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Hypoplastic Left Heart Syndrome
Ryan A. Moore, MD; Robert J. Fleck, 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

                    • Other Modality Findings

                      • Imaging Recommendations

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            • Gross Pathologic & Surgical Features

                              CLINICAL ISSUES

                              • Presentation

                                • Demographics

                                  • Natural History & Prognosis

                                    • Treatment

                                      Selected References

                                      1. 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
                                      2. 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
                                      3. 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
                                      4. Fredenburg TB et al: The Fontan procedure: anatomy, complications, and manifestations of failure. Radiographics. 31(2):453-63, 2011
                                      5. 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
                                      6. 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
                                      7. 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
                                      8. Hirsch JC et al: Fontan operation in the current era: a 15-year single institution experience. Ann Surg. 248(3):402-10, 2008
                                      9. 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
                                      10. 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
                                      11. 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
                                      12. 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

                                                        • Other Modality Findings

                                                          • Imaging Recommendations

                                                            DIFFERENTIAL DIAGNOSIS

                                                              PATHOLOGY

                                                              • General Features

                                                                • Gross Pathologic & Surgical Features

                                                                  CLINICAL ISSUES

                                                                  • Presentation

                                                                    • Demographics

                                                                      • Natural History & Prognosis

                                                                        • Treatment

                                                                          Selected References

                                                                          1. 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
                                                                          2. 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
                                                                          3. 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
                                                                          4. Fredenburg TB et al: The Fontan procedure: anatomy, complications, and manifestations of failure. Radiographics. 31(2):453-63, 2011
                                                                          5. 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
                                                                          6. 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
                                                                          7. 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
                                                                          8. Hirsch JC et al: Fontan operation in the current era: a 15-year single institution experience. Ann Surg. 248(3):402-10, 2008
                                                                          9. 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
                                                                          10. 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
                                                                          11. 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
                                                                          12. Norwood WI Jr: Hypoplastic left heart syndrome. Ann Thorac Surg. 52(3):688-95, 1991