Medscape is available in 5 Language Editions – Choose your Edition here.


Double Outlet Right Ventricle With Normally Related Great Arteries Workup

  • Author: Maggie L Likes, MD; Chief Editor: Stuart Berger, MD  more...
Updated: Jan 08, 2016

Laboratory Studies

See the list below:

  • 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

See the list below:

  • 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

See the list below[10] :

  • 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.


See the list below:

  • 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

See the list below:

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

Maggie L Likes, MD Pediatric Cardiologist, Seattle Children's Heart Center; Assistant Professor of Pediatrics, University of Washington School of Medicine

Maggie L Likes, MD is a member of the following medical societies: American Society of Echocardiography

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.

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, American Autonomic Society, American Physiological Society

Disclosure: Received grant/research funds from Lundbeck Pharmaceuticals for none.

Chief Editor

Stuart Berger, MD Medical Director of The Heart Center, Children's Hospital of Wisconsin; Associate Professor, Department of Pediatrics, Section of Pediatric Cardiology, Medical College 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, Society for Cardiovascular Angiography and Interventions

Disclosure: Nothing to disclose.

Additional Contributors

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, International Society for Heart and Lung Transplantation

Disclosure: Received honoraria from Actelion for speaking and teaching.


Rod Tarrago, MD Pediatric Intensivist, Children's Respiratory and Critical Care Specialists; Chief Medical Information Officer, 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.

  1. Witham AC. Double outlet right ventricle; a partial transposition complex. Am Heart J. 1957 Jun. 53(6):928-39. [Medline].

  2. Al-Muhaya MA, Ismail SR, Abu-Sulaiman RM, Kabbani MS, Najm HK. Short- and mid-term outcomes of total correction of Taussig-Bing anomaly. Pediatr Cardiol. 2012 Feb. 33(2):258-63. [Medline].

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

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

  5. 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].

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

  7. 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].

  8. 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. 2006 Jul. 152(1):163.e1-7. [Medline].

  9. 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. 2007 May. 35(4):221-5. [Medline].

  10. Shi K, Yang ZG, Chen J, Zhang G, Xu HY, Guo YK. Assessment of Double Outlet Right Ventricle Associated with Multiple Malformations in Pediatric Patients Using Retrospective ECG-Gated Dual-Source Computed Tomography. PLoS One. 2015. 10 (6):e0130987. [Medline]. [Full Text].

  11. 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. 2000 Apr. 18(3):245-53. [Medline].

  12. Li S, Ma K, Hu S, et al. Biventricular repair for double outlet right ventricle with non-committed ventricular septal defect. Eur J Cardiothorac Surg. 2015 Oct. 48 (4):580-7; discussion 587. [Medline].

  13. Tan LH, Du LZ, Carr MR, Kuzin JK, Moffett BS, Chang AC. Captopril induced reversible acute renal failure in a premature neonate with double outlet right ventricle and congestive heart failure. World J Pediatr. 2011 Feb. 7(1):89-91. [Medline].

  14. Dirks V, Prêtre R, Knirsch W, Valsangiacomo Buechel ER, Seifert B, Schweiger M, et al. Modified Blalock Taussig shunt: a not-so-simple palliative procedure. Eur J Cardiothorac Surg. 2013 Mar 28. [Medline].

  15. Lacour-Gayet F. Complexity stratification of the arterial switch operation: a second learning curve. Cardiol Young. 2012 Dec. 22(6):739-44. [Medline].

  16. 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. 2006 Apr. 29(4):545-50. [Medline].

  17. Takeuchi K, McGowan FX, Bacha EA, et al. Analysis of surgical outcome in complex double-outlet right ventricle with heterotaxy syndrome or complete atrioventricular canal defect. Ann Thorac Surg. 2006 Jul. 82(1):146-52. [Medline].

  18. 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].

