Sinus Venosus Atrial Septal Defects 

  • Author: Gary M Satou, MD, FASE; Chief Editor: Stuart Berger, MD   more...
 
Updated: Mar 30, 2012
 

Background

In simple terms, an atrial septal defect (ASD) is a deficiency of the atrial septum. Atrial septal defects account for about 10-15% of all congenital cardiac anomalies and are the most common congenital cardiac lesion presenting in adults.[1] Sinus venosus atrial septal defects account for only 10% of atrial septal defects. The remaining atrial septal defects are ostium secundum type (70%), ostium primum type (20%), and unroofed coronary sinus, or coronary sinus septal defects, (< 1%). Most children with sinus venosus atrial septal defects are asymptomatic but may develop symptoms as they age.

Excellent surgical results with a mortality rate near 0% can be expected. This is particularly true in patients who undergo repair when younger than 15 years. An atrial septal defect was the first lesion repaired using cardiopulmonary bypass in 1954 by John Gibbon, MD, at the Mayo Clinic.

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Pathophysiology

The more common sinus venosus type defect (often referred to as the "usual type") occurs in the upper atrial septum and is contiguous with the superior vena cava (SVC). The lesion is rostral and posterior to the fossa ovalis (where secundum type defects occur) and is separate from it. It is almost always associated with anomalous pulmonary venous drainage of the right upper pulmonary vein into the SVC. See the image below.

Panel A. Transesophageal echocardiogram (transversPanel A. Transesophageal echocardiogram (transverse view) of a patient with a sinus venosus defect of the superior vena cava (SVC) type. The original defect (white star burst) has been repaired by placing a baffle (arrows), which directs blood from the anomalously connected right upper pulmonary vein into the left atrium (LA). In this patient, the baffle was redundant so at a more rostral level (Panel B), it could be seen (black open arrows) to bulge into the superior vena cava (SVC)–right atrial (RA) junction (trio of white arrows). The remainder of the atrial septum is denoted by the duo of white open arrows. Panel C is a transesophageal echocardiogram, sagittal view. Doppler color flow mapping verifies that the protruding baffle (white closed arrows) results in a narrowing of the pathway from the SVC to the RA. The quartet of white open arrows points to the remainder of the atrial septum.

Less commonly, the defect may occur at the junction of the right atrium and inferior vena cava (IVC) and be associated with anomalous connection of the right lower pulmonary vein to the IVC. Rarely, sinus venosus defects occur posterior to the fossa ovalis without bordering the SVC or IVC. The predominant hemodynamic consequence is a left-to-right shunt through the defect. See the image below.

Panel A is a transesophageal echocardiogram, transPanel A is a transesophageal echocardiogram, transverse view. The white star burst shows the sinus venosus defect of the inferior vena cava (IVC) type, lying adjacent to the IVC junction with the right atrium (RA). The remainder of the atrial septum is just out of the view of this sector but is represented by the white open arrowheads. The leaflets of the closed tricuspid valve (TV) are visible. RV = right ventricle. Panel B is a transesophageal echocardiogram, sagittal view. This is the same patient as in Panel A. This view proves that the rostral portion of the atrial septum (which would be missing in a patient with a sinus venosus defect of the SVC type) is intact. ct = crista terminalis; svc = superior vena cava.
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Epidemiology

Frequency

United States

Sinus venosus defects represent approximately 1% of congenital cardiac lesions.

Mortality/Morbidity

Surgical repair in the first 2 decades of life is associated with a mortality rate near zero. Life expectancy approaches that of the general population if the defect is repaired during this time. Cardiac size rapidly regresses after surgery, and the functional result is excellent. In cases of repair during adulthood, life expectancy may be decreased despite successful repair. Surgical morbidity rates are related to early postoperative pericardial effusion, early postoperative pulmonary venous or systemic venous obstruction, and supraventricular arrhythmias. If the baffle directing pulmonary venous blood to the left atrium is not placed correctly, it may obstruct pulmonary venous drainage. If the baffle bulges into the SVC, it may obstruct SVC inflow, necessitating the placement of an augmentation patch on the anterior surface of the SVC and right atrial junction.

Untreated atrial septal defects are associated with a significantly shortened life expectancy. After age 20 years, the mortality rate is approximately 5% per decade with 90% of patients dead by age 60 years. These patients present with an increase in left-to-right shunting and occasionally with congestive heart failure with pulmonary hypertension in the fourth to sixth decades of life. Guidelines for the diagnosis and treatment of pulmonary artery hypertension have been established.[2] Late problems in untreated patients also include the risk of paradoxical embolus as well as atrial fibrillation, pulmonary hypertension, and right heart failure.

