Imaging Studies

The following imaging studies are helpful for the workup of isolated coronary sinus atrial septal defects (ASDs).

Chest radiography

Chest radiographic findings vary. Cardiac silhouette and pulmonary vascular markings are increased in proportion to the degree of left-to-right shunt. Pulmonary vascular markings may show peripheral extension with central prominence. Increased right atrial shadow and triangular-shaped cardiac silhouette may be observed.

Echocardiography

Note the following:

Transthoracic echocardiographic findings are diagnostic in most cases.
Transesophageal echocardiography is useful during defect repair, and findings may be diagnostic in relatively old or large patients with limited transthoracic acoustic windows.^{[4, 5]}
M-mode echocardiography shows right ventricular enlargement and flattened (sometimes paradoxical) septal motion. Two-dimensional imaging defines anatomic features well, and findings are diagnostic.
Subcostal views are most useful for defining the area of interatrial shunting characteristic of this defect and for assessing the degree of coronary sinus unroofing.
The apical 4-chamber view demonstrates the coronary sinus well but is unreliable for assessing the interatrial septum because of false septal drop-out.
The left superior vena cava (SVC) is best imaged from the suprasternal notch and subcostal views. Additional features include dilatation of the right atrium, right ventricle, and main pulmonary artery.
Care must be taken not to confuse either ostium primum ASD or sinus venosus defect of the inferior vena cava type with coronary sinus ASD.
Color and pulsed Doppler interrogation provides hemodynamic data regarding right-sided pressures, and the degree of shunting (Qp/Qs) can be estimated.
In infants and young children, echocardiography is the noninvasive method of choice to either rule out or further evaluate complex lesions in cases of heterotaxy associated with a coronary sinus defect.
Real-time 3-dimensional transthoracic echocardiography has been reported to be useful in diagnosing a surgically unroofed coronary sinus in a patient with poor acoustic windows; therefore, it may be useful in native coronary sinus ASD visualization.^[6]

Magnetic resonance imaging (MRI)

MRI may be useful, particularly in patients with heterotaxy syndrome or other complex anomalies incompletely defined by echocardiography.

Cineangiography

Optimal visualization of a coronary sinus ASD requires selective left SVC, right upper pulmonary vein, or left atrium contrast injection in the hepatoclavicular view. A coronary sinus defect may also be suggested by a catheter course through the coronary sinus into the left atrium and pulmonary vein.^[4]

Electrocardiography (ECG)

ECG most commonly shows normal sinus rhythm in young patients, with an increasing frequency of sinus-node dysfunction with increasing age, beginning in childhood. Righ ventricular hypertrophy, manifest by rsR', may be seen in moderate to large shunts.

A prolonged PR interval is relatively uncommon but not unusual.

Common findings include right-axis deviation, right atrial enlargement, and mild right ventricular enlargement manifested by an RSr' or rsR' pattern in precordial leads V3R and V1.

Holter monitoring

Holter monitoring is indicated in patients with a history of arrhythmias and before surgery in adults with newly diagnosed arrhythmias.

Cardiac catheterization

Cardiac catheterization is generally not necessary for diagnosis but may be necessary to evaluate hemodynamics in complicated cases, in patients with auscultatory or Doppler evidence of elevated pulmonary artery resistance, and in patients in whom transthoracic and transesophageal echocardiography is inconclusive.

