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Double Outlet Right Ventricle, Normally Related Great Arteries: Differential Diagnoses & Workup

Author: Rod Tarrago, MD, Pediatric Intensivist, Department of Pediatric Critical Care, Children's Hospitals and Clinics of Minnesota
Coauthor(s): Steven R Neish, MD, SM, Director of Pediatric Cardiology Fellowship Program, Associate Professor, Department of Pediatrics, Baylor College of Medicine
Contributor Information and Disclosures

Updated: Oct 6, 2008

Differential Diagnoses

Transposition of the Great Arteries
Truncus Arteriosus
Ventricular Septal Defect, General Concepts

Workup

Laboratory Studies

  • Routine laboratory studies are not required for the initial diagnosis and management in children with double outlet right ventricle (DORV).
  • Assess hemoglobin and hematocrit if children are thought to have polycythemia.
  • Monitor serum electrolyte levels after treating children with diuretics, glycosides, and afterload-reducing agents.

Imaging Studies

  • Chest radiography
    • Chest radiography findings usually correlate with clinical presentation.
    • Chest radiographs cannot be used to differentiate double outlet right ventricle from other forms of congenital heart disease (CHD).
    • Presence or absence of pulmonary stenosis and pulmonary vascular resistance determines if cardiomegaly and increased pulmonary vascularity are present.
    • Patients with subaortic ventricular septal defect (VSD) and severe pulmonary stenosis demonstrate diminished pulmonary vascularity and concave left heart border (similar to appearance associated with tetralogy of Fallot).
    • If pulmonary obstructive vascular disease is present, peripheral pulmonary vascularity may be reduced and proximal pulmonary arteries may be dilated.
    • The appearance in patients with subpulmonary VSD is similar to that in patients with transposition of the great arteries, revealing increased pulmonary vascularity and cardiomegaly.
    • In patients in whom the aorta is anterior and to the left, radiography may reveal the leftward position of the aorta.
  • Echocardiography
    • Echocardiography is the imaging technique most often used to diagnose double outlet right ventricle.
    • The principle diagnostic feature is appearance of both great arteries primarily committed to the right ventricle. Parasternal long-axis and short-axis views reveal the degree of commitment to the right ventricle.
    • Subcostal and apical 4-chamber views reveal the separation between semilunar and atrioventricular valves (ventriculoinfundibular fold).
    • Use multiple views to determine the relationship between the ventricular septum and the outlet septum.
    • Features that must be established with echocardiography include the primary commitment of both great arteries to the right ventricle, the spatial relationship of both great arteries, the location of the VSD and its relationship to semilunar valves, and the presence of associated anomalies such as coarctation, the straddle/override of atrioventricular valve in relation to the VSD, and the presence of restrictive VSD.
    • In addition, fetal echocardiography has been used to prenatally diagnose cases of double outlet right ventricle. This imaging modality can also reveal other congenital cardiac anomalies that may be present in addition to double outlet right ventricle. Prenatal quantification of left ventricular size is important. One study noted that, in patients with double outlet right ventricle and borderline left ventricular size, only 33% of pregnancies proceeded to live birth.7 Of those patients, only 25% successfully underwent biventricular repair. Patients with double outlet right ventricle may demonstrate other abnormalities on fetal ultrasonography. However, even in patients with isolated double outlet right ventricle, diagnosis is possible based solely on limited fetal echocardiography, provided a long-axis view is included.8  
  • MRI
    • MRI can help clarify ambiguities that remain after echocardiography.
    • MRI reveals the relationship between the great arteries, the anatomy of the outlet septum relative to the ventricular septum, and the relationship of the VSD to the great arteries.
    • One study reported that, in patients with doubly committed or noncommitted VSDs, MRI more reliably predicted the feasibility of a biventricular repair than did echocardiography.9
    • Limitations include the need for prolonged evaluation, deeper sedation, and incomplete atrioventricular valve definition. MRI may also fail to reveal the presence of aberrant chordae tendineae.

Other Tests

  • Abnormalities are often present on electrocardiography (ECG) but are not diagnostic of double outlet right ventricle.
  • If obtained after the newborn period, ECG reveals right ventricular hypertrophy.
  • Left ventricular hypertrophy may develop in the presence of a restrictive VSD that leads to left ventricular pressure overload or an increased pulmonary venous return that leads to left ventricular volume overload.
  • Right atrial enlargement is common.
  • Left atrial enlargement may be present if pulmonary venous return or mitral stenosis/atresia is increased.
  • Usually, left axis deviation of the frontal plane QRS is present because of displacement of the bundle of His posterior to VSD.

Procedures

  • Cardiac catheterization may delineate anatomy and hemodynamics. Objectives of catheterization include the following:
    • Evaluation of right and left ventricular volumes
    • Evaluation for possible gradient across VSD and pulmonary vascular resistance (PVR)
    • Evaluation of relationship between VSD and great arteries
    • Evaluation of coronary artery and aortic arch anatomy
    • Assessment of degree of mixing of the 2 circulations
  • If a restrictive atrial septal defect (ASD) is present, increased pulmonary blood flow with aortic saturations below 70%, or 10% less than pulmonary saturations, indicates the possibility of improvement with atrial septostomy (termed transposition physiology).
  • Diagnostic angiographic features of double outlet right ventricle include the following:
    • Opacification of both great arteries following right ventriculography
    • Similarity of aortic and pulmonary valve horizontal planes
    • Frequent anterior malposition of the aorta
    • Presence of a filling defect dividing the 2 outflow tracts

Histologic Findings

  • Findings vary depending on the clinical presentation; various physiologic effects determine histology of cardiac structures.

