Congenital Heart Disease Flashcards
what is congenital heart disease?
abnormality of the structure of the heart
present at birth
‘ a gross structural abnormality of the heart or intrathoracic great vessels that is actually or potentially of functional significance’
describe mild congenital heart disease
asymptomatic, may resolve spontaneously or may progress to moderate or severe in adulthood in some specific conditions
describe moderate congenital heart disease
require specialist intervention and monitoring in cardiac centre
describe severe congenital heart disease
present severely ill/ die in newborn period or early infancy
describe major congenital heart disease
requires surgery within the first year of life
how does it present?
screening - antenatal - newborn baby check well baby with clinical signs unwell baby - cyanosis - shock - cardiac failure
presentation day 1-2 baby check
murmurs
abnormal pulses
cyanosis
presentation day 3-7
sudden circulatory collapse
shock
cyanosis
sudden death
presentation 4-6 weeks
signs of cardiac failure- reduced feeding, failure to thrive, breathlessness, sweatiness
presentation 6-8 week GP check
incidental finding of murmurs at other clinical contacts
describe antenatal screening
US at 18-22 weeks gestation 4 chamber heart view and outflow tract view sensitivity very variable - operator experience and training - maternal characteristics
treatment of antenatally diagnosed duct dependant lesion
prostaglandin infusion
describe newborn screening
clinical examination at around 24 hours of age
femoral pulses, heart sounds and presence or murmurs
sometimes includes measurement of pre and post ductal saturations
will detect any condition causing a murmur, obvious cyanosis or abnormal pulses
what causes cyanosis in babies?
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
clinically causes bluish discolouration
differential diagnosis of cyanosis
cardiac disease- blue with little or no respiratory distress
resp disease- increased work of breathing, xray changes
PPHN- otherwise very unwell babies
collapse at time of duct closure
often between 2 and 7 days
severe cyanosis or pallor, tachypnoea, distress, rapid deterioration
pallor, prolonged CRT, poor or absent pulses, hepatomegaly, crepitations, increased work of breathing
profoundly acidotic
treatment of collapse at time of duct closure
ABC- support airway and breathing as necessary
prostglandin E2 to open duct
multisystem supportive treatment
transport to cardiac surgical centres
duct dependant systemic circulation
hypoplastic left heart
critical aortic stenosis
interrupted aortic arch
critical coarctation of aorta
duct dependant pulmonary circulation
tricuspid atresia
pulmonary atresia
presentation with cardiac failure
usually seen with moderate to large left to right shunts- increased pulmonary flow, increased ventricular load
tend to present after a few weeks as pulmonary pressures drop
clinical signs of failure in babies
failure to thrive slow/ reduced feeding breathlessness (especially when feeding) sweatiness hepatomegaly crepitations
describe moderate/ large ventricular septum defect
big defect- less gradient
often no murmur at baby check
murmur develops as pulmonary pressures drop over first weeks
increased pulmonary circulation, congestive cardiac failure
long term management of major congenital heart disease
surgical management - repair vs palliation developmental problems - hypoxia - bypass time need for surgery - valves, stenosis - transplant emotional/ social issues
patent ductus arteriosus repair
catheter procedure - closure by device couple of follow up appointments - ensure flow stopped - device in correct position discharged
ventricular septum defect repair
closure by patch
follow up during childhood/ adolescence
rhythm problems or problems
generally expect to go on to normal life
surgery for hypoplastic left heart syndrome
3 stage complex
total cavo-pulmonary connection
SVC and IVC both connected to right PA
intra atrial patch directs flow from IVC to right PA
significant mortality at each stage and between
ends with RV supplying systemic circulation
will fail over time
transplant
