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Double-outlet left ventricle: Diagnosis and management

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Double-outlet left ventricle (DOLV) is a rare congenital cardiac malformation in which both great arteries originate entirely or predominantly from the morphologic left ventricle. DOLV occurs most commonly in the form of atrial situs solitus with atrioventricular (AV) concordance. Ventricular septal defect (VSD) is most commonly subaortic, and 90% of these patients have associated pulmonary stenosis. In most cases, there is a right anterior position of the aorta relative to the pulmonary artery. Most patients with DOLV will have pulmonary outflow obstruction and present soon after birth with cyanosis. DOLV with two well-developed ventricles is conventionally treated surgically by VSD closure, closure of the native left ventricle-to-pulmonary outflow, and placement of a right ventricle-to-pulmonary artery extracardiac conduit. However, pulmonary root translocation may be used as an alternative to bi ventricular repair of DOLV. Patients with DOLV and functionally single-ventricle or AV valve atresia will require a Fontan procedure.

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References and Recommended Reading

  1. Tchervenkov CI, Walters HL 3rd, Chu VF: Congenital Heart Surgery Nomenclature and Database Project: double outlet left ventricle. Ann Thorac Surg 2000, 69(Suppl 4): S264–S269.

    Article  PubMed  CAS  Google Scholar 

  2. Bengur AR, Snider AR, Peters J, Merida-Asmus L: Two-dimensional echocardiographic features of double outlet left ventricle. J Am Soc Echocardiogr 1990, 3: 320–325.

    PubMed  CAS  Google Scholar 

  3. Van Praagh R, Weinberg PM, Srebro J: Double-outlet left ventricle. In Moss’ Heart Disease in Infants, Children, and Adolescents. Edited by Adams FH, Emmanouilides GC, Reimenschneider JA. Baltimore: Williams & Wilkins; 1989: 461–485.

    Google Scholar 

  4. Wilkinson J: Double outlet ventricle. In Pediatric Cardiology, vol 2. Edited by Anderson RH, Macartney FJ, Shinebourne EA, Tynan M. Edinburg: Churchill Livingstone; 1987: 889–911.

    Google Scholar 

  5. Sakakibara S, Takao A, Arai T, et al.: Both great vessels arising from the left ventricle. Bull Heart Inst Jpn 1967, (11): 66–86.

    Google Scholar 

  6. Bharati S, Lev M, Stewart R, et al.: The morphologic spectrum of double outlet left ventricle and its surgical significance. Circulation 1978, 58: 558–565.

    PubMed  CAS  Google Scholar 

  7. Manner J, Seidl W, Steding G: Embryological observations on the formal pathogenesis of double-outlet left ventricle with a right-ventricular infundibulum. Thorac Cardiovasc Surg 1997, 45: 172–177.

    Article  PubMed  CAS  Google Scholar 

  8. Hagler D: Double-outlet right ventricle and double-outlet left ventricle. In Moss and Adams’ Heart Disease in Infants, Children, and Adolescents, vol 2, edn 7. Edited by Allen HD, Driscoll DJ, Shaddy RE, Feltes TF. Philadelphia: Lippincott Williams & Wilkins; 2008: 1100–1127.

    Google Scholar 

  9. Gouton M, Bozio A, Rey C, et al.: Double outlet left ventricle: a rare and unusual cardiopathy. Apropos of 7 new cases [in French]. Arch Mal Coeur Vaiss 1996, 89: 553–559.

    PubMed  CAS  Google Scholar 

  10. Beitzke A, Suppan C: Double outlet left ventricle with intact ventricular septum. Int J Cardiol 1984, 5: 175–183.

    Article  PubMed  CAS  Google Scholar 

  11. Pacifico AD, Kirklin JW, Bargeron LM Jr, Soto B: Surgical treatment of double-outlet left ventricle. Report of four cases. Circulation 1973, 48(Suppl 1): III19–III23.

    PubMed  CAS  Google Scholar 

  12. Akagawa H, Yoshioka F, Isomura T, et al.: Surgical treatment of double-outlet left ventricle in situs inversus [I,D,D]. Ann Thorac Surg 1984, 37: 337–342.

    PubMed  CAS  Google Scholar 

  13. Subirana MT, de Leval M, Somerville J: Double-outlet left ventricle with atrioventricular discordance. Am J Cardiol 1984, 54: 1385–1388.

    Article  PubMed  CAS  Google Scholar 

  14. Alehan D, Dogan OF, Ozkutlu S: Echocardiographic diagnosis of an extremely rare case with double-outlet left ventricle, tricuspid atresia, and two balanced ventricles. Pediatr Cardiol 2007, 28: 418–419.

    Article  PubMed  CAS  Google Scholar 

  15. Lilje C, Weiss F, Lacour-Gayet F, et al.: Images in cardiovascular medicine. Double-outlet left ventricle. Circulation 2007, 115(3): e36–e37.

    Article  Google Scholar 

  16. 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.

    Article  PubMed  CAS  Google Scholar 

  17. McElhinney DB, Reddy VM, Hanley FL: Pulmonary root translocation for biventricular repair of double-outlet left ventricle with absent subpulmonic conus. J Thorac Cardiovasc Surg 1997, 114: 501–503.

    Article  PubMed  CAS  Google Scholar 

  18. Murphy DA, Gillis DA, Sridhara KS: Intraventricular repair of double-outlet left ventricle. Ann Thorac Surg 1981, 31: 364–369.

    Article  PubMed  CAS  Google Scholar 

  19. Kerr AR, Barcia A, Bargeron LM Jr, Kirklin JW: Double-outlet left ventricle with ventricular septal defect and pulmonary stenosis: report of surgical repair. Am Heart J 1971, 81: 688–693.

    Article  PubMed  CAS  Google Scholar 

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  1. Mayo Clinic College of Medicine, 200 First Street SW, Rochester, MN, 55905, USA

    Donald J. Hagler

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  1. Shaji C. Menon
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  2. Donald J. Hagler
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Correspondence to Donald J. Hagler.

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Menon, S.C., Hagler, D.J. Double-outlet left ventricle: Diagnosis and management. Curr Treat Options Cardio Med 10, 448–452 (2008). https://doi.org/10.1007/s11936-008-0037-6

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