Download Cardiology - Oxford Society of Paediatrics

Paediatric Cardiology
Abhishek Oswal
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
Vital signs
Innocent Murmurs
• Characteristics
•
•
•
•
•
•
•
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
Heart Failure
• Varied presentation
• Dependent on age
• 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
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
Normal (adult) circulation
From lungs
From body
RA
LA
RV
LV
To lungs
To body
Defects
• Acyanotic
•
•
•
•
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)
Things to know for each one
• Pink or blue?
• Presentation
• Murmur
• Other clinical signs
• ECG
• CXR
• Management
Atrial septal defect
From lungs
From body
RA
LA
RV
LV
To lungs
To body
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
Ventricular Septal Defect
From lungs
From body
RA
LA
RV
LV
To lungs
To body
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
(Eisenmenger syndrome)
From lungs
From body
RA
LA
RV
LV
To lungs
To body
(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
Persistent ductus arteriosus
From body
From lungs
RA
LA
RV
LV
To lungs
To body
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
Coarctation of the aorta
From lungs
From body
RA
From lungs
From body
RA
LA
RV
LV
LA
LV
RV
To lungs
To body
To lungs
To body
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
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
• Emergency!
• Admit to NICU for ?ventilation, PGE1 infusion, transfer to cardiac centre
• Surgery within 24hr to resect coarcted segment
Any questions so far?
Transposition of the great arteries
From lungs
From body
RA
LA
RV
LV
To body
To lungs
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
Tetralogy of Fallot
From lungs
From body
From lungs
From body
RA
LA
RA
LA
RV
LV
RV
LV
To lungs
To body
To lungs
To body
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
Tetralogy of Fallot
• Murmur
• 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
Summary flow diagram
Cyanotic?
Yes
No
Murmur?
Murmur?
Yes
No
PSM LLSE (±
thrill)
ESM ULSE +
fixed split S2
Continuous
machinery
VSD
ASD
PDA
Femoral
pulses?
ToF
Yes
TGA
No
CoA (preductal)
ESM  back
(UL vs LL BP)
CoA (postductal)
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
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
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
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
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
Any questions?
References
• RCH, Melbourne heart defects:
http://www.rch.org.au/cardiology/heart_defects/
• Lissauer’s Illustrated textbook of Paediatrics
• Beattie, Champion. Essential revision notes in Paediatrics for the
MRCPCH
• Medscape