Atrial Septal Defect, Sinus Venosus Follow-up

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

Further Inpatient Care

  • Patients with sinus venosus atrial septal defect (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.
  • Patients in heart failure may require short-term continued support until pulmonary edema resolves, myocardial function improves, and until pulmonary vascular resistance, if elevated, normalizes.
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Further Outpatient Care

  • Postoperative follow-up: This 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 is observed after repair of sinus venosus atrial septal defects.
    • Sinus node dysfunction screening should be part of outpatient follow-up care as sinus node dysfunction may become apparent years after repair of a sinus venosus atrial septal defect.
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Inpatient & Outpatient Medications

  • 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 who have had atrial septal defects repaired.
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Transfer

  • Patients with a sinus venosus atrial septal defect should be transferred to a center experienced in the repair of such a defect in children or adults.
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Complications

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Prognosis

  • As discussed above, the prognosis is excellent for young patients who undergo repair of uncomplicated defects. Repair delayed until the third decade of life is associated with a decrease in life expectancy.
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Patient Education

  • Patient education mainly focuses on preoperative and postoperative care and recovery, which are especially important in young children undergoing surgery. Centers with experienced child life personnel are invaluable in preparing children for open-heart surgery.
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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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