congenital heart disease Flashcards
what is congenital heart disease
Abnormality of the structure of the heart/great vessels present at birth
incidence
estimated between 4 or 13 per 1000 liver birth
Scottish is 8 per 1000, major defects 3/1000
Mild –
asymptomatic, may resolve spontaneously (may progress to moderate or severe in adulthood in some specific conditions)
Moderate –
require specialist intervention and monitoring in a cardiac centre
Severe –
present severely ill / die in newborn period or early infancy
Major congenital heart disease –
requires surgery within the first year of life
when does it present
Antenatally - pre birth
antenatal screening
- Ultrasound at 18-22 weeks gestation
- 4 chamber heart view and outflow tract view
- Sensitivity very variable
- Operator experience and training
- Maternal characteristics - Large centres in some countries achieve 80% detection rates
Newborn screening – The Baby Check
Clinical examination at around 24 hours of age
Femoral pulses, heart sounds and presence of murmurs
In some regions includes measurement of pre and post ductal saturations
Will detect any condition causing a murmur, obvious cyanosis or abnormal pulses
Effectiveness of newborn screening
More than half of infants with congenital heart disease missed by newborn clinical examination
Around 1/3 of infants with life threatening heart abnormality left hospital undiagnosed
Around half of babies with murmurs have underlying heart disease – murmurs common in normal babies
murmur at baby check
- small VSD (ventricular septal defect)
- hole blood flow from left to right cause murmur
- murmur early in life
- no haemodynamic consequences
- may close spontaneously
Cyanosis – Blue baby
Any condition causing deoxygenated blood to bypass the lungs and enter the systemic circulation
Any condition where mixed oxygenated and deoxygenated blood enters the systemic circulation from the heart
collapse at time of duct closure
- often between 2 and 7 days
- severe cyanosis or pallor, tachypnoea, distress, rapid deterioration to death
- clinical signs include pallor, prolonged CRT, poor or absent pulses, hepatomegaly, crepitations, increased work breathing
collapse at time of duct treatment
- ABC – support airway and breathing as necessary
- Prostaglandin E2 to open duct
- Multi system supportive treatment
- Transfer to cardiac surgical centre for definitive management
Duct dependent systemic circulation
Any condition where blood reaching the aorta is dependent on the duct being open
eg. Hypoplastic left heart, critical aortic stenosis, interrupted aortic arch, critical coarctation of aorta
