Flashcards in Congenital Heart Disease Deck (29)
What is congenital heart disease?
Abnormality of the structure of the heart
Present at birth
'Gross abnormality of the heart or intrathoracic great vessels that is actually or potentially of functional significance
Spectrum of severity for congenital heart disease?
Describe mild congenital heart disease?
May resolve spontaneously
(can progress to moderate or severe in specific conditions at adulthood)
Describe moderate congenital heart disease?
Require specialist intervention
Monitoring in cardiac center
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 is congenital heart disease picked up?
Screening- antenatal, newborn baby check
Well baby with clinical signs
Unwell baby - cyanosis, shock, cardiac failure
When congenital heart disease can present?
Soon after birth- cyanosis
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- reduced feeding, failure to thrive, breathlessness, sweaty
6-8 week GP check= Finding murmurs incidentally
What is involved in antenatal screening?
Ultrasound at 18-22 weeks
4 chamber heart view and outflow tract
Management of antenatally diagnosed congenital heart disease?
Plans in place with expert team available
May decide to deliver in cardiac surgical centre
Prostaglandin infusion duct dependant lesion
What is newborn screening?
Clinical examination at around 24 hours of age
Femoral Pulses, heart sounds, presence of murmurs
Murmur in new born health check?
Small muscular VSD
Murmur early in life
No haemodynamic consequences
May close spontaneously
What is cyanosis?
-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
What does cyanosis do clinically?
Differential diagnosis of cyanosis in newborn baby?
-Persistent pulmonary hypertension of the newborn
-Pre-post ductal differential - cardiac babies tend to be blue with little to no respiratory distress
Clinical sign of collapse at time of duct closure?
Poor or absent pulses
Increased work of breathing
Differential Diagnosis for collapse at time of duct closure?
When do babies often collapse due to duct closure?
2-7 days after birth
Treatment of babies who collapse on duct closure?
ABC- support airway and breathing as necessary
Prostaglandin E2 to open duct
Multisystem supportive treatment
Transfer to cardiac surgical centre for definitive management
Examples of duct dependent systemic circulation conditions?
Hypoplastic left heart
Critical aortic stenosis
Interrupted aortic arch
Critical coarctation of aorta
Examples of duct dependant conditions in relation to pulmonary circulation?
Clinical signs of cardiac failure in babies?
-Failure to thrive
-Breathlessness (especially when feeding)
-Hepatomegaly (enlargement of the liver)
Events in a moderate ventricular septal defect?
Often no murmur at baby check
Murmur develops as pulmonary pressures drop over first weeks
Effects of VSD? Ventricular septal defect
Increased pulmonary circulation
Congestive cardiac failure
Longer term management of major congenital heart disease?
Developmental problems - hypoxia, bypass time
Need for further surgery- valve, stenosis, transplant
Patent ductus arteriosus repair?
Follow up appointments- ensure flow stopped, device in correct position
Ventricular septal defect repair?
-Follow up during childhood/adolescence
-Rhythm problems or valve problems
-Generally expected to go onto normal life
Hypoplastic left heart syndrome repair?
3 stage complex surgery
Significant mortality at each stage and between
Ends with RV supplying systemic circulation
Will fail over time- transplant