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Endocardial Cushion Defects Workup

  • Author: Mary C Mancini, MD, PhD, MMM; Chief Editor: Park W Willis IV, MD  more...
 
Updated: Sep 15, 2014
 

Laboratory Studies

See the list below:

  • CBC count: Blood tests determine the presence of polycythemia in a potentially cyanotic condition.
  • Prothrombin time/activated partial thromboplastin time (PT/aPTT): In children with cyanotic heart disease, the coagulation profile may be abnormal because of associated polycythemia.
  • Electrolytes: This test detects any abnormalities incurred with treatment of CHF.
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Imaging Studies

See the list below:

  • Chest radiography
    • This test is a good general screening that shows cardiac enlargement, particularly of the right atrium and ventricle.
    • The main pulmonary artery usually is prominent with increased pulmonary vascular markings. After pulmonary hypertension develops, a reduction in pulmonary vascular markings is observed.
  • Echocardiography
    • M-mode shows diastolic movement of the mitral valve with enlarged right ventricle and paradoxical motion of the interventricular septum.
    • Two-dimensional echocardiography is highly reliable in identification of septal defects. Echocardiography identifies the absence of the interventricular septum. Findings may include right ventricular dilatation and paradoxical motion of the interventricular septum. The extent of septal defects as well as the left-to-right shunting and degree of valvular insufficiency can be determined as well as an estimate of pulmonary artery pressure. Lack of displacement of the left and right AV valves is a characteristic finding in this condition. Prolonged diastolic contact between the anterior mitral leaflet and the interventricular septum also may be noted. Associated defects that may require attention also can be detected.
    • Abnormalities in the AV valves can be identified reliably. Transesophageal echocardiography clearly identifies AV valve morphology.[5, 6, 7]
  • MRI: This test readily visualizes the deficiency in the ventricular septum as well as AV valve morphology.[8, 9]
  • Cardiac catheterization: This test is indicated when clinically significant questions remain unanswered after a comprehensive noninvasive evaluation. If other lesions are suspected or if operative planning cannot be performed adequately after noninvasive testing, then catheterization should be undertaken. Left ventricular angiography in the frontal plane shows an elongated left ventricular outflow tract, called a "gooseneck deformity," which is characteristic of this condition. Catheterization should involve quantitation of the shunts and valvular insufficiency and calculation of pulmonary vascular resistance. Aortography may be performed to determine whether a patent ductus arteriosus is present.
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Other Tests

See the list below:

  • Electrocardiography
    • The typical ECG in patients with partial AV septal defects shows first-degree AV block and left axis deviation (because of late left anterior fascicular depolarization). Patients with right ventricular dilatation usually have partial or complete right bundle-branch block. Complete AV block and atrial fibrillation commonly occur in older patients. See Medscape's Atrial Fibrillation Resource Center.
    • A prolonged PR interval accompanied by biventricular or left ventricular hypertrophy also may be seen.
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Contributor Information and Disclosures
Author

Mary C Mancini, MD, PhD, MMM Professor and Chief of Cardiothoracic Surgery, Department of Surgery, Louisiana State University School of Medicine in Shreveport

Mary C Mancini, MD, PhD, MMM is a member of the following medical societies: American Association for Thoracic Surgery, American College of Surgeons, American Surgical Association, Society of Thoracic Surgeons, Phi Beta Kappa

Disclosure: Nothing to disclose.

Coauthor(s)

Henry G Hanley, MD Chief of Cardiology Section, Freedman Memorial Cardiology; Professor, Department of Medicine, Louisiana State University Health Sciences Center

Henry G Hanley, MD is a member of the following medical societies: American College of Cardiology, Society for Cardiovascular Angiography and Interventions, American College of Physicians, American Heart Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Frank M Sheridan, MD 

Frank M Sheridan, MD is a member of the following medical societies: American College of Cardiology, American Heart Association, Society for Cardiovascular Angiography and Interventions

Disclosure: Nothing to disclose.

