Atrial Septal Defect, Sinus Venosus Treatment & Management

  • Author: Gary M Satou, MD, FASE; Chief Editor: Stuart Berger, MD   more...
 
Updated: Jun 12, 2009
 

Medical Care

  • Medical care of sinus venosus atrial septal defect (ASD) is primarily supportive and is not required for asymptomatic patients.
  • Patients presenting in heart failure should be stabilized in anticipation of elective repair.
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Surgical Care

Surgical correction is the mainstay of therapy.

  • Repair of the sinus venosus atrial septal defect is more complex than repair of the average secundum atrial septal defect. A patch (synthetic material or pericardium) is used to redirect blood flow from the right superior pulmonary vein into the left atrium. This effectively closes the interatrial communication while also correcting the anomalous pulmonary venous drainage. Sometimes, to avoid creating superior vena cava (SVC) obstruction, a patch is placed on the anterior surface of the SVC. Care is taken to avoid injuring the nearby sinus node. Ligation of the azygous vein may also be required to eliminate its drainage into the left atrium and to prevent the resulting residual right-to-left shunt.
  • When the location of the anomalous venous drainage is in the high SVC and is far from the atrial-caval junction, a different surgical approach can be used to decrease the probability of caval stenosis or pulmonary vein stenosis.
    • As described by Warden et al, the repair consists of division of the SVC just above the take off of the anomalous pulmonary vein.[4]
    • The distal caval end is oversewn or patched to assure no pulmonary vein compromise.
    • Next, the well-mobilized cava is anastomosed to the right atrial appendage after amputation of the most distal end.
    • The atrial septal defect is then closed by sewing a patch to cover the atrial septal defect and divided SVC orifice, thereby baffling the anomalous vein to the left atrium.
    • This method is very effective in patients with more complicated pulmonary venous anomalies.
    • Although a relatively recent advance in the treatment of high anomalous pulmonary venous drainage, this operation has become the procedure of choice for more difficult cases.
    • All reported series have demonstrated excellent results with little or no pulmonary venous or SVC stenosis.[5]
    • In addition, concern for injury to the conduction system or sinus node have not been observed to date.[6]
  • Asymptomatic children generally undergo repair when aged 3-5 years.
  • Sinus venosus defects do not close spontaneously.
  • Adults with left-to-right shunts greater than 1.5-2:1 benefit from surgical closure.
  • Patients with significant pulmonary hypertension and elevated pulmonary vascular resistance unresponsive to pulmonary vasodilator therapy (eg, oxygen, nitric oxide, calcium channel blockers,) may not be good candidates for surgical repair. Such patients may develop acute right ventricular failure if their heart no longer has the ability to shunt right to left at the atrial communication in response to increases in pulmonary vascular resistance.
  • Repair is performed most often through a standard median sternotomy. More cosmetic incisions may also be used, such as partial sternotomies, small right anterior thoracotomies, and inframammary incisions. All approaches still require the use of cardiopulmonary bypass for closure of the atrial septal defect.
  • Although transcatheter occlusion devices are currently used for closing secundum atrial septal defects, such devices are not indicated (at present) for the closure of sinus venosus atrial septal defects because of the position of the defect and because of the lack of surrounding tissue adequate to seat such an occlusion device. In addition, such a device may obstruct SVC flow and does not achieve redirection of the anomalous right pulmonary venous flow to the left atrium.
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Consultations

  • Pediatric cardiologist
  • Pediatric cardiac surgeon
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Diet

  • No dietary restrictions
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Activity

  • Physical activity should not be limited in patients who undergo early and complete correction.
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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.

Specialty Editor Board

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.

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

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.

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.

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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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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