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Hypoplastic Left Heart Syndrome
Jonathan Hero Chung, MD; Prabhakar Rajiah, MBBS, MD, FRCR, FSCMR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Abbreviations

              • Hypoplastic left heart syndrome (HLHS)
            • Synonyms

              • Aortic atresia
            • Definitions

              • Hypoplasia/atresia of ascending aorta, aortic valve, left ventricle (LV), and mitral valve
                • Secondary findings: Patent ductus arteriosus (PDA), juxtaductal coarctation
              • Most severe congenital heart lesion presenting in neonatal period with congestive heart failure, cardiogenic shock, and cyanosis
              • Category: Cyanotic, cardiomegaly, increased pulmonary vascularity
              • Hemodynamics
                • Severe obstruction of flow to systemic circulation (ductus dependent)
                • Retrograde flow in hypoplastic aortic arch and ascending aorta for cranial and coronary perfusion
                • Volume overload in pulmonary circulation
                • Left-to-right shunting through patent foramen ovale
                • Flow admixture in right atrium → severe cyanosis

            IMAGING

            • General Features

              • Radiographic Findings

                • CT Findings

                  • MR Findings

                    • Echocardiographic 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. Cheasty E et al: The use of cardiovascular CT for the follow up of paediatric hypoplastic left heart syndrome. J Cardiovasc Comput Tomogr. ePub, 2018
                                          2. Roeleveld PP et al: Hypoplastic left heart syndrome: from fetus to fontan. Cardiol Young. 28(11):1275-88, 2018
                                          3. Feinstein JA et al: Hypoplastic left heart syndrome: current considerations and expectations. J Am Coll Cardiol. 2012 Jan 3;59(1 Suppl):S1-42. Review. Erratum in: J Am Coll Cardiol. 59(5):544, 2012
                                          4. Boris JR: Primary care cardiology for patients with hypoplastic left heart syndrome. Cardiol Young. 21 Suppl 2:53-8, 2011
                                          5. Dadlani GH et al: Long-term management of patients with hypoplastic left heart syndrome: the diagnostic approach at All Children's Hospital. Cardiol Young. 21 Suppl 2:80-7, 2011
                                          6. Fuller S et al: Neonatal surgical reconstruction and peri-operative care for hypoplastic left heart syndrome: current strategies. Cardiol Young. 21 Suppl 2:38-46, 2011
                                          7. Nguyen T et al: Echocardiography of hypoplastic left heart syndrome. Cardiol Young. 21 Suppl 2:28-37, 2011
                                          8. 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
                                          9. 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
                                          10. Muthurangu V et al: Cardiac magnetic resonance imaging after stage I Norwood operation for hypoplastic left heart syndrome. Circulation. 112(21):3256-63, 2005
                                          11. Oye RG et al. Hypoplastic left heart syndrome. In Mavroudis C et al: Pediatric Cardiac Surgery. 3rd ed. Philadelphia: Mosby. 560-74, 2003
                                          12. Bardo DM et al: Hypoplastic left heart syndrome. Radiographics. 21(3):705-17, 2001
                                          13. 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
                                          14. Herman TE et al: Special imaging casebook. Hypoplastic left heart, prostaglandin therapy gastric focal foveolar hyperplasia and brown-fat necrosis. J Perinatol. 21(4):263-5, 2001
                                          15. Rosenthal A et al: Hypoplastic left heart syndrome. In Moller JH et al: Pediatric Cardiovascular Medicine. 1st ed. Philadelphia: Churchill Livingstone. 594-605, 2000
                                          16. Fellows KE et al: MR imaging and heart function in patients pre- and post-Fontan surgery. Acta Paediatr Suppl. 410:57-9, 1995
                                          17. Fogel MA et al: A study in ventricular-ventricular interaction. Single right ventricles compared with systemic right ventricles in a dual-chamber circulation. Circulation. 92(2):219-30, 1995
                                          18. Kondo C et al: Nuclear magnetic resonance imaging of the palliative operation for hypoplastic left heart syndrome. J Am Coll Cardiol. 18(3):817-23, 1991
                                          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

                                              • Top Differential Diagnoses

                                                • Pathology

                                                  • Clinical Issues

                                                    TERMINOLOGY

                                                    • Abbreviations

                                                      • Hypoplastic left heart syndrome (HLHS)
                                                    • Synonyms

                                                      • Aortic atresia
                                                    • Definitions

                                                      • Hypoplasia/atresia of ascending aorta, aortic valve, left ventricle (LV), and mitral valve
                                                        • Secondary findings: Patent ductus arteriosus (PDA), juxtaductal coarctation
                                                      • Most severe congenital heart lesion presenting in neonatal period with congestive heart failure, cardiogenic shock, and cyanosis
                                                      • Category: Cyanotic, cardiomegaly, increased pulmonary vascularity
                                                      • Hemodynamics
                                                        • Severe obstruction of flow to systemic circulation (ductus dependent)
                                                        • Retrograde flow in hypoplastic aortic arch and ascending aorta for cranial and coronary perfusion
                                                        • Volume overload in pulmonary circulation
                                                        • Left-to-right shunting through patent foramen ovale
                                                        • Flow admixture in right atrium → severe cyanosis

                                                    IMAGING

                                                    • General Features

                                                      • Radiographic Findings

                                                        • CT Findings

                                                          • MR Findings

                                                            • Echocardiographic 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. Cheasty E et al: The use of cardiovascular CT for the follow up of paediatric hypoplastic left heart syndrome. J Cardiovasc Comput Tomogr. ePub, 2018
                                                                                  2. Roeleveld PP et al: Hypoplastic left heart syndrome: from fetus to fontan. Cardiol Young. 28(11):1275-88, 2018
                                                                                  3. Feinstein JA et al: Hypoplastic left heart syndrome: current considerations and expectations. J Am Coll Cardiol. 2012 Jan 3;59(1 Suppl):S1-42. Review. Erratum in: J Am Coll Cardiol. 59(5):544, 2012
                                                                                  4. Boris JR: Primary care cardiology for patients with hypoplastic left heart syndrome. Cardiol Young. 21 Suppl 2:53-8, 2011
                                                                                  5. Dadlani GH et al: Long-term management of patients with hypoplastic left heart syndrome: the diagnostic approach at All Children's Hospital. Cardiol Young. 21 Suppl 2:80-7, 2011
                                                                                  6. Fuller S et al: Neonatal surgical reconstruction and peri-operative care for hypoplastic left heart syndrome: current strategies. Cardiol Young. 21 Suppl 2:38-46, 2011
                                                                                  7. Nguyen T et al: Echocardiography of hypoplastic left heart syndrome. Cardiol Young. 21 Suppl 2:28-37, 2011
                                                                                  8. 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
                                                                                  9. 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
                                                                                  10. Muthurangu V et al: Cardiac magnetic resonance imaging after stage I Norwood operation for hypoplastic left heart syndrome. Circulation. 112(21):3256-63, 2005
                                                                                  11. Oye RG et al. Hypoplastic left heart syndrome. In Mavroudis C et al: Pediatric Cardiac Surgery. 3rd ed. Philadelphia: Mosby. 560-74, 2003
                                                                                  12. Bardo DM et al: Hypoplastic left heart syndrome. Radiographics. 21(3):705-17, 2001
                                                                                  13. 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
                                                                                  14. Herman TE et al: Special imaging casebook. Hypoplastic left heart, prostaglandin therapy gastric focal foveolar hyperplasia and brown-fat necrosis. J Perinatol. 21(4):263-5, 2001
                                                                                  15. Rosenthal A et al: Hypoplastic left heart syndrome. In Moller JH et al: Pediatric Cardiovascular Medicine. 1st ed. Philadelphia: Churchill Livingstone. 594-605, 2000
                                                                                  16. Fellows KE et al: MR imaging and heart function in patients pre- and post-Fontan surgery. Acta Paediatr Suppl. 410:57-9, 1995
                                                                                  17. Fogel MA et al: A study in ventricular-ventricular interaction. Single right ventricles compared with systemic right ventricles in a dual-chamber circulation. Circulation. 92(2):219-30, 1995
                                                                                  18. Kondo C et al: Nuclear magnetic resonance imaging of the palliative operation for hypoplastic left heart syndrome. J Am Coll Cardiol. 18(3):817-23, 1991
                                                                                  19. Norwood WI Jr: Hypoplastic left heart syndrome. Ann Thorac Surg. 52(3):688-95, 1991