Treatment & Management

Martin SS, Shapiro EP, Mukherjee M. Atrial septal defects - clinical manifestations, echo assessment, and intervention. Clin Med Insights Cardiol. 2014. 8 (Suppl 1):93-8. [QxMD MEDLINE Link]. [Full Text].
Attenhofer Jost CH, Connolly HM, Danielson GK, Dearani JA, Warnes CA, Jamil Tajik A. Clinical features and surgical outcome in 25 patients with fenestrations of the coronary sinus. Cardiol Young. 2007 Dec. 17(6):592-600. [QxMD MEDLINE Link].
McMahon CJ, Feltes TF, Fraley JK, et al. Natural history of growth of secundum atrial septal defects and implications for transcatheter closure. Heart. 2002 Mar. 87(3):256-9. [QxMD MEDLINE Link]. [Full Text].
Huang XS. Images in cardiovascular medicine. Partially unroofed coronary sinus. Circulation. 2007 Oct 9. 116(15):e373. [QxMD MEDLINE Link].
Roberson DA, Cui W, Patel D, et al. Three-dimensional transesophageal echocardiography of atrial septal defect: a qualitative and quantitative anatomic study. J Am Soc Echocardiogr. 2011 Jun. 24(6):600-10. [QxMD MEDLINE Link].
Singh A, Nanda NC, Romp RL, Kirklin JK. Assessment of surgically unroofed coronary sinus by live/real time three-dimensional transthoracic echocardiography. Echocardiography. 2007 Jan. 24(1):74-6. [QxMD MEDLINE Link].
Wang JK, Chen SJ, Hsu JY, et al. Midterm follow-up results of transcatheter treatment in patients with unroofed coronary sinus. Catheter Cardiovasc Interv. 2014 Feb. 83 (2):243-9. [QxMD MEDLINE Link].
Kijima Y, Taniguchi M, Akagi T. Catheter closure of coronary sinus atrial septal defect using Amplatzer Septal Occluder. Cardiol Young. 2011 Jul 26. 1-4. [QxMD MEDLINE Link].
Di Bernardo S, Fasnacht M, Berger F. Transcatheter closure of a coronary sinus defect with an Amplatzer septal occluder. Catheter Cardiovasc Interv. 2003 Oct. 60 (2):287-90. [QxMD MEDLINE Link].
Stiles S. FDA issues Amplatzer ASO occluder safety alert over erosions. Medscape. Available at http://www.medscape.com/viewarticle/812813. Oct 18, 2013; Accessed: Oct 28, 2013.
FDA. St. Jude Amplatzer atrial septal occluder (ASO): safety communication - reports of tissue erosion. US Food and Drug Administration. Available at http://www.fda.gov/Safety/MedWatch/SafetyInformation/SafetyAlertsforHumanMedicalProducts/ucm371202.htm. Oct 17, 2013; Accessed: Oct 28, 2013.
FDA. Rare serious erosion events associated with St. Jude Amplatzer atrial septal occluder (ASO). US Food and Drug Administration. Available at http://www.fda.gov/MedicalDevices/Safety/AlertsandNotices/ucm371145.htm. Accessed: Oct 28, 2013.
Rao PS, Harris AD. Recent advances in managing septal defects: atrial septal defects. F1000Res. 2017. 6:2042. [QxMD MEDLINE Link]. [Full Text].
Quaegebeur J, Kirklin JW, Pacifico AD, Bargeron LM Jr. Surgical experience with unroofed coronary sinus. Ann Thorac Surg. 1979 May. 27(5):418-25. [QxMD MEDLINE Link].
Andersen M, Moller I, Lyngborg K, Wennevold A. The natural history of small atrial septal defects; long-term follow-up with serial heart catheterizations. Am Heart J. 1976 Sep. 92(3):302-7. [QxMD MEDLINE Link].
Bierman FZ, Williams RG. Subxiphoid two-dimensional imaging of the interatrial septum in infants and neonates with congenital heart disease. Circulation. 1979 Jul. 60(1):80-90. [QxMD MEDLINE Link].
Cockerham JT, Martin TC, Gutierrez FR, et al. Spontaneous closure of secundum atrial septal defect in infants and young children. Am J Cardiol. 1983 Dec 1. 52(10):1267-71. [QxMD MEDLINE Link].
Foster ED, Baeza OR, Farina MF, Shaher RM. Atrial septal defect associated with drainage of left superior vena cava to left atrium and absence of the coronary sinus. J Thorac Cardiovasc Surg. 1978 Nov. 76(5):718-20. [QxMD MEDLINE Link].
