Double Outlet Right Ventricle, With Transposition Treatment & Management

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

Medical Care

Medical treatment depends on the clinical presentation, which is determined by the different physiology of each type of double outlet right ventricle (DORV).

In DORV with no pulmonary valve stenosis (PS), direct medical management at reducing congestive heart failure (CHF) to improve the patient's condition prior to surgery. Management of CHF requires medications such as loop diuretics (eg, furosemide), potassium-sparing diuretics (eg, spironolactone), and digitalis. In addition, observe subacute bacterial endocarditis prophylaxis.

Infants with a subpulmonary ventricular septal defect (VSD) with a small or restrictive patent foramen ovale or atrial septal defect may require balloon atrial septostomy or blade atrial septostomy to improve interatrial mixing of saturated and desaturated blood and to decompress the left atrium.

In patients with DORV and PS with marked cyanosis and hypoxemia, initial medical management consists of increasing the fraction of inspired oxygen (FIO2), which may be up to 100%. This decreases pulmonary vascular resistance, thereby increasing the amount of blood flow in the lungs with consequent increase in overall organ oxygenation.

Next

Surgical Care

Two surgical approaches are appropriate, depending on the degree of CHF.

Palliative surgery

As with medical treatment, this approach helps improve the patient's clinical condition, allowing him or her to gain weight to achieve optimal conditions for definitive surgical repair.

Infants with no PS who have a subpulmonary VSD, subaortic VSD, or doubly committed VSD and who present with CHF may undergo pulmonary artery (PA) banding to decrease pulmonary blood flow (PBF).

Patients with subaortic or subpulmonary VSD with PS are cyanotic and have decreased PBF; therefore, they undergo a systemic-to-PA shunt to increase PBF.

Definitive surgery

The relationship of VSD to the great arteries and the distribution of coronary artery (CA) determine surgical strategies.

Biventricular repair can be achieved in most patients with DORV. If biventricular repair is not feasible (eg, in straddling or abnormal distribution of chordae tendineae of atrioventricular [AV] valves and/or severe underdevelopment of left ventricle [LV]), a Fontan-type operation is an option with redirection of systemic (deoxygenated) blood into the PA without traversing a ventricle.

Several surgical approaches are appropriate in subpulmonary VSD; surgery is usually completed by age 3-4 months to avoid development of increased pulmonary vascular resistance. The surgical approach with the lower mortality rate of approximately 10-15% is the arterial switch operation with creation of an interventricular tunnel directing LV outflow into the PA, which becomes a neo-aorta (AO) by virtue of the switch.

If the VSD is subaortic or doubly committed, the optimal approach is to create a tunnel between the VSD and the AO to direct oxygenated blood into systemic circulation and also to eliminate mixing of the 2 circulations. Timing for this surgery depends on the size and clinical condition of the patient, but it is generally completed by age 4-6 months.

Heart transplantation

If the anatomy of associated lesions is too complex to consider an anatomic repair or if a repair results in unsatisfactory hemodynamics and intractable symptoms, consider heart transplantation. According to a report from the Children's Hospital of Pittsburgh, 15.4% of patients undergoing transplant were born with some form of DORV.[8] These patients require lifelong immunosuppression and close follow-up care.

Previous
Next

Consultations

As with any other form of congenital heart disease (CHD), parents of patients born with DORV and transposition of the great arteries may meet with a geneticist to discuss the possibility of subsequent children having this or other forms of CHD. When CHD is detected, a detailed investigation for extracardiac malformation should be performed and vice versa. Also, issues such as preterm birth and stillbirth should be taken into account in risk assessment and risk stratification in patients born with CHD.

CHD belongs to the spectrum of birth defects and, despite technological advances, it significantly contributes to infant mortality. Because extracardiac anomalies occur in 15-45% of patients with CHD, these should always be investigated.

According to one study, the most prevalent extracardiac anomalies in general are the craniofacial malformations. However, the most prevalent associated with conotruncal heart defects are anomalies of the GI and genitourinary systems. Specifically, DORV may be associated with omphalocele, gastroschisis, facial clefting, and CHARGE (coloboma, heart disease, atresia choanae, retarded growth and retarded development and/or CNS anomalies, genital hypoplasia, and ear anomalies and/or deafness) syndrome.

