MRCPCH l Questíons

wíth lndívíduuí
Sub¡ett Summuríes
lourth Ldítíon
R Mark ßeattie
8Sc M88S PPCPCH MPCP
Sarah A L WiIIiams
MA M8 Ch8 MPCPCH
1oanne ßorbone
M8 Ch8 MPCPCH
ll
ª 20ll PASTLST LTD
Lgerton Court
Parkgate Lstate
Knutsford
Cheshlre
wAl6 8DX
Telephone: 0l565 752000
All rlghts reserved. No part of thls publlcatlon may be reproduced, stored ln a retrleval
system, or transmltted, ln any form or by any means, electronlc, mechanlcal, photocopylng,
recordlng or otherwlse wlthout the prlor permlsslon of the copyrlght owner.
Plrst publlshed l997
Second edltlon 2000
Thlrd edltlon 2006, Peprlnted 20l0
Pourth edltlon 20ll
|S8N: l905635745
978l905635740
A catalogue record for thls book ls avallable from the 8rltlsh Llbrary.
The lnformatlon contalned wlthln thls book was obtalned by the author from rellable
sources. However, whlle every enort has been made to ensure lts accuracy, no
responslblllty for loss, damage or ln[ury occasloned to any person actlng or refralnlng from
actlon as a result of lnformatlon contalned hereln can be accepted by the publlshers or
author.
PasTest Pevlslon 8ooks and |ntenslve Courses
PasTest has been establlshed ln the ñeld of undergraduate and postgraduate medlcal
educatlon slnce l972, provldlng revlslon books and lntenslve study courses for doctors
preparlng for thelr professlonal examlnatlons.
8ooks, courses and onllne revlslon avallable for:
Medlcal undergraduates, MPCGP, MPCP Parts l and 2, MPCPCH Parts l and 2, MPCS,
MPCOG, DPCOG, DCH, PPCA, Dentlstry.
Por further detalls contact:
PasTest, Preepost, Knutsford, Cheshlre wAl6 78P
7eI: 01565 752000 Fax: 01565 650264
www.pastest.co.uk enquiries@pastest.co.uk
Text prepared ln the UK by Carnegle 8ook Productlon, Lancaster
Prlnted and bound ln the UK by CP| Antony Powe, Chlppenham, wlltshlre
lll
Contents
Contrlbutors lv
|ntroductlon v
QULST|ONS
Chapter l. Cardlology l
Chapter 2. Communlty Paedlatrlcs and Chlld Psychlatry 26
Chapter 3. Dermatology 42
Chapter 4. Lndocrlnology and Syndromes 45
Chapter 5. Gastroenterology and Nutrltlon 59
Chapter 6. Haematology, Oncology and Polsonlng 79
Chapter 7. |nfectlous Dlseases and |mmunology 90
Chapter 8. Neonatology l07
Chapter 9. Nephrology l25
Chapter l0. Neurology l44
Chapter ll. Pesplratory l62
ANSwLPS AND SU81LCT SUMMAP|LS
Chapter l. Cardlology l83
Chapter 2. Communlty Paedlatrlcs and Chlld Psychlatry 229
Chapter 3. Dermatology 252
Chapter 4. Lndocrlnology and Syndromes 257
Chapter 5. Gastroenterology and Nutrltlon 286
Chapter 6. Haematology, Oncology and Polsonlng 328
Chapter 7. |nfectlous Dlseases and |mmunology 352
Chapter 8. Neonatology 396
Chapter 9. Nephrology 440
Chapter l0. Neurology 48l
Chapter ll. Pesplratory 5l4
|ndex 547
lv
Contríbutors to
lourth Ldítíon
Dr R Mark ßeattie
8Sc M88S PPCPCH MPCP
Consultant Paedlatrlc Gastroenterologlst, Paedlatrlc Medlcal Unlt, Southampton General
Hospltal, Southampton
Dr Sarah A L WiIIiams
MA M8 Ch8 MPCPCH
SpP Paedlatrlc Gastroenterology, Paedlatrlc Gastroenterology Department, Southampton
General Hospltal, Southampton
Dr 1oanne ßorbone
M8 Ch8 MPCPCH
SpP Paedlatrlcs, Southampton General Hospltal, Southampton
Dr ReyneIIa Anne Morenas
M88S 8Sc MPCPCH
SpP Paedlatrlcs, Poyal Hampshlre County Hospltal, Hampshlre
Dr HeIen C FieIder
M88S MPCPCH
Consultant Neonatologlst, Prlncess Anne Hospltal, Southampton
The publlshers and Authors would also llke to thank the followlng contrlbutors to the
prevlous edltlons for thelr contrlbutlon:
Dr Nick ßrown
Consultant Paedlatrlclan, Sallsbury Dlstrlct Hospltal, wlltshlre
Dr 7racey Farnon
Consultant Paedlatrlclan, Sallsbury Dlstrlct Hospltal, wlltshlre
Dr A ß Acharya
Senlor Peglstrar, Peterborough Dlstrlct Hospltal, Peterborough
Dr DL I £IIis
Senlor House Omcer, Peterborough Dlstrlct Hospltal, Peterborough
Dr L 1 PhiIIips
Senlor House Omcer, Peterborough Dlstrlct Hospltal, Peterborough
Dr A 7 7idsweII
Senlor House Omcer, Peterborough Dlstrlct Hospltal, Peterborough
v
lntroduttíon
we hope that thls text wlll prove lnvaluable ln your preparatlon for MPCPCH Part l or
Dlploma ln Chlld Health. we have personally found earller edltlons of thls book essentlal ln
the preparatlons for both of these quallñcatlons.
The format of the questlons ln thls edltlon has been totally revlsed ln accordance wlth the
structure of the new style examlnatlon, wlth a hlgher percentage of LMQs (extended
matchlng questlons) and 8OPs (best of ñves) whllst updatlng the multlple cholce questlons
to renect the dlstrlbutlon of questlon types you wlll encounter ln both Paper lA and l8. All
the materlal has been updated and over 20% of the questlons are entlrely new to thls
edltlon. All the sub[ect summarles are succlnct but comprehenslve overvlews of the areas
essentlal to cover ln preparatlon for the exam as well as cllnlcally relevant for all doctors
worklng ln paedlatrlcs today.
we would llke to acknowledge the many authors who have enthuslastlcally contrlbuted to
thls book over lts number of edltlons and pay speclal thanks to the slgnlñcant contrlbutlon
made ln thls edltlon by Peynella Morenas and Helen Plelder as well as Llly Martln at PasTest
for her patlence and expertlse ln helplng pull thls new edltlon together.
we hope that you wlll ñnd thls text [ust as lnvaluable ln your preparatlon as we dld ln the
past and wlsh you ever success ln both your examlnatlons and future careers.
Mark 8eattle
Sarah wllllams
1oanne 8orbone
October 20ll
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Curdíoíogy
Muítípíe 1rueluíse Questíons
1.1 Which of the foIIowing conditions can present in the neonataI period with
centraI cyanosis!
‰ A Llsenmenger syndrome
‰ 8 Pulmonary atresla
‰ C Hypoplastlc left heart
‰ D Transposltlon of the great arterles
‰ L Crltlcal aortlc stenosls
1.2 With regard to the paediatric £CC
‰ A Neonates have rlght axls devlatlon
‰ 8 Congenltal complete heart block ls assoclated wlth maternal systemlc lupus
erythematosus
‰ C Plght bundle-branch block ls seen ln coarctatlon of the aorta
‰ D Pomano-ward syndrome produces prolongatlon of the PP lnterval
‰ L A restlng heart rate ln newborns of l80 ls a tachycardla
1.3 Which of the foIIowing chest radiograph ñndings wouId support the paired
diagnosis!
