double outlet right ventricle


Summary: Incomplete transposition of the great vessels in which both the AORTA and the PULMONARY ARTERY arise from the RIGHT VENTRICLE. The only outlet of the LEFT VENTRICLE is a large ventricular septal defect (VENTRICULAR SEPTAL DEFECTS or VSD). The various subtypes are classified by the location of the septal defect, such as subaortic, subpulmonary, or noncommitted.

Top Publications

  1. Tan Z, Huang C, Xu Z, Yang J, Yang Y. Novel ZFPM2/FOG2 variants in patients with double outlet right ventricle. Clin Genet. 2012;82:466-71 pubmed publisher
    ..5-1% of all newborns and are the most common birth defects. Double outlet right ventricle (DORV) accounts for approximately 1-3% of all CHDs...
  2. Lacour Gayet F. Biventricular repair of double outlet right ventricle with noncommitted ventricular septal defect. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2002;5:163-72 pubmed
    b>Double outlet right ventricle (DORV) with noncommitted ventricular septal defect (VSD) (DORVncVSD) represents the most extreme form of DORV, raising challenging surgical difficulties for biventricular repair...
  3. Shin oka T, Kurosawa H, Imai Y, Aoki M, Ishiyama M, Sakamoto T, et al. Outcomes of definitive surgical repair for congenitally corrected transposition of the great arteries or double outlet right ventricle with discordant atrioventricular connections: risk analyses in 189 patients. J Thorac Cardiovasc Surg. 2007;133:1318-28, 1328.e1-4 pubmed
    ..types of surgical repairs for either congenitally corrected transposition of the great arteries or double outlet right ventricle with discordant atrioventricular connections, and to analyze the risk factors that affect early and ..
  4. Obler D, Juraszek A, Smoot L, Natowicz M. Double outlet right ventricle: aetiologies and associations. J Med Genet. 2008;45:481-97 pubmed publisher
    b>Double outlet right ventricle (DORV), a clinically significant congenital heart defect, occurs in 1-3% of individuals with congenital heart defects...
  5. Troise D, Ranieri L, Arciprete P. Surgical repair for double outlet right ventricle and intact ventricular septum. Ann Thorac Surg. 2001;71:1018-9 pubmed
    We report the case of a neonate with a complex cardiac anomaly that consisted of a double outlet right ventricle, intact ventricular septum, small left ventricle, and pulmonary stenosis who underwent surgical repair with a successful ..
  6. Fujii Y, Kotani Y, Takagaki M, Arai S, Kasahara S, Otsuki S, et al. The impact of the length between the top of the interventricular septum and the aortic valve on the indications for a biventricular repair in patients with a transposition of the great arteries or a double outlet right ventricle. Interact Cardiovasc Thorac Surg. 2010;10:900-5; discussion 905 pubmed publisher patients with transposition of the great arteries (TGA)/ventricular septal defect (VSD) or double outlet right ventricle (DORV)...
  7. Pace Napoleone C, Formigari R, Chiappini B, Frascaroli G, Gargiulo G. Surgical management of double outlet right ventricle with intact ventricular septum. Ann Thorac Surg. 2003;75:586-7 pubmed
    A very rare case of double outlet right ventricle with intact ventricular septum and unrestricted pulmonary flow was successfully palliated with pulmonary banding and delayed bidirectional cavopulmonary anastomosis and mitral avulsion...
  8. Ciccolo M, Bernstein J, Collazos J, Acherman R, Restrepo H, Winters J, et al. Argatroban anticoagulation for cardiac surgery with cardiopulmonary bypass in an infant with double outlet right ventricle and a history of heparin-induced thrombocytopenia. Congenit Heart Dis. 2008;3:299-302 pubmed publisher
    ..We report the use of the anticoagulant argatroban for cardiac surgery with cardiopulmonary bypass in an infant with double outlet right ventricle and a history of HIT.
  9. Ito M, Takagi N, Sugimoto S, Oosawa H, Abe T. Pregnancy after undergoing the Fontan procedure for a double outlet right ventricle: report of a case. Surg Today. 2002;32:63-5 pubmed
    We describe a 25-year-old woman who had a successful pregnancy after the repair of a double outlet right ventricle using the Fontan procedure...

