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Sinus Venosus Atrial Septal Defects Workup

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

Imaging Studies

General laboratory studies are rarely helpful in sinus venosus atrial septal defect (ASD). However, imaging studies help in the evaluation and diagnosis of suspected sinus venosus ASD.

Chest radiography

Findings on chest radiography may include the following:

  • Prominent right atrium
  • Prominent main pulmonary artery
  • Increased heart size and pulmonary vascularity

Echocardiography

Echocardiography (ECHO) reveals ASD and most of the pulmonary vein connections in most patients and is the diagnostic modality of choice.[3]

Two-dimensional ECHO with color flow Doppler reveals the position and size of the defect and the presence of anomalous pulmonary venous drainage (in many of these cases). It also helps identify associated anomalies and reveals the left-to-right (or right-to-left) direction of flow and the degree of right ventricular overload.

In children with difficult transthoracic windows, or in older or larger patients, transesophageal echocardiography may be helpful in imaging the defect and pulmonary vein connections.[4] In the current era, cardiac magnetic resonance angiography (MRA)/magnetic resonance imaging (MRI) may be alternatively used to complete the diagnostic information needed prior to surgery.

Cardiac MRI/MRA

Features that may be seen with MRI/MRA include the following:

  • Atrial septal defect size and location are shown.
  • Excellent delineation of individual pulmonary vein connections can be identified.
  • Right ventricle enlargement and indexing to body surface area (BSA) is available if helpful.
  • Flow-quantification may also be performed.

Electrocardiography

Note the following:

  • Right ventricular hypertrophy predominates, with a lengthened PR interval and incomplete right bundle branch block secondary to right ventricular dilation (small rSR').
  • Peaked P waves in lead II may demonstrate right atrial enlargement.
  • Abnormal P-wave axis (negative in lead AVF) would indicate a displaced sinus node inferiorly (non sinus atrial rhythm).
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Procedures

Cardiac catheterization

Cardiac catheterization is usually not required in the preoperative assessment of patients with sinus venosus atrial septal defect, but it may be considered in the following circumstances:

  • In any child in whom associated lesions are suspected or in whom pulmonary hypertension is suspected, catheterization is performed to measure pulmonary artery pressure and, if pulmonary resistance is elevated, the response to pulmonary vasodilators.
  • Adults who have the potential for associated coronary atherosclerotic lesions should undergo catheterization to exclude these abnormalities before surgical repair of the sinus venosus atrial septal defect.
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Histologic Findings

Patients with pulmonary hypertension and advanced pulmonary vascular obstructive disease may exhibit histologic changes similar to those seen in pulmonary vascular disease. Specifically, these include intimal and medial hypertrophy and, in more advanced lesions, luminal occlusion.

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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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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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