1. Paediatric Cardiology
Abhishek Oswal

2. Syllabus
Age related changes in heart rate and blood pressure: know approximate values for infants and toddlers
Innocent murmurs: definition and how to distinguish from pathological murmurs
Heart failure: symptoms and signs in infants and children, possible causes
Common forms of congenital heart disease: natural history and management of 
Acyanotic: ASD, VSD (small, medium and large), PDA, coarctation
Cyanotic: Fallot's, transposition
Infective endocarditis: which children are at risk, preventative measures

3. Vital signs

4. Innocent Murmurs Characteristics Examples Soft Systolic aSymptomatic
left Sternal edge
No thrills
No radiation
Otherwise normal heart sounds, no S3/S4
Examples
Still’s murmur – musical, vibratory
Venous hum – through cardiac cycle
Carotid flow murmur – in the neck

5. Heart Failure Varied presentation Dependent on age Clinical signs:
Newborn – cyanosis, circulatory collapse, may need NICU
Infant – FTT, sweating, SOB (particularly on feeding)
Child – recurrent chest infections, palpitations, syncope, exercise limitation
Clinical signs:
Tachypnoea, Tachycardia
Cool peripheries
Cardiomegaly
Heart murmur, gallop rhythm
Hepatomegaly

6. Causes of HF Newborn – obstructed outflow tract e.g.
Preductal CoA
Critical aortic stenosis
Infant – high pulmonary flow e.g.
VSD
Large PDA
Older child – right or left or congestive cardiac failure e.g.
Eisenmenger
Cardiomyopathy

7. Normal (adult) circulation
From body
From lungs RA RV LA LV
To lungs
To body

8. Defects Acyanotic Cyanotic Atrial septal defect (ASD)
Ventricular septal defect (VSD)
Persistent ductus arteriosus (PDA)
Coarctation of the aorta (CoA)
Cyanotic
Transposition of the great arteries (TGA)
Tetralogy of Fallot (ToF)

9. Things to know for each one
Pink or blue?
Presentation
Murmur
Other clinical signs
ECG
CXR
Management

10. Atrial septal defect
From body
From lungs LA LV RA RV
To lungs
To body

11. Atrial septal defect Pink
Presentation (often in adulthood): asymptomatic, syncope palpitations, decreased exercise tolerance
20% of the population
Murmur
Soft systolic murmur ULSE
+ (pan systolic murmur if AVSD affecting AV valves)
Fixed split S2
ECG
Partial RBBB (RSR’ in V1) in 90% of ASD
RAD/RVH
Right atrial enlargement - Tall P waves
CXR
Increased pulmonary vascular markings Rx
Conservative if asymptomatic
Closure age 3-5 (before starting school)
Cath lab device closure for 80%
Some may require surgical closure if associated with other defects or too large for device

12. Ventricular Septal Defect
From body
From lungs RA RV LA LV
To lungs
To body

13. Ventricular septal defect
Pink
Presentation: Asymptomatic adult  heart failure in an infant
Murmur
Small: blowing pan systolic murmur LLSE, may be associated with a thrill
Large: ?above + rumbling mid diastolic murmur, loud P2, RV heave
ECG
Large: biventricular hypertrophy by age 2/12, peaked P waves (LA hypertrophy)
CXR
Large: increased pulmonary vascular markings, cardiomegaly, ?pulmonary oedema Rx
Conservative if asymptomatic. Many close spontaneously before 2 years of age
Large: diuretics, captopril, calories so ready for surgical closure at 3-5/12

14. (Eisenmenger syndrome)
From body
From lungs RA RV LA LV
To lungs
To body

15. (Eisenmenger syndrome)
Irreversible conversion of a LR shunt disease into a RL shunt, cyanotic disease due to pulmonary hypertension and RV hypertrophy
Blue
Presentation: cyanosed teen/early 20s, SOB, reduced exercise tolerance, fatigue, HF, erythrocytosis
CV exam
Clubbing
RV heave, Raised JVP
PSM of VSD disappears as the shunt reverses. May have early diastolic murmur of pulmonary regurgitation
ECG
Right heart hypertrophy (right axis deviation)
CXR
Dilated pulmonary arteries, normal pulmonary vascular markings, normal heart size Rx
Supportive therapy, night time O2
Pulmonary vasodilators e.g. prostaglandin analogues, endothelin antagonists, sildenafil
Only definitive is heart lung transplant

16. Persistent ductus arteriosus
From body
From lungs RA RV LA LV
To lungs
To body

17. Persistent ductus arteriosus
Pink
Presentation: asymptomatic, recurrent LRTI, FTT (rarely HF)
CV exam
Continuous machinery murmur left infraclavicular region
Bounding pulses
ECG
Normal, LVH
CXR
Normal, increased pulmonary markings Rx
Conservative – wait and let it close
Medical – NSAIDs e.g. indomethacin, aspirin
Small – cath lab closure with coil/plug at 1 year
Large – surgical ligation age 1-3mo

