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Double Outlet Right Ventricle

  • Ravi S. Samraj
  • Ross M. Ungerleider
  • Inder MehtaEmail author
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Abstract

Double outlet right ventricle (DORV) is a relatively rare group of cardiac anomalies characterized by both great arteries (pulmonary and aorta) arising primarily from the right ventricle. It includes a broad spectrum of anatomical variants and associated malformations. The multiple anatomic variations of this lesion are commonly classified by the relationship of the great vessels to one another and/or by the location of the ventricular septal defect to the great vessels. The location of the ventricular septal defect and its size are critical to the repair. Surgical options include one-stage biventricular repair, biventricular repair with a conduit, or staged palliative single-ventricle surgery. Surgical treatment can be offered to most patients with low operative mortality and excellent long-term survival.

Keywords

Double outlet right ventricle Heterotaxy syndromes Rastelli procedure Ventriculoarterial connection Ventricular septal defect 

References

  1. 1.
    Obler D, Juraszek AL, Smoot LB, Natowicz MR. Double outlet right ventricle: aetiologies and associations. J Med Genet. 2008;45:481–97.PubMedCrossRefPubMedCentralGoogle Scholar
  2. 2.
    Botto LD, Correa A. Decreasing the burden of congenital heart anomalies: an epidemiologic evaluation of risk factors and survival. Prog Pediatr Cardiol. 2003;18:111–21.CrossRefGoogle Scholar
  3. 3.
    Loffredo CA. Epidemiology of cardiovascular malformations: prevalence and risk factors. Am J Med Genet. 2000;9:319–25.CrossRefGoogle Scholar
  4. 4.
    Srivastava D, Olson EN. A genetic blueprint for cardiac development. Nature. 2000;407:221–6.PubMedCrossRefPubMedCentralGoogle Scholar
  5. 5.
    Van Praagh S, Davidoff A, Chin A, Shiel SF, Reynolds J, Van Praagh R. Double outlet right ventricle: anatomic types and developmental implications based on a study of 101 autopsied cases. Coeur. 1982;13:389–439.Google Scholar
  6. 6.
    Witham AC. Double outlet right ventricle; a partial transposition complex. Am Heart J. 1957;53:928–39.PubMedCrossRefPubMedCentralGoogle Scholar
  7. 7.
    McGoon DC. Origin of both great vessels from the right ventricle. Surg Clin North Am. 1961;41:1113–20.CrossRefGoogle Scholar
  8. 8.
    Walters HL III, Mavroudis C, Tchervenkov CI, Jacobs JP, Lacour-Gayet F, Jacobs ML. Congenital Heart Surgery Nomenclature and Database Project: double outlet right ventricle. Ann Thorac Surg. 2000;69(4 Suppl):S249–63.PubMedCrossRefPubMedCentralGoogle Scholar
  9. 9.
    Moorman A, Webb S, Brown NA, Lamers W, Anderson RH. Development of the heart: (1) formation of the cardiac chambers and arterial trunks. Heart. 2003;89:806–14.PubMedPubMedCentralCrossRefGoogle Scholar
  10. 10.
    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;43:307–12.PubMedCrossRefPubMedCentralGoogle Scholar
  11. 11.
    Patel CR, Muise KL, Redline RW. Double-outlet right ventricle with intact ventricular septum in a foetus with trisomy-18. Cardiol Young. 1999;9:419–22.PubMedCrossRefPubMedCentralGoogle Scholar
  12. 12.
    Khositseth A, Tocharoentanaphol C, Khowsathit P, Ruangdaraganon N. Chromosome 22q11 deletions in patients with conotruncal heart defects. Pediatr Cardiol. 2005;26:570–3.PubMedCrossRefPubMedCentralGoogle Scholar
  13. 13.
    Wilkinson JL, Wilcox BR, Anderson RH. The anatomy of double outlet right ventricle. Pediatr Cardiol. 1981;5:397–407.Google Scholar
  14. 14.
    Lev M, Bharati S, Meng CC, Liberthson RR, Paul MH, Idriss F. A concept of double-outlet right ventricle. J Thorac Cardiovasc Surg. 1972;64:271–81.PubMedCrossRefPubMedCentralGoogle Scholar
  15. 15.
