb GD, Harris L. Atrial arrhythmia after surgical closure of atrial septal defects in adults. N Engl J Med. 1999;40:839–46.

    Article  Google Scholar 

  5. Kidd L, Driscoll DJ, Gersony WM, Hayes CJ, Keane JF, O’Fallon WM, Pieroni DR, Wolfe RR, Weidman WH. Second natural history study of congenital heart defects. Results of treatment of patients with ventricular septal defects. Circulation. 1993;87:38–51.

    Google Scholar 

  6. Rhodes LA, Keane JF, Keane JP, Fellows KE, Jonas RA, Castaneda AR, Nadas AS. Long follow-up (to 43 years) of ventricular septal defect with audible aortic regurgitation. Am J Cardiol. 1990;66:340–5.

    Article  CAS  PubMed  Google Scholar 

  7. Menting ME, Cuypers JA, Opic P, Utens EM, Witsenburg M, van den Bosch AE, van Domburg RT, Meijboom FJ, Boersma E, Bogers AJ, Roos-Hesselink JW. The unnatural history of the ventricular septal defect: outcome up to 40 years after surgical closure. J Am Coll Cardiol. 2015;65:1941–51.

    Article  PubMed  Google Scholar 

  8. Oliver JM, Garrido A, Gonzalez A, Benito F, Mateos M, Aroca A, Sanz E. Rapid progression of midventricular obstruction in adults with double-chambered right ventricle. J Thorac Cardiovasc Surg. 2003;126:711–7.

    Article  PubMed  Google Scholar 

  9. Rigby ML, Redington AN. Primary transcatheter umbrella closure of perimembranous ventricular septal defect. Br Heart J. 1994;72:368–71.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  10. Engelfriet P, Boersma E, Oechslin E, Tijssen J, Gatzoulis MA, Thilen U, Kaemmerer H, Moons P, Meijboom F, Popelova J, Laforest V, Hirsch R, Daliento L, Thaulow E, Mulder B. The spectrum of adult congenital heart disease in Europe: morbidity and mortality in a 5 year follow-up period. The Euro Hear Survey on adult congenital heart disease. Eur Heart J. 2005;26:2325–33.

    Article  PubMed  Google Scholar 

  11. Bando K, Turrentine MW, Sun K, Sharp TG, Ensing GJ, Miller AP, Kesler KA, Binford RS, Carlos GN, Hurwitz RA, Caldwell RL, Darragh RK, Hubbard J, Cordes TM, Girod DA, King H, Brown JW. Surgical management of complete atrioventricular septal defects. A twenty-year experience. J Thorac Cardiovasc Surg. 1995;110:1543–52; discussion 1552.

    Article  CAS  PubMed  Google Scholar 

  12. Gatzoulis MA, Hechter S, Webb GD, Williams WG. Surgery for partial atrioventricular septal defect in the adult. Ann Thorac Surg. 1999;67:504510.

    Article  Google Scholar 

  13. Michielon G, Stellin G, Rizzoli G, Milanesi O, Rubino M, Moreolo GS, Casarotto D. Left atrioventricular valve incompetence after repair of common atrioventricular canal defects. Ann Thorac Surg. 1995;60:S604–9.

    Article  CAS  PubMed  Google Scholar 

  14. El-Najdawi EK, Driscoll DJ, Puga FJ, Dearani JA, Spotts BE, Mahoney DW, Danielson GK. Operation for partial atrioventricular septal defect: a forty-year review. J Thorac Cardiovasc Surg. 2000;119:880–9; discussion 889–890.

    Article  CAS  PubMed  Google Scholar 

  15. Rosenhek R, Binder T, Porenta G, Lang I, Christ G, Schemper M, Maurer G, Baumgartner H. Predictors of outcome in severe, asymptomatic aortic stenosis. N Engl J Med. 2000;343:611–7.

    Article  CAS  PubMed  Google Scholar 

  16. Tzemos N, Therrien J, Yip J, Thanassoulis G, Tremblay S, Jamorski MT, Webb GD, Siu SC. Outcomes in adults with bicuspid aortic valves. JAMA. 2008;300:1317–25.

    Article  CAS  PubMed  Google Scholar 

  17. Urban Z, Zhang J, Davis EC, Maeda GK, Kumar A, Stalker H, Belmont JW, Boyd CD, Wallace MR. Supravalvular aortic stenosis: genetic and molecular dissection of a complex mutation in the elastin gene. Hum Genet. 2001;109:512–20.

    Article  CAS  PubMed  Google Scholar 

  18. Baumgartner H, Hung J, Bermejo J, Chambers JB, Edvardsen T, Goldstein S, Lancellotti P, LeFevre M, Miller F Jr, Otto CM. Recommendations on the echo-cardiographic assessment of aortic valve stenosis: a focused update from the European Association of Cardiovascular Imaging and the American Society of Echocardiography. Eur Heart J Cardiovasc Imaging. 2017;18:254–75.

    Article  PubMed  Google Scholar 

  19. van der Linde D, Takkenberg JJ, Rizopoulos D, Heuvelman HJ, Budts W, van Dijk AP, Witsenburg M, Yap SC, Bogers AJ, Silversides CK, Oechslin EN, Roos-Hesselink JW. Natural history of discrete subaortic stenosis in adults: a multi-Centre study. Eur Heart J. 2013;34:1548–56.