More on Double Outlet Right Ventricle, Normally Related Great Arteries

Overview: Double Outlet Right Ventricle, Normally Related Great Arteries
Differential Diagnoses & Workup: Double Outlet Right Ventricle, Normally Related Great Arteries
Treatment & Medication: Double Outlet Right Ventricle, Normally Related Great Arteries
Follow-up: Double Outlet Right Ventricle, Normally Related Great Arteries
Multimedia: Double Outlet Right Ventricle, Normally Related Great Arteries
References
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References

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  2. Brown JW, Ruzmetov M, Okada Y, et al. Surgical results in patients with double outlet right ventricle: a 20- year experience. Ann Thorac Surg. Nov 2001;72(5):1630-5. [Medline].

  3. Kirby ML, Waldo KL. Role of neural crest in congenital heart disease. Circulation. Aug 1990;82(2):332-40. [Medline].

  4. Goldmuntz E, Clark BJ, Mitchell LE, et al. Frequency of 22q11 deletions in patients with conotruncal defects. Journal of the American College of Cardiology. 1999;32:499-501. [Medline].

  5. Khositseth A, Tocharoentanaphol C, Khowsathit P, Ruangdaraganon N. Chromosome 22q11 deletions in patients with conotruncal heart defects. Pediatr Cardiol. Sep-Oct 2005;26(5):570-3. [Medline].

  6. Momma K, Kondo C, Matsuoka R, Takao A. Cardiac anomalies associated with a chromosome 22q11 deletion in patients with conotruncal anomaly face syndrome. American Journal of Cardiology. 1996;78:591-594. [Medline].

  7. Pitkanen OM, Hornberger LK, Miner SE, et al. Borderline left ventricles in prenatally diagnosed atrioventricular septal defect or double outlet right ventricle: echocardiographic predictors of biventricular repair. Am Heart J. Jul 2006;152(1):163.e1-7. [Medline].

  8. Tongsong T, Chanprapaph P, Sittiwangkul R, Khunamornpong S. Antenatal diagnosis of double outlet of right ventricle without extracardiac anomaly: a report of 4 cases. J Clin Ultrasound. May 2007;35(4):221-5. [Medline].

  9. Beekmana RP, Roest AA, Helbing WA, et al. Spin echo MRI in the evaluation of hearts with a double outlet right ventricle: usefulness and limitations. Magn Reson Imaging. Apr 2000;18(3):245-53. [Medline].

  10. Artrip JH, Sauer H, Campbell DN, et al. Biventricular repair in double outlet right ventricle: surgical results based on the STS-EACTS International Nomenclature classification. Eur J Cardiothorac Surg. Apr 2006;29(4):545-50. [Medline].

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  13. Bartelings MM, Gittenberger-de Groot AC. Morphogenetic considerations on congenital malformations of the outflow tract. Part 2: Complete transposition of the great arteries and double outlet right. International Journal of Cardiology. 1991;33:5-26. [Medline].

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  21. Takeuchi K, McGowan FX Jr, Moran AM, et al. Surgical outcome of double-outlet right ventricle with subpulmonary VSD. Ann Thorac Surg. Jan 2001;71(1):49-52; discussion 52-3. [Medline].

  22. Tchervenkov CI, Korkola SJ, Beland MJ. Single-stage anatomical repair of complete atrioventricular canal, double-outlet right ventricle, and cor triatriatum using ventricular septal defect translocation. Ann Thorac Surg. Apr 2002;73(4):1317-20. [Medline].

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Further Reading

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Keywords

double outlet right ventricle, normally related great arteries, DORV, both great arteries originating from the right ventricle, partial transposition complex of the great arteries, transposition of the aorta and levoposition of the pulmonary artery, congenital heart defect, CHD, ventricular septal defect, VSD, Taussig-Bing anomaly, tetralogy of Fallot, DiGeorge syndrome, DiGeorge's syndrome, neural crest, congestive heart failure, subaortic stenosis, arch obstruction, atrioventricular canal defect, mitral stenosis, coarctation of the aorta, interrupted aortic arch, mitral atresia, atrial septal defect, ASD, tricuspid regurgitation, Ebstein malformation, transposition of the great arteries, hypercyanotic spells, polycythemia, failure to thrive

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Contributor Information and Disclosures

Author

Rod Tarrago, MD, Pediatric Intensivist, Department of Pediatric Critical Care, Children's Hospitals and Clinics of Minnesota
Rod Tarrago, MD is a member of the following medical societies: Society of Critical Care Medicine
Disclosure: Nothing to disclose.

Coauthor(s)

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.

Medical Editor

Juan Carlos Alejos, MD, Associate Clinical Professor, Department of Pediatrics, Division of Cardiology, University of California at Los Angeles
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

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

Managing Editor

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.

CME Editor

Gilbert Herzberg, MD, Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College
Gilbert 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.

 
 
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