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Double-Outlet Right Ventricle
Randy R. Richardson, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          TERMINOLOGY

          • Abbreviations

            • Double-outlet right ventricle (DORV)
          • Definitions

            • Form of abnormal ventriculoarterial connection in which both great arteries arise completely or predominately from morphologic right ventricle (RV)
            • 16 variants based on relationship of great arteries & position/location of ventricular septal defect (VSD)
            • Fibrinous discontinuity between mitral & semilunar valves or bilateral conus
            • May be part of complex lesions coexisting with ventricular anomalies, valve stenosis or atresia, abnormal atrioventricular valve, aortic valve anomalies, coarctation, coronary anomalies, & anomalies of systemic & pulmonary venous return
            • Taussig-Bing anomaly with DORV is subpulmonary VSD where left ventricular outflow is directed toward pulmonary artery with preferential flow

          IMAGING

          • General Features

            • Radiographic Findings

              • CTA or MRA

                • Echocardiographic Findings

                  • Angiographic Findings

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          CLINICAL ISSUES

                          • Presentation

                            • Demographics

                              • Treatment

                                Selected References

                                1. Kumar P et al: Role of computed tomography in pre- and postoperative evaluation of a double-outlet right ventricle. J Cardiovasc Imaging. 29(3):205-27, 2021
                                2. Young AA et al: Fetal double outlet right ventricle without heterotaxy syndrome: diagnostic spectrum, associated extracardiac pathology and clinical outcomes. Prenat Diagn. 41(9):1118-26, 2021
                                3. Shearn AIU et al: Use of 3D models in the surgical decision-making process in a case of double-outlet right ventricle with multiple ventricular septal defects. Front Pediatr. 7:330, 2019
                                4. Goo HW: Coronary artery anomalies on preoperative cardiac CT in children with tetralogy of Fallot or Fallot type of double outlet right ventricle: comparison with surgical findings. Int J Cardiovasc Imaging. 34(12):1997-2009, 2018
                                5. Ryan J et al: 3D printing for congenital heart disease: a single site's initial three-yearexperience. 3D Print Med. 4(1):10, 2018
                                6. Yim D et al: Essential modifiers of double outlet right ventricle: revisit with endocardial surface images and 3-dimensional print models. Circ Cardiovasc Imaging. 11(3):e006891, 2018
                                7. Meng H et al: Biventricular repair of double outlet right ventricle: preoperative echocardiography and surgical outcomes. World J Pediatr Congenit Heart Surg. 8(3):354-60, 2017
                                8. Pang KJ et al: Echocardiographic classification and surgical approaches to double-outlet right ventricle for great arteries arising almost exclusively from the right ventricle. Tex Heart Inst J. 44(4):245-51, 2017
                                9. Farooqi KM et al: Use of 3-dimensional printing to demonstrate complex intracardiac relationships in double-outlet right ventricle for surgical planning. Circ Cardiovasc Imaging. 8(5), 2015
                                10. Singla M et al: Double-outlet right ventricle without interventricular communication: an unusual and challenging problem. Pediatr Cardiol. 34(8):1941-4, 2013
                                11. Kha LC et al: Multimodality cardiac imaging of a double chambered right ventricle with intrapulmonary shunting: a case report. BMC Res Notes. 5:516, 2012
                                12. Mershina EA et al: [Radial diagnosis MRT and MSCT in assessing the outcomes of surgical correction for complicated congenital heart defects: discordant atrioventricular connections with double outlet right ventricle.] Angiol Sosud Khir. 18(1):39-44, 2012
                                13. Frank L et al: Cardiovascular MR imaging of conotruncal anomalies. Radiographics. 30(4):1069-94, 2010
                                14. Saleeb SF et al: Anatomic, imaging, and clinical characteristics of double-inlet, double-outlet right ventricle. Am J Cardiol. 105(4):542-9, 2010
                                15. Uehara M et al: Double outlet right ventricle demonstrated by multislice computed tomography. Int J Cardiol. 121(2):218-20, 2007
                                16. Brown JW et al: Surgical results in patients with double outlet right ventricle: a 20-year experience. Ann Thorac Surg. 72(5):1630-5, 2001
                                17. Hagler DJ. Double-outlet right ventricle. In Allen HD et al: Heart Disease in Infants, Children, & Adolescents. Lippincott Williams & Wilkins. 1102-28, 2001
                                Related Anatomy
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                                Related Differential Diagnoses
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                                References
                                Tables

                                Tables

                                KEY FACTS

                                • Terminology

                                  • Imaging

                                    • Pathology

                                      • Clinical Issues

                                        TERMINOLOGY

                                        • Abbreviations

                                          • Double-outlet right ventricle (DORV)
                                        • Definitions

