Hypoplastic Left Heart
Syndrome (HLHS)
Epidemiology:
Common sites:
Gross features:
- left ventricular hypoplasia (varying degrees)
- left ventricular endocardial sclerosis
- stenosis / atresia
of mitral valve and aorta
- hypoplasia of ascending aorta
- patent ductus arteriosus (main
conduit for systemic and coronary circulation)
- patent foramen ovale (usually)
- dilated and
hypertrophied right heart and pulmonary trunk
- normal birthweight usually
- no extracardiac malformations mostly
- often congenital
brain anomalies
Histologic features:
Immunophenotype:
Marker:
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Sensitivity:
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Specificity:
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Molecular features:
Other features:
- death within first
hours or days
- association with
Turner syndrome
References: