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Hypoplastic Left Heart Syndrome
Ryan A. Moore, MD; Randy R. Richardson, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Clinical Issues

        TERMINOLOGY

        • Abbreviations

          • Hypoplastic left heart syndrome (HLHS)
        • Synonyms

          • 4 classic types: Mitral atresia/aortic atresia, mitral atresia/aortic stenosis, mitral stenosis/aortic atresia, mitral stenosis/aortic stenosis
        • Definitions

          • Hypoplasia/atresia of ascending aorta, aortic valve, left ventricle (LV), & mitral valve (MV)
            • Secondary findings: Patent ductus arteriosus (PDA), juxtaductal coarctation
          • Most severe congenital heart lesion
            • Presents in neonatal period with congestive heart failure, cardiogenic shock, & cyanosis
          • Category: Cyanotic, cardiomegaly, ↑ pulmonary vascularity
          • Hemodynamics
            • Severe obstruction of flow to systemic circulation (ductal dependent)
            • Retrograde flow in hypoplastic aortic arch & ascending aorta for cranial & coronary perfusion
            • Volume overload in pulmonary circulation
            • Obligate left-to-right shunt at atrial level via patent foramen ovale (PFO) or secundum atrial septal defect (ASD)
              • Restrictive atrial septum in neonate is highly lethal & requires emergent catheter-based balloon atrial septostomy
            • Flow admixture in right atrium → severe cyanosis

        IMAGING

        • General Features

          • Radiographic Findings

            • Echocardiographic Findings

              • CT Findings

                • MR Findings

                  • Angiographic Findings

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Gross Pathologic & Surgical Features

                            CLINICAL ISSUES

                            • Presentation

                              • Demographics

                                • Natural History & Prognosis

                                  • Treatment

                                    Selected References

                                    1. Baş S et al: Ventriculocoronary fistulas with hypoplastic left heart in a neonate: imaging with cardiac CT. Case Rep Radiol. 2021:6657447, 2021
                                    2. Bellsham-Revell H: Noninvasive imaging in interventional cardiology: hypoplastic left heart syndrome. Front Cardiovasc Med. 8:637838, 2021
                                    3. Wang AP et al: Ventriculotomy decreases agreement between assessment of right ventricular function by echocardiography and cardiac magnetic resonance imaging in patients with hypoplastic left heart syndrome. Pediatr Cardiol. 42(4):951-9, 2021
                                    4. Friedberg MK et al: Right ventricular failure in congenital heart disease. Curr Opin Pediatr. 31(5):604-10, 2019
                                    5. Kovaćević-Kuśmierek K et al: Lung perfusion scintigraphy in the assessment of pulmonary circulation after completion of surgical treatment of a hypoplastic left heart syndrome (HLHS). Nucl Med Rev Cent East Eur. 22(2):81-4, 2019
                                    6. Chen SA et al: Digital design and 3D printing of aortic arch reconstruction in HLHS for surgical simulation and training. World J Pediatr Congenit Heart Surg. 9(4):454-8, 2018
                                    7. Salehi Ravesh M et al: Longitudinal deformation of the right ventricle in hypoplastic left heart syndrome: a comparative study of 2D-feature tracking magnetic resonance imaging and 2D-speckle tracking echocardiography. Pediatr Cardiol. 39(6):1265-75, 2018
                                    8. Ruotsalainen H et al: Right ventricular systolic function in hypoplastic left heart syndrome: a comparison of velocity vector imaging and magnetic resonance imaging. Eur Heart J Cardiovasc Imaging. 17(6):687-92, 2016
                                    9. Nassar MS et al: Technical and anatomical factors affecting the size of the branch pulmonary arteries following first-stage Norwood palliation for hypoplastic left heart syndrome. Interact Cardiovasc Thorac Surg. 20(5):631-5, 2015
                                    10. Bellsham-Revell HR et al: Serial magnetic resonance imaging in hypoplastic left heart syndrome gives valuable insight into ventricular and vascular adaptation. J Am Coll Cardiol. 61(5):561-70, 2013
                                    11. Fredenburg TB et al: The Fontan procedure: anatomy, complications, and manifestations of failure. Radiographics. 31(2):453-63, 2011
                                    12. Valsangiacomo Buechel ER et al: Congenital cardiac defects and MR-guided planning of surgery. Magn Reson Imaging Clin N Am. 19(4):823-40; viii, 2011
                                    13. Valverde I et al: Comprehensive four-dimensional phase-contrast flow assessment in hemi-Fontan circulation: systemic-to-pulmonary collateral flow quantification. Cardiol Young. 21(1):116-9, 2011
                                    14. Grosse-Wortmann L et al: Borderline hypoplasia of the left ventricle in neonates: insights for decision-making from functional assessment with magnetic resonance imaging. J Thorac Cardiovasc Surg. 136(6):1429-36, 2008
                                    15. Hirsch JC et al: Fontan operation in the current era: a 15-year single institution experience. Ann Surg. 248(3):402-10, 2008
                                    16. Muthurangu V et al: Cardiac magnetic resonance imaging after stage I Norwood operation for hypoplastic left heart syndrome. Circulation. Nov 22;112(21):3256-63, 2005; erratum in: Circulation. 113(5):e70, 2006
                                    17. Sundareswaran KS et al: Impaired power output and cardiac index with hypoplastic left heart syndrome: a magnetic resonance imaging study. Ann Thorac Surg. 82(4):1267-75; discussion 1275-7, 2006
                                    18. Cheatham JP: Intervention in the critically ill neonate and infant with hypoplastic left heart syndrome and intact atrial septum. J Interv Cardiol. 14(3):357-66, 2001
                                    19. Norwood WI Jr: Hypoplastic left heart syndrome. Ann Thorac Surg. 52(3):688-95, 1991
                                    Related Anatomy
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                                    Related Differential Diagnoses
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                                    References
                                    Tables