                                          • Form of abnormal ventriculoarterial connection in which both great arteries arise completely or predominately from morphologic right ventricle (RV)
                                          • 16 variants based on relationship of great arteries & position/location of ventricular septal defect (VSD)
                                          • Fibrinous discontinuity between mitral & semilunar valves or bilateral conus
                                          • May be part of complex lesions coexisting with ventricular anomalies, valve stenosis or atresia, abnormal atrioventricular valve, aortic valve anomalies, coarctation, coronary anomalies, & anomalies of systemic & pulmonary venous return
                                          • Taussig-Bing anomaly with DORV is subpulmonary VSD where left ventricular outflow is directed toward pulmonary artery with preferential flow

                                        IMAGING

                                        • General Features

                                          • Radiographic Findings

                                            • CTA or MRA

                                              • Echocardiographic Findings

                                                • Angiographic Findings

                                                  • Imaging Recommendations

                                                    DIFFERENTIAL DIAGNOSIS

                                                      PATHOLOGY

                                                      • General Features

                                                        CLINICAL ISSUES

                                                        • Presentation

                                                          • Demographics

                                                            • Treatment

                                                              Selected References

                                                              1. Kumar P et al: Role of computed tomography in pre- and postoperative evaluation of a double-outlet right ventricle. J Cardiovasc Imaging. 29(3):205-27, 2021
                                                              2. Young AA et al: Fetal double outlet right ventricle without heterotaxy syndrome: diagnostic spectrum, associated extracardiac pathology and clinical outcomes. Prenat Diagn. 41(9):1118-26, 2021
                                                              3. Shearn AIU et al: Use of 3D models in the surgical decision-making process in a case of double-outlet right ventricle with multiple ventricular septal defects. Front Pediatr. 7:330, 2019
                                                              4. Goo HW: Coronary artery anomalies on preoperative cardiac CT in children with tetralogy of Fallot or Fallot type of double outlet right ventricle: comparison with surgical findings. Int J Cardiovasc Imaging. 34(12):1997-2009, 2018
                                                              5. Ryan J et al: 3D printing for congenital heart disease: a single site's initial three-yearexperience. 3D Print Med. 4(1):10, 2018
                                                              6. Yim D et al: Essential modifiers of double outlet right ventricle: revisit with endocardial surface images and 3-dimensional print models. Circ Cardiovasc Imaging. 11(3):e006891, 2018
                                                              7. Meng H et al: Biventricular repair of double outlet right ventricle: preoperative echocardiography and surgical outcomes. World J Pediatr Congenit Heart Surg. 8(3):354-60, 2017
                                                              8. Pang KJ et al: Echocardiographic classification and surgical approaches to double-outlet right ventricle for great arteries arising almost exclusively from the right ventricle. Tex Heart Inst J. 44(4):245-51, 2017
                                                              9. Farooqi KM et al: Use of 3-dimensional printing to demonstrate complex intracardiac relationships in double-outlet right ventricle for surgical planning. Circ Cardiovasc Imaging. 8(5), 2015
                                                              10. Singla M et al: Double-outlet right ventricle without interventricular communication: an unusual and challenging problem. Pediatr Cardiol. 34(8):1941-4, 2013
                                                              11. Kha LC et al: Multimodality cardiac imaging of a double chambered right ventricle with intrapulmonary shunting: a case report. BMC Res Notes. 5:516, 2012
                                                              12. Mershina EA et al: [Radial diagnosis MRT and MSCT in assessing the outcomes of surgical correction for complicated congenital heart defects: discordant atrioventricular connections with double outlet right ventricle.] Angiol Sosud Khir. 18(1):39-44, 2012
                                                              13. Frank L et al: Cardiovascular MR imaging of conotruncal anomalies. Radiographics. 30(4):1069-94, 2010
                                                              14. Saleeb SF et al: Anatomic, imaging, and clinical characteristics of double-inlet, double-outlet right ventricle. Am J Cardiol. 105(4):542-9, 2010
                                                              15. Uehara M et al: Double outlet right ventricle demonstrated by multislice computed tomography. Int J Cardiol. 121(2):218-20, 2007
                                                              16. Brown JW et al: Surgical results in patients with double outlet right ventricle: a 20-year experience. Ann Thorac Surg. 72(5):1630-5, 2001
                                                              17. Hagler DJ. Double-outlet right ventricle. In Allen HD et al: Heart Disease in Infants, Children, & Adolescents. Lippincott Williams & Wilkins. 1102-28, 2001