Double Outlet Right Ventricle, With Transposition Workup

  • Author: M Silvana Horenstein, MD; Chief Editor: Steven R Neish, MD, SM   more...
 
Updated: Aug 11, 2010
 

Laboratory Studies

Clinical laboratory studies (eg, hematologic analysis, urinalysis) are not likely to be of diagnostic help; late findings may include polycythemia, but this and other findings of chronic cyanosis are nonspecific.

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Imaging Studies

Echocardiography is used to evaluate anatomy, hemodynamics, and function of the heart after surgical repair or palliation, and it is the most important means of establishing diagnosis of double outlet right ventricle (DORV) with transposition of the great arteries. Four important findings are as follows:

  • Both great arteries arise from the right ventricle (RV).
  • The aorta (AO) is to the right of or anterior to the pulmonary artery (PA).
  • No course of egress of blood from the left ventricle (LV) other than a ventricular septal defect (VSD) is present.
  • Discontinuity of mitral and semilunar valves is present.

In experienced centers, the accuracy of the prenatal echocardiographic diagnosis (and prognosis) of fetuses with conotruncal anomalies in general is good (ie, correct diagnosis in 77% of cases in a major center).[6] However, defining the exact spatial relationship of the great arteries can be problematic in some fetuses (ie, 7 of 17 fetuses with DORV anatomy, of which 6 were thought to have a subpulmonary VSD, had incorrect prenatal assessment of the great artery relationships).[6]

Some authors have described real-time 3-dimensional echocardiography as a way to improve cardiac imaging and diagnosis of complex congenital heart disease (CHD) through a clear display of cardiac morphology using volumetric views combined with sequential segmental approach.[7] However, others have recently concluded that information provided by real-time 2-dimensional echocardiography in fetuses with and without CHD were consistent with that provided by real-time 3-dimensional echocardiography. Therefore, no clear advantage of real-time 3-dimensional echocardiography over real-time 2-dimensional echocardiography has been documented.

Chest radiography may provide valuable clues for the diagnosis of DORV with transposition of the great arteries. Chest radiography for patients with either subaortic or subpulmonary VSD without PS shows cardiomegaly with increased pulmonary vascular markings; the main PA segment may be prominent. These findings are not specific for DORV. If PS is present, chest radiography reveals a normal heart size and normal-to-decreased pulmonary vascular markings.

MRI may serve as an adjunct tool to echocardiography for determination of visceral and atrial situs as vasculo-vascular and vasculo-visceral relationships. In some patients with DORV with remote VSD, MRI may aid in defining the spatial relationship between VSD and the semilunar valves. A new modality in MRI is the 3-dimensional MRI, which is increasingly used as an adjuvant to echocardiography and angiography for such purposes.

Angiography (see images below) may add anatomic and physiologic details to information found by echocardiography.

This is an angiogram obtained during catheterizatiThis is an angiogram obtained during catheterization of a patient with double outlet right ventricle (DORV) with transposition of the great arteries. Injection of contrast though the catheter (arrow) into the left ventricle (LV) shows that blood is directed toward the right ventricle (RV) through a remote or doubly committed ventricular septal defect (VSD). The aorta (AO) is anterior to the pulmonary artery (PA) and both clearly arise from the RV. This is an angiogram obtained during catheterizatiThis is an angiogram obtained during catheterization of a patient with double outlet right ventricle (DORV) with transposition of the great arteries (see Media file 2). Blood fills the aorta (AO) and pulmonary artery (PA) almost simultaneously, which is another indicator of a remote or doubly committed ventricular septal defect (VSD) (curved arrow). LV indicates the left ventricle, RV indicates the right ventricle, and the small arrow to the left indicates the catheter.
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Other Tests

ECG in patients with DORV with transposition of the great arteries reveals no specific findings. Usually, normal sinus rhythm and possible prolonged PR interval are present. Right axis deviation and right ventricular hypertrophy (RVH) are likely to be present. In the absence of these findings, question the diagnosis or consider special circumstances such as an associated atrioventricular (AV) canal if left axis deviation is present.

Some ECG variations may be noted, depending on the type of DORV with transposition of the great arteries. ECG in patients with subaortic VSD with no PS may show superior QRS axis (-30° to -170°) with either RVH or biventricular hypertrophy and left atrial enlargement. First-degree AV block may be present with this lesion. ECG in patients with subpulmonic VSD or in those with subaortic VSD and PS reveals right axis deviation, RVH, and often right atrial enlargement.

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Procedures

Echocardiography has mostly eliminated the need to perform cardiac catheterization in these patients; however, catheterization may still be necessary in certain circumstances.

Catheterization may be required for the following reasons:

  • Need for further definition of coronary artery anatomy
  • Need to determine coexistent conditions that cannot be elucidated by echocardiography
  • Need to confirm restrictive VSD by measuring ventricular pressures
  • Need to determine pulmonary vascular resistance (and reactivity) in patients suspected of having increased resistance
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Contributor Information and Disclosures
Author

M Silvana Horenstein, MD  Assistant Professor, Department of Pediatrics, University of Texas Medical School Houston; Medical Doctor Consultant, Legacy Department, Best Doctors, Inc

M Silvana Horenstein, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, and American Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

Michael L Epstein, MD  Director, Division of Pediatric Cardiology, Department of Pediatrics, Children's Hospital of Michigan; Professor, Wayne State University School of Medicine

Michael L Epstein, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, and American Heart Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Juan Carlos Alejos, MD  Clinical Professor, Department of Pediatrics, Division of Cardiology, University of California, Los Angeles, David Geffen School of Medicine

Juan Carlos Alejos, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Medical Association, and International Society for Heart and Lung Transplantation

Disclosure: Actelion Honoraria Speaking and teaching

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Pharmacy Editor, eMedicine

Disclosure: Nothing to disclose.

Julian M Stewart, MD, PhD  Associate Chairman of Pediatrics, Director, Center for Hypotension, Westchester Medical Center; Professor of Pediatrics and Physiology, New York Medical College

Julian M Stewart, MD, PhD is a member of the following medical societies: American Academy of Pediatrics

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

Steven R Neish, MD, SM  Director of Pediatric Cardiology Fellowship Program, Associate Professor, Department of Pediatrics, Baylor College of Medicine

Steven R Neish, MD, SM is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, and American Heart Association

Disclosure: Nothing to disclose.

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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).
This is an angiogram obtained during catheterization of a patient with double outlet right ventricle (DORV) with transposition of the great arteries. Injection of contrast though the catheter (arrow) into the left ventricle (LV) shows that blood is directed toward the right ventricle (RV) through a remote or doubly committed ventricular septal defect (VSD). The aorta (AO) is anterior to the pulmonary artery (PA) and both clearly arise from the RV.
This is an angiogram obtained during catheterization of a patient with double outlet right ventricle (DORV) with transposition of the great arteries (see Media file 2). Blood fills the aorta (AO) and pulmonary artery (PA) almost simultaneously, which is another indicator of a remote or doubly committed ventricular septal defect (VSD) (curved arrow). LV indicates the left ventricle, RV indicates the right ventricle, and the small arrow to the left indicates the catheter.
 
 
 
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