Chief Editor

Park W Willis IV, MD Sarah Graham Distinguished Professor of Medicine and Pediatrics, University of North Carolina at Chapel Hill School of Medicine

Park W Willis IV, MD is a member of the following medical societies: American Society of Echocardiography

Disclosure: Nothing to disclose.

Additional Contributors

Park W Willis IV, MD Sarah Graham Distinguished Professor of Medicine and Pediatrics, University of North Carolina at Chapel Hill School of Medicine

Park W Willis IV, MD is a member of the following medical societies: American Society of Echocardiography

Disclosure: Nothing to disclose.

References
  1. Person AD, Klewer SE, Runyan RB. Cell biology of cardiac cushion development. Int Rev Cytol. 2005. 243:287-335. [Medline].

  2. Cooper RS. Endocardial cushion defects: embryology, anatomy and pathophysiology. Adv Cardiol. 2004. 41:118-26. [Medline].

  3. Marelli AJ, Ionescu-Ittu R, Mackie AS, Guo L, Dendukuri N, Kaouache M. Lifetime prevalence of congenital heart disease in the general population from 2000 to 2010. Circulation. 2014 Aug 26. 130(9):749-56. [Medline].

  4. [Guideline] Marino BS, Lipkin PH, Newburger JW, Peacock G, Gerdes M, Gaynor JW, et al. Neurodevelopmental outcomes in children with congenital heart disease: evaluation and management: a scientific statement from the American Heart Association. Circulation. 2012 Aug 28. 126(9):1143-72. [Medline].

  5. Tardif JC, Schwartz SL, Vannan MA, et al. Clinical usefulness of multiplane transesophageal echocardiography: comparison to biplanar imaging. Am Heart J. 1994 Jul. 128(1):156-66. [Medline].

  6. Weyman AE, Wann LS, Caldwell RL, et al. Negative contrast echocardiography: a new method for detecting left-to-right shunts. Circulation. 1979 Mar. 59(3):498-505. [Medline].

  7. Williams RG, Rudd M. Echocardiographic features of endocardial cushion defects. Circulation. 1974 Mar. 49(3):418-22. [Medline].

  8. Holmvang G, Palacios IF, Vlahakes GJ, et al. Imaging and sizing of atrial septal defects by magnetic resonance. Circulation. 1995 Dec 15. 92(12):3473-80. [Medline].

  9. Jacobstein MD, Fletcher BD, Goldstein S, Riemenschneider TA. Evaluation of atrioventricular septal defect by magnetic resonance imaging. Am J Cardiol. 1985 Apr 15. 55(9):1158-61. [Medline].

  10. Hanley FL, Fenton KN, Jonas RA, et al. Surgical repair of complete atrioventricular canal defects in infancy. Twenty-year trends. J Thorac Cardiovasc Surg. 1993 Sep. 106(3):387-94; discussion 394-7. [Medline].

  11. Prêtre R, Dave H, Kadner A, Bettex D, Turina MI. Direct closure of the septum primum in atrioventricular canal defects. J Thorac Cardiovasc Surg. 2004 Jun. 127(6):1678-81. [Medline].

  12. Silverman N, Levitsky S, Fisher E, et al. Efficacy of pulmonary artery banding in infants with complete atrioventricular canal. Circulation. 1983 Sep. 68(3 Pt 2):II148-53. [Medline].

  13. Studer M, Blackstone EH, Kirklin JW, et al. Determinants of early and late results of repair of atrioventricular septal (canal) defects. J Thorac Cardiovasc Surg. 1982 Oct. 84(4):523-42. [Medline].

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Anatomy of the endocardial cushion defect (ie, complete form); note the common atrioventricular valve straddling the atrial septal and ventricular septal defects.
Repair of the endocardial cushion defect. The patch is covering the ostium primum atrial septal defect.
 
 
 
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