Paediatric cardiology Flashcards
Five features of innocent murmurs
Soft Short Systolic Symptomless Situation dependent (e.g. if quieter when standing or only appears when the child is unwell/feverish)
Features of murmurs that prompt further investigations
Louder than 2/6
Diastolic murmurs
Louder on standing
Symptoms such as failure to thrive, feeding difficulty, cyanosis or shortness of breath
Investigations to determine the cause of a murmur
ECG
Chest X-Ray
Echo
PDA murmur features
Continuous crescendo-decrescendo “machinery” murmur
Tetralogy of Fallot murmur features
Ejection systolic murmur loudest in the pulmonary area (second intercostal space, left sternal border) –arising from the pulmonary stenosis
VSD murmur features
Pan systolic murmur heard at the left lower sternal border
ASD murmur features
Mid-systolic crescendo-decrescendo murmur heard loudest at the upper left sternal border with a fixed split second heart sound
What is a fixed split second heart sound?
Splitting of the second heart sound can be normal with inspiration, however a “fixed split” second heart sound means the split does not change with inspiration and expiration.
What are the causes of cyanotic heart disease?
Anything that can result in a right to left shunt:
- Transposition of the great arteries
- TOF can also cause cyanosis
- VSD
- ASD
- PDA
- These three don’t usually cause cyanosis because the pressure in the left side of the heart is much greater than the right side, and blood will flow from the area of high pressure to the area of low pressure. This prevents a right-to-left shunt. If the pulmonary pressure increases beyond the systemic pressure blood will start to flow from right-to-left across the defect, causing cyanosis. This is called Eisenmenger syndrome.
Risk factors for PDA
Prematurity
Maternal infections (e.g. rubella)
Genetic element
PDA presentation
May be asymptomatic Shortness of breath Difficulty feeding Poor weight gain Lower RTIs Murmur Heart failure symptoms in adulthood (rarely)
PDA murmur
continuous crescendo-decrescendo “machinery” murmur that may continue during the second heart sound, making the second heart sound difficult to hear.
Investigations to diagnose PDA
Echocardiogram - can also assess effect of PDA on the heart e.g. RVH or LVH
Doppler during the echo to assess size of left to right shunt
Management of PDA
Indomethacin to promote its closure
If that is ineffective:
Monitoring with Echo until 1 year of age
After 1 year old highly unlikely to close spontaneously so surgical/transcatheter closure
Complications of ASD
Stroke (VTE)
AF or atrial flutter
Pulmonary hypertension and right sided heart failure
Eisenmenger syndrome
Symptoms/presentation of ASD
May be asymptomatic in childhood SOB (dyspnoea) Difficulty feeding Poor weight gain/failure to thrive Lower RTIs
May be picked up on antenatal scans or hearing a murmur in newborn baby check
In adulthood it can present with dyspnoea, heart failure or stroke
ASD murmur features
Mid systolic crescendo-decrescendo murmur loudest at upper left sternal border
Fixed split second heart sound
ASD management
Refer to paediatric cardiologist
If small can watch and wait
Surgical closure (open or transcatheter)
Anticoagulants (aspirin, warfarin, NOACs) used in adults to prevent VTE/Stroke
VSD is commonly associated with what conditions
Down’s syndrome
Turner’s syndrome
Presentation of VSD
Often initially symptomless and patients can present as late as adulthood
May be picked up on antenatal scans or hearing a murmur in newborn baby check
Poor feeding
Tachypnoea
Dyspnoea
Failure to thrive
VSD murmur features
pan systolic murmur heard loudest at the left lower sternal border in 3rd and 4th intercostal spaces
Treatment of VSD
Referral to paediatric cardiologist
Small VSDs with no symptoms can watch and wait - often close spontaneously
Surgery to close - either transcatheter or open heart surgery (with antibiotic prophylaxis to reduce risk of infective endocarditis)
Risks of VSD, complications of VSD
Pulmonary hypertension and right sided heart failure
Eisenmenger syndrome
Infective endocarditis
What is Eisenmenger syndrome?
ASD/VSD leads to left to right shunt
Which leads to pulmonary hypertension
When pulmonary pressure exceeds systemic pressure the shunt switches to right to left
Deoxygenated blood bypasses the lungs leading to cyanosis
What is coarctation of the aorta?
