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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
- Hypoplasia/atresia of ascending aorta, aortic valve, left ventricle (LV), & mitral valve (MV)
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
- 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
- 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
- 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
- Fredenburg TB et al: The Fontan procedure: anatomy, complications, and manifestations of failure. Radiographics. 31(2):453-63, 2011
- 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
- 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
- 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
- Hirsch JC et al: Fontan operation in the current era: a 15-year single institution experience. Ann Surg. 248(3):402-10, 2008
- 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
- 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
- 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
- Norwood WI Jr: Hypoplastic left heart syndrome. Ann Thorac Surg. 52(3):688-95, 1991
Related Anatomy
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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
- Hypoplasia/atresia of ascending aorta, aortic valve, left ventricle (LV), & mitral valve (MV)
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
- 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
- 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
- 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
- Fredenburg TB et al: The Fontan procedure: anatomy, complications, and manifestations of failure. Radiographics. 31(2):453-63, 2011
- 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
- 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
- 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
- Hirsch JC et al: Fontan operation in the current era: a 15-year single institution experience. Ann Surg. 248(3):402-10, 2008
- 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
- 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
- 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
- Norwood WI Jr: Hypoplastic left heart syndrome. Ann Thorac Surg. 52(3):688-95, 1991