                                    Tables

                                    KEY FACTS

                                    • Terminology

                                      • Imaging

                                        • Clinical Issues

                                          TERMINOLOGY

                                          • Abbreviations

                                            • Hypoplastic left heart syndrome (HLHS)
                                          • Synonyms

                                            • 4 classic types: Mitral atresia/aortic atresia, mitral atresia/aortic stenosis, mitral stenosis/aortic atresia, mitral stenosis/aortic stenosis
                                          • Definitions

                                            • Hypoplasia/atresia of ascending aorta, aortic valve, left ventricle (LV), & mitral valve (MV)
                                              • Secondary findings: Patent ductus arteriosus (PDA), juxtaductal coarctation
                                            • Most severe congenital heart lesion
                                              • Presents in neonatal period with congestive heart failure, cardiogenic shock, & cyanosis
                                            • Category: Cyanotic, cardiomegaly, ↑ pulmonary vascularity
                                            • Hemodynamics
                                              • Severe obstruction of flow to systemic circulation (ductal dependent)
                                              • Retrograde flow in hypoplastic aortic arch & ascending aorta for cranial & coronary perfusion
                                              • Volume overload in pulmonary circulation
                                              • Obligate left-to-right shunt at atrial level via patent foramen ovale (PFO) or secundum atrial septal defect (ASD)
                                                • Restrictive atrial septum in neonate is highly lethal & requires emergent catheter-based balloon atrial septostomy
                                              • Flow admixture in right atrium → severe cyanosis

