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Hypoplastic Left Heart

Definition

Hypoplasia (underdevelopment of left side of heart)
Incidence 0.16/1000 live births, 9% of congenital heart disease
Diagnosis Ultrasound
Gestation at diagnosis 18-20 weeks
Known chromosomal or genetic association Yes 2%, commonest Turner's syndrome (45XO), but also trisomy 18.  Alternatively may be autosomally recessive
Further investigations

Karyotype and Fetal Echocardiogram

Differential Diaganosis

Double inlet left ventricle

Severe aortic stenosis

Coarctation of the aorta

Prognosis Good initially (~80%) with newer surgical techniques, but then may require heart transplantation
Recurrance risk 2% (unless autosomally recessive)
Research Studies  

Further information for clinicians please click here

Patient information leaflet please click here

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