More Information


  1. Lacour Gayet F, Haun C, Ntalakoura K, Belli E, Houyel L, Marcsek P, et al. Biventricular repair of double outlet right ventricle with non-committed ventricular septal defect (VSD) by VSD rerouting to the pulmonary artery and arterial switch. Eur J Cardiothorac Surg. 2002;21:1042-8 pubmed
    Biventricular repair of double outlet right ventricle non-committed ventricular septal defect (DORVncVSD) is usually achieved by a VSD rerouting to the aorta...
  2. Meadows J, Pigula F, Lock J, Marshall A. Transcatheter creation and enlargement of ventricular septal defects for relief of ventricular hypertension. J Thorac Cardiovasc Surg. 2007;133:912-8 pubmed
    ..Recurrent obstruction is common but responds to repeated intervention. Further studies are required to establish clinical benefit. ..
  3. De Luca A, Sarkozy A, Ferese R, Consoli F, Lepri F, Dentici M, et al. New mutations in ZFPM2/FOG2 gene in tetralogy of Fallot and double outlet right ventricle. Clin Genet. 2011;80:184-90 pubmed publisher
    ..with CTD, including tetralogy of Fallot with or without pulmonary atresia (TOF, 178 patients), double outlet right ventricle (DORV, 13 patients), and truncus arteriosus (11 patients)...
  4. Murphy M, Austin C, Bapat V, Morgan G. Use of an Edwards Sapien S3 valve to replace a dysfunctional mechanical mitral valve in an 11-year old boy: another small step for surgical and interventional collaboration. Eur J Cardiothorac Surg. 2016;50:577-9 pubmed publisher
    An 11-year old boy, with complex left ventricular morphology in the setting of repaired double outlet right ventricle developed progressive mitral regurgitation leading to a repair which failed, necessitating replacement of the valve ..
  5. Uemura H, Yagihara T, Kadohama T, Kawahira Y, Yoshikawa Y. Repair of double outlet right ventricle with doubly-committed ventricular septal defect. Cardiol Young. 2001;11:415-9 pubmed
    To investigate our surgical results of intraventricular rerouting in patients having double outlet right ventricle with doubly-committed ventricular septal defect. We undertook repair in 8 patients with this particular feature...
  6. Agematsu K, Naito Y, Aoki M, Fujiwara T. Total cavo-pulmonary connection without foreign material for asplenic heart associated with partial anomalous pulmonary venous connection. Interact Cardiovasc Thorac Surg. 2008;7:344-6 pubmed
    The presented case was a 3-year-old boy diagnosed with asplenia (SLL), double outlet right ventricle, pulmonary stenosis, atrioventricular septal defect, hypoplastic left ventricle and partial anomalous pulmonary venous connection to the ..
  7. Dai S, Inubashiri E, Hanaoka U, Kanenishi K, Yamashiro C, Tanaka H, et al. Three- and four-dimensional volume-rendered imaging of fetal double-outlet right ventricle using inversion mode. Ultrasound Obstet Gynecol. 2006;28:347-9 pubmed
  8. D Alessandro L, Latney B, Paluru P, 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;161A:792-802 pubmed publisher
    ..Patients with TGA (n = 169), double outlet right ventricle (DORV; n = 89), common atrioventricular canal (CAVC; n = 41), and heterotaxy (n = 54) underwent ..
  9. Stoica S, Carpenter E, Campbell D, Mitchell M, Da Cruz E, Ivy D, et al. Morbidity of the arterial switch operation. Ann Thorac Surg. 2012;93:1977-83 pubmed publisher
    ..The only anatomic incremental risk factors for morbidity were aortic arch repair and malaligned commissures, but not primary diagnosis, weight less than 2.5 kg, or coronary patterns. ..
  10. Ono M, Goerler H, Boethig D, Breymann T. Neonatal Kawashima intraventricular repair for Taussig-Bing anomaly with oblique relationship of the great arteries. Thorac Cardiovasc Surg. 2008;56:492-5 pubmed publisher
  11. Sparks J, Seefelder C, Shamberger R, McGowan F. The perioperative management of a patient with complex single ventricle physiology and pheochromocytoma. Anesth Analg. 2005;100:972-5 pubmed
    ..We report the perioperative management with doxazosin of a patient with single ventricle physiology and cavo-pulmonary and aorto-pulmonary lung perfusion who presented for resection of a pheochromocytoma. ..
  12. Brown J, Park H, Turrentine M. Arterial switch operation: factors impacting survival in the current era. Ann Thorac Surg. 2001;71:1978-84 pubmed
    ..002). The arterial switch operation for TGA and Taussig-Bing anomaly has early low and late mortality and reoperation rates. Technical modifications in coronary reimplantation have minimized coronary artery pattern-related risks. ..
  13. Parvathy U, Balakrishnan K, Ranjith M, Moorthy J. False aneurysm following modified Blalock-Taussig shunt. Pediatr Cardiol. 2002;23:178-81 pubmed
    ..Computerized tomographic scan and angiography showed a false aneurysm of the subclavian artery at the insertion of the shunt. Successful surgical management is discussed. ..
  14. Sersar S, Baslaim G. Concealed neo-aortic incompetence and new right ventricular outflow tract obstruction after arterial switch operation in a late presenter. Heart Lung Circ. 2008;17:419-21 pubmed
    ..Aortic valve replacement (AVR) was done on the 11th postoperative day and he was then extubated and had uneventful hospital course in spite of two emergency procedures: drainage of sub-dural haematoma and appendectomy. ..
  15. Murakami T, Takei K, Ueno M, Takeda A, Yakuwa S, Nakazawa M. Aortic reservoir function after arterial switch operation in elementary school-aged children. Circ J. 2008;72:1291-5 pubmed
    ..The preserved DR suggests that dilatation of the aorta compensates for aortic reservoir function. Because large artery dysfunction predicts future cardiovascular diseases, careful follow-up is crucial. ..
  16. Fennell L, Church S, Tyrell D, Forbes G, Charles J, McCowan C, et al. Double-outlet right ventricle in a 10-month-old Friesian filly. Aust Vet J. 2009;87:204-9 pubmed publisher
    ..In horses, complex cardiac disease presents a diagnostic challenge to the clinician. Appropriate therapy must be based on an accurate diagnosis. ..
  17. Hörer J, Haas F, Cleuziou J, Schreiber C, Kostolny M, Vogt M, et al. Intermediate-term results of the Senning or Mustard procedures combined with the Rastelli operation for patients with discordant atrioventricular connections associated with discordant ventriculoarterial connections or double outlet right ventricle. Cardiol Young. 2007;17:158-65 pubmed
  18. McClung H, Prasad R, Schwartz R. Anesthetic management of tracheoesophageal fistula repair in a newborn with a double-outlet right ventricle. J Clin Anesth. 2011;23:643-5 pubmed publisher
    ..A case of a newborn with a double-outlet right ventricle and tracheoesophageal fistula is presented. The pathophysiology of double-outlet right ventricle and its anesthetic implications for tracheoesophageal fistula repair are discussed. ..
  19. Miyaji K, Nagata N, Miyamoto T, Kitahori K. Combined therapy with inhaled nitric oxide and intravenous epoprostenol (prostacyclin) for critical pulmonary perfusion after the Fontan procedure. J Thorac Cardiovasc Surg. 2003;125:437-9 pubmed
  20. Ong J, Brizard C, D Udekem Y, Weintraub R, Robertson T, Cheung M, et al. Repair of atrioventricular septal defect associated with tetralogy of Fallot or double-outlet right ventricle: 30 years of experience. Ann Thorac Surg. 2012;94:172-8 pubmed publisher
    ..022). Complete AVSD associated with TOF or DORV can be repaired with good survival. Detachment of the superior bridging leaflet provided excellent exposure. However, reoperation rate remains high. ..
  21. Bobylev D, Breymann T, Boethig D, Haverich A, Ono M. Semilunar valve replacement with decellularized homograft after Damus-Kaye-Stansel anastomosis and fontan procedure. Ann Thorac Surg. 2014;97:1792-5 pubmed publisher
    ..During follow-up of more than 2 years, the valve function is excellent, and no infectious or thromboembolic complications were seen. The decellularized homograft seems to be an ideal material in this situation. ..
