eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiology

Atrial Septal Defect, Sinus Venosus: Differential Diagnoses & Workup

Author: Gary M Satou, MD, FASE, Director, Pediatric Echocardiography, Mattel Children's Hospital at University of California at Los Angeles; Associate Clinical Professor, Department of Pediatrics, David Geffen School of Medicine at University of California at Los Angeles
Coauthor(s): Brian L Reemtsen, MD, Assistant Professor of Cardiothoracic Surgery, Keck School of Medicine, University of Southern California
Contributor Information and Disclosures

Updated: Jun 12, 2009

Differential Diagnoses

Atrial Septal Defect, Coronary Sinus
Partial Anomalous Pulmonary Venous Connection
Atrial Septal Defect, Ostium Primum
Pulmonary Stenosis, Valvar
Atrial Septal Defect, Ostium Secundum
Atrioventricular Septal Defect, Partial and Intermediate
Cor Triatriatum

Workup

Laboratory Studies

  • General laboratory studies are rarely helpful in sinus venosus atrial septal defect (ASD).

Imaging Studies

  • Chest radiography
    • Prominent right atrium
    • Prominent main pulmonary artery
    • Increased heart size and pulmonary vascularity
  • Echocardiography
    • Echocardiography (ECHO) reveals atrial septal defect and most of the pulmonary vein connections in most patients and is the diagnostic modality of choice.
    • Two-dimensional ECHO with color flow Doppler reveals the position and size of the defect and the presence of anomalous pulmonary venous drainage (in many of these cases). It also helps identify associated anomalies and reveals the left-to-right (or right-to-left) direction of flow and the degree of right ventricular overload.
    • In children with difficult transthoracic windows, or in older or larger patients, transesophageal echocardiography may be helpful in imaging the defect and pulmonary vein connections.3 In the current era, cardiac magnetic resonance angiography (MRA)/MRI may be alternatively used to complete the diagnostic information needed prior to surgery.

Other Tests

  • Electrocardiogram
    • Right ventricular hypertrophy predominates, with a lengthened PR interval and incomplete right bundle branch block (small rSR').
    • P wave morphology may demonstrate atrial enlargement.
  • Cardiac MRI/MRA
    • Atrial septal defect size and location are shown.
    • Excellent delineation of individual pulmonary vein connections can be identified.
    • Right ventricle enlargement and indexing to body surface area (BSA) is available if helpful.
    • Flow-quantification may also be performed.

Procedures

Cardiac catheterization is usually not required in the preoperative assessment of patients with sinus venosus atrial septal defect, but it may be considered in the following circumstances:

  • In any child in whom associated lesions are suspected or in whom pulmonary hypertension is suspected, catheterization is performed to measure pulmonary artery pressure and, if pulmonary resistance is elevated, the response to pulmonary vasodilators.
  • Adults who have the potential for associated coronary atherosclerotic lesions should undergo catheterization to exclude these abnormalities before surgical repair of the sinus venosus atrial septal defect.

Histologic Findings

  • Patients with pulmonary hypertension and advanced pulmonary vascular obstructive disease may exhibit histologic changes similar to those seen in pulmonary vascular disease. Specifically, these include intimal and medial hypertrophy and, in more advanced lesions, luminal occlusion.

More on Atrial Septal Defect, Sinus Venosus

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

  1. Alpendurada F, Wage R, Mohiaddin R. Evaluation of a sinus venosus atrial septal defect by magnetic resonance: a case report. Rev Port Cardiol. Oct 2008;27(10):1317-21. [Medline].

  2. [Guideline] Galie N, Torbicki A, Barst R, et al. Guidelines on diagnosis and treatment of pulmonary arterial hypertension. The Task Force on Diagnosis and Treatment of Pulmonary Arterial Hypertension of the European Society of Cardiology. Eur Heart J. Dec 2004;25(24):2243-78. [Medline]. [Full Text].

  3. Crystal MA, Al Najashi K, Williams WG, Redington AN, Anderson RH. Inferior sinus venosus defect: echocardiographic diagnosis and surgical approach. J Thorac Cardiovasc Surg. Jun 2009;137(6):1349-55. [Medline].

  4. Warden HE, Gustafson RA, Tarnay TJ, Neal WA. An alternative method for repair of partial anomalous pulmonary venous connection to the superior vena cava. Ann Thorac Surg. Dec 1984;38(6):601-5. [Medline].

  5. Gustafson RA, Warden HE, Murray GF. Partial anomalous pulmonary venous connection to the superior vena cava. Ann Thorac Surg. Dec 1995;60(6 Suppl):S614-7. [Medline].