Preterm infants have been shown to have more than twice as many cardiovascular malformations as do term infants, and 16% of all infants with cardiovascular malformations are preterm.

Prevalence of CHD is high among late stillbirths. In particular, a greater incidence of coarctation of the AO, double-inlet left ventricle, hypoplastic left heart, truncus arteriosus, DORV, and AV septal defect is noted among stillbirths.

Previous
Next

Activity

Patients with DORV and transposition of the great arteries have no specific activity restrictions; their physiology may limit their exercise tolerance. After surgical intervention, some restrictions may be required depending on the hemodynamic result; however, these patients can usually participate in all age-appropriate activities.

Lifelong antibiotic prophylaxis is necessary prior to any potentially contaminated procedure, especially dental work.

Previous
Proceed to Medication
 
 
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.

References

  1. Derbent M, Yilmaz Z, Baltaci V, et al. Chromosome 22q11.2 deletion and phenotypic features in 30 patients with conotruncal heart defects. Am J Med Genet A. Jan 15 2003;116(2):129-35. [Medline].

  2. Goldmuntz E, Clark BJ, Mitchell LE, et al. Frequency of 22q11 deletions in patients with conotruncal defects. J Am Coll Cardiol. Aug 1998;32(2):492-8. [Medline].

  3. Sergi C, Serpi M, Muller-Navia J, et al. CATCH 22 syndrome: report of 7 infants with follow-up data and review of the recent advancements in the genetic knowledge of the locus 22q11. Pathologica. Jun 1999;91(3):166-72. [Medline].

  4. Siman CM, Gittenberger-De Groot AC, Wisse B, Eriksson UJ. Malformations in offspring of diabetic rats: morphometric analysis of neural crest-derived organs and effects of maternal vitamin E treatment. Teratology. May 2000;61(5):355-67. [Medline].

  5. Obler D, Juraszek A, Smoot LB, Natowicz MR. Double Outlet Right Ventricle: Aetiologies and Associations. J Med Genet. May 2 2008;[Medline].

  6. Tometzki AJ, Suda K, Kohl T, et al. Accuracy of prenatal echocardiographic diagnosis and prognosis of fetuses with conotruncal anomalies. J Am Coll Cardiol. May 1999;33(6):1696-701. [Medline].

  7. Chen GZ, Huang GY, Liang XC, et al. Methodological study on real-time three-dimensional echo-cardiography and its application in the diagnosis of complex congenital heart disease. Chin Med J (Engl). Jul 20 2006;119(14):1190-4. [Medline].

  8. Devine WA, Webber SA, Anderson RH. Congenitally malformed hearts from a population of children undergoing cardiac transplantation: comments on sequential segmental analysis and dissection. Pediatr Dev Pathol. Mar-Apr 2000;3(2):140-54. [Medline].

  9. Kim N, Friedberg MK, Silverman NH. Diagnosis and prognosis of fetuses with double outlet right ventricle. Prenat Diagn. Aug 2006;26(8):740-5. [Medline].

  10. Gelehrter S, Owens ST, Russell MW, van der Velde ME, Gomez-Fifer C. Accuracy of the fetal echocardiogram in double-outlet right ventricle. Congenit Heart Dis. Jan 2007;2(1):32-7. [Medline].

  11. Bajolle F, Zaffran S, Kelly RG, et al. Rotation of the myocardial wall of the outflow tract is implicated in the normal positioning of the great arteries. Circ Res. Feb 17 2006;98(3):421-8. [Medline].

  12. Baschat AA, Gembruch U, Knopfle G, Hansmann M. First-trimester fetal heart block: a marker for cardiac anomaly. Ultrasound Obstet Gynecol. Nov 1999;14(5):311-4. [Medline].

  13. 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].

  14. Benito Bartolome F, Sanchez Fernandez-Bernal C, Torres Feced V. [Catheter ablation of accessory pathways in patients with congenital cardiopathies]. Rev Esp Cardiol. Nov 1999;52(11):1028-31. [Medline].

