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Sinus Venosus Atrial Septal Defects Treatment & Management

  • Author: Gary M Satou, MD, FASE; Chief Editor: Howard S Weber, MD, FSCAI  more...
 
Updated: Sep 10, 2015
 

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. Consult with a pediatric cardiologist and a pediatric cardiac surgeon.

Patients with a sinus venosus ASD should be transferred to a center experienced in the repair of such a defect in children or adults. Patients with sinus venosus ASD in heart failure may require short-term continued support until pulmonary edema resolves, myocardial function improves, and until pulmonary vascular resistance, if elevated, normalizes.

Patients with sinus venosus ASD require a brief postoperative admission to a pediatric cardiac intensive care unit. The patient who undergoes uncomplicated surgical repair is usually discharged home within several days.

There are no dietary restrictions, and physical activity should not be limited in patients who undergo early and complete correction.

Pharmacotherapy

No long-term medication is required after repair of an uncomplicated atrial septal defect. Some surgeons prescribe aspirin or other anticoagulation regimens for several weeks in patients in whom a prosthetic patch was used to close the defect. This allows for endothelial ingrowth over the thrombogenic surface of the patch. Long-term anticoagulation is not indicated.

Antibiotic prophylaxis is not required in patients 6 months following repair of atrial septal defects.

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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.[5]

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.[6]  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.[7]  In addition, concern for injury to the conduction system or sinus node have not been observed to date.[8]

A very small right upper pulmonary vein that drains into the high SVC could be left intact and not incorporated into the surgical repair if it is deemed high risk for late pulmonary vein obstruction. 

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 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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Long-Term Monitoring

Postoperative follow-up

Postoperative follow-up usually involves an office visit with the pediatric cardiologist (and possibly the cardiac surgeon) 1-3 weeks after hospital discharge.

Echocardiography is used to effectively evaluate the repair for evidence of residual shunting, superior vena cava (SVC) or pulmonary vein obstruction, pericardial effusion, and ventricular function.

The potential for late postoperative narrowing of the SVC and possible superior vena cava syndrome is observed after repair of sinus venosus atrial septal defects.

Sinus node dysfunction screening should be part of outpatient follow-up care, because sinus node dysfunction may become apparent years after repair of a sinus venosus atrial septal defect.

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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, Society of Pediatric Echocardiography, American College of Cardiology, American Heart Association, American Society of 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, Western Thoracic Surgical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

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 Emeritus Professor of Pediatrics, 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, Society for Developmental Biology, American Heart Association

Disclosure: Nothing to disclose.

Chief Editor

Howard S Weber, MD, FSCAI Professor of Pediatrics, Section of Pediatric Cardiology, Pennsylvania State University College of Medicine; Director of Interventional Pediatric Cardiology, Penn State Hershey Children's Hospital

Howard S Weber, MD, FSCAI is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, Society for Cardiovascular Angiography and Interventions

Disclosure: Received income in an amount equal to or greater than $250 from: St. Jude Medical.

Additional Contributors

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, Heart Rhythm Society, Cardiac Electrophysiology Society, Pediatric and Congenital Electrophysiology Society, American College of Cardiology, American Heart Association, Society for Pediatric Research

Disclosure: Received grant/research funds from Medtronic for consulting.

Acknowledgements

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
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  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. 2004 Dec. 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. 2010 Apr. 23(4):457.e4-6. [Medline].

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  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. 1984 Dec. 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. 1995 Dec. 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. 2006 Jan. 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. 2011 Sep. 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. 1970 Nov. 32(6):820-6. [Medline].

  12. Driscoll DJ. Left-to-right shunt lesions. Pediatr Clin North Am. 1999 Apr. 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. 1984 Aug. 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. 1988 Jul. 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. 1978 Jan. 25(1):44-50. [Medline].

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  17. Mas MS, Bricker JT. Clinical Physiology of Left-to-Right Shunts. Garson A, Bricker JT, McNamara DG, eds. The Science and Practice of Pediatric Cardiology. Lippincott Williams & Wilkins; 1990. Vol 2: 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. 1990 Dec 13. 323(24):1645-50. [Medline].

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  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. 2001 May 15. 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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