  19. Battistessa S, Soto B. Double outlet right ventricle with discordant atrioventricular connexion: an angiographic analysis of 19 cases. International Journal of Cardiology. 1990. 27:253-267. [Medline].

  20. Belli E, Serraf A, Lacour-Gayet F, et al. Biventricular repair for double-outlet right ventricle. Results and long-term follow-up. Circulation. 1998. 98 (19 supplement):360-367. [Medline].

  21. Belli E, Serraf A, Lacour-Gayet F, et al. Double-outlet right ventricle with non-committed ventricular septal defect. Eur J Cardiothorac Surg. 1999 Jun. 15(6):747-52. [Medline].

  22. D'Alessandro LC, Latney BC, Paluru PC, Goldmuntz E. The phenotypic spectrum of ZIC3 mutations includes isolated d-transposition of the great arteries and double outlet right ventricle. Am J Med Genet A. 2013 Apr. 161A(4):792-802. [Medline]. [Full Text].

  23. De Luca A, Sarkozy A, Ferese R, Consoli F, Lepri F, Dentici ML, et al. New mutations in ZFPM2/FOG2 gene in tetralogy of Fallot and double outlet right ventricle. Clin Genet. 2011 Aug. 80(2):184-90. [Medline].

  24. Drenthen W, Pieper PG, van der Tuuk K, et al. Fertility, pregnancy and delivery in women after biventricular repair for double outlet right ventricle. Cardiology. 2008. 109(2):105-9. [Medline].

  25. Manner J, Seidl W, Steding G. Embryological observations on the morphogenesis of double-outlet right ventricle with subaortic ventricular septal defect and normal arrangement of the great arteries. Thorac Cardiovasc Surg. 1995 Dec. 43(6):307-12. [Medline].

  26. Oppido G, Napoleone CP, Loforte A, et al. Complex double-outlet right ventricle repair in a neonate with complete tracheal agenesis. J Thorac Cardiovasc Surg. 2004 Jan. 127(1):283-5. [Medline].

  27. Patel CR, Steele MA, Stewart JW. Double-outlet right ventricle with partial anomalous pulmonary venous connection:prenatal diagnosis. J Ultrasound Med. 2005 Jun. 24(6):861-4. [Medline].

  28. Silka MJ. Double-outlet ventricles. The Science and Practice of Pediatric Cardiology. 2nd ed. 1997. 1132, 1505-23.

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

  30. Tan ZP, Huang C, Xu ZB, Yang JF, Yang YF. Novel ZFPM2/FOG2 variants in patients with double outlet right ventricle. Clin Genet. 2011 Sep 15. [Medline].

  31. 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. 2002 Apr. 73(4):1317-20. [Medline].

  32. Walters HL, Mavroudis C, Tchervenkov CI, et al. Congenital Heart Surgery Nomenclature and Database Project: double outlet right ventricle. Ann Thorac Surg. 2000 Apr. 69(4 Suppl):S249-63. [Medline].

  33. Wernovsky G, Hanley FL. Double outlet right ventricle. Pediatric Cardiac Intensive Care. 1st ed. 1998. 301-3.

Neonate with double outlet right ventricle. Chest radiograph shows a mildly enlarged heart with symmetrically slightly increased pulmonary vasculature.
Double outlet right ventricle with subaortic ventricular septal defect. Arrow shows flow of oxygenated blood from left ventricle to aorta.
Repair of double outlet right ventricle with subaortic ventricular septal defect.
Double outlet right ventricle with subpulmonary ventricular septal defect (Taussig-Bing anomaly).
Complex repair of double outlet right ventricle with subpulmonary ventricular septal defect.
Double outlet right ventricle with doubly committed ventricular septal defect.
Repair of double outlet right ventricle with doubly committed ventricular septal defect showing VSD patch and intraventricular baffle.
Double outlet right ventricle with noncommitted ventricular septal defect.
Repair of double outlet right ventricle with noncommitted ventricular septal defect using a long ventricular septal defect patch.
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.