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Pediatrics
Diagnosis
Cardiac
Congenital Heart Disease
Double-Outlet Right Ventricle
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
            • 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. 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
                                2. Singla M et al: Double-outlet right ventricle without interventricular communication: an unusual and challenging problem. Pediatr Cardiol. 34(8):1941-4, 2013
                                3. 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
                                4. 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
                                5. Frank L et al: Cardiovascular MR imaging of conotruncal anomalies. Radiographics. 30(4):1069-94, 2010
                                6. Saleeb SF et al: Anatomic, imaging, and clinical characteristics of double-inlet, double-outlet right ventricle. Am J Cardiol. 105(4):542-9, 2010
                                7. Uehara M et al: Double outlet right ventricle demonstrated by multislice computed tomography. Int J Cardiol. 121(2):218-20, 2007
                                8. 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
                                9. Hagler DJ. Double-outlet right ventricle. In Allen HD et al: Heart Disease in Infants, Children, and Adolescents. Philadelphia: 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
                                          • 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. 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
                                                              2. Singla M et al: Double-outlet right ventricle without interventricular communication: an unusual and challenging problem. Pediatr Cardiol. 34(8):1941-4, 2013
                                                              3. 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
                                                              4. 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
                                                              5. Frank L et al: Cardiovascular MR imaging of conotruncal anomalies. Radiographics. 30(4):1069-94, 2010
                                                              6. Saleeb SF et al: Anatomic, imaging, and clinical characteristics of double-inlet, double-outlet right ventricle. Am J Cardiol. 105(4):542-9, 2010
                                                              7. Uehara M et al: Double outlet right ventricle demonstrated by multislice computed tomography. Int J Cardiol. 121(2):218-20, 2007
                                                              8. 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
                                                              9. Hagler DJ. Double-outlet right ventricle. In Allen HD et al: Heart Disease in Infants, Children, and Adolescents. Philadelphia: Lippincott Williams & Wilkins. 1102-28, 2001