Paediatric Cardiology Flashcards
What congenital heart problems can children have?
Septal defects PDA Valve stenosis (A or P) Coarctation of the aorta Tetralogy of Fallot Transposition of the great arteries
How do congenital heart defects present?
With heart failure, a murmur, shock, or cyanosis.
What is the most common congenital heart defect?
Ventricular septal defect, by far.
If a newborn present with the symptoms of heart failure, what do we suspect? (In order of likelihood)
- Ventricular septal defect
- Patent ductus arteriosus
- Atrioventricular septal defect
Tell me about a ventricular septal defect.
Can be membranous or muscular.
May be asymptomatic, and many close spontaneously.
O/E harsh pansystolic murmur at lower left sternal edge + parasternal thrill.
If a newborn has down’s syndrome and presents with heart failure, what should we suspect?
Atrioventricular septal defect - 40% are associated with Down’s.
How can we look for AVSD?
ECG
What will an ECG of AVSD look like?
Superior QRS axis
A baby is brought into A and E by her father because she turned blue.
Which 2 systems are we trying to work with here?
Cardiovascular and respiratory
A baby is brought into A and E by her father because she turned blue.
What cardiac causes are there for a blue baby?
- Heart failure
- Heart murmurs
- Congenital heart disease (L->R shunting)
What are the 5 S’s of innocent murmurs in children?
- Short
- Symptomless
- Systolic
- Soft
- Sitting/standing (vary with position)
A baby is brought into A and E by her father because she turned blue.
What respiratory causes are there for a blue baby?
- IRDS
- Birth asphyxia/injury/haemorrhage
- Transient tachypnoea of the newborn
- Pneumothorax
- Meconium aspiration
- Pulmonary oedema
- CDH
- Pleural effusion
- URT obstruction
A newborn is centrally cyanosed. How quickly should this clear after birth?
Within a few minutes.
A child is blue, and not because they’re dressing up as a smurf.
What assessment should we do?
ABCDE - look for cardiac or respiratory causes as you go, as well as other potential causes.
A child is blue, and not because they’re dressing up as a smurf.
What initial management can we do after A to E?
- Bloods - FBC, U and Es, ABG, glucose, cultures.
- Urine dip/culture
- Lumbar puncture if indicated
- ECG
- Echocardiogram
- CXR
How does heart failure manifest in a child?
Signs of poor tissue perfusion (Fatigue, poor exercise tolerance, confusion)
and/or
Signs of congestion (SoB, pleural effusion, hepatomegaly, oedema)
What are the 4 categories of pathologies underlying paediatric heart failure?
- Increased afterload
- Increased preload
- Myocardial abnormalities
- Tachyrrythmias
A child presents with symptoms of heart failure.
What broad differential should be top of our list?
Congenital structural defect
What is a hypoplastic left heart?
Where the left side of the heart does no form properly.
This results in an aortic arch being present but the left ventricle, and aortic and mitral valves are atresic or very small.
What causes of myocardial ischaemia are there in children?
- Kawasaki disease
- Anomalous left coronary artery
What common murmur can be heard best at the lower sternal edge in a child?
VSD
What common murmur can be heard best at the upper sternal edge in a child?
Aortic or pulmonary stenosis
What common murmur can be heard best at the base of the neck in a child?
Aortic valve lesion
How do we classify the volume of a heart murmur?
Out of 6:
1- quiet murmur, not always audible
2- clearly audible but quiet murmur
3- loud murmur, no palpable thrill
4- loud murmur with palpable thrill
5- loud murmur with palpable thrill (can hear with only rim of stethoscope)
6- loud murmur with palpable thrill (can hear with stethoscope just lifted off chest)
What is a cyanotic spell?
A period of time where a child becomes blue around the lips/mouth/nail beds
Which 2 cardiac conditions are associated most with cyanotic spells?
- Tetralogy of Fallot
- Pulmonary atresia
When do most cyanotic spells occur?
Early in the morning, or in the context of stress or dehydration
A child presents with cyanotic spells.
What should form part of your initial assessment?
- Severity of cyanosis/pallor
- Distress (resp) - what could be causing it (pain?)
- Dehydration
- ?structural heart disease
- ?heart murmur
Full Hx and Examination
How do you manage cyanotic spells initially?
- Knee to chest position
- High flow O2
- Avoid stress/making stress worse
- Morphine I.M.
- ECG monitoring (cont)
- Obs
Find they underlying cause.
What are the 4 elements of tetralogy of Fallot?
- Large VSD
- Overriding aorta
- RV outflow obstruction
- RV hypertrophy
What does the degree of cyanosis depend on in tetralogy of Fallot?
Degree of RV outflow obstruction
An infant presents with episodic cyanotic spells, and clubbing of her fingers.
What is the top differential?
Tetralogy of Fallot
What is the early management of tetralogy of Fallot?
If severe, prostaglandin E infusion and surgery to insert a shunt.
What is the definitive management of tetralogy of Fallot?
