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Norwood Procedure
Randy R. Richardson, MD
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KEY FACTS

  • Terminology

    • Imaging

      TERMINOLOGY

      • Definitions

        • 3-stage procedure to palliate single ventricle physiology (most commonly hypoplastic left heart syndrome)
          • Staged approach is necessary due to high pulmonary vascular resistance in neonatal period
          • Developed in early 1980s, initially as 2 stages
        • Goals of Norwood procedure
          • Utilize single right ventricle (RV) [or, less frequently, single left ventricle (LV)] as systemic pump & reconstruct systemic arterial outflow
          • Ensure unobstructed pulmonary venous return to right heart
          • Reroute systemic venous return directly to lungs
        • Stage 1 Norwood
          • Performed within 1st few days after birth
          • Neoaorta construction: Divide main pulmonary artery (PA) & anastomose to aortic root
            • Single, unobstructed arterial trunk from RV to systemic circulation is now in place
          • Blalock-Taussig (BT) or Sano shunt is created to provide blood flow to high-resistance PA circulation
          • Ductus arteriosus is ligated
          • Atrial septum is resected
            • Common atrium receives blood from superior & inferior vena cavae (SVC & IVC) & pulmonary veins
        • Stage 2 Norwood
          • Performed between 3-6 months of age
            • Pulmonary vascular resistance has ↓ by this time to normal levels
          • BT or Sano shunt is excised
          • Bidirectional Glenn shunt is created
            • End-to-side anastomosis of SVC to PAs
        • Stage 3 Norwood
          • Typically performed between 18-36 months of age
          • Lateral tunnel or extracardiac type of Fontan is now performed; atriopulmonary type is not common today
            • IVC blood flow is directed to PAs

      IMAGING

      • General Features

        • Radiographic Findings

          • CT Findings

            • MR Findings

              DIFFERENTIAL DIAGNOSIS

                CLINICAL ISSUES

                • Natural History & Prognosis

                  Selected References

                  1. Dillman JR et al: Imaging of Fontan-associated liver disease. Pediatr Radiol. 50(11):1528-41, 2020
                  2. Vitanova K et al: Choice of shunt type for the Norwood I procedure: does it make a difference? Interact Cardiovasc Thorac Surg. 30(4):630-5, 2020
                  3. Devlin PJ et al: Intervention for arch obstruction after the Norwood procedure: prevalence, associated factors, and practice variability. J Thorac Cardiovasc Surg. 157(2):684-695.e8, 2019
                  4. Nakamura Y et al: The Norwood procedure with valvular pulmonary stenosis. Ann Thorac Surg. 107(1):e49-50, 2019
                  5. Brida M et al: Systemic right ventricle in adults with congenital heart disease: anatomic and phenotypic spectrum and current approach to management. Circulation. 137(5):508-18, 2018
                  6. Téllez L et al: Fontan-associated liver disease: a review. Ann Hepatol. 17(2):192-204, 2018
                  7. Kiraly L et al: Three-dimensional printed prototypes refine the anatomy of post-modified Norwood-1 complex aortic arch obstruction and allow presurgical simulation of the repair. Interact Cardiovasc Thorac Surg. 22(2):238-40, 2016
                  8. Kutty S et al: Role of imaging in the evaluation of single ventricle with the Fontan palliation. Heart. 102(3):174-83, 2016
                  9. Poterucha JT et al: Magnetic resonance elastography: a novel technique for the detection of hepatic fibrosis and hepatocellular carcinoma after the Fontan operation. Mayo Clin Proc. 90(7):882-94, 2015
                  10. Sandler KL et al: Optimizing CT angiography in patients with Fontan physiology: single-center experience of dual-site power injection. Clin Radiol. 69(12):e562-7, 2014
                  11. Dillman JR et al: Cardiovascular magnetic resonance imaging of hypoplastic left heart syndrome in children. Pediatr Radiol. 40(3):261-74; quiz 379-80, 2010
                  Related Anatomy
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                  Related Differential Diagnoses
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                  References
                  Tables

