Congenital Heart Disease Flashcards
what is congenital heart disease?
= abnormality of structure of the heart
when does congenital heart disease present?
= at birth
what are the 4 levels to the spectrum of severity?
1) mild
2) moderate
3) severe
4) Major
what does mild and moderate congenital heart disease present with?
MILD
= asymptomatic
= may resolve spontaneously
- may progress to moderate or severe in adulthood in some specific conditions
MODERATE
= requires specialist intervention and monitoring in cardiac centre
what does severe and major congenital heart disease present with and requruie?
SEVERE
= presents severely ill/die in new-born period or early infancy
MAJOR
= requires surgery within first year of life
how can congenital heart disease be picked up?
1) Screening
- antenatal
- newborn baby check
2) well baby with clinical signs
3) unwell baby;
= cyanosis
= shock
= cardiac failure
when can congenital heart disease present?
1) Antenatally
2) Soon after birth
e. g. cyanosis
3) Day 1-2 baby check
4) Day 3-7
5) 4-6weeks
6) 6-8weeks
= GP check
= incidental finding of murmurs or other clinical contacts
what could be find in a baby with congenital disease check in day 1-2
= murmur
= abnormal pulses
= cyanosis
what could be find inn a baby with congenital heart disease in days 3-7?
= sudden circulatory collapse
= shock
= cyanosis
= sudden death
what could be found in a baby with congenital heart disease in 4-6weeks?
= signs of cardiac failure = reduced feeding = failure to thrive = breathessness = sweatiness
when is antenatal screening done?
= ultrasound at 18-22 weeks gestation
what happens in an antenatal screen?
= 4 chambers heart view and outlaw tract view
what should be given if there is a duct dependent lesion?
= prostaglandin infusion
when should a clinical examination of a new born be done?
= 24hours of age
what should a newborn clinical examination involve?
- femoral pulses
- heart sounds
- presence of murmur
= some includes measurement of pre and post ductal saturations
what should a newborn screen be able to detect?
= murmur
= obvious cyanosis
= abnormal pulses
give 4 examples of murmurs that cold be detected at baby checks?
1) small muscular VSD
2) murmur early in life
3) no haemo-dynamic consequences
4) may close spontaneously
what does cyanosis do to the baby?
= blueish dicolouration
what is cyanosis? (2)
1) any condition causing deoxygenated blood to bypass the lungs and enter the systemic circulation
2) any condition any condition where mixed oxygenated and deoxygenated blood enters the systemic circulation from heart
what are 3 differential diagnosis of cyanosis in newborns?
1) cardiac disease
2) respiratory disease
3) persistent pulmonary hypertension of the newborn (PPHN)
what do cardiac diseased babies tend to present with?
= blue with little or no respiratory distress
- may have pre-post ducal differential
what do respiratory disease babies present with?
= increased work of breathing
= X-ray changes
what does PPHN usually present with?
= often seen inn unwell babies.
= large pre and post ductal differential
when is the time of collapse when theres duct closure?
between 2-7days
what happens when there is duct closure?
- cyanosis or pallor
- tachypnoea
- distress
- rapid deterioration to death
what are clinical signs of duct closure?
- pallor
- prolonger CRT
- poor or absent pulses
- hepatomegaly
- crepitations
- increased work of breathing
what do babies with duct closure become?
= profoundly acidotic
what are 2 differential diagnosis of duct closure?
- sepsis
- metabolic conditions
how would you treat duct closure?
- ABC = support airwave & breathings
- prostaglandin E2 to open duct
- multi-system support
what are the 2 examples of duct dependent conditions?
1) duct dependent systemic circulation
2) duct dependent pulmonary circulation
what causes duct dependent systemic circulation?
- hypo plastic left heart
- critical aortic stenosis
- interrupted aortic arch
- critical coarctation of aorta
what causes duct dependent pulmonary circulation?
- tricuspid atresia
- pulmonary atresia
what does the left to right shunt in cardiac failure do??
- increases pulmonary flow
- increases ventricular load
when does cardiac failure tend to present?
= presents after a few weeks as pulmonary pressures drop
what are the clinical signs of cardiac failure in babies?
- failure to thrive
- slow/reduced feeding
- breathlessness (especially when feeding)
- sweatiness
- hepatomegaly
- crepitations
what sort of effect does a moderate or large ventricular septal defect have?
= big defect - less gradient
= often no murmur at baby check
- murmur develops as pulmonary pressure drops over first weeks
- increased pulmonary circulative, congestive cardiac failure
what is the long term management of major congenital heart disease?
1) surgical management
- repair vs palliation
2) developmental problems
- hypoxia
- bypass time
3) need for further surgery
- valves, stenosis
- transplant
4) emotional/social issues
how do you repair patent ductus arteriosus?
1) catheter procedure
- requires follow up to ensure flow stopped and device is in correct position
= go on to completely normal life
how would you repair VSD?
= restores anatomical normality to heart
how would you repair hyper plastic left heart syndrome (HLHS)?
- 3 stage complex surgery
- significant mortality at each stage and between
- ends with RV supplying systemic circulation
= will fail over time and you will require a transplant