  22. Pannová J, Kováčová H, Matura D, Gruszka T, Pavlicek J, Delongova P. [Ectopia cordis - case report]. Ceska Gynekol. 2015;80:214-7 pubmed
    ..Detailed examination of the heart revealed a double outlet right ventricle with a complete atrioventricular septal defect...
  23. Matsuhisa H, Yoshimura N, Niimi H, Ichida F. Open-heart surgery in an infant with heterozygous factor VII deficiency. Interact Cardiovasc Thorac Surg. 2010;10:1037-8 pubmed publisher
    ..Consequently, the prophylactic replacement therapy with recombinant factor VII is recommended during cardiac operations. ..
  24. Varghese J, Kutty S, Bisselou Moukagna K, Craft M, Abdullah I, Hammel J. Five-year experience with immediate extubation after arterial switch operations for transposition of great arteries. Eur J Cardiothorac Surg. 2017;51:728-734 pubmed publisher
    ..the 14 patients with ventricular septal defect (VSD) underwent IE, whereas none of the patients with double outlet right ventricle or aortic arch obstruction ( n  = 4) did...
  25. Lu C, Wang W, Wang Q, Liu X, Yang Y. A Novel MEF2C Loss-of-Function Mutation Associated with Congenital Double Outlet Right Ventricle. Pediatr Cardiol. 2018;39:794-804 pubmed publisher
    ..R15C, was detected in an index patient with congenital double outlet right ventricle (DORV) as well as ventricular septal defect...
  26. Joseph T, Raccuglia S, Kort S, Oviasu F, Mangion J. Anomalous right coronary artery from the main pulmonary artery in a patient with double-chambered right ventricle. Echocardiography. 2002;19:687-90 pubmed
    ..To the best of our knowledge, this is the first reported case describing the association of an anomalous right coronary artery coming off the main pulmonary artery in a patient with DCRV. ..
  27. Manuel D, Ghosh G, Joseph G, Lahiri A, George P. Criss-cross heart: Transthoracic echocardiographic features. Indian Heart J. 2018;70:71-74 pubmed publisher
    ..Ventriculo-arterial discordance was seen in three cases, concordance in one and double outlet right ventricle in one. Three cases had pulmonary stenosis and the other two had pulmonary arterial hypertension...
  28. Shibata M, Itatani K, Hayashi T, Honda T, Kitagawa A, Miyaji K, et al. Flow Energy Loss as a Predictive Parameter for Right Ventricular Deterioration Caused by Pulmonary Regurgitation After Tetralogy of Fallot Repair. Pediatr Cardiol. 2018;39:731-742 pubmed publisher
    ..82; p?=?0.014) and the diastolic FEL (r?=?0.70; p?=?0.052) in Group I. FEL by VFM is an effective tool for evaluating ventricular deterioration caused by RV workload. ..
  29. Patrick W, Mainwaring R, Reinhartz O, Punn R, Tacy T, Hanley F. Major Aortopulmonary Collateral Arteries With Anatomy Other Than Pulmonary Atresia/Ventricular Septal Defect. Ann Thorac Surg. 2017;104:907-916 pubmed publisher
    ..patients with MAPCAs and two ventricles included 5 with CAVC, 4 with corrected transposition, 3 with double outlet right ventricle, 3 with scimitar syndrome, and 2 with complex D-transposition...
  30. Meot M, Lefort B, El Arid J, Soulé N, Lothion Boulanger J, Lengellé F, et al. Intraoperative Stenting of Pulmonary Artery Stenosis in Children With Congenital Heart Disease. Ann Thorac Surg. 2017;104:190-196 pubmed publisher
    ..atresia with ventricular septal defect (13), tetralogy of Fallot (10), troncus arteriosus (4), double outlet right ventricle (2), and single left or right ventricle (4). A total of 44 balloon-expandable stents (mean diameter, 9...
  31. Baumeister F, Engelsberger I, Schulze A. Pancreatic agenesis as cause for neonatal diabetes mellitus. Klin Padiatr. 2005;217:76-81 pubmed
    ..A patient with pancreatic agenesis and double outlet right ventricle is reported. This association has not previously been reported...