                  Tables

                  KEY FACTS

                  • Terminology

                    • Imaging

                      TERMINOLOGY

                      • Definitions

                        • 3-stage procedure to palliate single ventricle physiology (most commonly hypoplastic left heart syndrome)
                          • Staged approach is necessary due to high pulmonary vascular resistance in neonatal period
                          • Developed in early 1980s, initially as 2 stages
                        • Goals of Norwood procedure
                          • Utilize single right ventricle (RV) [or, less frequently, single left ventricle (LV)] as systemic pump & reconstruct systemic arterial outflow
                          • Ensure unobstructed pulmonary venous return to right heart
                          • Reroute systemic venous return directly to lungs
                        • Stage 1 Norwood
                          • Performed within 1st few days after birth
                          • Neoaorta construction: Divide main pulmonary artery (PA) & anastomose to aortic root
                            • Single, unobstructed arterial trunk from RV to systemic circulation is now in place
                          • Blalock-Taussig (BT) or Sano shunt is created to provide blood flow to high-resistance PA circulation
                          • Ductus arteriosus is ligated
                          • Atrial septum is resected
                            • Common atrium receives blood from superior & inferior vena cavae (SVC & IVC) & pulmonary veins
                        • Stage 2 Norwood
                          • Performed between 3-6 months of age
                            • Pulmonary vascular resistance has ↓ by this time to normal levels
                          • BT or Sano shunt is excised
                          • Bidirectional Glenn shunt is created
                            • End-to-side anastomosis of SVC to PAs
                        • Stage 3 Norwood
                          • Typically performed between 18-36 months of age
                          • Lateral tunnel or extracardiac type of Fontan is now performed; atriopulmonary type is not common today
                            • IVC blood flow is directed to PAs

                      IMAGING

                      • General Features

                        • Radiographic Findings

                          • CT Findings

                            • MR Findings

                              DIFFERENTIAL DIAGNOSIS

                                CLINICAL ISSUES

                                • Natural History & Prognosis

                                  Selected References

                                  1. Dillman JR et al: Imaging of Fontan-associated liver disease. Pediatr Radiol. 50(11):1528-41, 2020
                                  2. Vitanova K et al: Choice of shunt type for the Norwood I procedure: does it make a difference? Interact Cardiovasc Thorac Surg. 30(4):630-5, 2020
                                  3. Devlin PJ et al: Intervention for arch obstruction after the Norwood procedure: prevalence, associated factors, and practice variability. J Thorac Cardiovasc Surg. 157(2):684-695.e8, 2019
                                  4. Nakamura Y et al: The Norwood procedure with valvular pulmonary stenosis. Ann Thorac Surg. 107(1):e49-50, 2019
                                  5. Brida M et al: Systemic right ventricle in adults with congenital heart disease: anatomic and phenotypic spectrum and current approach to management. Circulation. 137(5):508-18, 2018
                                  6. Téllez L et al: Fontan-associated liver disease: a review. Ann Hepatol. 17(2):192-204, 2018
                                  7. Kiraly L et al: Three-dimensional printed prototypes refine the anatomy of post-modified Norwood-1 complex aortic arch obstruction and allow presurgical simulation of the repair. Interact Cardiovasc Thorac Surg. 22(2):238-40, 2016
                                  8. Kutty S et al: Role of imaging in the evaluation of single ventricle with the Fontan palliation. Heart. 102(3):174-83, 2016
                                  9. Poterucha JT et al: Magnetic resonance elastography: a novel technique for the detection of hepatic fibrosis and hepatocellular carcinoma after the Fontan operation. Mayo Clin Proc. 90(7):882-94, 2015
                                  10. Sandler KL et al: Optimizing CT angiography in patients with Fontan physiology: single-center experience of dual-site power injection. Clin Radiol. 69(12):e562-7, 2014
                                  11. Dillman JR et al: Cardiovascular magnetic resonance imaging of hypoplastic left heart syndrome in children. Pediatr Radiol. 40(3):261-74; quiz 379-80, 2010