18. Coarctation of the aorta
From body
From lungs
From body
From lungs RA RV LA LV RA RV LA LV
To lungs
To lungs
To body
To body

19. Coarctation of the aorta (post ductal)
Pink
Adult type (rare)
Presentation: asymptomatic  progressive hypertension, resistant to drugs
CV exam
Ejection systolic murmur, upper sternal edge
Continuous rumbling murmur on auscultation of the back
Radiofemoral delay
Upper limb hypertension, lower limb hypotension (difference >20mmHg)
ECG
LVH
CXR
rib notching
3 sign – visible notch of coarctation in descending aorta Rx
Cath lab angioplasty/stenting age 3-5 if detected early and no HF as reduces long term risk of HTN
Surgical repair in adulthood if detected late

20. Coarctation of the aorta (pre ductal)
Emergency!
Blue  grey
Presentation
May be detected antenatally
Collapsed, cyanosed, shocked within the first 2 weeks of life
Absent femoral pulses
O2 sat of right arm >> left arm >> feet
Murmur
None
ECG + CXR: likely normal, ?cardiomegaly + HF Rx
Admit to NICU for ?ventilation, PGE1 infusion, transfer to cardiac centre
Surgery within 24hr to resect coarcted segment

21. Any questions so far?

22. Transposition of the great arteries
From body
From lungs RA RV LA LV
To body
To lungs

23. Transposition of the great arteries
Emergency!
Blue
Presentation
Should be diagnosed antenatally
Collapsed, shocked, very blue
Murmur
None
Single loud S2
ECG
Normal
CXR
Egg on side
Increased pulmonary vascular markings Rx
Antibiotics + PGE2 infusion
Catheter atrial septostomy
Corrective surgery at 1-2/52

24. Tetralogy of Fallot From body From lungs RA RV LA LV From body
To lungs
To body
From lungs RA RV LA LV
To lungs
To body

25. Tetralogy of Fallot
Malformation of the outflow tract septum leading to
VSD
Overriding aorta
Pulmonary stenosis
Right ventricular hypertrophy
Blue
Presentation
Antenatal detection
Severe cyanosis at birth
Older children with reduced exercise tolerance, squatting on exercise
Tet (hypercyanotic) spells

26. Tetralogy of Fallot Murmur ECG CXR Rx
Harsh ejection systolic murmur ULSE
ECG
Normal at birth
RVH when older
CXR
Small, boot shaped heart
Pulmonary bay (reduced flow through pulmonary artery)
Reduced pulmonary vasculature Rx
Surgical (BT) shunt in neonate if severely cyanosed
Aim for elective repair at 6-9/12
Tet spells – if >15min need: intubation + ventilation, fluids, IV pain relief, beta blocker

27. Summary flow diagram Cyanotic? Yes Murmur? ToF No Femoral pulses? TGA
CoA (pre-ductal)
PSM LLSE (± thrill)
VSD
ESM ULSE + fixed split S2
ASD
Continuous machinery
PDA
(UL vs LL BP)
ESM  back
CoA (post-ductal)

28. Syndromic associations
Down’s
VSD/AVSD (30%)
Turner’s
Bicuspid aortic valve, Coarctation
Fragile X
Mitral valve prolapse, mitral regurgitation, aortic regurgitation
Williams’
Supravalvular aortic/pulmonary stenosis

29. Infective Endocarditis
At risk?
Any congenital heart defects, particularly CoA, VSD, PDA
Any surgical implants, coils, devices, replacement valves
Consider in anyone with a persistent fever/ESR
Preventative measures
Good dental hygiene
Avoid piercings/tattoos/IVDU
No need for prophylactic Abx in dental/any other surgical procedures

30. MCQ 1
An uncomplicated VSD in a 5-year-old boy may be associated with which one of the following?
A. A collapsing pulse
B. Wide and fixed splitting of the second heart sound
C. Clubbing of the fingers
D. A pansystolic murmur of grade 4/6 in intensity
E. Splenomegaly

31. MCQ 2
A four year old child is found to have a continuous machinery murmur heard loudest below the left clavicle. He is underweight for age but otherwise well. Considering the likely diagnosis, which of the following would you recommend for this patient?
A. Recommend early operative closure
B. Review the child constantly, expecting spontaneous closure within the next five years
C. Recommend prophylactic penicillin until operation is performed
D. Delay operation until the child has reached its expected weight for age
E. Explain to the parents that this is of little significance and can be ignored

32. MCQ 3
A previously well 3-day-old becomes unresponsive and dusky on the neonatal unit. A CXR shows an “egg on side” appearance of the heart. Which of the following 3 are correct?
A. Femoral pulses may be palpable
B. ECG will show RV hypertrophy
C. There is a loud, single second heart sound
D. There is a ejection systolic murmur at the ULSE
E. Give an immediate prostaglandin infusion

33. Any questions?

34. References
RCH, Melbourne heart defects:
Lissauer’s Illustrated textbook of Paediatrics
Beattie, Champion. Essential revision notes in Paediatrics for the MRCPCH
Medscape