  32. Ravi P, Fruitman D, Mills L, Colen T, Hornberger L. Prenatal Diagnosis of the Criss-Cross Heart. Am J Cardiol. 2017;119:916-922 pubmed publisher
    ..Prenatal diagnosis correctly predicted postnatal presentation and outcomes in 1 with double outlet right ventricle and coarctation, 2 presenting with complete (D) transposition of the great arteries physiology, and 1 ..
  33. Fraser C. The Neonatal Arterial Switch Operation: Technical Pearls. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2017;20:38-42 pubmed publisher
    ..septum, transposition with ventricular septal defect with or without aortic arch hypoplasia, and double outlet right ventricle with subpulmonary ventricular septal defect (Taussig-Bing anomaly)...
  34. Say B, Guzoglu N, Uras N, Candemir Z, Akin I, Dilmen U. Partial trisomy 3p and partial monosomy 11q associated with double outlet right ventricle and septum pellucidum et vergae: a case report. Genet Couns. 2013;24:387-91 pubmed
    ..In this paper, we report a newborn who has dysmorphic facial features, double outlet right ventricle, hypotonia, hypospadias, neonatal thrombocytopenia, hydroureteronephrosis, talipes equinovarus and ..
  35. Goldberg S, McCanta A, Campbell D, Carpenter E, Clarke D, Da Cruz E, et al. Implications of incising the ventricular septum in double outlet right ventricle and in the Ross-Konno operation. Eur J Cardiothorac Surg. 2009;35:589-93; discussion 593 pubmed publisher
    ..This retrospective study evaluates the risk of a ventricular septal incision in patients undergoing double outlet right ventricle (DORV) repair and Ross-Konno procedure...
  36. Hibino N, Imai Y, Aoki M, Shin oka T, Hiramatsu T. Double switch operation for superior-inferior ventricles. Ann Thorac Surg. 2001;72:2119-21 pubmed
    ..This is the first report we have been able to locate of a double switch operation for superior-inferior ventricles. ..
  37. Langley S, Winlaw D, Stumper O, Dhillon R, De Giovanni J, Wright J, et al. Midterm results after restoration of the morphologically left ventricle to the systemic circulation in patients with congenitally corrected transposition of the great arteries. J Thorac Cardiovasc Surg. 2003;125:1229-41 pubmed
    ..Continued surveillance is necessary for patents with valved conduits and to determine the longer-term function of the aortic valve and the morphologically left ventricle in the systemic circulation. ..
  38. Boss R, Holmes K, Althaus J, Rushton C, McNee H, McNee T. Trisomy 18 and complex congenital heart disease: seeking the threshold benefit. Pediatrics. 2013;132:161-5 pubmed publisher
    ..There was conflict among the medical team about what threshold of neonatal benefit could outweigh maternal and neonatal treatment burdens. A prenatal ethics consultation was requested. ..
  39. Yang J, Hu D, Hu J, Yin B, Zhou X, Zhou W, et al. Surgical treatment of double outlet ventricle: report on 72 cases. Chin Med J (Engl). 2005;118:344-7 pubmed
  40. Kim N, Friedberg M, Silverman N. Diagnosis and prognosis of fetuses with double outlet right ventricle. Prenat Diagn. 2006;26:740-5 pubmed
    To examine the echocardiographic features, associated anomalies, and outcomes of fetuses diagnosed with double outlet right ventricle (DORV)...
  41. Ishino K, Kawada M, Yoshizumi K, Sano S. Combined aortic arch repair and arterial switch without coronary reimplantation. J Thorac Cardiovasc Surg. 2004;127:577-8 pubmed
  42. Park C, Lee C, Lee C, Kwak J. Anatomical repair of double outlet right ventricle associated with complete atrioventricular septal defect and pulmonary stenosis: extending the indications for aortic translocation. J Card Surg. 2012;27:231-4 pubmed publisher
    The combination of double outlet right ventricle (DORV) and complete atrioventricular septal defect (CAVSD) remains a surgical challenge for anatomical repair...