    Taussig HB, Bing RJ. Complete transposition of the aorta and a levoposition of the pulmonary artery; clinical, physiological, and pathological findings. Am Heart J. 1949;37:551–9.PubMedCrossRefPubMedCentralGoogle Scholar
  16. 16.
    Stellin G, Ho SY, Anderson RH, Zuberbuhler JR, Siewers RD. The surgical anatomy of double-outlet right ventricle with concordant atrioventricular connection and noncommitted ventricular septal defect. J Thorac Cardiovasc Surg. 1991;102:849–55.PubMedCrossRefPubMedCentralGoogle Scholar
  17. 17.
    Lacour-Gayet F. Intracardiac repair of double outlet right ventricle. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2008;11:39–43.CrossRefGoogle Scholar
  18. 18.
    Anderson RH, Ho SY, Wilcox BR. The surgical anatomy of ventricular septal defect part IV: double outlet ventricle. J Card Surg. 1996;11:2–11.PubMedCrossRefPubMedCentralGoogle Scholar
  19. 19.
    Kirklin JK, Westaby S, Blackstone EH, Kirklin JW, Chenoweth DE, Pacifico AD. Complement and the damaging effects of cardiopulmonary bypass. J Thorac Cardiovasc Surg. 1983;86:845–57.PubMedCrossRefPubMedCentralGoogle Scholar
  20. 20.
    Wilcox BR, Ho SY, Macartney FJ, Becker AE, Gelis LM, Anderson RH. Surgical anatomy of double-outlet right ventricle with situs solitus and atrioventricular concordance. J Thorac Cardiovasc Surg. 1981;82:405–17.PubMedCrossRefPubMedCentralGoogle Scholar
  21. 21.
    Stark J, de Levai M. Surgery for congenital heart defects. Philadelphia: WB Saunders; 1994.Google Scholar
  22. 22.
    Replogle RL, Campbell DJ, Campbell CD, Arcilla RA. Double-outlet ventricles. In: Arciniegas E, editor. Pediatric cardiac surgery. Chicago: Year Book Medical Publishers; 1985.Google Scholar
  23. 23.
    Judson JP, Danielson GK, Puga FJ, Mair DD, McGoon DC. Double-outlet right ventricle. Surgical results, 1970–1980. J Thorac Cardiovasc Surg. 1983;85:32–40.PubMedCrossRefPubMedCentralGoogle Scholar
  24. 24.
    Marin-Garcia J, Neches WH, Park SC, Lenox CC, Zuberbuhler JR, Bahnson HT. Double-outlet right ventricle with restrictive ventricular septal defect. J Thorac Cardiovasc Surg. 1978;76:853–8.PubMedCrossRefPubMedCentralGoogle Scholar
  25. 25.
    Cherian KM, John TA, Abraham KA. Transatrial correction of origin of both great vessels from right ventricle with pulmonary hypertension. J Thorac Cardiovasc Surg. 1982;84:783–6.PubMedCrossRefPubMedCentralGoogle Scholar
  26. 26.
    Kirklin JW, Barratt-Boyce B. Cardiac surgery. New York: Wiley Medical Publications; 1986.Google Scholar
  27. 27.
    Singh A, Letsky EA, Stark J. Hemolysis following correction of double-outlet right ventricle. J Thorac Cardiovasc Surg. 1976;71:226–9.PubMedCrossRefPubMedCentralGoogle Scholar
  28. 28.
    Judson JP, Danielson GK, Puga FJ, et al. Double outlet right ventricle: surgical results 1970–1980. J Thorac Cardiovasc Surg. 1983;85:32–40.PubMedCrossRefPubMedCentralGoogle Scholar
  29. 29.
    Pigott JD, Chin AJ, Weinberg PM, Wagner HR, Norwood WI. Transposition of the great arteries with aortic arch obstruction. Anatomical review and report of surgical management. J Thorac Cardiovasc Surg. 1987;94:82–6.PubMedCrossRefPubMedCentralGoogle Scholar
  30. 30.
    Yacoub MH, Radley-Smith R. Anatomic correction of the Taussig-Bing anomaly. J Thorac Cardiovasc Surg. 1984;88:380–8.PubMedCrossRefPubMedCentralGoogle Scholar
  31. 31.
    Quaegebeur JM, Rohmer J, Ottenkamp J, et al. The arterial switch operation. An eight-year experience. J Thorac Cardiovasc Surg. 1986;92(3 Pt 1):361–84.PubMedCrossRefPubMedCentralGoogle Scholar
  32. 32.
    Kanter KR, Anderson RH, Lincoln C, Rigby ML, Shinebourne EA. Anatomic correction for complete transposition and double-outlet right ventricle. J Thorac Cardiovasc Surg. 1985;90:690–9.PubMedCrossRefPubMedCentralGoogle Scholar
  33. 33.