‰ A Atrloseptal defect and coeur en sabot (heart ln a boot)
‰ 8 Patent ductus arterlosus (PDA) and rlb notchlng
‰ C Truncus arterlosus and absent thymus
‰ D Total anomalous pulmonary venous dralnage and cottage loaf appearance
‰ L Sclmltar syndrome and dextrocardla
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1.4 Which of the foIIowing conditions are associated with an increased
incidence of heart disease!
‰ A Kawasakl dlsease
‰ 8 Congenltal rubella
‰ C Marfan syndrome
‰ D Turner syndrome
‰ L Petlt mal epllepsy
1.5 Which of the foIIowing statements are true!
‰ A The most common congenltal cardlac leslon ls an ASvD
‰ 8 Tetralogy of Pallot ls assoclated wlth plethorlc lung ñelds
‰ C The 8lalock-Tausslg shunt glves rlse to a contlnuous murmur
‰ D Nltrous oxlde can be used to treat perslstent pulmonary hypertenslon
‰ L Lbsteln anomaly ls an anomaly of the trlcuspld valve
1.6 Concerning the fetaI circuIation
‰ A The umblllcal veln carrles deoxygenated blood to the placenta from the fetus
‰ 8 Petal superlor vena caval blood preferentlally nows across the foramen ovale lnto
the left atrlum
‰ C Porty per cent of rlght ventrlcular outnow enters the lungs vla the pulmonary
arterles
‰ D The descendlng aorta ls connected to the pulmonary artery vla the ductus
venosus
‰ L Gas exchange occurs ln the fetal lungs
1.7 Which of the foIIowing statements are true!
‰ A Patlents wlth cyanotlc congenltal heart dlsease grow normally
‰ 8 |mmunlsatlon ls contralndlcated ln chlldren wlth congenltal heart dlsease
‰ C The comblned oral contraceptlve plll ls contralndlcated ln patlents wlth cyanotlc
congenltal heart dlsease
‰ D Two per cent of patlents wlth congenltal heart dlsease have a chromosomal
abnormallty
‰ L Thlrty per cent of lnfants wlth chromosomal defects have heart defects
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1.8 Which of the foIIowing cardiac defects and teratogens are correctIy paired!
‰ A Alcohol and transposltlon of the great arterles
‰ 8 Sodlum valproate and tetralogy of Pallot
‰ C Purosemlde and PDA
‰ D Phenytoln and coarctatlon of the aorta
‰ L Llthlum and Lbsteln anomaly
1.9 Which of the foIIowing are causes of circuIatory faiIure in the ñrst week of
Iife!
‰ A Arrhythmlas
‰ 8 Hypoplastlc left heart
‰ C 8lrth asphyxla
‰ D Severe anaemla
‰ L Pluld overload
ßest ol líve Questíons
1.10 With regard to advanced Iife support and resuscitation in chiIdren with
cardiac arrest, which of the foIIowing statements is most factuaIIy correct!
‰ A 8aslc llfe support wlth hlgh concentratlon oxygen should be contlnued
throughout any resuscltatlon wlth the mlnlmum of lnterruptlons
‰ 8 ventrlcular ñbrlllatlon ls the most common cardlac arrest rhythm ln chlldren
‰ C |t ls lmportant that a chlld's alrway ls secured durlng an arrest sltuatlon, so lt ls
acceptable for baslc llfe support to be lnterrupted for as long as lt takes to
lntubate the chlld
‰ D The resuscltatlon dose of adrenallne ls 0.l Pg/kg and should be admlnlstered at
4-mlnute lntervals ln asystollc arrests wlth the thlrd dose belng glven as a hlgher
dose of l mg/kg lf no response ls seen
‰ L |n a paedlatrlc arrest assoclated wlth PLA a 2 1/kg synchronlsed DC shock should
be glven every 2 mlnutes whlle reverslble causes are belng sought and treated
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1.11 A 14-year-oId girI is referred to the paediatric outpatient cIinic with a
4-month history of paIpitations.
She ls normally ñt and well and has not experlenced any recent lllness or welght
loss. She ls ln her ñnal year at an all glrls' prlvate school but ls generally achlevlng
good grades ln all her classes. She descrlbes her symptoms as a raclng heart rate
that comes on gradually and wlll slowly decrease back to normal after several hours.
She has not had assoclated chest paln or collapses and her symptoms usually
present durlng school hours. She has had no eplsodes assoclated wlth exerclse or
durlng her tlme at home. She has a sensatlon of dlmculty breathlng durlng the
attacks and has descrlbed 'plns and needles' ln her hands durlng her eplsodes. She
has no slgnlñcant past medlcal hlstory of cardlac dlsease but does have mlld
asthma. She has no famlly hlstory of heart dlsease, sudden death or deafness. She
had an entlrely normal examlnatlon lncludlng a normal pulse rate (70 beats/mln
regular), blood pressure and l2-lead LCGln cllnlc were wlthln normal llmlts.
Which of the foIIowing outcomes fromthis consuItation is the most
appropriate!
‰ A Peferral to paedlatrlc cardlologlst for further lnvestlgatlon of cardlac arrhythmla
‰ 8 P8C, U&Ls, TPTs and 24-hour urlnary catecholamlne collectlon wlth follow-up ln
general paedlatrlc cllnlc to exclude medlcal causes for her palpltatlons
‰ C Peassurance that her palpltatlons donot have a slgnlñcant cause, advlce about
relaxatlonandstress control durlngexamseason, anddlscharge tofollow-upfromcllnlc
‰ D Peferral to chlld and adolescent psychlatry for treatment of anxlety dlsorder
‰ L |nltlatlon of propranolol treatment for panlc attacks
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1.12 An 18-month-oId girI is admitted to the paediatric assessment unit with a
3-week history of worsening shortness of breath and dimcuIty feeding
accompanied by weight Ioss.
She had had symptoms of a cold and mlld gastroenterltls a week before the onset
of symptoms from whlch she had lnltlally appeared to recover. She had become
lncreaslngly letharglc, seemed unsettled and had lncreased work of breathlng
when lald nat, relleved by belng ln an uprlght posltlon or sleeplng ln a propped-
up posltlon ln her buggy. She had prevlously been a well, thrlvlng chlld wlth no
slgnlñcant past medlcal hlstory. On examlnatlon she had a low-grade fever of
37.8ºC and a heart rate of l90 beats/mln whlle settled. She had lncreased work of
breathlng wlth blbasllar crepltatlons. She had a resplratory rate of 50 beats/mln
wlth mlld lntercostal recesslon and normal oxygen saturatlons of 98% ln alr. On
auscultatlon of her heart no murmurs were heard but she was thought to have a
gallop rhythm. On examlnatlon of her abdomen a 4 cm palpable llver was found.
Fromthe foIIowing Iist choose the most IikeIy expIanation for her current
symptoms.
‰ A Supraventrlcular tachycardla
‰ 8 Pneumonla wlth hyperlnnatlon of her thorax
‰ C vlral myocardltls
‰ D Large vSD wlth cardlac fallure
‰ L Cystlc ñbrosls
1.13 Which of the foIIowing statements about £CCchanges associated with
eIectroIyte abnormaIities is most factuaIIy correct!
‰ A LCG changes assoclated wlth hyperkalaemla are progresslve wlth the severlty of
hyperkalaemla leadlng to the absence of p waves, broadenlng of QPS complexes,
and vP or vT
‰ 8 Hyperkalaemla ls assoclated wlth lncreased helght of U waves
‰ C LCG changes seen ln hypocalcaemla mlmlc those seen ln hypokalaemla
‰ D Hypernatraemla ls assoclated wlth reduced QT lnterval on LCG
‰ L Hypocalcaemla ls assoclated wlth a shortened QTc and ln severe cases sudden
death
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1.14 With regard to aortic stenosis, which of the foIIowing statements is most
factuaIIy correct!