Where there is narrowing of the aortic arch
What is coarctation of the aorta associated with
Often associated with an underlying genetic condition - particularly Turner’s syndrome
Presentation of coarctation of the aorta
Weak femoral pulses
Systolic murmur heard below the left clavicle
Difference in blood pressure between limbs
Tachypnoea and increased work of breathing
Poor feeding
Grey floppy baby
Management of coarctation of the aorta
Mild cases - can live symptom free until adulthood without need for surgery
Severe cases - emergency surgery
In critical cases where there is a risk of heart failure and death shortly after birth - can give prostaglandin E to keep ductus arteriosus open
Presentation of congenital aortic stenosis
May be incidental finding due to murmur with no symptoms
Fatigue
SOB
Dizziness and fainting
Worse on exertion
Murmur and signs in Aortic stenosis
Ejection systolic murmur heard loudest in second intercostal space, right sternal border (aortic area)
Crescendo-decrescendo murmur which radiates to the carotids
Other signs:
- Ejection click before the murmur
- Palpable thrill during systole
- Slow rising pulse and narrow pulse pressure
Investigation for Aortic stenosis
Echo
Also will need ECG and exercise testing at follow up with a paeds cardiologist
Management for aortic stenosis
Regular follow up as it is a progressive condition usually
May need to restrict physical activities
Treatment options:
- Percutaneous balloon aortic valvuloplasty
- Surgical aortic valvotomy
- Valve replacement
Complications of Aortic stenosis in children
Left ventricular outflow tract obstruction Heart failure Ventricular arrhythmia Bacterial endocarditis Sudden death, often on exertion
Congenital pulmonary valve stenosis associations
Congenital pulmonary valve stenosis often occurs without any associations. It can be associated with other conditions such as:
Tetralogy of Fallot
William syndrome
Noonan syndrome
Congenital rubella syndrome
Symptoms in pulmonary valve stenosis
Often asymptomatic
More severe cases can present with fatigue on exertion, SOB, dizziness and fainting
Signs that may be present in pulmonary valve stenosis
Ejection systolic murmur heard loudest at the pulmonary area (second intercostal space, left sternal border)
Palpable thrill in the pulmonary area
Right ventricular heave due to right ventricular hypertrophy
Raised JVP with giant a waves
Investigations in suspected pulmonary valve stenosis or any congenital heart disease for that matter
Echocardiogram
ECG
Management of P valve stenosis
Mild - can watch and wait with regular follow up by cardiologist
Symptomatic or more significant stenosis - balloon valvuloplasty or open heart valve repair
Four pathologies in tetralogy of Fallot
PROV
Pulmonary valve stenosis
Right ventricular hypertrophy
Overriding aorta
Ventricular septal defect
Type of shunt in TOF
Right to left cardiac shunt
Therefore cyanosis can occur
Risk factors for TOF
Rubella infection
Increased age of the mother (>40 years)
Alcohol consumption in pregnancy
Diabetic mother
How does TOF present?
Most cases picked up on antenatal scans
Ejection systolic murmur caused by pulmonary stenosis
Cyanosis Poor feeding Poor weight gain Tet spells - Spells where r-L shunt worsens and cyanosis occurs. These episodes may be precipitated by waking, physical exertion or crying. The child will become irritable, cyanotic and short of breath. Severe spells can lead to reduced consciousness, seizures and potentially death. Clubbing
Treatment of tet spells
Encourage child to squat or bring knees to chest in younger children
Oxygen
Beta blockers
IV fluids
Management of TOF
Prostaglandin infusion in neonates to maintain PDA then total surgical repair by open heart surgery
Ebstein’s anomaly - what is it and what is the presentation?
Tricuspid valve is set lower in the right side of the heart causing a bigger right atrium and small right ventricle and therefore poor flow to the pulmonary vessels
Cyanosis (due to right to left shunt)
Evidence of heart failure e.g. oedems
SOB and tachypnoea
Poor feeding
Associated with WPW syndrome
What is transposition of the great arteries?
Attachments of the aorta and pulmonary trunk are swapped
What can transposition of the great arteries also be associated with?
VSD
Coarctation of the aorta
Pulmonary stenosis
How does transposition of the great arteries present?
Often diagnosed during antenatal ultrasound scans
As cyanosis at birth or in first days of life - PDA can initially compensate for this
Management
Prostaglandin infusion can be used to maintain the ductus arteriosus
Balloon septostomy involves inserting a catheter into the foramen ovale via the umbilicus, and inflating a balloon to create a large atrial septal defect. This allows blood returning from the lungs (on the left side) to flow to the right side of the heart and out through the aorta to the body.
Open heart surgery is the definitive management - ASAP
