congenital heart disease Flashcards
>def of congenital heart disease >spectrum of severity of CHD >different ways in which CHDs can present >differences between cyanotic and cyanotic CHD >concept of duct dependent lesions and the emergency management
what is a congenital heart disease
abnormality of the structure of the heart that is present at birth
spectrum of severity
> mild - asymptomatic
may resolve spontaneously (can progress to become >moderate at adult life)
moderate - enquire specialist intervention and monitoring in a cardiac centre
severe - present severely il / die in newborn period or early infancy
hoes does it present
- screening (antenatal / newborn baby check)
- well babies with clinical signs
- unwell babies may be cyanosed, shock, have cardiac failure
when does it present
> antenatally (ie picked up in a scan)
soon after birth (may be cyanosed)
day 1-2 baby check (murmurs, abnormal pulses, cyanosis)
day 3-7 (sudden circulatory collapse, shock, cyanosis, sudden death)
4-6 weeks (signs of cardiac failure ie reduced feeding, failure to thrive, breathlessness, sweatiness)
6-8 weeks GP check / at other clinical checks (incidental finding of murmurs)
antenatal screening
an ultrasound offered at 18-22 weeks of gestation
>4 chamber heart view and outflow tract view
>sensitivity very variable ie material characteristics and skill of operator
management of antenatally diagnosed CHD
> major advantage is allowing treatment before babies becomes critically ill
expert counselling required at time of diagnosis
newborn screening - the baby check
clinical examination at around 24 hours of age
>femoral pulses, heart sounds and presence of murmurs
transposition of the great arteries
one of the common causes of cyanosis in newborns
>ie an atrial septal defect - like the PFO being bigger than normal and so blood can go back and forth between LA and RA
>a large PFO can be presented as normal
collapse at time of duct closure
often between 2-7 days severe cyanosis or pallor, tachypnoea, distress, rapid deteriorations clinical sings include: pallor prolonger CRT poor or absent pulses hapetomegaly crepitations increased work of breathing
treatment of duct collapse
ABC - supportive airway and breathing as necessary
prostaglandin E2
multisystem supportive treatment
transfer to cardiac surgical centre for definitive management
duct dependent conditions - systemic circulation
any condition where blood reaching the aorta is dependent on the duct being open
>hypoplastic LH, critical aortic stenosis, interrupted aortic arch, critical coarctation of aorta
duct dependent pulmonary circulation
any condition where blood reaching the pulmonary arteries is dependent on the duct being open
- tricuspid atresia
- pulmonary atresia
hypo plastic left heart syndrome
while the duct is open the aorta is supplied by the right ventricle via the DA
when the duct closes there is no route for blood from the heart to the aorta
vsd
ventricular septal defect
pulmonary atresia
when the duct is open the blood supply to the lungs comes from the aorta via the ductus arteriosus
when the duct closes there is no way for blood to get to the lungs