‰ A Aortlc stenosls ls the most common cardlac leslon assoclated wlth Down
syndrome
‰ 8 Plght ventrlcular hypertrophy ls a common ñndlng ln a chlld wlth aortlc stenosls
‰ C An e[ectlon cllck on auscultatlon suggests that the stenosls ls supravalvular
‰ D The most common treatment for aortlc stenosls ln chlldhood ls aortlc valve
replacement
‰ L There ls an assoclatlon wlth sudden death ln patlents wlth aortlc stenosls
1.15 Which of the foIIowing statements about WiIIiams syndrome is most
factuaIIy correct!
‰ A The most common cardlac leslon assoclated wlth wllllams syndrome ls an
atrloseptal defect
‰ 8 wllllams syndrome ls caused by a mlcrodeletlon on chromosome ll, whlch
encodes for the elastln gene
‰ C Dlagnosls of wllllams syndrome can be conñrmed by nuorescence |n s|tu
hybrldlzatlon (P|SH) studles
‰ D wllllams syndrome ls assoclated wlth normal development and |Q
‰ L Hypercalcaemla ls requlred to make the dlagnosls of wllllams syndrome ln a
neonate
1.16 Which of the foIIowing statements about cardiovascuIar disease associated
with Down syndrome is most factuaIIy correct!
‰ A The most common cardlac leslon assoclated wlth Down syndrome ls an atrlal
septal defect
‰ 8 Lchocardlography ls lndlcated ln all cases of Down syndrome for ldentlñcatlon of
congenltal cardlac dlsease even lf a murmur ls not present
‰ C Up to 20% of all cases of Down syndrome have assoclated heart defects
‰ D Cardlac dlsease ln Down syndrome becomes symptomatlc earller than lsolated
congenltal heart dlsease due to the hlgh prevalence of pulmonary arterlal
hypertenslon
‰ L The cardlac defects assoclated wlth Down syndrome are the sole cause of
pulmonary vascular dlsease ln thls populatlon
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1.17 Which of the foIIowing statements concerning transposition of the great
arteries is most factuaIIy correct!
‰ A Transposltlon of the great arterles ls assoclated wlth decreased pulmonary blood
now and pulmonary ollgaemla on chest radlograph
‰ 8 Transposltlon of the great arterles ls the most common cause of cyanotlc
congenltal heart dlsease ln the neonatal perlod
‰ C Transposltlon of the great arterles ls assoclated wlth a metabollc alkalosls at
presentatlon
‰ D The vast ma[orlty of cases of transposltlon of the great arterles are dlagnosed on
routlne antenatal ultrasonography
‰ L Transposltlon of the great arterles occurs together wlth a recognlzed paedlatrlc
syndrome ln 80% of cases
1.18 Which of the foIIowing statements regarding tetraIogy of FaIIot is most
factuaIIy correct!
‰ A The murmur heard ln a patlent wlth tetralogy of Pallot becomes louder durlng
cyanotlc spells
‰ 8 Cyanotlc spells assoclated wlth tetralogy of Pallot usually begln around 4-6
months of age
‰ C An atrlal septal defect ls one of the four ma[or components of tetralogy of Pallot
‰ D Plnger clubblng usually develops wlthln the ñrst few months of llfe
‰ L Most patlents wlth tetralogy of Pallot have a left-to-rlght shunt across the vSD
1.19 Which of the foIIowing statements concerning atriaI septaI defects is most
factuaIIy correct!
‰ A Ostlum prlmum defects are more common than ostlum secundum defects
‰ 8 Atrlal ñbrlllatlon ls a common assoclated compllcatlon ln the ñrst decade of llfe
‰ C |n a secundum defect the LCG shows rlght bundle-branch block and left axls
devlatlon
‰ D |n chlldren wlth ASDs the pulmonary vascular reslstance lncreases ln early
chlldhood, leadlng to pulmonary hypertenslon typlcally ln the ñrst decade of llfe
‰ L A pulmonary to systemlc now ratlo of more than 2:l ls an lndlcatlon for surglcal or
more commonly percutaneous transcatheter devlce closure
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1.20 Which of the foIIowing statements about ventricuIar septaI defects (VSDs) is
most factuaIIy correct!
‰ A vSDs are most commonly located ln the muscular part of the ventrlcular septum
‰ 8 vSDs wlll cause an audlble now murmur across the defect, usually audlble from
blrth
‰ C |nfectlve endocardltls ls a compllcatlon seen ln approxlmately l0% of all chlldren
wlth vSDs
‰ D vSDs are assoclated wlth hlgher oxygen content ln the blood of the rlght ventrlcle
than the rlght atrlum
‰ L vSDs are assoclated wlth rlght ventrlcular volume overload
1.21 Which of the foIIowing interventions wouId produce the best cIinicaI
outcome for a chiId at risk of deveIoping £isenmenger syndrome!
‰ A Surglcal correctlon of a vSD when cardlac catheter demonstrates slgnlñcant
pulmonary hypertenslon
‰ 8 Nocturnal oxygen supplementatlon
‰ C Slldenañl to help control pulmonary hypertenslon
‰ D Correctlon of the vSD before the development of pulmonary hypertenslon
‰ L Total heart and lung transplantatlon
1.22 Which of the foIIowing statements about patent ductus arteriosus is most
factuaIIy correct!
‰ A The lncldence of perslstent ductus arterlosus ls lncreased ln males compared wlth
females
‰ 8 Cardlac catheter coll placement ls the treatment of cholce for symptomatlc lnfants
wlth perslstent ductus arterlosus post term
‰ C PDA can be treated wlth prostaglandlns
‰ D PDA usually closes spontaneously ln term lnfants
‰ L The lncldence of PDA ls lncreased ln preterm lnfants and those wlth perlnatal
hypoxla and/or dlstress
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1.23 Which of the foIIowing statements concerning coarctation of the aorta is
most factuaIIy correct!
‰ A |t ls rare to dlagnose duct-dependent coarctatlon on antenatal ultrasonography
‰ 8 Coarctatlon of the aorta ls assoclated wlth rlb notchlng on chest radlograph ln
lnfancy
‰ C Coarctatlon ls commonly assoclated wlth a trlcuspld aortlc valve
‰ D Coarctatlon of the aorta ls more common ln females
‰ L Adult-type coarctatlon of the aorta ls assoclated wlth blood pressure dlscrepancy
between the upper and lower llmbs
1.24 Which of the foIIowing statements about chiIdhood hypertension is most
factuaIIy correct!
‰ A A systollc pressure greater than the 75th centlle for age and sex ls deñned as
hypertenslon
‰ 8 Prlmary hypertenslon ls more common than secondary hypertenslon ln chlldren
‰ C |n lnfants the most common cause of hypertenslon ls renal parenchymal dlsease
‰ D Chlldren wlth hypertenslon are usually symptomatlc
‰ L More than 25% of chlldren wlth secondary hypertenslon wlll have symptoms of
headache at presentatlon
1.25 Which of the foIIowing statements concerning infective endocarditis is most
factuaIIy correct!
‰ A |nfectlve endocardltls ls assoclated wlth a l3-20% mortallty rate
‰ 8 Antlblotlc prophylaxls ls lndlcated to cover dental procedures ln chlldren wlth
congenltal heart dlsease
‰ C Spllnter haemorrhages ln the nallbeds are an almost unlversal ñndlng
‰ D The dlagnosls of lnfectlve endocardltls ls excluded lf echocardlography ls normal
‰ L The rlght slde of the heart ls most commonly anected
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1.26 Which of the foIIowing statements about cardiomyopathy is the most
factuaIIy correct!
‰ A Hypertrophlc obstructlve cardlomyopathy ls most commonly lnherlted as an
X-llnked dlsorder
‰ 8 Dllated cardlomyopathy ls assoclated wlth doxorublcln toxlclty ln chlldren
‰ C Pestrlctlve cardlomyopathy ls the commonest cardlomyopathy ln chlldhood
‰ D Lndocardlal ñbroelastosls ls a form of hypertrophlc cardlomyopathy
‰ L There ls an lncreased lncldence of dllated cardlomyopathy ln lnfants of mothers
wlth dlabetes
1.27 Which of the foIIowing statements about rheumatic fever is most factuaIIy
correct!
‰ A Pheumatlc fever ls caused by an lnfectlon wlth group A u-haemolytlc
5tteµtococcus specles
‰ 8 The most common cardlac manlfestatlon of rheumatlc fever ls an lsolated
endocardltls, occurrlng ln over 50% of cases
‰ C The most commonly occurrlng ma[or crlterlon for dlagnoslng rheumatlc fever ls
polyarthrltls
‰ D PP prolongatlon on the LCG ls one of the ma[or dlagnostlc crlterla
‰ L Prophylactlc antlblotlcs should be stopped after 3 months
1.28 A 5-year-oId girI is referred to a paediatric outpatient cIinic for assessment
of a murmur heard incidentaIIy when she was reviewed by her CP with a 48-
hour history of fever and cough.
|n cllnlc she ls heard to have a 2/6 systollc murmur, heard loudest ln the left
sternal edge. The murmur ls loudest on lylng nat but ls audlble ln the slttlng
posltlon. She has no symptoms of breathlessness or exerclse lntolerance. She ls
actlve - dances and swlms after school wlth her older slbllngs. There ls no famlly
hlstory of cardlac dlsease and she has never been admltted to hospltal.
What is the most appropriate foIIowup for this young girI with regard to her
murmur!
‰ A |mmedlate referral for paedlatrlc cardlology revlew
‰ 8 LCG and chest radlograph
‰ C Lxerclse stress test
‰ D Outpatlent referral to paedlatrlc cardlology
‰ L Peassurance that the murmur ls lnnocent and dlscharge from follow-up
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1.29 A 3-month-oId chiId presents to the emergency department with a
20-minute history of severe shock - paIe but responsive with a peripheraI
capiIIary reñII time of 4 seconds and a centraI capiIIary reñII time of 3
seconds.
She ls found to have a tachycardla of 290 beats/mln wlth a narrow complex on the
LCG. vascular access ls dlmcult to obtaln. Hlgh-now oxygen has been
admlnlstered by the ambulance.
What is the most appropriate next management step!
‰ A Asynchronous DC shock of 2 1/kg
‰ 8 |ntramuscular ceftrlaxone
‰ C Place an lntraosseous needle and admlnlster adenoslne l00 Pg/kg
‰ D Synchronous shock of l 1/kg
‰ L Amlodarone glven vla an lntraosseous needle
1.30 Which of the foIIowing statements about WoIñ-Parkinson-White syndrome
is most accurate!
‰ A woln-Parklnson-whlte syndrome ls most commonly found ln patlents wlth
congenltal heart dlsease, most commonly wlth corrected transposltlon of the
great arterles
‰ 8 The LCG of a patlent wlth woln-Parklnson-whlte syndrome, when the patlent ls
ln normal rhythm, ls lndlstlngulshable from normal
‰ C Dlgoxln ls useful ln the long-term treatment of patlents wlth woln-Parklnson-
whlte syndrome
‰ D Padlofrequency ablatlon of the accessory pathway ls potentlally curable
‰ L woln-Parklnson-whlte syndrome has an equal sex dlstrlbutlon
1.31 Which of the foIIowing statements about the paediatric £CCis most
accurate!
‰ A The PP lnterval ls prolonged ln hyperkalaemla
‰ 8 The normal PP lnterval ln lnfancy ls 0.2-0.3 s
‰ C |t ls normal to have uprlght T wave ln lead vl untll approxlmately 3 months of age
‰ D A prolonged QTc ls consldered to be > 0.35 s
‰ L The normal range for the axls of a neonatal QPS complex ls l60-2l0º
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1.32 Which of the foIIowing statements about proIonged Ç7 syndrome is most
factuaIIy correct!
‰ A There ls a slgnlñcant rlsk of sudden death assoclated wlth prolonged QT
syndrome but 90% of cases of sudden death wlll have had symptoms before the
fatal event
‰ 8 Lrythromycln should be avolded ln patlents wlth prolonged QT syndrome
‰ C Slxty per cent of cases of prolonged QT syndrome wlll present between 30 and 50
years of age
‰ D Pomano-ward syndrome ls an autosomally domlnant, lnherlted syndrome
assoclated wlth prolonged QT and congenltal deafness
‰ L A normal LCG excludes the dlagnosls of prolonged QT syndrome ln a patlent wlth
a posltlve famlly hlstory
1.33 Which symptomfromthe foIIowing Iist is the MDS7 important when
considering a diagnosis of kawasaki disease!
‰ A Gallbladder hydrops
‰ 8 Hlgh-grade fever for >5 days
‰ C Thrombocytosls
‰ D Petechlal rash
‰ L 8llateral exudatlve con[unctlvltls
1.34 Which is a sign of subacute bacteriaI endocarditis (Sߣ)!
‰ A Spllnter haemorrhages
‰ 8 Lxtensor surface nodules
‰ C Loslnophllla
‰ D Cervlcal nodes
‰ L Penal artery brult
1.35 A 3-month-oId baby boy is admitted to hospitaI breathIess with poor
feeding. Dn arrivaI the main ñndings are: paIe with puIse of 240/min, Iiver
enIarged at 3 cmbeIowcostaI margin and saturation in air of 88%. Which is
the MDS7 appropriate ñrst action!
‰ A |ntravenous adenoslne
‰ 8 |ntravenous µ blocker
‰ C Appllcatlon of lce pack over face
‰ D Oxygen
‰ L |ntranasal adenoslne
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1.36 A 4-day-oId baby girI is admitted fromhome with increasing cyanosis. Dn
examination, she is deepIy cyanosed (saturation in air 78%), and has miId
subcostaI recession. She is aIert and not distressed. What is the MDS7 IikeIy
diagnosis!
‰ A Large ventrlcular septal defect
‰ 8 Trlcuspld atresla
‰ C Dlaphragmatlc hernla
‰ D Perslstent fetal clrculatlon
‰ L Transposltlon of the great arterles
1.37 A 4-month-oId is referred to the outpatient cIinic with stridor since birth and
faItering growth. 7he baby is thin with a soft ejection systoIic and diastoIic
murmur. What is the investigation MDS7 IikeIy to yieId a unifying diagnosis
in this cIinicaI setting!
‰ A Sweat test
‰ 8 8arlum swallow
‰ C Dlrect laryngoscopy
‰ D LCG
‰ L Arterlography
1.38 A 2-year-oId, fuIIy immunised boy is referred with suspected measIes. He
has a week-Iong history of fever and maIaise and has deveIoped a
generaIised morbiIIiformrash.
On arrlval he ls mlserable, febrlle (temperature 40.lºC), cervlcal lymphadenopathy,
con[unctlvltls, desquamatlon over palms. 8loods apart froma hlgh plasma vlscoslty
are normal.
What is the MDS7 appropriate ñrst-Iine management!
‰ A Pull lnfectlon screen lncludlng lumbar puncture and urlne and broad-spectrum
antlblotlc cover
‰ 8 vltamln A
‰ C Hlgh-dose asplrln and parenteral lmmunoglobulln
‰ D Parenteral sterolds
‰ L Hlgh-dose asplrln alone
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Curdíoíogy Answers
M1l Answers
1.1 NeonataI cyanosis
Answets: 8 C 0l
Llsenmenger syndrome ls an acqulred defect secondary to pulmonary hypertenslon.