  15. Black MD, Shukla V, Freedom RM. Direct neonatal ventriculo-arterial connections (REV): early results and future implications. Ann Thorac Surg. Apr 1999;67(4):1137-41. [Medline].

  16. Blume ED, Altmann K, Mayer JE, et al. Evolution of risk factors influencing early mortality of the arterial switch operation. J Am Coll Cardiol. May 1999;33(6):1702-9. [Medline].

  17. Bosi G, Scorrano M, Tosato G, et al. The Italian Multicentric Study on Epidemiology of Congenital Heart Disease: first step of the analysis. Working Party of the Italian Society of Pediatric Cardiology. Cardiol Young. May 1999;9(3):291-9. [Medline].

  18. Breymann T, Boethig D, Goerg R, Thies WR. The Contegra bovine valved jugular vein conduit for pediatric RVOT reconstruction: 4 years experience with 108 patients. J Card Surg. Sep-Oct 2004;19(5):426-31. [Medline].

  19. Casaldaliga J, Girona JM, Sanchez C, et al. [Doppler echocardiography in the postoperative assessment of the arterial switch in the repair of transposition of the great arteries and double outlet right ventricle]. Rev Esp Cardiol. Feb 1996;49(2):117-23. [Medline].

  20. Casta AR, Jonas RA, Mayer JE. Double-Outlet Right Ventricle. In: Cardiac Surgery of the Neonate and Infant. Philadelphia, PA: WB Saunders Co; 1994:445-59.

  21. Chaoui R, Korner H, Bommer C, et al. [Prenatal diagnosis of heart defects and associated chromosomal aberrations]. Ultraschall Med. Oct 1999;20(5):177-84. [Medline].

  22. Dearani JA, Danielson GK, Puga FJ, et al. Late follow-up of 1095 patients undergoing operation for complex congenital heart disease utilizing pulmonary ventricle to pulmonary artery conduits. Ann Thorac Surg. Feb 2003;75(2):399-410; discussion 410-1. [Medline].

  23. Digilio MC, Marino B, Ammirati A, et al. Cardiac malformations in patients with oral-facial-skeletal syndromes: clinical similarities with heterotaxia. Am J Med Genet. Jun 4 1999;84(4):350-6. [Medline].

  24. Donnelly LF, Higgins CB. MR imaging of conotruncal abnormalities. AJR Am J Roentgenol. Apr 1996;166(4):925-8. [Medline].

  25. Franco D, Campione M. The role of Pitx2 during cardiac development. Linking left-right signaling and congenital heart diseases. Trends Cardiovasc Med. May 2003;13(4):157-63. [Medline].

  26. Gober V, Berdat P, Pavlovic M, et al. Adverse mid-term outcome following RVOT reconstruction using the Contegra valved bovine jugular vein. Ann Thorac Surg. Feb 2005;79(2):625-31. [Medline].

  27. Gonçalves LF, Nien JK, Espinoza J, Kusanovic JP, Lee W, Swope B, et al. What does 2-dimensional imaging add to 3- and 4-dimensional obstetric ultrasonography?. J Ultrasound Med. Jun 2006;25(6):691-9. [Medline]. [Full Text].

  28. Gucer S, Ince T, Kale G, et al. Noncardiac malformations in congenital heart disease: a retrospective analysis of 305 pediatric autopsies. Turk J Pediatr. Apr-Jun 2005;47(2):159-66. [Medline].

  29. Haas F, Wottke M, Poppert H, Meisner H. Long-term survival and functional follow-up in patients after the arterial switch operation. Ann Thorac Surg. Nov 1999;68(5):1692-7. [Medline].

  30. Hagler DJ. Double-Outlet Right Ventricle. In: Heart Disease in Infants, Children, and Adolescents: Including the Fetus and Young Adult. Vol 2. Baltimore, MD: Williams and Wilkins; 1995:1246-70.

  31. Kleinert S, Sano T, Weintraub RG, et al. Anatomic features and surgical strategies in double-outlet right ventricle. Circulation. Aug 19 1997;96(4):1233-9. [Medline].

