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Double-Outlet Right Ventricle
Whitnee Hogan, MD; Michael D. Puchalski, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Abbreviations

              • Double-outlet right ventricle (DORV)
            • Definitions

              • Both great arteries arise predominantly from right ventricle (RV)
                • Neither semilunar valve is in fibrous continuity with atrioventricular (AV) valve
                • Ventricular septal defect (VSD) usually present

            IMAGING

            • Echocardiographic Findings

              • Imaging Recommendations

                DIFFERENTIAL DIAGNOSIS

                  PATHOLOGY

                  • General Features

                    CLINICAL ISSUES

                    • Demographics

                      • Natural History & Prognosis

                        • Treatment

                          DIAGNOSTIC CHECKLIST

                          • Image Interpretation Pearls

                            Selected References

                            1. Corno AF et al: Narrative review of assessing the surgical options for double outlet right ventricle. Transl Pediatr. 10(1):165-76, 2021
                            2. Oladunjoye O et al: Repair of double outlet right ventricle: midterm outcomes. J Thorac Cardiovasc Surg. S0022-5223(19)31694-0, 2019
                            3. 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
                            4. Li S et al: Surgical outcomes of 380 patients with double outlet right ventricle who underwent biventricular repair. J Thorac Cardiovasc Surg. 148(3):817-24, 2014
                            5. Hartge DR et al: Prenatal detection and postnatal management of double outlet right ventricle (DORV) in 21 singleton pregnancies. J Matern Fetal Neonatal Med. 25(1):58-63, 2012
                            6. Lagopoulos ME et al: Impact of prenatal diagnosis and anatomical subtype on outcome in double outlet right ventricle. Am Heart J. 160(4):692-700, 2010
                            7. Obler D et al: Double outlet right ventricle: aetiologies and associations. J Med Genet. 45(8):481-97, 2008
                            8. Gelehrter S et al: Accuracy of the fetal echocardiogram in double-outlet right ventricle. Congenit Heart Dis. 2(1):32-7, 2007
                            9. Kim N et al: Diagnosis and prognosis of fetuses with double outlet right ventricle. Prenat Diagn. 26(8):740-5, 2006
                            Related Anatomy
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                            Related Differential Diagnoses
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                            References
                            Tables

                            Tables

                            KEY FACTS

                            • Terminology

                              • Imaging

                                • Pathology

                                  • Clinical Issues

                                    • Diagnostic Checklist

                                      TERMINOLOGY

                                      • Abbreviations

                                        • Double-outlet right ventricle (DORV)
                                      • Definitions

                                        • Both great arteries arise predominantly from right ventricle (RV)
                                          • Neither semilunar valve is in fibrous continuity with atrioventricular (AV) valve
                                          • Ventricular septal defect (VSD) usually present

                                      IMAGING

                                      • Echocardiographic Findings

                                        • Imaging Recommendations

                                          DIFFERENTIAL DIAGNOSIS

                                            PATHOLOGY

                                            • General Features

                                              CLINICAL ISSUES

                                              • Demographics

                                                • Natural History & Prognosis

                                                  • Treatment

                                                    DIAGNOSTIC CHECKLIST

                                                    • Image Interpretation Pearls

                                                      Selected References

                                                      1. Corno AF et al: Narrative review of assessing the surgical options for double outlet right ventricle. Transl Pediatr. 10(1):165-76, 2021
                                                      2. Oladunjoye O et al: Repair of double outlet right ventricle: midterm outcomes. J Thorac Cardiovasc Surg. S0022-5223(19)31694-0, 2019
                                                      3. 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
                                                      4. Li S et al: Surgical outcomes of 380 patients with double outlet right ventricle who underwent biventricular repair. J Thorac Cardiovasc Surg. 148(3):817-24, 2014
                                                      5. Hartge DR et al: Prenatal detection and postnatal management of double outlet right ventricle (DORV) in 21 singleton pregnancies. J Matern Fetal Neonatal Med. 25(1):58-63, 2012
                                                      6. Lagopoulos ME et al: Impact of prenatal diagnosis and anatomical subtype on outcome in double outlet right ventricle. Am Heart J. 160(4):692-700, 2010
                                                      7. Obler D et al: Double outlet right ventricle: aetiologies and associations. J Med Genet. 45(8):481-97, 2008
                                                      8. Gelehrter S et al: Accuracy of the fetal echocardiogram in double-outlet right ventricle. Congenit Heart Dis. 2(1):32-7, 2007
                                                      9. Kim N et al: Diagnosis and prognosis of fetuses with double outlet right ventricle. Prenat Diagn. 26(8):740-5, 2006