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Azygos and Hemiazygos Continuation of the IVC
Sherief H. Garrana, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Synonyms

              • Azygos continuation of inferior vena cava (IVC)
              • Interruption of IVC
              • Absence of hepatic segment of IVC with azygos continuation
            • Definitions

              • Caused by persistence of embryonic right supracardinal vein and failure of development of suprarenal part of subcardinal vein
                • IVC interrupted above renal veins
                • Hepatic veins drain directly into right atrium
              • Venous return to right atrium
                • Large azygos vein carries venous return
                • Occasionally, large hemiazygos vein carries venous return
                • Both azygos and hemiazygos veins may carry venous return to right atrium
              • Azygos continuation of IVC associated with congenital heart disease and situs abnormalities, especially heterotaxy syndrome with polysplenia

            IMAGING

            • General Features

              • Radiographic Findings

                • CT Findings

                  • MR Findings

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Gross Pathologic & Surgical Features

                            CLINICAL ISSUES

                            • Presentation

                              • Demographics

                                • Natural History & Prognosis

                                  • Treatment

                                    DIAGNOSTIC CHECKLIST

                                    • Consider

                                      • Image Interpretation Pearls

                                        Selected References

                                        1. Kiran VS et al: The spectrum of cardiac anomalies associated with heterotaxy: a single-center study of a large series based on computed tomography. Pediatr Cardiol. 41(7):1414-24, 2020
                                        2. Banka P et al: Changes in prognosis of heterotaxy syndrome over time. pediatrics. 146(2), 2020
                                        3. Iezzi R et al: Multidetector-row CT imaging evaluation of superior and inferior vena cava normal anatomy and caval variants: report of our cases and literature review with embryologic correlation. Phlebology. 34(2):77-87, 2019
                                        4. Oliveira JD et al: Congenital systemic venous return anomalies to the right atrium review. Insights Imaging. 10(1):115, 2019
                                        5. Bass JE et al: Spectrum of congenital anomalies of the inferior vena cava: cross-sectional imaging findings. Radiographics. 20(3):639-52, 2000
                                        6. Applegate KE et al: Situs revisited: imaging of the heterotaxy syndrome. RadioGraphics 19:837-52, 1999
                                        7. Liu Y et al: Radiological features of azygos and hemiazygos continuation of inferior vena cava: a case report. Medicine (Baltimore). 97(17):e0546, 2018
                                        8. Yildirim A et al: An unusual case of heterotaxy and polysplenia syndrome associated with hemiazygous continuation of the left-sided vena cava inferior, dilated azygous vein and large venous ectasia. Congenit Heart Dis. 6(3):262-5, 2011
                                        9. Mamidipally S et al: Azygous continuation of inferior vena cava. J Am Coll Cardiol. 56(21):e41, 2010
                                        10. Demos TC et al: Venous anomalies of the thorax. AJR Am J Roentgenol. 182(5):1139-50, 2004
                                        11. Yilmaz E et al: Interruption of the inferior vena cava with azygos/hemiazygos continuation accompanied by distinct renal vein anomalies: MRA and CT assessment. Abdom Imaging. 28(3):392-4, 2003
                                        12. Lagrotta G et al: Heterotaxy Polysplenia Syndrome in Adulthood: Focused Review and a Case Report. Cureus. 12(1):e6822, 2020
                                        13. Gayer G et al: Polysplenia syndrome detected in adulthood: report of eight cases and review of the literature. Abdom Imaging. 24(2):178-84, 1999
                                        14. Jelinek JS et al: MRI of polysplenia syndrome. Magn Reson Imaging. 7(6):681-6, 1989
                                        15. Munechika H et al: Hemiazygos continuation of a left inferior vena cava: CT appearance. J Comput Assist Tomogr. 12(2):328-30, 1988
                                        16. Cohen MI et al: Accessory hemiazygos continuation of left inferior vena cava: CT demonstration. J Comput Assist Tomogr. 8(4):777-9, 1984
                                        17. Schultz CL et al: Azygos continuation of the inferior vena cava: demonstration by NMR imaging. J Comput Assist Tomogr. 8(4):774-6, 1984
                                        18. Siegfried MS et al: Diagnosis of inferior vena cava anomalies by computerized tomography. Comput Radiol. 7(2):119-23, 1983
                                        19. Allen HA et al: Case report. Left-sided inferior vena cava with hemiazygos continuation. J Comput Assist Tomogr. 5(6):917-20, 1981
                                        20. Ginaldi S et al: Absence of hepatic segment of the inferior vena cava with azygous continuation. J Comput Assist Tomogr. 4(1):112-4, 1980
                                        21. O'Reilly RJ et al: The lateral chest film as an unreliable indicator of azygos continuation of the inferior vena cava. Circulation. 53(5):891-5, 1976
                                        Related Anatomy
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                                        Related Differential Diagnoses
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                                        References
                                        Tables

                                        Tables

                                        KEY FACTS

                                        • Terminology

                                          • Imaging

                                            • Top Differential Diagnoses

                                              • Clinical Issues

                                                • Diagnostic Checklist

                                                  TERMINOLOGY

                                                  • Synonyms

                                                    • Azygos continuation of inferior vena cava (IVC)
                                                    • Interruption of IVC
                                                    • Absence of hepatic segment of IVC with azygos continuation
                                                  • Definitions