    Pacifico AD. Double outlet right ventricle. In: Sabiston Jr DC, editor. Davis-Christopher textbook of surgery. 12th ed. Philadelphia: W.B. Saunders Co; 1981.Google Scholar
  34. 34.
    Ebert PA, Robinson SJ, Stanger P, Engle MA. Pulmonary artery conduits in infants younger than six months of age. J Thorac Cardiovasc Surg. 1976;72:351–6.PubMedCrossRefPubMedCentralGoogle Scholar
  35. 35.
    Angell WW, Angell JD, Oury JH, Lamberti JJ, Grehl TM. Long-term follow-up of viable frozen aortic homografts. A viable homograft valve bank. J Thorac Cardiovasc Surg. 1987;93:815–22.PubMedCrossRefPubMedCentralGoogle Scholar
  36. 36.
    O’Brien MF, Stafford EG, Gardner MA, Pohlner PG, McGiffin DC. A comparison of aortic valve replacement with viable cryopreserved and fresh allograft valves, with a note on chromosomal studies. J Thorac Cardiovasc Surg. 1987;94:812–23.PubMedCrossRefPubMedCentralGoogle Scholar
  37. 37.
    Kirklin JW, Pacifico AD, Blackstone EH, Kirklin JK, Bargeron LM Jr. Current risks and protocols for operations for double-outlet right ventricle. Derivation from an 18 year experience. J Thorac Cardiovasc Surg. 1986;92:913–30.PubMedCrossRefPubMedCentralGoogle Scholar
  38. 38.
    Piccoli G, Pacifico AD, Kirklin JW, Blackstone EH, Kirklin JK, Bargeron LM Jr. Changing results and concepts in the surgical treatment of double-outlet right ventricle: analysis of 137 operations in 126 patients. Am J Cardiol. 1983;52:549–54.PubMedCrossRefPubMedCentralGoogle Scholar
  39. 39.
    Sondheimer HM, Freedom RM, Olley PM. Double outlet right ventricle: clinical spectrum and prognosis. Am J Cardiol. 1977;39:709–14.PubMedCrossRefPubMedCentralGoogle Scholar
  40. 40.
    Stewart RW, Kirklin JW, Pacifico AD, Blackstone EH, Bargeron LM Jr. Repair of double-outlet right ventricle. An analysis of 62 cases. J Thorac Cardiovasc Surg. 1979;78:502–14.PubMedCrossRefPubMedCentralGoogle Scholar
  41. 41.
    Mazzucco A, Faggian G, Stellin G, et al. Surgical management of double-outlet right ventricle. J Thorac Cardiovasc Surg. 1985;90:29–34.PubMedCrossRefPubMedCentralGoogle Scholar
  42. 42.
    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;29:545–50.PubMedCrossRefPubMedCentralGoogle Scholar
  43. 43.
    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 Suppl):II360–5.PubMedPubMedCentralGoogle Scholar
  44. 44.
    Bradley TJ, Karamlou T, Kulik A, et al. Determinants of repair type, reintervention, and mortality in 393 children with double-outlet right ventricle. J Thorac Cardiovasc Surg. 2007;134:967–73 e6.PubMedCrossRefPubMedCentralGoogle Scholar
  45. 45.
    Brown JW, Ruzmetov M, Okada Y, Vijay P, Turrentine MW. Surgical results in patients with double outlet right ventricle: a 20-year experience. Ann Thorac Surg. 2001;72:1630–5.PubMedCrossRefPubMedCentralGoogle Scholar
  46. 46.
    Vogt PR, Carrel T, Pasic M, Arbenz U, von Segesser LK, Turina MI. Early and late results after correction for double-outlet right ventricle: uni- and multivariate analysis of risk factors. Eur J Cardiothorac Surg. 1994;8:301–7.PubMedCrossRefPubMedCentralGoogle Scholar
  47. 47.
    Kreutzer C, De Vive J, Oppido G, et al. Twenty-five-year experience with rastelli repair for transposition of the great arteries. J Thorac Cardiovasc Surg. 2000;120:211–23.PubMedCrossRefPubMedCentralGoogle Scholar
  48. 48.
    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. 1999;68:1692–7.PubMedCrossRefPubMedCentralGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2020

Authors and Affiliations

  • Ravi S. Samraj
    • 1
  • Ross M. Ungerleider
    • 2
  • Inder Mehta
    • 2
    Email author
  1. 1.Department of Anesthesiology and Pediatric Critical CareUTMB, Driscoll Children’s HospitalCorpus ChristiUSA
  2. 2.Department of Cardiothoracic SurgeryDriscoll Children’s HospitalCorpus ChristiUSA

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