  43. Tidholm A, Ljungvall I, Michal J, Häggström J, Höglund K. Congenital heart defects in cats: A retrospective study of 162 cats (1996-2013). J Vet Cardiol. 2015;17 Suppl 1:S215-9 pubmed publisher
    ..6% of cats: double chamber right ventricle, double outlet right ventricle, endocardial fibroelastosis, dextroposition of the aorta, persistent right aortic arch, and pulmonary ..
  44. Mascio C, Wayment M, Colaizy T, Mahoney L, Burkhart H. The modified Fontan procedure and prolonged pleural effusions. Am Surg. 2009;75:175-7 pubmed
    ..A higher preoperative mean pulmonary artery pressure may be predictive of these effusions. Fenestration, type of Fontan reconstruction, and size of extracardiac conduit did not predispose to postoperative effusions. ..
  45. Beekmana R, Roest A, Helbing W, Hazekamp M, Schoof P, Bartelings M, et al. Spin echo MRI in the evaluation of hearts with a double outlet right ventricle: usefulness and limitations. Magn Reson Imaging. 2000;18:245-53 pubmed
    The surgical approach to a double outlet right ventricle (DORV) is dependent on the spatial relationship of the semilunar valves, outflow tracts and ventricular septal defect (VSD)...
  46. Gao W, Wang Z, Zhang R, Zhu H, Gong H, Wang J, et al. [Surgical treatment on residual shunt after repair of ventricular septal defect]. Zhonghua Wai Ke Za Zhi. 2004;42:462-4 pubmed 37 patients with congenital heart disease including ventricular septal defect, tetralogy of Fallot, double outlet right ventricle in 19, 17 and 1 patients, respectively. It accounted for 0...
  47. Pang K, Meng H, Wang H, Hu S, Hua Z, Pan X, et al. [Value of a novel categorization of congenital double-outlet right ventricle on guiding the choice of surgical approaches]. Zhonghua Xin Xue Guan Bing Za Zhi. 2015;43:969-74 pubmed
    ..This novel DORV categorization can accurately diagnose DORV lesions, and guide the clinic therapy choice. ..
  48. Al Muhaya M, Ismail S, Abu Sulaiman R, Kabbani M, Najm H. Short- and mid-term outcomes of total correction of Taussig-Bing anomaly. Pediatr Cardiol. 2012;33:258-63 pubmed publisher
    ..Residual lesion and development of VOT obstruction can occur, requiring close follow-up and intervention for residual lesion. ..
  49. Kurosawa H. [Double outlet right ventricle]. Nihon Geka Gakkai Zasshi. 2001;102:578-83 pubmed
    ..The appropriate biventricular repair can be selected in the setting of two types of double outlet right ventricle, the dextroposition and conotruncal criss-cross type.
  50. Latson L, Mumtaz M. Cyanosis after Kawashima-Fontan: hybrid approach to mixing and matching flows. Ann Thorac Surg. 2011;92:e81-3 pubmed publisher
    ..A dilatable pulmonary artery band reduced cyanosis by limiting flow to the affected lung until resolution of pulmonary arteriovenous malformations...
  51. Asano M, Saito T, Nomura N, Mishima A. Double-outlet technique for tetralogy of Fallot-type disease with an anomalous coronary artery. Pediatr Cardiol. 2005;26:710-2 pubmed
    ..If the anomalous coronary artery exists in the RV outflow tract, the procedure can not be done. ..
  52. Combs L, Gupta S, Mehta A. Evaluation of spleen in children with heterotaxia and congenital heart disease. Tenn Med. 2004;97:161-3 pubmed
    ..Hepatoiminodiacetic acid (HIDA) scan in conjunction with radiocolloid scan is the best available choice today to document the presence or absence of a functional spleen in children with heterotaxia and CHD...
  53. Suchonska B, Borowiecka E, Szymusik I, Krzemien Wiczynska S, Borowiecka Elwertowska A. The course of pregnancy and delivery in a patient after a double-outlet right ventricle Fontan repair and the influence of this procedure on her general condition: report of a case. Surg Today. 2008;38:853-6 pubmed publisher
    ..After a 4-year follow-up it was clear that pregnancy and delivery had a harmful effect on her cardiovascular function. ..