  32. Lee Y, Song AJ, Baker R, et al. Jumonji, a nuclear protein that is necessary for normal heart development. Circ Res. May 12 2000;86(9):932-8. [Medline]. [Full Text].

  33. Napoleone RM, Varela M, Andersson HC. Complex congenital heart malformations in mosaic tetrasomy 8p: case report and review of the literature. Am J Med Genet. Dec 19 1997;73(3):330-3. [Medline].

  34. Niezen RA, Beekman RP, Helbing WA, et al. Double outlet right ventricle assessed with magnetic resonance imaging. Int J Card Imaging. Aug 1999;15(4):323-9. [Medline].

  35. Ohuchi H, Hiraumi Y, Tasato H, et al. Comparison of the right and left ventricle as a systemic ventricle during exercise in patients with congenital heart disease. Am Heart J. Jun 1999;137(6):1185-94. [Medline].

  36. Pretre R, Ye Q, Fasnacht M, et al. Recent experience with the arterial switch operation in transposition of the great arteries. Schweiz Med Wochenschr. Oct 9 1999;129(40):1443-9. [Medline].

  37. Reddy VM, Hanley FL. Cardiac surgery in infants with very low birth weight. Semin Pediatr Surg. May 2000;9(2):91-5. [Medline].

  38. Scalzo G, Cavallaro A, Pulvirenti A, et al. [Physiopathologic findings and surgical treatment in transposition of great vessels: our experience]. Pediatr Med Chir. Nov-Dec 1998;20(6):377-80. [Medline].

  39. Serraf A, Nakamura T, Lacour-Gayet F, et al. Surgical approaches for double-outlet right ventricle or transposition of the great arteries associated with straddling atrioventricular valves. J Thorac Cardiovasc Surg. Mar 1996;111(3):527-35. [Medline].

  40. Silka MJ. Double-Outlet Ventricles. In: The Science and Practice of Pediatric Cardiology. Vol 2. Baltimore, MD: Williams & Wilkins; 1997:1505-23.

  41. Sivanandam S, Glickstein JS, Printz BF, et al. Prenatal diagnosis of conotruncal malformations: diagnostic accuracy, outcome, chromosomal abnormalities, and extracardiac anomalies. Am J Perinatol. May 2006;23(4):241-5. [Medline].

  42. Smith RS, Comstock CH, Kirk JS, et al. Double-outlet right ventricle: an antenatal diagnostic dilemma. Ultrasound Obstet Gynecol. Nov 1999;14(5):315-9. [Medline].

  43. Snider AR. Abnormalities of Ventriculoarterial Connection. In: Echocardiography in Pediatric Heart Disease. St. Louis, Mo: Mosby-Year Book; 1997:323-42.

  44. Sorensen TS, Korperich H, Greil GF, et al. Operator-independent isotropic three-dimensional magnetic resonance imaging for morphology in congenital heart disease: a validation study. Circulation. Jul 13 2004;110(2):163-9. [Medline]. [Full Text].

  45. [Best Evidence] Tanner K, Sabrine N, Wren C. Cardiovascular malformations among preterm infants. Pediatrics. Dec 2005;116(6):e833-8. [Medline].

  46. Tchervenkov CI, Tahta SA, Cecere R, Beland MJ. Single-stage arterial switch with aortic arch enlargement for transposition complexes with aortic arch obstruction. Ann Thorac Surg. Dec 1997;64(6):1776-81. [Medline].

  47. Walters HL 3rd, Mavroudis C, Tchervenkov CI, et al. Congenital Heart Surgery Nomenclature and Database Project: double outlet right ventricle. Ann Thorac Surg. Apr 2000;69(4 Suppl):S249-63. [Medline].

  48. Walters HL, Pacifico AD. Double Outlet Ventricles. In: Mavroudis C, Backer CL, eds. Pediatric Cardiac Surgery. 3rd ed. Stamford, CT: Appleton & Lange; 1994:305-38.

  49. Yoo SJ, Kim YM, Choe YH. Magnetic resonance imaging of complex congenital heart disease. Int J Card Imaging. Apr 1999;15(2):151-60. [Medline].

 
Previous
Next
 
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.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.