Surgery carried out from 4 months of age onwards
What is the prognosis for treated ToF?
Good - unrestricted lives, although may need pulmonary valve replacement in teenage years.
May have conductive defects post-op.
When does the ductus arteriosus close?
After roughly 2-3 days of life (although can take a few days longer)
How does pulmonary blood flow increase after birth?
In utero, pulomary vascular resistance is high.
After birth when the lungs inflate and expel the fluid within them, pulmonary vasculature resistance decreases so blood flow can increase through the lungs.
What compound is responsible for closing the ductus arteriosus?
How can we use this to our advantage?
Prostaglandins (lack of/lower levels of).
These can be given to a newborn with cyanotic heart disease to maintain the patency of the ductus arteriosus so mixing can occur.
When do most cases of CHD get picked up?
Antenatal screening picks up ~70% of cases of CHD
What are the 5 major ways in which CHD can present?
- Cyanosis
- Heart failure
- Murmur
- Collapse
- Oxygen saturation screening
What other broad differentials should be on your mind when considering CHD?
Why?
Sepsis and respiratory disease.
It is often hard to differentiate them in a newborn.
Which heart defects are associated most with Turner’s syndrome?
-Co-arctation of the aorta
-Biscuspid aortic valve
(-Mitral stenosis)
Which heart defects are associated most with Down’s syndrome?
- AVSD
- Tetralogy of Fallot
Which heart defects are associated most with Edward syndrome?
VSD
Which heart defects are associated most with Patau syndrome?
VSD
Which heart defects are associated most with DiGeorge syndrome?
- Truncus arteriosus
- Tetralogy of Fallot
- Interrupted aortic arch
Which heart defects are associated most with Williams syndrome?
- Pulmonary stenosis
- Supravalvular aortic stenosis
Which heart defects are associated most with Noonan syndrome?
- Pulmonary stenosis
- ASD
- Hypertrophic cardiomyopathy
Which heart defects are associated most with Foetal alcohol syndrome?
VSD
After a full hx and examination of a child with suspected CHD, what investigations would you want to request?
- Chest X-ray
- ECG
- Echo
- Consider genetic testing if indicated
What would a boot shaped heart indicate if it was seen on a CXR of a child with suspected CHD?
Tetralogy of Fallot
What would an “egg on side” heart indicate if it was seen on a CXR of a child with suspected CHD?
Transposition of the Great Arteries
Are heart murmurs always pathological in a newborn?
No.
What might an ejection systolic murmur indicate in a newborn?
Aortic stenosis
Co-arctation of the aorta
What might a pansystolic murmur indicate in a newborn?
A/V SD
What part of a cardiovascular exam am I prone to missing out that must not be missed out, and might help differentiate one CHD from another?
Feeling the femoral pulses - are they strong, is there delay, are they symmetrical?
How can we differentiate between cardiac and non-cardiac causes of unresolved cyanosis in a newborn?
Nitrogen washout test.
Give 100% oxygen for 10 mins, then do an ABG.
If pO2 is less than 15 kPa, cyanotic congenital heart disease is indicated.
What kind of defect is a PDA?
Patent ductus arteriosus is a form of acyanotic congenital heart defect.
What happens if PDA does not resolve?
Eventually results in late cyanosis in lower extremities.
Who is PDA more common in?
Premature babies
Babies born at hgh altitude
After maternal rubella infection in the first trimester
You come to do an examination on a newborn who started to become peripherally cyanosed a few days after birth. What do you expect to find O/E if PDA is present?
- L subclavicular thrill
- Continuous machinery murmur
- Pulse has large volume, bounding, and collapsing.
- Heaving apex beat
How do we manage a PDA?
Indomethacin = prostaglandin synthesis inhibitor, unless another heart defect which requires the duct to be open for survival is present.
Why is it important to close a PDA if it doesn’t close on its own?
It can increase risk of infective endocarditis
What are the risk factors for having a congenital heart defect?
- First degree relative with congenital heart defect
- Consanguineous unions
- Intrauterine infection/drug/toxins
- Certain genetic conditions
- Maternal DM
How should a murmur or suspected congenital heart defect be investigated first line?
Echocardiography
What are the risks and complications of congenital heart disease?
Infective endocarditis Failure to thrive Paradoxical embolism -> stroke. Pulmonary hypertension Polycythaemia
If a child presents with a murmur, what do we need to ask to help distinguish if the murmur is innocent or pathological?
- Are there any other symptoms/signs of heart disease?
- Are there any predisposing medical conditions?
- What are the characteristics of the murmur?
In an older child, what might indicate heart disease?
- Exercise intolerance
- Palpitations
- Chest pain
- Syncope
- Pedal oedema
- Positive FHx
In a young child/infant, what symptoms might indicate heart disease?
- SoB
- Poor feeding
- Excessive sweating
- Blue episodes
- General unwellness
- Not gaining weight
- Positive FHx
In a young child/infant, what signs might indicate heart disease?