                                          IMAGING

                                          • General Features

                                            • Radiographic Findings

                                              • Echocardiographic Findings

                                                • CT Findings

                                                  • MR Findings

                                                    • Angiographic Findings

                                                      • Imaging Recommendations

                                                        DIFFERENTIAL DIAGNOSIS

                                                          PATHOLOGY

                                                          • General Features

                                                            • Gross Pathologic & Surgical Features

                                                              CLINICAL ISSUES

                                                              • Presentation

                                                                • Demographics

                                                                  • Natural History & Prognosis

                                                                    • Treatment

                                                                      Selected References

                                                                      1. Baş S et al: Ventriculocoronary fistulas with hypoplastic left heart in a neonate: imaging with cardiac CT. Case Rep Radiol. 2021:6657447, 2021
                                                                      2. Bellsham-Revell H: Noninvasive imaging in interventional cardiology: hypoplastic left heart syndrome. Front Cardiovasc Med. 8:637838, 2021
                                                                      3. Wang AP et al: Ventriculotomy decreases agreement between assessment of right ventricular function by echocardiography and cardiac magnetic resonance imaging in patients with hypoplastic left heart syndrome. Pediatr Cardiol. 42(4):951-9, 2021
                                                                      4. Friedberg MK et al: Right ventricular failure in congenital heart disease. Curr Opin Pediatr. 31(5):604-10, 2019
                                                                      5. Kovaćević-Kuśmierek K et al: Lung perfusion scintigraphy in the assessment of pulmonary circulation after completion of surgical treatment of a hypoplastic left heart syndrome (HLHS). Nucl Med Rev Cent East Eur. 22(2):81-4, 2019
                                                                      6. Chen SA et al: Digital design and 3D printing of aortic arch reconstruction in HLHS for surgical simulation and training. World J Pediatr Congenit Heart Surg. 9(4):454-8, 2018
                                                                      7. Salehi Ravesh M et al: Longitudinal deformation of the right ventricle in hypoplastic left heart syndrome: a comparative study of 2D-feature tracking magnetic resonance imaging and 2D-speckle tracking echocardiography. Pediatr Cardiol. 39(6):1265-75, 2018
                                                                      8. Ruotsalainen H et al: Right ventricular systolic function in hypoplastic left heart syndrome: a comparison of velocity vector imaging and magnetic resonance imaging. Eur Heart J Cardiovasc Imaging. 17(6):687-92, 2016
                                                                      9. Nassar MS et al: Technical and anatomical factors affecting the size of the branch pulmonary arteries following first-stage Norwood palliation for hypoplastic left heart syndrome. Interact Cardiovasc Thorac Surg. 20(5):631-5, 2015
                                                                      10. Bellsham-Revell HR et al: Serial magnetic resonance imaging in hypoplastic left heart syndrome gives valuable insight into ventricular and vascular adaptation. J Am Coll Cardiol. 61(5):561-70, 2013
                                                                      11. Fredenburg TB et al: The Fontan procedure: anatomy, complications, and manifestations of failure. Radiographics. 31(2):453-63, 2011
                                                                      12. Valsangiacomo Buechel ER et al: Congenital cardiac defects and MR-guided planning of surgery. Magn Reson Imaging Clin N Am. 19(4):823-40; viii, 2011
                                                                      13. Valverde I et al: Comprehensive four-dimensional phase-contrast flow assessment in hemi-Fontan circulation: systemic-to-pulmonary collateral flow quantification. Cardiol Young. 21(1):116-9, 2011
                                                                      14. Grosse-Wortmann L et al: Borderline hypoplasia of the left ventricle in neonates: insights for decision-making from functional assessment with magnetic resonance imaging. J Thorac Cardiovasc Surg. 136(6):1429-36, 2008
                                                                      15. Hirsch JC et al: Fontan operation in the current era: a 15-year single institution experience. Ann Surg. 248(3):402-10, 2008
                                                                      16. Muthurangu V et al: Cardiac magnetic resonance imaging after stage I Norwood operation for hypoplastic left heart syndrome. Circulation. Nov 22;112(21):3256-63, 2005; erratum in: Circulation. 113(5):e70, 2006
                                                                      17. Sundareswaran KS et al: Impaired power output and cardiac index with hypoplastic left heart syndrome: a magnetic resonance imaging study. Ann Thorac Surg. 82(4):1267-75; discussion 1275-7, 2006
                                                                      18. Cheatham JP: Intervention in the critically ill neonate and infant with hypoplastic left heart syndrome and intact atrial septum. J Interv Cardiol. 14(3):357-66, 2001
                                                                      19. Norwood WI Jr: Hypoplastic left heart syndrome. Ann Thorac Surg. 52(3):688-95, 1991