Franz C, Mennicken U, Dalichau H, Hirsch H. Abnormal communication between the left atrium and the coronary sinus. Presentation of 2 cases and review of the literature. Thorac Cardiovasc Surg. 1985 Apr. 33(2):113-7. [QxMD MEDLINE Link].
Freedom RM, Culham JA, Rowe RD. Left atrial to coronary sinus fenestration (partially unroofed coronary sinus). Morphological and angiocardiographic observations. Br Heart J. 1981 Jul. 46(1):63-8. [QxMD MEDLINE Link].
Garson A, Neish S. Anomalies of systemic venous return. Bricker T, Fisher D, eds. The Science and Practice of Pediatric Cardiology. 2nd ed. Baltimore, MD: Williams and Wilkins; 1998. 1667-75.
Garson A, Neish S. Defects of the atrial septum including atrioventricular septal defects. Bricker T, Fisher D, eds. The Science and Practice of Pediatric Cardiology. 2nd ed. Baltimore, MD: Williams and Wilkins; 1998. 1141-79.
Garson, A, Neish S. Cardiac malposition and heterotaxy. Bricker T, Fisher D, eds. The Science and Practice of Pediatric Cardiology. 2nd ed. Baltimore, MD: Williams and Wilkins; 1997. 1539-61.
Graham TP Jr, Bricker JT, James FW, Strong WB. 26th Bethesda conference: recommendations for determining eligibility for competition in athletes with cardiovascular abnormalities. Task Force 1: congenital heart disease. J Am Coll Cardiol. 1994 Oct. 24(4):867-73. [QxMD MEDLINE Link].
Lee ME, Sade RM. Coronary sinus septal defect. Surgical considerations. J Thorac Cardiovasc Surg. 1979 Oct. 78(4):563-9. [QxMD MEDLINE Link].
Mantini E, Grondin CM, Lillehie CW. Congenital anomalies involving the coronary sinus. Circulation. 1966. 33:317.
Munoz-Castellanos L, Espinola-Zavaleta N, Kuri-Nivon M, et al. Atrial septal defect: anatomoechocardiographic correlation. J Am Soc Echocardiogr. 2006 Sep. 19(9):1182-9. [QxMD MEDLINE Link].
Murphy JG, Gersh BJ, McGoon MD, et al. Long-term outcome after surgical repair of isolated atrial septal defect. Follow-up at 27 to 32 years. N Engl J Med. 1990 Dec 13. 323(24):1645-50. [QxMD MEDLINE Link].
Perloff JK. Pregnancy and congenital heart disease. J Am Coll Cardiol. 1991 Aug. 18(2):340-2. [QxMD MEDLINE Link].
Raghib G, Ruttenberg HD, Anderson RC, et al. Termination of left superior vena cava in left atrium, atrial septal defect, and absence of coronary sinus; a developmental complex. Circulation. 1965 Jun. 31:906-18. [QxMD MEDLINE Link].
Sealy WC, Farmer JC, Young WG Jr, Brown IW Jr. Atrial dysrhythmia and atrial secundum defects. J Thorac Cardiovasc Surg. 1969 Feb. 57(2):245-50. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Troost E, Gewillig M, Budts W. Percutaneous closure of a persistent left superior vena cava connected to the left atrium. Int J Cardiol. 2006 Jan 26. 106(3):365-6. [QxMD MEDLINE Link].
Muthialu N, Fajardo D, Sullivan ID, Tsang V. Repair of persistent left superior vena cava to unroofed coronary sinus defect by retro-aortic implantation (modified Warden type procedure). J Card Surg. 2016 Feb. 31(2):103-5. [QxMD MEDLINE Link].
Kawamukai M, Muranaka A, Yuda S, et al. Utility of three-dimensional transesophageal echocardiography for diagnosis of unroofed coronary sinus. J Med Ultrason (2001). 2016 Jan. 43(1):91-4. [QxMD MEDLINE Link].
Kim HR, Yoo SM, Lee HY, et al. A case of complete unroofed coronary sinus syndrome combined with coronary sinus stenosis leading to asymptomatic presentation. Iran J Radiol. 2015 Jul. 12(3):e16063. [QxMD MEDLINE Link].
Mallula KK, Patel ND, Abdulla RI, Bokowski JW. Tetralogy of Fallot with left superior vena cava and coronary sinus atrial septal defect: a rare association. Pediatr Cardiol. 2015 Jun. 36(5):1100-1. [QxMD MEDLINE Link].
Mahmoud H, Nicolescu AM, Filip C, et al. Complex atrial septal defect closure in children. Rom J Morphol Embryol. 2019. 60(1):49-57. [QxMD MEDLINE Link]. [Full Text].