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Atrial Septal Defect, Coronary Sinus: Differential Diagnoses & Workup

Author: Louis I Bezold, MD, Associate Professor, Department of Pediatrics, University of Kentucky College of Medicine; Chief, Division of Pediatric Cardiology, Medical Director, Kentucky Children's Hospital
Contributor Information and Disclosures

Updated: Nov 14, 2008

Differential Diagnoses

Atrial Septal Defect, Ostium Primum
Atrial Septal Defect, Ostium Secundum
Atrial Septal Defect, Patent Foramen Ovale
Atrial Septal Defect, Sinus Venosus

Workup

Laboratory Studies

  • No specific laboratory studies are required for the workup of isolated coronary sinus atrial septal defects (ASDs).

Imaging Studies

  • 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
    • 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.3
    • 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 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.4    
  • 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.3

Other Tests

  • ECG most commonly shows normal sinus rhythm in young patients, with an increasing frequency of sinus-node dysfunction with increasing age, beginning in childhood.
    • 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 is indicated in patients with a history of arrhythmias and before surgery in adults with newly diagnosed arrhythmias.

Procedures

  • 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.

More on Atrial Septal Defect, Coronary Sinus

Overview: Atrial Septal Defect, Coronary Sinus
Differential Diagnoses & Workup: Atrial Septal Defect, Coronary Sinus
Treatment & Medication: Atrial Septal Defect, Coronary Sinus
Follow-up: Atrial Septal Defect, Coronary Sinus
References
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References

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  2. McMahon CJ, Feltes TF, Fraley JK, et al. Natural history of growth of secundum atrial septal defects and implications for transcatheter closure. Heart. Mar 2002;87(3):256-9. [Medline]. [Full Text].

  3. Huang Xin-Sheng. Partially Unroofed Coronary Sinus. Circulation. October/2007;116(15):e373. [Medline].

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  5. Quaegebeur J, Kirklin JW, Pacifico AD, Bargeron LM Jr. Surgical experience with unroofed coronary sinus. Ann Thorac Surg. May 1979;27(5):418-25. [Medline].

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Further Reading

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Keywords

coronary sinus atrial septal defect, coronary sinus ASD, unroofed coronary sinus, interatrial shunting, persistent left superior vena cava draining to the coronary sinus, LSVC, heterotaxy syndrome, abnormalities of atrial situs, anomalies of systemic venous return, venous emboli, paradoxical emboli, communication between the right and left atria, congenital heart disease, CHD, cardiovascular disease, heart disease, tricuspid atresia, pulmonary atresia, right ventricular hypertrophy, mitral stenosis, atrial hypertension, pulmonary vascular disease, mitral valve prolapse, mitral regurgitation, ostium secundum ASD, exercise intolerance, tachycardia, bacterial endocarditis, failure to thrive, reactive airway disease

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Contributor Information and Disclosures

Author

Louis I Bezold, MD, Associate Professor, Department of Pediatrics, University of Kentucky College of Medicine; Chief, Division of Pediatric Cardiology, Medical Director, Kentucky Children's Hospital
Louis I Bezold, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Society of Echocardiography, and Society of Pediatric Echocardiography
Disclosure: Nothing to disclose.

Medical Editor

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.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

Managing Editor

Alvin J Chin, MD, Professor of Pediatrics, Division of Cardiology, The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine
Alvin J Chin, MD is a member of the following medical societies: American Association for the Advancement of Science and American Heart Association
Disclosure: Nothing to disclose.

CME Editor

Gilbert Herzberg, MD, Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College
Gilbert 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.

 
 
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