Sinus Venosus Atrial Septal Defects Clinical Presentation

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

History

Sinus venosus atrial septal defects, like most atrial septal defects, are diagnosed upon detection of a murmur, a split second heart sound, and/or right heart enlargement on EKG in the usually asymptomatic patient.

  • Symptoms of atrial septal defects are typically a function of the size of the associated shunt.
  • As many as 60% of apparently asymptomatic patients may have easy fatigability and dyspnea. Such symptoms usually indicate a relatively large shunt.
  • Adults may not come to medical attention until symptoms occur. Arrhythmias, dyspnea, and a decrease in exercise tolerance are common symptoms.
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Physical

  • A cardiac murmur secondary to increased pulmonary artery blood flow is heard over the left sternal border. The murmur is usually a grade 2-3/6 systolic ejection murmur. A prominent right ventricular impulse may also be noted along the left sternal border. A diastolic flow murmur may be present at the left lower sternal border and the tricuspid area and is indicative of a large left-to-right shunt.
  • The second heart sound is widely split and may be fixed or may vary little with respiration. The pulmonic component of the second heart sound is usually normal in intensity but may increase in intensity if pulmonary hypertension is present.
  • Patients with atrial septal defects may present with the "gracile habitus." These patients are thin for their height.
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Causes

  • During normal embryonic development, the right horn of the sinus venosus encompasses the right superior vena cava (SVC) and inferior vena cava (IVC). If abnormal resorption of the sinus venosus occurs, an atrial septal defect results near the orifice of either the SVC or IVC.
  • Atrial septal defects occur as associated anomalies in many major complex congenital lesions but sinus venosus atrial septal defects occur more often as an isolated abnormality.
  • Other abnormalities may exacerbate an atrial septal defect. For instance, systemic hypertension in an adult with a sinus venosus atrial septal defect may result in left ventricular hypertrophy and reduce left ventricular compliance, which, in turn, exacerbates the atrial level left-to-right shunt. Mitral stenosis, which is either congenital or acquired, may also exacerbate the atrial level left-to-right shunt.
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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

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  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].

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  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].

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  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].

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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. 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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