Paediatric Cardiology Flashcards
Name the eight commonest congenital heart defects
- Ventricular septal defect
- Patent ductus arteriosus
- Atrial septal defect
- Pulmonary stenosis
- Aortic stenosis
- Coarctation of the aorta
- Transposition of the great arteries
- Tetralogy of Fallot
What is the aetiology of congenital heart defects
- Genetic susceptibility
- Teratogenic insult (18-60 days post conception)
- Drugs: alcohol, amphetamines, cocain, ecstasy, phenytoin and lithium
- Infections: toxoplasma, rubella, CMV and herpes
- Maternal: diabetes and systemic lupus
- Chromosomal abnormalities
Which chromosomal abnormalities are associated with congenital heart defects?
- Trisomy 13 and 18
- Trisomy 21 (AVSD)
- Turner (Co-arctation of the aorta)
- Noonan (pulmonary stenosis)
- Williams (supravalvular aortic stenosis)
- 22q11 deletion syndrome (De George syndrome)
What features might be present in a history in a child with congenital heart defect?
- Feeding, weight and development
- Cyanosis (central - L to R shunt)
- Tachypnoea and dyspnoea
- Exercise tolerance
- Chest pain
- Syncope
- Palpitation
- Joint problems
What would you examine in a child with a possible congenital heart defect?
- Weight and height
- Dysmorphic features
- Cyanosis
- Clubbing
- Tachy/dyspnoea
- Pulses/apex beat
- Heart sounds
- Murmurs
What investigations can you do for a child with a suspected congenital heart defect?
- Blood pressure
- O2 sats, ABG
- ECG
- CXR
- ECHO
- Catheter
- Angiography
- MRI/A
- Exercise testing
What are the features of heart failure in children?
- Tachypnoea
- Hepatomegaly
- Tachycardia
What are the treatment principles for congenital heart defects in children?
- If you can fix it then fix it
- If you can’t fix it then improve the situation medication, palliative procedures (BT shunt, balloon valvoplasty etc.)
- If you can do neither then replace it
How are murmurs characterised?
- Timing in cardiac cycle: systolic/diastole/continuous
- Duration: early/mid/late and ejection/pansystolic
- Pitch/quality
What are the common features of the innocent murmurs?
- Systolic murmur
- No other signs of cardiac disease
- Soft murmur
- Vibratory, musical
- Localised
- Varies with position, respiration and exercise
What are the features of Still’s murmur?
- LV outflow murmur
- Age 2-7 years
- Soft systolic, vibratory, musical, twangy
- Apex: left sternal border
- Increases in supine position and with exercise
What are the features of a pulmonary outflow murmur?
- Age 8-10 years
- Soft systolic vibratory
- Upper left sternal border
- Well localised
- Not radiating to back
- Increases in supine position and with exercise
- Often children with narrow chest
What are the features of carotid/brachiocephalic arterial bruits?
- Age 2-10yrs
- Systolic and harsh
- Supraclavicular
- Radiates to the neck
- Increases with exercise,decreases on turning head or extending neck
What are the features of a venous hum?
- Age 3-8 years
- Soft and indistinct
- Continuous (sometimes has diastolic accentuation)
- Supraclavicular
- Only in upright position, disappears on lying down or turning head
What are the three main types of ventricular septal defects (L to R shunt)?
- Subaortic
- Perimembranous
- Muscular
How does a ventral septal defect present?
- Pansystolic murmur at the lower left sternal edge +/- thrill
- Small VSDs: early systolic murmur
- Large VSDs: diastolic rumble (relative mitral stenosis)
- Signs of cardiac failure in large VSDs: biventricular hypertrophy and pulmonary hypertension
What are the consequences of VSD if left untreated?
- LVH
- RVH
- Pulmonary hypertension
How can VSD be treated?
- Amplatzer or other occlusion device: trans catheter
- Patch closure: open heart surgery
How do atrial septal defects present?
- Few clinical signs in early childhood (may spontaneously close)
- May present in adulthood with AF, HF or pulmonary hypertension
- Splitting of 2nd heart sound, pulmonary flow murmur
How can an ASD be treated?
Occlusion device
What is the biggest probelm with an AVSD?
There is only one AV valve
How does pulmonary stenosis present?
- Asymptomatic in mild
- Exertional dyspnoea and fatigue in moderate to severe
- Ejection systolic murmur upper left sternal border with radiation to the back
- Can get worse as children grow (
How can pulmonary stenosis be managed?
Balloon valvoplasty
How does aortic stenosis present?
- Mostly symptomatic
- If severe: reduced exercise tolerance, exertional chest pain and syncope
- Ejection systolic murmur, upper right sternal border and radiation into the carotids
How can aortic stenosis be treated?
Balloon aortic valvuloplasty
What changes occur in the fetal circulation at birth?
- Pulmonary vascular resistance falls
- Pulmonary blood flow rises
- Systemic vascular resistance is increased
- Ductus arteriosus closes
- Foramen ovale closes
- Ductus venosus closes
In the foetus which blood vessels take blood to and from the heart from the placentan through the umbilicus?
- Umbilical vein goes to the heart
- Umbilical artery returns the bloods to the placenta
How can patent ductus arteriosus be treated in pre- term babies?
- Fluid restriction/diuretics
- Prostaglandin inhibitors (ibuprofen, indomethacin)
- Surgical ligation
What happens to term babies that have patent ductus arteriosus?
- Most have spontaneous closure
- Not prostaglandin sensitive
How can coarctation of the aorta be investigated?
- ECHO
- MRI
How can coarctation of the aorta be managed?
- Reopen DA with prostaglandins
- Resection with end to end anastomosis
- Subclavian patch repair
- Balloon aortoplasty
How can transposition of the great arteries be managed?
- Rashkind’s atrial septostomy
- Switch procedure
What are the features of tetralogy of Fallot?
- Pulmonary valve stenosis (+right outflow obstruction): determines prognosis
- RVH
- VSD
- Overiding aorta