Cyanosls occurs ln the hypoplastlc left heart syndrome secondary to clrculatory fallure and
worsens when the duct closes. Severe (crltlcal) aortlc stenosls can present ln the neonatal
perlod wlth cyanosls by the same mechanlsm.
Cardlac condltlons presentlng wlth neonatal cyanosls:
x Decreased pulmonary now
- pulmonary atresla
- tetralogy of Pallot
- Lbsteln anomaly (abnormallty of trlcuspld valve posltlon and functlon)
x |ncreased pulmonary now
- hypoplastlc left heart
- trlcuspld atresla
- truncus arterlosus
- total anomalous pulmonary venous dralnage
- double-outlet ventrlcle
- slngle ventrlcle
- poor mlxlng
- transposltlon of the great arterles.
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1.2 Paediatric £CCs
Answets: A 8 C l
Menn ÇR5
x At blrth - l25º
x l month - 90º
x 3 years - 50º.
The rlght axls devlatlon seen ln newborns ls due to rlght ventrlcular domlnance ln the
fetus. Plght ventrlcular devlatlon ln older chlldren lmplles rlght ventrlcular hypertrophy.
Left axls devlatlon (superlor axls) ln the neonatal perlod ls seen ln the followlng cardlac
leslons:
x Trlcuspld atresla
x AvSD
x Pulmonary stenosls.
Righr bunJle-brnnch bleck
Plght bundle-branch block ls the most common conductlon dlsturbance seen ln chlldren.
The abnormallty ls usually due to rlght ventrlcular overload prolonglng rlght ventrlcular
depolarlsatlon as a result of lengthenlng of the conductlon pathway.
Crlterla for rlght bundle-branch block lnclude:
x prolonged QPS
x rlght axls devlatlon
x termlnal slurrlng of the QPS over the rlght ventrlcular leads v3P, v4P and vl
x ST depresslon and T-wave lnverslon ln adults (rarely ln chlldren).
Causes of rlght-bundle branch block lnclude:
x AvSD
x Lbsteln anomaly
x coarctatlon of the aorta (lnfants)
x endocardlal cushlon defects
x post rlght ventrlculotomy
x partlal anomalous pulmonary venous dralnage
x normal varlant.
Cengenirnl henrr bleck
Congenltal complete heart block (complete atrloventrlcular dlssoclatlon) occurs ln lnfants
of mothers wlth systemlc lupus erythematosus (SLL), partlcularly those wlth antl-Po (SS-A)
and antl La (SS-8) antlbodles. The damage to the conductlon pathway ls lrreverslble.
Antenatal dlagnosls ls posslble because of perslstent fetal bradycardla, 50% have an
assoclated structural defect, usually congenltally corrected transposltlon. The condltlon ls
usually well tolerated and often does not requlre a pacemaker.
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Renrr rnre
Heart rate varles wlth the age and status of the patlent. A rate of ll0-l50 ls normal ln the
newborn and the adult rate of 60-l00 ls achleved by the age of 6 years.
Ç7 inrervnl
Pomano-ward ls a congenltal long QT syndrome.
1.3 Chest radiographs
Answets: C 0l
Ceeur en snber (henrr in n beer}
Seen ln tetralogy of Pallot, due to hypoplasla of the maln pulmonary artery and the consequent
upturnlng of the apex away fromthe dlaphragm. Arlght-slded aortlc arch ls seen ln 25%.
Rib nerching
A feature of coarctatlon of the aorta, lt occurs as a consequence of the lncrease ln slze of
the lntercostal vessels whlch functlon as collaterals. The upper two or three rlbs are spared
because thelr posterlor lntercostal arterles do not arlse from the aorta. |t ls rarely seen ln
chlldren aged <5 years.
0rher chesr rnJiegrnµh chnnges eI cenrcrnrien
x Dllatatlon of the ascendlng aorta, descendlng aorta (post-stenotlc dllatatlon)
x Cardlomegaly
x |ncreased pulmonary vascular shadowlng.
7runcus nrreriesus
The features on the chest radlograph lnclude a rlght-slded aortlc arch, absent thymus (30%
of cases have Dl George syndrome), cardlomegaly and a promlnent ascendlng aorta.
7ernl nnemnleus µulmennry veneus Jrninnge
There are two types:
l. Unobstructed - cardlomegaly, lncreased pulmonary vascular marklngs
2. Obstructed - normal heart slze, lncreased pulmonary vascular marklngs (severe
pulmonary oedema).
The leslon may be supracardlac, cardlac, lnfracardlac or mlxed. Obstructed leslons are usually
lnfracardlac. |n the supracardlac leslon, unobstructed total anomalous pulmonary venous
dralnage, and dllatatlon of the left and rlght superlor vena cavas and the left lnnomlnate veln
glve rlse to a 'cottage loaf' or 'snowman' appearance on the chest radlograph.
5cimirnr synJreme
Thls ls a form of partlal anomalous pulmonary venous dralnage whereby the velns from the
rlght lung draln dlrectly lnto the lnferlor vena cava (|vC). The rlght lung ls hypoplastlc as a
consequence and thls allows movement of the heart to the rlght (dextroposltlon). The
slngle veln dralnlng the rlght lung produces a (sclmltar) shape on the posteroanterlor (PA)
chest radlograph as lt heads towards the rlght cardlodlaphragmatlc angle.
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1.4 Conditions associated with an increased risk of heart disease
Answets: A 8 C 0
Knwnsnki Jisense
The cardlac leslons appear ln the second week of the lllness as proxlmal coronary artery
aneurysms heallng by ñbrosls and thrombosls. Leslons are most common on the left slde.
Protectlon ls onered by the early admlnlstratlon of lntravenous lmmunoglobulln. Other
cardlac leslons can occur lncludlng aortlc and mltral regurgltatlon, myocardltls, perlcardltls,
perlcardlal enuslon and myocardlal lnfarctlon.
Cengenirnl rubelln
|nfectlon wlth rubella durlng the ñrst trlmester causes the classlc trlad of sensorlneural
deafness, ophthalmologlcal defects (cataracts, lnfantlle glaucoma and plgmentary retlnltls)
and cardlac anomalles. Cardlac defects lnclude PDA (patent ductus arterlosus) and
perlpheral pulmonary artery stenosls most commonly, and are present ln 50% of bables
lnfected ln the ñrst l0 weeks of pregnancy. Parer cardlac manlfestatlons of ñrst trlmester
lnfectlon lnclude coarctatlon of the aorta, myocardltls, vSD and ASD. Other abnormalltles
seen ln the congenltal rubella syndrome lnclude mlcrocephaly and learnlng dlsablllty,
mlcrophthalmla, lntrauterlne growth retardatlon, hepatltls and neonatal
thrombocytopenla.
MnrInn synJreme
Thls ls an lnherlted dlsorder of connectlve tlssue. Cardlac manlfestatlons lnclude:
x mltral valve prolapse
x dllatatlon/dlssectlon of the ascendlng aorta
x aortlc regurgltatlon
x pulmonary artery aneurysm
x mltral valve prolapse.
7urner synJreme
The most common cardlac leslon ls coarctatlon of the aorta (l5-30%). Other cardlac leslons
seen lnclude aortlc stenosls, AvSD (atrloventrlcular septal defect) and blcuspld aortlc
valves. Hypertenslon ls not uncommon. Leslons commonly seen ln Noonan syndrome
lnclude pulmonary stenosls and obstructlve cardlomyopathy. Por examlnatlon purposes lt
ls worth rememberlng that, ln Noonan syndrome, rlght-slded heart leslons are seen and, ln
Turner syndrome, left-slded heart leslons.
There ls no lncrease ln the lncldence of congenltal heart dlsease ln petlt mal epllepsy.
1.5 CongenitaI heart disease
Answets: C l
The most common form of congenltal heart dlsease ls a vSD.
The lung ñelds are ollgaemlc ln tetralogy of Pallot as a consequence of the pulmonary stenosls.
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8lnleck-7nussig shunr
Thls ls an anastomosls between the subclavlan artery and the pulmonary artery and ls used
for palllatlon ln condltlons wlth severe restrlctlon pulmonary blood now, e.g. pulmonary
atresla and tetralogy of Pallot. The murmur ls contlnuous. Contlnuous murmurs
characterlstlcally pass through the second heart sound lnto dlastole.
£bsrein nnemnly
The trlcuspld valve ls malformed and leaks. The septal and posterlor leanets of the
trlcuspld valve are set further lnto the rlght ventrlcle, causlng atrlallsatlon of part of the
rlght ventrlcle. |n severe cases, chlldren can present wlth cyanosls and cardlac fallure ln the
neonatal perlod. Arrhythmlas are common lncludlng woln-Parklnson-whlte syndrome.
Llthlum durlng pregnancy ls a rlsk factor.
Nltrlc and not nltrous oxlde ls used to treat perslstent pulmonary hypertenslon.
Cnuses eI µlerheric lung 6elJs
Acyonot|c
x AvSD
x vSD
x PDA
x Lndocardlal cushlon defect
x Partlal anomalous pulmonary venous dralnage.
Cyonot|c
x Slngle ventrlcle
x Truncus arterlosus
x Hypoplastlc left heart
x Transposltlon of the great arterles
x Total anomalous pulmonary venous dralnage.
Cnuses eI cenrinueus murmurs
x 8lalock-Tausslg shunt
x Av malformatlon
x Aneurysm
x Collateral vessels
x PDA
x venous hum
x Perlpheral pulmonary stenosls.
1.6 FetaI circuIation
Answet: none cottect
Oxygenated blood fromthe placenta returns to the fetus vla the umblllcal veln: 50%enters the
hepatlc clrculatlon and 50%bypasses the llver vla the ductus venosus. As lt enters the rlght
atrlummost of the lnferlor caval blood ls dlrected through the foramen ovale lnto the left
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atrlum. The rlght atrlumcontalns blood fromthe superlor vena cava (SvC), coronary slnus and
some fromthe |vC. Plght atrlal blood enters the rlght ventrlcle, and 85%of rlght ventrlcular
blood passes lnto the descendlng aorta vla the ductus arterlosus and l5%enters the fetal lungs.
Changes ln the fetal clrculatlon occur at blrth: the ductus venosus closes and loss of the
low-reslstance placenta results ln an lncrease ln systemlc vascular reslstance. There ls a
functlonal closure of the foramen ovale. Lung expanslon results ln a fall ln pulmonary
vascular reslstance, lncreased pulmonary blood now and lncreased dellvery of blood to the
left atrlum. Plow through the ductus arterlosus changes from the pulmonary to the
systemlc clrculatlon to the systemlc to the pulmonary clrculatlon. The hlgh concentratlon
of oxygen ln the blood causes smooth muscle contractlon of the duct and closure.
1.7 CongenitaI heart disease
Answets: C l
Poor growth (helght and welght) ls common ln chlldren wlth cyanotlc congenltal heart
dlsease. Catch-up growth after correctlve surgery ls common. Pallure to thrlve and feedlng
dlmcultles are common ln lnfants wlth a symptomatlc congenltal heart dlsease. The
contralndlcatlons to lmmunlsatlon are as for the normal populatlon, lmmunlsatlon should
be encouraged to prevent potentlally serlous and/or deadly dlseases. The oral
contraceptlve plll ls contralndlcated ln glrls wlth cyanotlc congenltal heart dlsease due to
the rlsk of thrombosls. The coll ls also contralndlcated because lt ls a potentlal focus of
lnfectlon predlsposlng to endocardltls. Unplanned pregnancy for those wlth congenltal
heart dlsease can be dlsastrous, so lnformed and approprlate advlce for thls populatlon ls
vltal. A chromosomal abnormallty ls present ln 6-l0% of chlldren wlth congenltal heart
dlsease and 30% of newborn lnfants wlth chromosomal abnormalltles have heart defects.
1.8 7eratogens
Answets: A 8 0l
The followlng are teratogens for the defects shown.
Alcohol ASD, vSD, PDA, TGA
Amphetamlnes ASD, vSD, PDA, TGA
Llthlum Lbsteln anomaly
Oestrogens/progesterones vSD, TGA, TOP
Phenytoln Pulmonary stenosls, aortlc stenosls, PDA, coarctatlon, ASD, vSD
Thalldomlde TOP, truncus arterlosus
Sodlum valproate TOP, vSD, coarctatlon
warfarln TOP, vSD
ASD, atrlal septal defect, vSD, ventrlcular septal defect, PDA, patent ductus arterlosus, TGA,
transposltlon of the great arterles, TOP, tetralogy of Pallot.
Maternal dlseases llnked to an lncreased rlsk of congenltal heart dlsease lnclude:
x Dlabetes - transposltlon, septal defects, coarctatlon, translent cardlomyopathy
x Phenylketonurla - tetralogy of Pallot
x SLL - congenltal heart block.
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1.9 CircuIatory faiIure in the neonataI period
Answets: A 8 C 0l
Acute clrculatory fallure (shock) ls characterlsed by lnadequate tlssue and organ perfuslon.
Organ perfuslon relles on a functlonlng pump (heart), an adequate dellvery system
(lntegrlty of structure and vasomotor tone of all vascular beds - venous, arterlal and
caplllary) and an adequate substrate dellverlng nutrlents and removlng toxlns and waste.
Pallure of any of the three components ln lsolatlon or ln comblnatlon wlll lead to shock.
Cardlogenlc causes of neonatal shock:
x Crltlcal aortlc stenosls
x Hypoplastlc left heart
x Coarctatlon
x Myocardlal lschemla (hypoxlc lschaemlc encephalopathy, hypoglycaemla)
x Cardlomyopathy
x Arrhythmla
x Arterlovenous ñstula
x Obstructed pulmonary venous dralnage
x TGA wlth vSD.
8y the end of the ñrst week, when pulmonary vascular reslstance falls, left-to-rlght shunts
become an lmportant cause of heart fallure lncludlng vSD, PDA, AvSD and truncus
arterlosus.
Non-cardlogenlc causes of neonatal shock:
x Hypovolaemla (blood, nuld or electrolyte losses)
x Dlssoclatlve shock (severe anaemla or methaemogloblnaemla)
x Dlstrlbutlve (sepsls, vasodllators, myocardlal depresslon or endothellal ln[ury)
x Obstructlve shock (tenslon pneumothorax or cardlac tamponade).
The treatment of neonatal clrculatory fallure depends on ldentlfylng the cause and treatlng
accordlngly.
ßCl Answers
1.10 Cardiac arrest
Answet A: 8os|c l|le suµµott w|th h|çh concenttot|on oxyçen shoulJ oe cont|nueJ thtouçhout
ony tesusc|tot|on w|th the m|n|mumol |ntettuµt|ons
|n all states of cardlac arrest the establlshment and contlnulty of baslc llfe support (8LS)
should take precedence over any advanced management.
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Asysrele
Thls ls the most common arrest rhythm ln chlldren:
l. Clear alrway
2. ventllatlon wlth hlgh-concentratlon oxygen and cardlac massage (l5:2)
3. Attach monltor
4. Adrenallne dose every 4 mlnutes (avold hlgher doses as assoclated wlth poorer
outcomes) lntravenously or lntraosseously (l0 Pg/kg ¬ 0.l ml/kg of l ln l0 000)
plus nush! (LTT dose ls l0×)
5. |ntubate and proceed wlth unlnterrupted CPP (cardlopulmonary resuscltatlon)
6. Conslder reverslble causes (pulseless electrlcal actlvlty or PLA).
Pulseless elecrricnl ncriviry (incluJing elecrre-mechnnicnl Jissecinrien}
|t should be treated as asystole. |t can be secondary to a treatable cause:
x Severe hypovolaemla
x Tenslon pneumothorax
x Perlcardlal tamponade
x Hypothermla
x Hypoxla
x Acldosls
x Toxlc
x Hypocalcaemla/hyperkalaemla/hypokalaemla
x Pulmonary thromboembolus.
venrriculnr 6brillnrien
Thls ls rare ln chlldhood. Suspect ln cases of sudden collapse or wlth underlylng cardlac
dlsease, hypothermla and/or overdose wlth a trlcycllc antldepressant.
x 8LS wlth hlgh-now oxygen should be establlshed stralghtaway and contlnued
throughout the resuscltatlon wlth the mlnlmum pauses posslble.
x A slngle asynchronous DC shock (4 1/kg) ls glven at 2-mlnute lntervals throughout
the resuscltatlon wlth lmmedlate resumptlon of CPP after each shock, and a short
pause for rhythm reassessment lmmedlately before the next shock ls dellvered.
x |ntubatlon and the establlshment of vascular access should be secured as soon as
posslble wlthout compromlslng CPP or the DC shocklng protocol.
x Adrenallne ls glven lmmedlately before the thlrd shock (l0 Pg/kg).
x Amlodarone 5 mg/kg ls glven before the fourth DC shock at 5 mg/kg.
x Purther adrenallne can be glven every other shock (every 4 mlnutes) and reverslble
causes should be sought.
Pulseless vT should be treated as vP. Prolonged resuscltatlon may be lndlcated ln
hypothermla.
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1.11 PaIpitations
Answet C: keossutonce thot het µolµ|tot|ons Jo not hove o s|çn|hcont couse, oJv|ce ooout
teloxot|on onJ sttess conttol Jut|nç exomseoson, onJ J|schotçe to lollow-uµ ltomcl|n|c
Thls young glrl has got physlologlcal tachycardla assoclated wlth sltuatlon-speclñc stress
and requlres strong reassurance not further lnvestlgatlon or referral.
Palpltatlons are common ln paedlatrlcs. A mlnorlty wlll have true arrhythmlas (l3%).
Dlnerentlal dlagnosls lncludes:
x Cardlac condltlons
x Potentlally llfe threatenlng:
- SvT - wPw syndrome
- prolonged QT syndrome
- hypertrophlc cardlomyopathy
- myocardltls
- slck slnus syndrome
- arrhythmlas assoclated wlth congenltal heart dlsease
x Not lmmedlately/non-llfe-threatenlng cardlac causes:
- premature atrlal contractlons
- premature ventrlcular contractlons
- acute rheumatlc fever wlth valvular dlsease
x Non-cardlac condltlons
- potentlally llfe threatenlng
- hypoglycaemla
- drug/toxln exposure
- phaeochromocytoma
x Not lmmedlately/non-llfe threatenlng
- fever
- anaemla
- exerclse
- emotlonal arousal
- anxlety/hyperventllatlon/panlc attacks
- drug lngestlon
- past orthostatlc tachycardla syndrome
- hyperthyroldlsm.
A careful hlstory and examlnatlon are essentlal to correctly ldentlfy the aetlology:
x Cardlac arrhythmlas are often descrlbed as havlng abrupt onset and onset.
x Chlldren wlth premature atrlal or ventrlcular beats may descrlbe a nlp-nop feellng
when the heart stops and then compensates wlth a larger stroke volume on the
followlng beat.
x Palpltatlons assoclated wlth excesslve perlodlc sweatlng, headaches and
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hypertenslon may suggest the rare underlylng dlagnosls of phaeochromocytoma.
x Palpltatlons wlth heat lntolerance, sweatlng and welght loss may suggest
hyperthyroldlsm.
x Syncope (wlth ln[ury), exerclse lntolerance, and a past hlstory or famlly hlstory of
cardlac dlsease, sudden death or deafness (PQT) may lndlcate slgnlñcant underlylng
cardlac pathology.
x Tachycardla out of proportlon to fever may lndlcated myocardltls.
A thorough systemlc examlnatlon ls vltal ln all cases and a l2-lead LCG should be
performed.
Conslder referral to cardlologlst plus 24-hour LCG or event monltor may help to clarlfy
aetlology ln concernlng cases.
1.12 Newonset cardiac faiIure
Answet C: v|tol myocotJ|t|s
Thls baby presents wlth a short hlstory of new onset cardlac fallure assoclated wlth
falterlng growth at l8 months of age followlng shortly after a vlral lllness. Thls ls conslstent
wlth an acqulred cardlac fallure not a congenltal cause:
x Myocardltls - lnnammatlon of the heart muscle
x Cause of sudden death ln prevlously healthy chlldren
x Can lead to acute dllated cardlomyopathy
x vlral myocardltls ls the leadlng cause ln the western world:
- adenovlrus (up to 39% of cases)
- Coxsacklevlrus (Coxsackle 8 enterovlrus)
x Other causes lnclude:
- toxlns
- autolmmune processes
- other lnfectlon (vlral, bacterlal, protozoal, parasltlc)
x Cllnlcal presentatlon: prodromal vlraemla (70-90%) - fever, myalgla, coryzal and/or
gastroenterltls
x LCG changes:
- non-speclñc ST-segment and T-wave abnormalltles
- pathologlcal Q waves
- T-wave lnverslon
- low QPS voltages (<5mm ln any precordlal leads)
- vT and frequent ventrlcular ectoplcs can be seen
x Symptoms and slgns accompanylng LCG abnormalltles:
- asymptomatlc
- palpltatlons
- presyncope/syncope
x Symptoms and slgns (heart fallure):
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- dyspnoea
- sweatlng
- poor feedlng
- orthopnoea/PND (paroxysmal nocturnal dyspnoea)
- falterlng growth
- tachypnoea
- tachycardla (out of proportlon to fever)
- gallop rhythm
- murmur of mltral regurgltatlon
- hepatomegaly
- perlpheral oedema
- ralsed 1vP ([ugular venous pressure)
Notutol h|stoty ol the J|seose JeµenJs on sevet|ty ol µtesentot|on
x Asymptomatlc patlents/LCG changes generally recover completely
x Mlld left ventrlcular systollc lmpalrment generally recover wlthln weeks to months
x Severe left ventrlcular dysfunctlon:
- 25% recover
- 25% progress to transplantatlon
- 50% develop chronlc dllated cardlomyopathy (DCM)
0|oçnos|s
x Needs hlgh lndex of susplclon
x LCG
x Lchocardlography
x Troponln T/| level
x vlral serology and PCP (polymerase chaln reactlon)
x Lndomyocardlal blopsy:
- hlstologlcal
- vlral PCP
- lmmunohlstochemlstry
Vonoçement
x Supportlve: lonotroplc support, antlcoagulatlon, afterload reductlon and dluresls
x Asslst devlces or LCMO ln severe cases
x Speclñc heart fallure treatments: ACL (anglotensln-convertlng enzyme) lnhlbltors, µ
blockers
x |mmune-medlated therapy
- |v|G (lntravenous lmmunoglobulln)
- lmmunosuppresslon wlth prednlsolone ln lmmune-medlated dlsease.
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1.13 £CCchanges in eIectroIyte disturbances
Answet A: lCCchonçes ossoc|oteJ w|th hyµetloloem|o ote µtoçtess|ve w|th the sevet|ty ol
hyµetloloem|o leoJ|nç to the oosence ol µ woves, otooJen|nç ol Çk5 comµlexes, onJ vl ot v7
Ryµeknlnemin
lCCchonçes
x ST depresslon
x Plattened or lnverted T waves
x U waves
x Cardlac arrhythmlas, atrlal and ventrlcular ectoplcs, atrlal tachycardla, heart blocks,
vP and vT
x |ncreased P-wave amplltude and duratlon
x QPS prolongatlon.
