II. Definitions
- Hypoplastic Left Heart Syndrome
- Under-developed entire left heart (left atrium, left ventricle, aorta, aortic valve, mitral valve)
- Severe symptom onset as the ductus arteriosus closes
III. Pathophysiology
- Ductus dependent lesion (along with reliance on right ventricle)
- Characteristic Anomalies
- Left ventricle hypoplasia
- Ascending aorta hypoplasia
- Aortic Stenosis (or hypoplasia or atresia)
- Mitral Stenosis (or hypoplasia or atresia)
IV. Associated Conditions
- Atrial Septal Defect (ASD) or Patent Foramen Ovale
- Patent Ductus Arteriousus
- Aortic Coarctation
V. Management: Surgery
- Uniformly fatal without surgical correction
- See Single Ventricle Defect Management
- Includes Acute decompensation following first Norwood Procedure
VI. References
- Civitarese and Crane (2016) Crit Dec Emerg Med 30(1): 14-23
- Sloas and Orman in Majoewsky (2013) EM:Rap 13(9): 10-11
- Rao in Berger (2013) Pediatric Hypoplastic Left Heart Syndrome, Emedicine