    Article  PubMed  Google Scholar 

  20. Padang R, Dennis M, Semsarian C, Bannon PG, Tanous DJ, Celermajer DS, Puranik R. Detection of serious complications by MR imaging in asymptomatic young adults with repaired coarctation of the aorta. Heart Lung Circ. 2014;23:332–8.

    Article  PubMed  Google Scholar 

  21. Fisher RG, Moodie DS, Sterba R, Gill CC. Patent ductus arteriosus in adults- long-term follow-up: nonsurgical versus surgical treatment. J Am Coll Cardiol. 1986;8:280–4.

    Article  CAS  PubMed  Google Scholar 

  22. Vejlstrup N, Sorensen K, Mattsson E, Thilen U, Kvidal P, Johansson B, Iversen K, Sondergaard L, Dellborg M, Eriksson P. Long-term outcome of Mustard/Senning correction for transposition of the great arteries in Sweden and Denmark. Circulation. 2015;132:633–8.

    Article  PubMed  Google Scholar 

  23. Zaragoza-Macias E, Zaidi AN, Dendukuri N, Marelli A. Medical therapy for systemic right ventricles: a systematic review (part 1) for the 2018 AHA/ACC Guideline for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. J Am Coll Cardiol. 2019;73:1564–78.

    Article  PubMed  Google Scholar 

  24. Kempny A, Wustmann K, Borgia F, Dimopoulos K, Uebing A, Li W, Chen SS, Piorkowski A, Radley-Smith R, Yacoub MH, Gatzoulis MA, Shore DF, Swan L, Diller GP. Outcome in adult patients after arterial switch operation for transposition of the great arteries. Int J Cardiol. 2013;167:2588–93.

    Article  PubMed  Google Scholar 

  25. Caldarone CA, McCrindle BW, Van Arsdell GS, Coles JG, Webb G, Freedom RM, Williams WG. Independent factors associated with longevity of prosthetic pulmonary valves and valved conduits. J Thorac Cardiovasc Surg. 2000;120:10221030; discussion 1031.

    Article  Google Scholar 

  26. Nakamura A, Horigome H, Seo Y, Ishizu T, Sumazaki R. Right ventricular remodeling due to pulmonary regurgitation is associated with reduced left ventricular free wall strain in surgically repaired tetralogy of fallot. Circ J. 2014;78:1960–6.

    Article  PubMed  Google Scholar 

  27. Babu-Narayan SV, Diller GP, Gheta RR, Bastin AJ, Karonis T, Li W, Pennell DJ, Uemura H, Sethia B, Gatzoulis MA, Shore DF. Clinical outcomes of surgical pulmonary valve replacement after repair of tetralogy of Fallot and potential prognostic value of preoperative cardiopulmonary exercise testing. Circulation. 2014;129:18–27.

    Article  PubMed  Google Scholar 

  28. Freling HG, Willems TP, van Melle JP, van Slooten YJ, Bartelds B, Berger RM, van Veldhuisen DJ, Pieper PG. Effect of right ventricular outflow tract obstruction on right ventricular volumes and exercise capacity in patients with repaired tetralogy of Fallot. Am J Cardiol. 2014;113:719–23.

    Article  PubMed  Google Scholar 

  29. Samad MD, Wehner GJ, Arbabshirani MR, Jing L, Powell AJ, Geva T, Haggerty CM, Fornwalt BK. Predicting deterioration of ventricular function in patients with repaired tetralogy of Fallot using machine learning. Eur Heart J Cardiovas Imag. 2018;19:730–8.

    Article  Google Scholar 

  30. Wald RM, Valente AM, Marelli A. Heart failure in adult congenital heart disease: emerging concepts with a focus on tetralogy of Fallot. Trends Cardiovasc Med. 2015;25:422–32.

    Article  PubMed  Google Scholar 

  31. Presnell LB, Blankenship A, Cheatham SL, Owens GE, Staveski SL. An overview of pulmonary atresia and major aortopulmonary collateral arteries. World J Pediatr Congenit Heart Surg. 2015;6:630–9.

    Article  PubMed  Google Scholar 

  32. Khairy P, Poirier N, Mercier LA. Univentricular heart. Circulation. 2007;115:800–12.

    Article  PubMed  Google Scholar 

  33. Ro PS, Rychik J, Cohen MS, Mahle WT, Rome JJ. Diagnostic assessment before Fontan operation in patients with bidirectional cavopulmonary anastomosis: are noninvasive methods sufficient? J Am Coll Cardiol. 2004;44:184–7.

    Article  PubMed  Google Scholar 

  34. Khairy P, Fernandes SM, Mayer JE Jr, Triedman JK, Walsh EP, Lock JE, Landzberg MJ. Long-term survival, modes of death, and predictors of mortality in patients with Fontan surgery. Circulation. 2008;117:85–92.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Michael A. Gatzoulis .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2022 The Author(s), under exclusive license to Springer Nature Switzerland AG

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Hammad, B.A., Gatzoulis, M.A. (2022). Cardiac Defects. In: Flocco, S.F., Habibi, H., Dellafiore, F., Sillman, C. (eds) Guide for Advanced Nursing Care of the Adult with Congenital Heart Disease. Springer, Cham. https://doi.org/10.1007/978-3-031-07598-8_2

Download citation

  • DOI: https://doi.org/10.1007/978-3-031-07598-8_2

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-031-07597-1

  • Online ISBN: 978-3-031-07598-8

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics