Highlights & Basics
- Interatrial communications (shunts between the atrial chambers) are often referred to as atrial septal defects (ASDs), although only secundum and vestibular defects are true defects within the atrial septum.
- Other atrial level shunts that result in similar physiologic effects are sinus venosus defects, ostium primum defects, and unroofed coronary sinus defects.
- Corrective closure is recommended if Qp:Qs (ratio of pulmonary blood flow to systemic blood flow) is ≥1.5.
- Sinus venosus defect with partial anomalous pulmonary drainage and unroofed coronary sinus (especially if associated with a persistent left superior vena cava) should be corrected irrespective of shunt volume.
- Correction is usually performed between ages 2 to 4 years, but can be done in younger patients if hemodynamically not well tolerated.
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Definition
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Images
Subtypes of interatrial communications. A: sinus venosus defect; B: ostium secundum defect; C: ostium primum defect; D: unroofed coronary sinus defect
Apical 4-chamber echocardiographic image demonstrating right ventricular enlargement in a patient with an interatrial communication. L: lateral; LV: left ventricle; RA: right atrium; RV: right ventricle; S: superior
Apical 4-chamber echocardiographic image of an ostium primum defect (arrows). LA: left atrium; LV: left ventricle; RA: right atrium
Parasternal short-axis echocardiographic image demonstrating right ventricular enlargement in a patient with an interatrial communication. LV: left ventricle; RV: right ventricle
Transesophageal echocardiographic image of an interatrial communication occluder device (arrow). LA: left atrium; RA: right atrium; SVC: superior vena cava
Citations
Jone PN, Ivy DD, Hauck A, et al. Pulmonary hypertension in congenital heart disease: a scientific statement from the American Heart Association. Circ Heart Fail. 2023 Jul;16(7):e00080.[Abstract][Full Text]
Baumgartner H, De Backer J, Babu-Narayan SV, et al; ESC Scientific Document Group. 2020 ESC guidelines for the management of adult congenital heart disease. Eur Heart J. 2021 Feb 11;42(6):563-645.[Abstract][Full Text]
Stout KK, Daniels CJ, Aboulhosn JA, et al. 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. Circulation. 2019 Apr 2;139(14):e698-800.[Abstract][Full Text]
Abrahamyan L, Dharma C, Alnasser S, et al. Long-term outcomes after atrial septal defect transcatheter closure by age and against population controls. JACC Cardiovasc Interv. 2021 Mar 8;14(5):566-75.[Abstract][Full Text]
1. Naqvi N, McCarthy KP, Ho SY. Anatomy of the atrial septum and interatrial communications. J Thorac Dis. 2018 Sep;10(suppl 24):S2837-47.[Abstract][Full Text]
2. Redington AN, Anderson RH, Spicer DE. Interatrial communications. In: Wernovsky G, Anderson RH, Kumar K, et al, eds. Anderson's Pediatric Cardiology. 4th edition. Philadelphia, PA: Elsevier; 2019.
3. Smallhorn JF, de Leval M, Stark J, et al. Isolated anterior mitral cleft. Two dimensional echocardiographic assessment and differentiation from "clefts" associated with atrioventricular septal defect. Br Heart J. 1982 Aug;48(2):109-16.[Abstract]
4. Kanani M, Anderson RH, Elliott MJ. Clinical and morphologic evidence points to closure of the zone of apposition in atrioventricular septal defects. Eur J Cardiothorac Surg. 2005 May;27(5):932-3.[Abstract]
5. al Zaghal AM, Li J, Anderson RH, et al. Anatomical criteria for the diagnosis of sinus venosus defects. Heart. 1997 Sep;78(3):298-304.[Abstract]
6. Yoo S-J, Lee S, de la Mora R, et al. (2022). New insights into sinus venosus defects from cross-sectional imaging. Congenital Heart Disease. 2022;17(1):5-23. [Full Text]
7. Loomba RS, Tretter JT, Mohun TJ, et al. Identification and morphogenesis of vestibular atrial septal defects. J Cardiovasc Dev Dis. 2020 Sep 10;7(3):35.[Abstract][Full Text]
8. John Sutton MG, Tajik AJ, McGoon DC. Atrial septal defects in patients ages 60 years or older: operative results and long-term postoperative follow-up. Circulation. 1981 Aug;64(2):402-9.[Abstract][Full Text]
9. Steele PM, Fuster V, Cohen M, et al. Isolated atrial septal defect with pulmonary vascular obstructive disease - long-term follow-up and prediction of outcome after surgical correction. Circulation. 1987 Nov;76(5):1037-42.[Abstract][Full Text]
10. Jone PN, Ivy DD, Hauck A, et al. Pulmonary hypertension in congenital heart disease: a scientific statement from the American Heart Association. Circ Heart Fail. 2023 Jul;16(7):e00080.[Abstract][Full Text]
11. van der Linde D, Konings EE, Slager MA, et al. Birth prevalence of congenital heart disease worldwide: a systematic review and meta-analysis. J Am Coll Cardiol. 2011 Nov 15;58(21):2241-7.[Abstract][Full Text]
12. Reller MD, Strickland MJ, Riehle-Colarusso T, et al. Prevalence of congenital heart defects in metropolitan Atlanta, 1998-2005. J Pediatr. 2008 Dec;153(6):807-13.[Abstract][Full Text]
13. Brida M, Chessa M, Celermajer D, et al. Atrial septal defect in adulthood: a new paradigm for congenital heart disease. Eur Heart J. 2022 Jul 21;43(28):2660-71.[Abstract]
14. Verheugt CL, Uiterwaal CS, van der Velde ET, et al. Gender and outcome in adult congenital heart disease. Circulation. 2008 Jul 1;118(1):26-32.[Abstract][Full Text]
15. Kuijpers JM, Mulder BJ, Bouma BJ. Secundum atrial septal defect in adults: a practical review and recent developments. Neth Heart J. 2015 Apr;23(4):205-11.[Abstract][Full Text]
16. Li QY, Newbury-Ecob RA, Terrett JA, et al. Holt-Oram syndrome is caused by mutation in TBX5, a member of the Brachyury (T) gene family. Nat Genet. 1997 Jan;15(1):21-9.[Abstract]
17. Srivastava D, Olson EN. A genetic blueprint for cardiac development. Nature. 2000 Sep 14;407(6801):221-6.[Abstract]
18. Ching YH, Ghosh TK, Cross SJ, et al. Mutation in myosin heavy chain 6 causes atrial septal defect. Nat Genet. 2005 Apr;37(4):423-8.[Abstract]
19. Harvey DC, Baer RJ, Bandoli G, et al. Association of alcohol use diagnostic codes in pregnancy and offspring conotruncal and endocardial cushion heart defects. J Am Heart Assoc. 2022 Jan 18;11(2):e022175.[Abstract][Full Text]
20. Parthiban A, Sachdeva R. Atrial septal defects. In: Shaddy RE, Penny DJ, Feltes TF, et al, eds. Moss and Adams' heart disease in infants, children, and adolescents: including the fetus and young adult. 10th edition. Philadelphia, PA: Lippincott Williams & Wilkins; 2021.
21. Butts RJ, Crean AM, Hlavacek AM, et al. Veno-venous bridges: the forerunners of the sinus venosus defect. Cardiol Young. 2011 Dec;21(6):623-30.[Abstract][Full Text]
22. Yang J, Qiu H, Qu P, et al. Prenatal alcohol exposure and congenital heart defects: a meta-analysis. PLoS One. 2015;10(6):e0130681.[Abstract][Full Text]
23. Baumgartner H, De Backer J, Babu-Narayan SV, et al; ESC Scientific Document Group. 2020 ESC guidelines for the management of adult congenital heart disease. Eur Heart J. 2021 Feb 11;42(6):563-645.[Abstract][Full Text]
24. Hamilton WT, Haffajee CI, Dalen JE, et al. Atrial septal defect secundum: clinical profile with physiologic correlates. In: Roberts WC, ed. Adult congenital heart disease. Philadelphia, PA: FA Davis; 1987:395-407.
