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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
- Performed between 3-6 months of age
- 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
- 3-stage procedure to palliate single ventricle physiology (most commonly hypoplastic left heart syndrome)
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
DIFFERENTIAL DIAGNOSIS
CLINICAL ISSUES
Natural History & Prognosis
Selected References
- Dillman JR et al: Imaging of Fontan-associated liver disease. Pediatr Radiol. 50(11):1528-41, 2020
- 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
- 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
- Nakamura Y et al: The Norwood procedure with valvular pulmonary stenosis. Ann Thorac Surg. 107(1):e49-50, 2019
- 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
- Téllez L et al: Fontan-associated liver disease: a review. Ann Hepatol. 17(2):192-204, 2018
- 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
- Kutty S et al: Role of imaging in the evaluation of single ventricle with the Fontan palliation. Heart. 102(3):174-83, 2016
- 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
- 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
- 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
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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
- Performed between 3-6 months of age
- 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
- 3-stage procedure to palliate single ventricle physiology (most commonly hypoplastic left heart syndrome)
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
DIFFERENTIAL DIAGNOSIS
CLINICAL ISSUES
Natural History & Prognosis
Selected References
- Dillman JR et al: Imaging of Fontan-associated liver disease. Pediatr Radiol. 50(11):1528-41, 2020
- 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
- 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
- Nakamura Y et al: The Norwood procedure with valvular pulmonary stenosis. Ann Thorac Surg. 107(1):e49-50, 2019
- 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
- Téllez L et al: Fontan-associated liver disease: a review. Ann Hepatol. 17(2):192-204, 2018
- 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
- Kutty S et al: Role of imaging in the evaluation of single ventricle with the Fontan palliation. Heart. 102(3):174-83, 2016
- 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
- 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
- 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