  6. Shahriari A, Rodefeld MD, Turrentine MW, Brown JW. Caval division technique for sinus venosus atrial septal defect with partial anomalous pulmonary venous connection. Ann Thorac Surg. Jan 2006;81(1):224-9; discussion 229-30. [Medline].

  7. Black MD, Pike N, Tede N, Popper R. Video-enhanced repair of sinus venosus atrial defects: with/without anomalous pulmonary venous drainage. Heart Surg Forum. 2003;6 S1:S28. [Medline].

  8. Campbell M. Natural history of atrial septal defect. Br Heart J. Nov 1970;32(6):820-6. [Medline].

  9. Driscoll DJ. Left-to-right shunt lesions. Pediatr Clin North Am. Apr 1999;46(2):355-68, x. [Medline].

  10. Freed MD, Nadas AS, Norwood WI, Castaneda AR. Is routine preoperative cardiac catheterization necessary before repair of secundum and sinus venosus atrial septal defects?. J Am Coll Cardiol. Aug 1984;4(2):333-6. [Medline].

  11. Fukazawa M, Fukushige J, Ueda K. Atrial septal defects in neonates with reference to spontaneous closure. Am Heart J. Jul 1988;116(1 Pt 1):123-7. [Medline].

  12. Kyger ER 3rd, Frazier OH, Cooley DA, et al. Sinus venosus atrial septal defect: early and late results following closure in 109 patients. Ann Thorac Surg. Jan 1978;25(1):44-50. [Medline].

  13. Li J, Al Zaghal AM, Anderson RH. The nature of the superior sinus venosus defect. Clin Anat. 1998;11(5):349-52. [Medline].

  14. Mas MS, Bricker JT. Clinical Physiology of Left-to-Right Shunts. In: Garson A, Bricker JT, McNamara DG, eds. The Science and Practice of Pediatric Cardiology. Vol 2. Lippincott Williams & Wilkins; 1990:999-1001.

  15. 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. Dec 13 1990;323(24):1645-50. [Medline].

  16. Radzik D, Davignon A, van Doesburg N, et al. Predictive factors for spontaneous closure of atrial septal defects diagnosed in the first 3 months of life. J Am Coll Cardiol. Sep 1993;22(3):851-3. [Medline].

  17. Sachweh JS, Daebritz SH, Hermanns B, et al. Hypertensive pulmonary vascular disease in adults with secundum or sinus venosus atrial septal defect. Ann Thorac Surg. Jan 2006;81(1):207-13. [Medline].

  18. Walker RE, Mayer JE, Alexander ME, et al. Paucity of sinus node dysfunction following repair of sinus venosus defects in children. Am J Cardiol. May 15 2001;87(10):1223-6; A8. [Medline].

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Further Reading

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Keywords

sinus venosus, atrial septal defect, ASD, superior vena cava type subcaval ASD, SVASD, atrial septum, congenital heart defect, congenital cardiac anomaly, congestive heart failure, murmur, treatment, diagnosis, heart problems, heart disease, heart anomaly

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Contributor Information and Disclosures

Author

Gary M Satou, MD, FASE, Director, Pediatric Echocardiography, Mattel Children's Hospital at University of California at Los Angeles; Associate Clinical Professor, Department of Pediatrics, David Geffen School of Medicine at University of California at Los Angeles
Gary M Satou, MD, FASE is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Society of Echocardiography, and Society of Pediatric Echocardiography
Disclosure: Nothing to disclose.

Coauthor(s)

Brian L Reemtsen, MD, Assistant Professor of Cardiothoracic Surgery, Keck School of Medicine, University of Southern California
Brian L Reemtsen, MD is a member of the following medical societies: American Medical Association, Society of Thoracic Surgeons, and Western Thoracic Surgical Association
Disclosure: Nothing to disclose.

Medical Editor

Charles I Berul, MD, Associate Professor of Pediatrics, Harvard Medical School; Senior Associate, Department of Cardiology, Children's Hospital of Boston
Charles I Berul, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, and Society for Pediatric Research
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 financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

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 Z Herzberg, MD, Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College; Consulting Staff, Department of Pediatrics, Sound Shore Medical Center
Gilbert Z Herzberg, MD is a member of the following medical societies: American Academy of Pediatrics
Disclosure: Nothing to disclose.

Chief Editor

Stuart Berger, MD, Professor of Pediatrics, Division of Cardiology, Medical College of Wisconsin; Chief of Pediatric Cardiology, Medical Director of Pediatric Heart Transplant Program, Medical Director of The Heart Center, Children's Hospital of Wisconsin
Stuart Berger, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American College of Chest Physicians, American Heart Association, and Society for Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.

 
 
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