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Double Outlet Right Ventricle Surgery Workup

  • Author: Abdullah A Alghamdi, MD, FRCSC; Chief Editor: Jonah Odim, MD, PhD, MBA  more...
 
Updated: Jan 30, 2015
 

Laboratory Studies

Routine laboratory testing in patients with double outlet right ventricle (DORV) includes CBC count, electrolyte levels, renal profile, hepatic function, coagulation profile, and assessment of nutritional status if indicated.

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Imaging Studies

See the list below:

  • Echocardiography of the heart and great vessels
    • Echocardiography generally provides enough information for accurate and adequate diagnosis and provides the needed information to plan the surgical approach in neonates and young infants.
    • Sanders et al reported that standard transthoracic echocardiography was used to diagnose conotruncal malformation in 109 of 113 infants.[6] Of the 12 infants in whom double outlet right ventricle was diagnosed and confirmed with angiography, 11 previously received a diagnosis based on subxiphoid 2-dimensional echocardiography.
    • Echocardiography can be used to correctly identify the relative position of the great arteries, the degree of subsemilunar narrowing, the position of the VSD, and the status of the mitral valve and left ventricle.
  • Cardiac angiography
    • Cardiac catheterization, once the criterion standard for confirming DORV, is now rarely required in the evaluation or preoperative planning of this cardiac disorder.
    • Angiography has several advantages, when indicated.
      • In the older child with long-standing disease, hemodynamic parameters can be directly measured.
      • In the setting of possible aberrant coronary anatomy (3-5% of cases), the actual anatomic variation can be defined. This knowledge can affect surgical planning when Rastelli-type repair is contemplated.
      • In the setting of pulmonary vascular anomalies, angiography can help in defining the main pulmonary branches, the pulmonary vascular tree, and the collateral vessels to the lungs.
  • MRI: This has been used in the diagnosis of double outlet right ventricle but is not yet a routine or well-established diagnostic modality for double outlet right ventricle. MRI is useful to obtain additional anatomical information such as relationship of both ventricles.
  • Chest radiography
    • Regardless of the end of the clinical spectrum (tetralogy of Fallot [TOF] or transposition of the great arteries [TGA]) at which double outlet right ventricle occurs, findings on anteroposterior and lateral chest radiography depend on the degree of pulmonary (or subpulmonary) stenosis.
    • In the setting of severe stenosis, the pulmonary parenchyma is relatively oligemic, whereas in the setting of minimal pulmonary stenosis (especially with a Taussig-Bing heart), findings are likely to be consistent with congestive heart failure. Either way, the chest image shows cardiomegaly.
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Other Tests

See the list below:

  • ECG findings are rarely diagnostic for double outlet right ventricle.
  • Common findings in a child with double outlet right ventricle include right ventricular hypertrophy, right axis deviation, and, occasionally, evidence of left ventricular hypertrophy.
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Diagnostic Procedures

See the list below:

  • No additional procedures are indicated.
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Histologic Findings

See the list below:

  • No particular histologic findings have been reported.
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Staging

See the list below:

  • No staging has been applied for this anomaly.
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Contributor Information and Disclosures
Author

Abdullah A Alghamdi, MD, FRCSC Fellow, Division of Pediatric Cardiac Surgery, University of Toronto, Hospital for Sick Children

Disclosure: Nothing to disclose.

Coauthor(s)

Christopher A Caldarone, MD Chair, Division of Cardiac Surgery, Professor of Surgery, University of Toronto; Staff Surgeon, Cardiovascular Surgery, Hospital for Sick Children, Toronto

Christopher A Caldarone, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Surgeons, American Medical Association

Disclosure: Nothing to disclose.

Phillip C Camp, Jr, MD Instructor in Surgery, Department of Surgery, Harvard Medical School; Associate Surgeon, Department of Surgery, Divisions of Thoracic Surgery and Surgical Critical Care, Brigham and Women's Hospital; Staff Surgeon, Children’s Hospital of Boston, Milford Regional Medical Center, Faulkner Hospital, Dana-Farber Cancer Institute, and South Shore Hospital; Associate Medical Director, New England Organ Bank

Phillip C Camp, Jr, MD is a member of the following medical societies: American College of Surgeons, American Heart Association, American Society for Artificial Internal Organs, International Society for Heart and Lung Transplantation, Society of Thoracic Surgeons, Southern Thoracic Surgical Association

Disclosure: Nothing to disclose.

Gregory B Dalshaug, MD Assistant Professor, Division of Cardiovascular Surgery, Royal University Hospital

Gregory B Dalshaug, MD is a member of the following medical societies: American College of Surgeons, American Medical Association, Canadian Medical Association, Iowa Medical Society, Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Osami Honjo, MD, PhD Staff Cardiovascular Surgeon, Division of Cardiovascular Surgery, The Hospital for Sick ChildrenAssistant professor, Department of Surgery, The University of Toronto

Osami Honjo, MD, PhD is a member of the following medical societies: Japan Surgical Society

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.

Chief Editor

Jonah Odim, MD, PhD, MBA Section Chief of Clinical Transplantation, Transplantation Branch, Division of Allergy, Immunology, and Transplantation, National Institute of Allergy and Infectious Diseases (NIAID), National Institutes of Health (NIH)

Jonah Odim, MD, PhD, MBA is a member of the following medical societies: American College of Cardiology, American College of Chest Physicians, American Association for Physician Leadership, American College of Surgeons, American Heart Association, American Society for Artificial Internal Organs, American Society of Transplant Surgeons, Association for Academic Surgery, Association for Surgical Education, International Society for Heart and Lung Transplantation, National Medical Association, New York Academy of Sciences, Royal College of Physicians and Surgeons of Canada, Society of Critical Care Medicine, Society of Thoracic Surgeons, Canadian Cardiovascular Society

Disclosure: Nothing to disclose.

Additional Contributors

Daniel S Schwartz, MD, FACS Medical Director of Thoracic Oncology, St Catherine of Siena Medical Center, Catholic Health Services

Daniel S Schwartz, MD, FACS is a member of the following medical societies: Society of Thoracic Surgeons, Western Thoracic Surgical Association, American College of Chest Physicians, American College of Surgeons

Disclosure: Nothing to disclose.

References
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Double outlet right ventricle (DORV) with transposition of the great arteries accounts for 26% of cases of DORV. The aorta (AO) is anterior and to the right of the pulmonary artery (PA), and both arteries arise from the right ventricle (RV). The only outflow from the left ventricle (LV) is a ventricular septal defect (VSD), which diverts blood toward the RV. Pulmonary veins drain into the left atrium (LA) after blood has been oxygenated in the lungs (L). Systemic venous return is to the right atrium (RA).
 
 
 
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