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Fontan Operation
Prakash M. Masand, MD; Daniel Podberesky, MD
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KEY FACTS

  • Terminology

    • Imaging

      TERMINOLOGY

      • Synonyms

        • Total cavopulmonary connection
      • Definitions

        • Goal is to direct systemic venous return to pulmonary circulation directly, bypassing right heart
        • Initially described by Dr. Francis Fontan in 1968
        • Used in single ventricle physiology
          • Tricuspid atresia
          • Hypoplastic left heart syndrome
          • Double-inlet ventricle
          • Some heterotaxies
        • Stage 3 of Norwood procedure
        • Typically performed between 18-36 months of age
        • Originally described as extracardiac valved conduit between right atrium & left pulmonary artery
          • In combination with Glenn shunt, creates physiologic correction of blood flow
          • Superior vena cava (SVC) blood directed to right pulmonary artery & inferior vena cava (IVC) blood directed to left pulmonary artery
          • Complications of classic Fontan
            • Right atrial enlargement & hypertension
            • Impaired ventricular function
            • ↓ pulmonary vascular blood flow
            • Tricuspid valve insufficiency
        • In order to preserve ventricular & pulmonary vascular function, modified Fontan now favored
          • Lateral tunnel Fontan
            • Intraatrial tunnel created in right atrium using prosthetic material
            • IVC anastomosed to caudal aspect of tunnel
            • Pulmonary artery anastomosed to cephalad aspect of tunnel
          • Extracardiac conduit Fontan (typically favored)
            • IVC divided from right atrium
            • Synthetic graft tube connected to IVC inferiorly & right pulmonary artery superiorly; travels along side of right atrium
          • In either form, IVC blood directed to pulmonary circulation, bypassing right heart structures
        • Small fenestration often created between Fontan circuit & right atrium
          • Prevents volume overload to lungs
          • Buffers any ↑ in systemic venous pressure
        • Excellent early, mid, & late-term outcomes, with mortality rates < 5-10%

      IMAGING

      • General Features

        • CT Findings

          • MR Findings

            DIFFERENTIAL DIAGNOSIS

              Selected References

              1. Kutty S et al: Role of imaging in the evaluation of single ventricle with the Fontan palliation. Heart. 102(3):174-83, 2016
              2. Burkhart HM et al: The Fontan operation: is timing everything? Semin Thorac Cardiovasc Surg. 27(2):175-6, 2015
              3. Navarro-Aguilar V et al: Fontan procedure: imaging of normal post-surgical anatomy and the spectrum of cardiac and extracardiac complications. Clin Radiol. 70(3):295-303, 2015
              4. Harris MA et al: Pre-Fontan cardiac magnetic resonance predicts post-Fontan length of stay and avoids ionizing radiation. J Thorac Cardiovasc Surg. 138(4):941-7, 2009
              5. Gaca AM et al: Repair of congenital heart disease: a primer-part 1. Radiology. 247(3):617-31, 2008
              6. Goo HW et al: Time-resolved three-dimensional contrast-enhanced magnetic resonance angiography in patients who have undergone a Fontan operation or bidirectional cavopulmonary connection: initial experience. J Magn Reson Imaging. 25(4):727-36, 2007
              7. Rodríguez E et al: Postoperative imaging in cyanotic congenital heart diseases: part 1, Normal findings. AJR Am J Roentgenol. 189(6):1353-60, 2007
              8. Festa P et al: The role of magnetic resonance imaging in the evaluation of the functionally single ventricle before and after conversion to the Fontan circulation. Cardiol Young. 15 Suppl 3:51-6, 2005
              Related Anatomy
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              Related Differential Diagnoses
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              References
              Tables

              Tables

              KEY FACTS

              • Terminology

                • Imaging

                  TERMINOLOGY

                  • Synonyms

                    • Total cavopulmonary connection
                  • Definitions

                    • Goal is to direct systemic venous return to pulmonary circulation directly, bypassing right heart
                    • Initially described by Dr. Francis Fontan in 1968
                    • Used in single ventricle physiology
                      • Tricuspid atresia
                      • Hypoplastic left heart syndrome
                      • Double-inlet ventricle
                      • Some heterotaxies
                    • Stage 3 of Norwood procedure
                    • Typically performed between 18-36 months of age
                    • Originally described as extracardiac valved conduit between right atrium & left pulmonary artery
                      • In combination with Glenn shunt, creates physiologic correction of blood flow
                      • Superior vena cava (SVC) blood directed to right pulmonary artery & inferior vena cava (IVC) blood directed to left pulmonary artery
                      • Complications of classic Fontan
                        • Right atrial enlargement & hypertension
                        • Impaired ventricular function
                        • ↓ pulmonary vascular blood flow
                        • Tricuspid valve insufficiency
                    • In order to preserve ventricular & pulmonary vascular function, modified Fontan now favored
                      • Lateral tunnel Fontan
                        • Intraatrial tunnel created in right atrium using prosthetic material
                        • IVC anastomosed to caudal aspect of tunnel
                        • Pulmonary artery anastomosed to cephalad aspect of tunnel
                      • Extracardiac conduit Fontan (typically favored)
                        • IVC divided from right atrium
                        • Synthetic graft tube connected to IVC inferiorly & right pulmonary artery superiorly; travels along side of right atrium
                      • In either form, IVC blood directed to pulmonary circulation, bypassing right heart structures
                    • Small fenestration often created between Fontan circuit & right atrium
                      • Prevents volume overload to lungs
                      • Buffers any ↑ in systemic venous pressure
                    • Excellent early, mid, & late-term outcomes, with mortality rates < 5-10%

                  IMAGING

                  • General Features

                    • CT Findings

                      • MR Findings

                        DIFFERENTIAL DIAGNOSIS

                          Selected References

                          1. Kutty S et al: Role of imaging in the evaluation of single ventricle with the Fontan palliation. Heart. 102(3):174-83, 2016
                          2. Burkhart HM et al: The Fontan operation: is timing everything? Semin Thorac Cardiovasc Surg. 27(2):175-6, 2015
                          3. Navarro-Aguilar V et al: Fontan procedure: imaging of normal post-surgical anatomy and the spectrum of cardiac and extracardiac complications. Clin Radiol. 70(3):295-303, 2015
                          4. Harris MA et al: Pre-Fontan cardiac magnetic resonance predicts post-Fontan length of stay and avoids ionizing radiation. J Thorac Cardiovasc Surg. 138(4):941-7, 2009
                          5. Gaca AM et al: Repair of congenital heart disease: a primer-part 1. Radiology. 247(3):617-31, 2008
                          6. Goo HW et al: Time-resolved three-dimensional contrast-enhanced magnetic resonance angiography in patients who have undergone a Fontan operation or bidirectional cavopulmonary connection: initial experience. J Magn Reson Imaging. 25(4):727-36, 2007
                          7. Rodríguez E et al: Postoperative imaging in cyanotic congenital heart diseases: part 1, Normal findings. AJR Am J Roentgenol. 189(6):1353-60, 2007
                          8. Festa P et al: The role of magnetic resonance imaging in the evaluation of the functionally single ventricle before and after conversion to the Fontan circulation. Cardiol Young. 15 Suppl 3:51-6, 2005