25. Expert Panels on Cardiac Imaging and Pediatric Imaging, Krishnamurthy R, Suman G, et al. ACR Appropriateness Criteria® congenital or acquired heart disease. J Am Coll Radiol. 2023 Nov;20(11s):S351-81.[Abstract][Full Text]
26. Wasserman MA, Shea E, Cassidy C, et al. Recommendations for the adult cardiac sonographer performing echocardiography to screen for critical congenital heart disease in the newborn: from the American Society of Echocardiography. J Am Soc Echocardiogr. 2021 Mar;34(3):207-22.[Abstract][Full Text]
27. Stout KK, Daniels CJ, Aboulhosn JA, et al. 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. Circulation. 2019 Apr 2;139(14):e698-800.[Abstract][Full Text]
28. Silvestry FE, Cohen MS, Armsby LB, et al. Guidelines for the echocardiographic assessment of atrial septal defect and patent foramen ovale: from the American Society of Echocardiography and Society for Cardiac Angiography and Interventions. J Am Soc Echocardiogr. 2015 Aug;28(8):910-58.[Abstract][Full Text]
29. Butera G, Biondi-Zoccai G, Sangiorgi G, et al. Percutaneous versus surgical closure of secundum atrial septal defects: a systematic review and meta-analysis of currently available clinical evidence. EuroIntervention. 2011 Jul;7(3):377-85.[Abstract]
30. Vida VL, Zanotto L, Zanotto L, et al. Minimally invasive surgery for atrial septal defects: a 20-year experience at a single centre. Interact Cardiovasc Thorac Surg. 2019 Jun 1;28(6):961-7.[Abstract][Full Text]
31. Campbell M. Natural history of atrial septal defect. Br Heart J. 1970 Nov;32(6):820-6.[Abstract][Full Text]
32. Barst RJ, Rubin LJ, Long WA, et al. A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. N Engl J Med. 1996 Feb 1;334(5):296-301.[Abstract][Full Text]
33. Delgado V, Ajmone Marsan N, de Waha S, et al. 2023 ESC guidelines for the management of endocarditis. Eur Heart J. 2023 Oct 14;44(39):3948-4042.[Full Text]
34. John AS, Jackson JL, Moons P, et al. Advances in managing transition to adulthood for adolescents with congenital heart disease: a practical approach to transition program design: a scientific statement from the American Heart Association. J Am Heart Assoc. 2022 Apr 5;11(7):e025278.[Abstract][Full Text]
35. Marelli A, Beauchesne L, Colman J, et al. Canadian Cardiovascular Society 2022 guidelines for cardiovascular interventions in adults with congenital heart disease. Can J Cardiol. 2022 Jul;38(7):862-96.[Abstract][Full Text]
36. Fuller SM, He X, Jacobs JP, et al. Estimating mortality risk for adult congenital heart surgery: an analysis of The Society of Thoracic Surgeons Congenital Heart Surgery Database. Ann Thorac Surg. 2015 Nov;100(5):1728-35;discussion 1735-6.[Abstract][Full Text]
37. DiBardino DJ, McElhinney DB, Kaza AK, et al. Analysis of the US Food and Drug Administration Manufacturer and User Facility Device Experience database for adverse events involving Amplatzer septal occluder devices and comparison with the Society of Thoracic Surgery congenital cardiac surgery database. J Thorac Cardiovasc Surg. 2009 Jun;137(6):1334-41.[Abstract][Full Text]
38. Abrahamyan L, Dharma C, Alnasser S, et al. Long-term outcomes after atrial septal defect transcatheter closure by age and against population controls. JACC Cardiovasc Interv. 2021 Mar 8;14(5):566-75.[Abstract][Full Text]
39. McElhinney DB, Quartermain MD, Kenny D, et al. Relative risk factors for cardiac erosion following transcatheter closure of atrial septal defects: a case-control study. Circulation. 2016 May 3;133(18):1738-46.[Abstract][Full Text]
40. Prystowsky EN, Benson DW Jr, Fuster V, et al. Management of patients with atrial fibrillation: a statement for healthcare professionals from the Subcommittee on Electrocardiography and Electrophysiology, American Heart Association. Circulation. 1996 Mar 15;93(6):1262-77.[Abstract][Full Text]
