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
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
Roeleveld PP et al: Hypoplastic left heart syndrome: from fetus to fontan. Cardiol Young. 28(11):1275-88, 2018
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
Boris JR: Primary care cardiology for patients with hypoplastic left heart syndrome. Cardiol Young. 21 Suppl 2:53-8, 2011
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
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
Nguyen T et al: Echocardiography of hypoplastic left heart syndrome. Cardiol Young. 21 Suppl 2:28-37, 2011
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
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
Muthurangu V et al: Cardiac magnetic resonance imaging after stage I Norwood operation for hypoplastic left heart syndrome. Circulation. 112(21):3256-63, 2005
Oye RG et al. Hypoplastic left heart syndrome. In Mavroudis C et al: Pediatric Cardiac Surgery. 3rd ed. Philadelphia: Mosby. 560-74, 2003
Bardo DM et al: Hypoplastic left heart syndrome. Radiographics. 21(3):705-17, 2001
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
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
Rosenthal A et al: Hypoplastic left heart syndrome. In Moller JH et al: Pediatric Cardiovascular Medicine. 1st ed. Philadelphia: Churchill Livingstone. 594-605, 2000
Fellows KE et al: MR imaging and heart function in patients pre- and post-Fontan surgery. Acta Paediatr Suppl. 410:57-9, 1995
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
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
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
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
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
Roeleveld PP et al: Hypoplastic left heart syndrome: from fetus to fontan. Cardiol Young. 28(11):1275-88, 2018
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
Boris JR: Primary care cardiology for patients with hypoplastic left heart syndrome. Cardiol Young. 21 Suppl 2:53-8, 2011
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
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
Nguyen T et al: Echocardiography of hypoplastic left heart syndrome. Cardiol Young. 21 Suppl 2:28-37, 2011
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
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
Muthurangu V et al: Cardiac magnetic resonance imaging after stage I Norwood operation for hypoplastic left heart syndrome. Circulation. 112(21):3256-63, 2005
Oye RG et al. Hypoplastic left heart syndrome. In Mavroudis C et al: Pediatric Cardiac Surgery. 3rd ed. Philadelphia: Mosby. 560-74, 2003
Bardo DM et al: Hypoplastic left heart syndrome. Radiographics. 21(3):705-17, 2001
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
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
Rosenthal A et al: Hypoplastic left heart syndrome. In Moller JH et al: Pediatric Cardiovascular Medicine. 1st ed. Philadelphia: Churchill Livingstone. 594-605, 2000
Fellows KE et al: MR imaging and heart function in patients pre- and post-Fontan surgery. Acta Paediatr Suppl. 410:57-9, 1995
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
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
Norwood WI Jr: Hypoplastic left heart syndrome. Ann Thorac Surg. 52(3):688-95, 1991
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