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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
- Hypoplasia/atresia of ascending aorta, aortic valve, left ventricle (LV), and mitral valve
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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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
- Hypoplasia/atresia of ascending aorta, aortic valve, left ventricle (LV), and mitral valve
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