- Tachypnoea
- Tachycardia
- Hepatomegaly
- Poor peripheral pulses
- Low o2 sats
- Faltering growth (chart)
In an older child, what signs might indicate heart disease?
- Tachypnoea
- Tachycardia
- Hepatomegaly
- Poor peripheral pulses
- Elevated JVP
- Pedal oedema
- Basal lung creps
What are the 6 features of a heart murmur that indicate pathology?
- Pansystolic
- Harsh
- Abnormal heart sounds
- Early/mid-systolic click
- Grade 3+
- Heard over upper left sternal border
What are the common paediatric innocent murmurs?
- Still’s
- Pulmonary flow murmur
- Venous hum
- Carotid bruit
- Peripheral pulmonary stenosis
What are the features of Still’s murmur?
Mid-left sternal border, mid-systolic, grade 2-3, twanging string, musical, vibratory sound
What are the features of pulmonary flow murmur?
Upper left sternal border, mid-systolic, grade 1-3, grating
What are the features of venous hum murmur?
Right and/or left infraclavicular, continuous, only heard in upright position, diastolic component louder than systolic
What are the features of carotid bruit?
Supraclavicular area, ejection systolic, grade 2-3
What are the features of peripheral pulmonary stenosis murmur?
Upper left sternal border, grade 1-2, radiates to axillae and back, usually disappears by 6 months of age
What happens in an ASD?
Left to right shunting of blood between the atria.
What are the 2 types of ASD?
- Secundum = 80%, basically a patent foramen ovale
- Partial AVSD = minority, defect is usually at inferior atrial border, ad=nd often involves the tricuspid valve.
How do ASDs commonly present?
- Commonly asymptomatic
- Recurrent chest infections / wheeze
- Heart failure
- Arrythmias – not until >4th decade of life
What signs are present on examination of an ASD?
- Split second heart sound
- Ejection systolic murmur
How should ASD be managed?
- Cardiac catheterisation to close defect of secundum ASD.
- Surger usually required for partial AVSD between age 3 and 5
How are VSDs classified?
Based upon size and proportion of septum involved.
Which way does blood flow in VSD?
From left to right initially, but can become R-> L if pulmonary resistance increases above the level of ystemic resistance.
How does blood flow into the right side of the heart affect the child in VSD?
Increased volume of blood flows out through right outflow tract, causing pulmonary congestion or Eisenmenger’s syndrome.
By which week of development are the ventricles usually divided?
By week 8.
What are the 2 sections of the ventricular septum, and which is where?
Muscular and membranous.
Membranous is the portion closer to the atria.
How common are VSDs?
Most common congenital heart problem, making up 50% of all congenital heart disease.
How do VSDs present?
Varies depending on size, ventricular pressures, and pulmonary resistance.
Usually picked up on antenatal scans.
If a VSD isn’t picked up on antenatal scans, how do they present?
- Small - murmur heard on routine examination
- Moderate - 5-6 weeks feeding difficulties (slows, SoB) so weight gain slows
- Large - severe feeding difficulties and weight stagnation.
How does a VSD present on examination?
- Small - harsh (pan)systolic murmur at left sternal edge
- Moderate - parasternal heave, 5/6 systolic murmur
- Large - may have no murmur.
What evidence of VSD might be seen on an ECG?
- Usually normal with small VSD
- LV hypertrophy can be seen in larger lesions
- R axis deviation and RVH may be seen
How do we manage VSD medically?
- Depends on symptoms
- If in HF, diuretics and high energy feeds necessary.
- ACEIs can reduced afterlod
How do we manage VSD surgically?
Surigcal repair if uncontrolled HF or poor growth.
If risk of other problems e.g. aortic valve prolapse because of location, close surgically.
How are most VSDs repaired surgically?
Patch from the right ventricular side.
What complications can occur due to VSDs?
- Aortic valve prolapse
- Aortic regurg
- Outflow tract obstruction
- Eisenmenger’s syndrome
- Bacterial endocarditis is untreated
What is transposition of the great arteries?
Congenital heart condition in which the aorta and pulmonary artery arise from the opposite sides if the heart to usual i.e. the aorto from the right ventricle, and the pulmonary artery fro the left ventricle.
What does survival of the infant depend on in transposition of the great arteries?
Mixing of blood through shunts e.g. ASD/VSD/PDA
Is transposition of the great arteries cyanotic or acyanotic?
Cyanotic
How soon after birth does the cyanosis associated with transposition of the great arteries appear?
Within the first week of life, uness there is a large lesion allowing mixing
What happens to an infant with transposition of the great arteries if they aren’t treated?
They develop metabolic acidosis and become severely ill
When is TGA usually diagnosed?
Antenatal scans
What is the classic finding on CXR for TGA?
Egg on a string appearance with increased vascular lung markings
What is the best way to diagnose a congenital heart defect after birth?
Echocardiography
How should TGA be managed?
- Maintain ductal patency with prostaglandins
- Transfer to cardiac centre for urgent atrial spetostomy to buy time
- Definitive corrective procedure = arterial switch procedure at 3 days of age ideally.
