The baseline prevalence of congenital heart disease (excluding PDA in preterm infants, mitral valve prolapse, and biscuspid aortic valves) is approximately 0.5-0.8% of all live-births.

The recurrence risk for siblings of children with congenital cardiac malformations have been variably estimated in the region between 1% and 4%, provided there is no strong family history of that particular malformation. The risk is higher in first degree relative  where the lesion is Hypoplastic Left Heart Syndrome (HLHS, 19.3%) and coarctation of the aorta (9.4%). ¹ The most common finding in relatives was of a bicuspid aortic valve (BAV), but some individuals had significant left-sided obstructive lesions including HLHS and coarctation.

If a neonate is born who has a first degree relative (parent or sibling) who has been diagnosed with HLHS, coarctation, or BAV:

• The baby requires a complete cardiovascular examination, ideally on the first day but also later in the first week.
• Infants with symptoms or signs of congenital heart disease should be assessed as indicated.
• Those infants with a normal examination should be referred to the Paediatric and Congenital Cardiac Service outpatient clinic at Starship Hospital for assessment, preferably within the first few weeks of life when sedation for an echocardiogram should not be necessary.

See also the management of antentally diagnosed congenital heart disease