                                                    • Caused by persistence of embryonic right supracardinal vein and failure of development of suprarenal part of subcardinal vein
                                                      • IVC interrupted above renal veins
                                                      • Hepatic veins drain directly into right atrium
                                                    • Venous return to right atrium
                                                      • Large azygos vein carries venous return
                                                      • Occasionally, large hemiazygos vein carries venous return
                                                      • Both azygos and hemiazygos veins may carry venous return to right atrium
                                                    • Azygos continuation of IVC associated with congenital heart disease and situs abnormalities, especially heterotaxy syndrome with polysplenia

                                                  IMAGING

                                                  • General Features

                                                    • Radiographic Findings

                                                      • CT Findings

                                                        • MR Findings

                                                          • Imaging Recommendations

                                                            DIFFERENTIAL DIAGNOSIS

                                                              PATHOLOGY

                                                              • General Features

                                                                • Gross Pathologic & Surgical Features

                                                                  CLINICAL ISSUES

                                                                  • Presentation

                                                                    • Demographics

                                                                      • Natural History & Prognosis

                                                                        • Treatment

                                                                          DIAGNOSTIC CHECKLIST

                                                                          • Consider

                                                                            • Image Interpretation Pearls

                                                                              Selected References

                                                                              1. Kiran VS et al: The spectrum of cardiac anomalies associated with heterotaxy: a single-center study of a large series based on computed tomography. Pediatr Cardiol. 41(7):1414-24, 2020
                                                                              2. Banka P et al: Changes in prognosis of heterotaxy syndrome over time. pediatrics. 146(2), 2020
                                                                              3. Iezzi R et al: Multidetector-row CT imaging evaluation of superior and inferior vena cava normal anatomy and caval variants: report of our cases and literature review with embryologic correlation. Phlebology. 34(2):77-87, 2019
                                                                              4. Oliveira JD et al: Congenital systemic venous return anomalies to the right atrium review. Insights Imaging. 10(1):115, 2019
                                                                              5. Bass JE et al: Spectrum of congenital anomalies of the inferior vena cava: cross-sectional imaging findings. Radiographics. 20(3):639-52, 2000
                                                                              6. Applegate KE et al: Situs revisited: imaging of the heterotaxy syndrome. RadioGraphics 19:837-52, 1999
                                                                              7. Liu Y et al: Radiological features of azygos and hemiazygos continuation of inferior vena cava: a case report. Medicine (Baltimore). 97(17):e0546, 2018
                                                                              8. Yildirim A et al: An unusual case of heterotaxy and polysplenia syndrome associated with hemiazygous continuation of the left-sided vena cava inferior, dilated azygous vein and large venous ectasia. Congenit Heart Dis. 6(3):262-5, 2011
                                                                              9. Mamidipally S et al: Azygous continuation of inferior vena cava. J Am Coll Cardiol. 56(21):e41, 2010
                                                                              10. Demos TC et al: Venous anomalies of the thorax. AJR Am J Roentgenol. 182(5):1139-50, 2004
                                                                              11. Yilmaz E et al: Interruption of the inferior vena cava with azygos/hemiazygos continuation accompanied by distinct renal vein anomalies: MRA and CT assessment. Abdom Imaging. 28(3):392-4, 2003
                                                                              12. Lagrotta G et al: Heterotaxy Polysplenia Syndrome in Adulthood: Focused Review and a Case Report. Cureus. 12(1):e6822, 2020
                                                                              13. Gayer G et al: Polysplenia syndrome detected in adulthood: report of eight cases and review of the literature. Abdom Imaging. 24(2):178-84, 1999
                                                                              14. Jelinek JS et al: MRI of polysplenia syndrome. Magn Reson Imaging. 7(6):681-6, 1989
                                                                              15. Munechika H et al: Hemiazygos continuation of a left inferior vena cava: CT appearance. J Comput Assist Tomogr. 12(2):328-30, 1988
                                                                              16. Cohen MI et al: Accessory hemiazygos continuation of left inferior vena cava: CT demonstration. J Comput Assist Tomogr. 8(4):777-9, 1984
                                                                              17. Schultz CL et al: Azygos continuation of the inferior vena cava: demonstration by NMR imaging. J Comput Assist Tomogr. 8(4):774-6, 1984
                                                                              18. Siegfried MS et al: Diagnosis of inferior vena cava anomalies by computerized tomography. Comput Radiol. 7(2):119-23, 1983
                                                                              19. Allen HA et al: Case report. Left-sided inferior vena cava with hemiazygos continuation. J Comput Assist Tomogr. 5(6):917-20, 1981
                                                                              20. Ginaldi S et al: Absence of hepatic segment of the inferior vena cava with azygous continuation. J Comput Assist Tomogr. 4(1):112-4, 1980
                                                                              21. O'Reilly RJ et al: The lateral chest film as an unreliable indicator of azygos continuation of the inferior vena cava. Circulation. 53(5):891-5, 1976