Race

No racial predilection is known.

Sex

Atrial septal defects affect females more often than males. Female-to-male ratio is 2:1. No difference in outcome is associated with sex.

Age

Sinus venosus atrial septal defects are congenital lesions present at birth. The age at presentation depends on the size of the left-to-right shunt. Atrial septal defects in infancy are usually asymptomatic. They are usually detected by echocardiography while undergoing a cardiac evaluation.

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Contributor Information and Disclosures
Author

Gary M Satou, MD, FASE  Director, Pediatric Echocardiography, Co-Director, Fetal Cardiology Program, Mattel Children's Hospital; Associate Clinical Professor, Department of Pediatrics, University of California, Los Angeles, David Geffen School of Medicine

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.

Specialty Editor Board

Charles I Berul, MD  Professor of Pediatrics and Integrative Systems Biology, George Washington University School of Medicine; Chief, Division of Cardiology, Children's National Medical Center

Charles I Berul, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, Cardiac Electrophysiology Society, Heart Rhythm Society, Pediatric and Congenital Electrophysiology Society, and Society for Pediatric Research

Disclosure: Johnson & Johnson Consulting fee Consulting

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Alvin J Chin, MD  Professor of Pediatrics, University of Pennsylvania School of Medicine; Attending Physician, Cardiology Division, Children's Hospital of Philadelphia

Alvin J Chin, MD, is a member of the following medical societies: American Association for the Advancement of Science, American Heart Association, and Society for Developmental Biology

Disclosure: Nothing to disclose.

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.

Additional Contributors

The authors and editors of eMedicine gratefully acknowledge the contributions of previous authors Jeff L Myers, MD, PhD, and James Jaggers, MD, to the writing and development of this article.

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. Chen CA, Wang JK, Hsu JY, Hsu HH, Chen SJ, Wu MH. Diagnosis of inferior sinus venosus atrial septal defects using transthoracic three-dimensional echocardiography. J Am Soc Echocardiogr. Apr 2010;23(4):457.e4-6. [Medline].

  4. 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].

  5. Gajjar TP, Hiremath CS, Desai NB. Surgical closure of sinus venosus atrial septal defect using a single patch--transcaval repair technique. J Card Surg. Jul 2011;26(4):429-34. [Medline].

  6. 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].

  7. 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].

  8. 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].

  9. Banka P, Bacha E, Powell AJ, Benavidez OJ, Geva T. Outcomes of inferior sinus venosus defect repair. J Thorac Cardiovasc Surg. Sep 2011;142(3):517-22. [Medline].

  10. 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].

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

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

  13. 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].

  14. 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].

  15. 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].

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

  17. 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.

  18. 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].

  19. 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].

  20. 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].

  21. 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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Panel A. Transesophageal echocardiogram (transverse view) of a patient with a sinus venosus defect of the superior vena cava (SVC) type. The original defect (white star burst) has been repaired by placing a baffle (arrows), which directs blood from the anomalously connected right upper pulmonary vein into the left atrium (LA). In this patient, the baffle was redundant so at a more rostral level (Panel B), it could be seen (black open arrows) to bulge into the superior vena cava (SVC)–right atrial (RA) junction (trio of white arrows). The remainder of the atrial septum is denoted by the duo of white open arrows. Panel C is a transesophageal echocardiogram, sagittal view. Doppler color flow mapping verifies that the protruding baffle (white closed arrows) results in a narrowing of the pathway from the SVC to the RA. The quartet of white open arrows points to the remainder of the atrial septum.
Panel A is a transesophageal echocardiogram, transverse view. The white star burst shows the sinus venosus defect of the inferior vena cava (IVC) type, lying adjacent to the IVC junction with the right atrium (RA). The remainder of the atrial septum is just out of the view of this sector but is represented by the white open arrowheads. The leaflets of the closed tricuspid valve (TV) are visible. RV = right ventricle. Panel B is a transesophageal echocardiogram, sagittal view. This is the same patient as in Panel A. This view proves that the rostral portion of the atrial septum (which would be missing in a patient with a sinus venosus defect of the SVC type) is intact. ct = crista terminalis; svc = superior vena cava.
 
 
 
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