Ryµerknlnemin
LCG changes occur ln a sequence assoclated wlth progresslon on hyperkalaemla:
x Larly LCG changes lnclude:
- peaked T waves,
- decreased QT lnterval
- ST-segment depresslon/elevatlon
x Progresslon of hyperkalaemla leads to:
- wldenlng of QPS complex 2º to bundle-branch blocks
- lncreased PP lnterval (ñrst-degree heart block)
- decreased amplltude of P wave
x wlthout treatment further progresslon leads to:
- P waves dlsappearlng
- QPS wldenlng to a slne wave morphology
- vP or asystole follows
x These LCG changes generally correct wlth potasslum
x Potentlally llfe-threatenlng arrhythmlas can occur at almost any level of
hyperkalaemla wlthout the warnlng sequence of LCG changes descrlbed above.
Ryµemngnesnemin (£CCchnnges resemble hyµeknlnemin}
x May be assoclated wlth non-speclñc LCG changes lncludlng:
- ST depresslon
- nattenlng T waves
- promlnent U waves
- loss of voltage
x Severe magneslum deñclency may cause:
- PP prolongatlon
- wldenlng of QPS complex
- tachycardla
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- rarely premature ventrlcular contractlon and ñbrlllatlon.
Ryµermngnesnemin (£CCchnnges mny resemble hyµerknlnemin}
x Prolonged PP lnterval
x wldened QPS (lntraventrlcular conductlon delay).
Ryµercnlcnemin
x Peduced QT lnterval on LCG
x T-wave duratlon unanected
x ST-segment duratlon shortened
x May present wlth a varlety of arrhythmlas
x Slgnlñcant hypercalcaemla may present wlth LCG changes mlmlcklng myocardlal
lschaemla.
Ryµecnlcnemin
x The maln LCG change ls a prolonged QT lnterval
x No change ln T-wave duratlon but ST segment ls prolonged.
Ryµer-/hyµennrrnemin
x No slgnlñcant LCG abnormalltles are assoclated
1.14 Aortic stenosis
Answet l: 7hete |s on ossoc|ot|on w|th suJJen Jeoth |n µot|ents w|th oott|c stenos|s
Aerric vnlve srenesis
Thls accounts for 5% of congenltal heart defects. |t ls more common ln males.
Assoc|ot|ons ol oott|c stenos|s
x Turner syndrome
x wllllams syndrome
x Coarctatlon of the aorta
x Other cardlac abnormalltles, e.g. hypoplastlc left ventrlcle, mltral valve abnormalltles.
The stenosls can be elther supravalvular, valvular or subvalvular. There ls often an
assoclated blcuspld valve. Aortlc stenosls ls usually asymptomatlc but ln lts most severe
form can cause congestlve cardlac fallure, arrhythmlas and sudden death ln lnfancy (rare).
The murmur ls best heard ln the aortlc area (upper left sternal edge) and radlates to the
neck. An e[ectlon cllck suggests valvular stenosls. A palpable thrlll ls usually present ln the
suprasternal notch. A2 (the aortlc component of the second heart sound) ls qulet.
Although left ventrlcular hypertrophy ls common the LCG can be normal.
Assessment ol sevet|ty
x Symptoms - anglna-llke paln
x Syncope/dlzzlness on exertlon
x Palpltatlons on exertlon
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x LCG evldence of left ventrlcular straln
x Lxerclse test posltlve
x ST and T-wave changes on the LCG durlng exerclse.
7teotment
Treatment ls conservatlve ln most cases avoldlng valve replacement ln the young patlent. |f
the gradlent across the valve ls >60 mmHg treatment ls lndlcated. Thls ls usually ln the
form of a balloon valvoplasty at cardlac catheter, or surglcal valvuloplasty.
1.15 WiIIiams syndrome
Answet C: 0|oçnos|s ol w|ll|oms synJtome con oe conhtmeJ oy huotescence |n s|tu
hyot|J|zot|on (ll5lï stuJ|es
wllllams syndrome ls a neurodevelopmental dlsorder. |t ls caused by a mlcro deletlon
lnvolvlng the elastln gene on the long arm of chromosome 7. Dlagnosls can be conñrmed
by P|SH studles.
lenrures
x Characterlstlc elñn facles (whlch may be dlmcult to detect ln young bables):
- stellate lrls
- nattened nasal brldge wlth small upturned nose
- long phlltrum
- wlde (carp-shaped) mouth
- partlally mlsslng teeth, defectlve tooth enamel or small, wldely spaced teeth
x Characterlstlc anect - over-frlendly wlth better verbal than vlsuospatlal skllls
x 8ehavloural problems wlth poor concentratlon and dlstractlblllty
x Normal blrth welght wlth post-natal growth retardatlon secondary to poor feedlng
(fallure to thrlve)
x Neurodevelopmental delay wlth delayed motor mllestone and |Q of 50-60
x |dlopathlc hypercalcaemla ln approxlmately l5% (presents as lrrltablllty, vomltlng
and constlpatlon ln bables) - resolves at age 2 years, management ls wlth low
calclum and vltamln D lntake, and aetlology unknown
x Hypercalclurla (nephrocalclnosls)
x Squlnts
x Hernla
x Pectal prolapse
x Cardlovascular abnormalltles (75%):
- supravalvular aortlc stenosls
- perlpheral pulmonary artery stenosls
- others lncludlng valvular and septal defects have been reported
- hypertenslon.
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1.16 Down syndrome
Answet 8: lchocotJ|oçtoµhy |s |nJ|coteJ |n oll coses ol 0own synJtome lot |Jent|hcot|on ol
conçen|tol cotJ|oc J|seose even |l o mutmut |s not µtesent
CnrJinc lesiens in Ðewn synJreme
Cardlac leslons are present ln 40-50% of chlldren wlth Down syndrome. Porty-ñve per cent
are AvSDs and 35% are lsolated ventrlcular septal defects (vSDs). Others commonly seen
lnclude tetralogy of Pallot, PDA and secundum ASDs. Of all chlldren wlth an AvSD, 25%
have Down syndrome. All chlldren wlth Down syndrome should have an echocardlogram
to detect congenltal heart dlsease (CHD). The symptoms assoclated wlth a rlght-to-left
cardlac shunt can be delayed ln chlldren wlth Down syndrome due to the perslstence of
hlgh pulmonary vascular reslstance.
Arrievenrriculnr seµrnl JeIecr
Partlal AvSD refers to an ostlum prlmum ASD and ls present wlth or wlthout a cleft ln the
mltral valve. Complete AvSD refers to a common atrloventrlcular valve wlth clefts ln both
the pulmonary and the mltral valves. Treatment of AvSD and large vSD lncludes:
x dluretlcs to control heart fallure
x nutrltlonal support to maxlmlse growth
x delayed closure at 3-6 months.
dµµer nirwny ebsrrucrien
Obstructlve sleep apnoea ls common ln chlldren wlth Down syndrome, anectlng 30-50%.
The aetlology lncludes hypotonlc upper alrway muscles, adenotonslllar hypertrophy,
macroglossla, glossoptosls, nattened mldface and narrowed nasopharynx. Laryngomalacla
and gastro-oesophageal renux dlsease (GOPD) are both lncreased ln chlldren wlth Down
syndrome whlch may worsen alrway dlsease. Pecurrent hypoxaemla ls a contrlbutlng
factor to the development of pulmonary arterlal hypertenslon (PAH).
Pulmennry nrrerinl hyµerrensien in chilJren wirh Ðewn synJreme
There ls an lncreased lncldence of pulmonary vascular dlsease ln chlldren wlth Down
syndrome, both wlth and wlthout assoclated CHD. The prlnclpal aetlology ls cardlac
(lncreased pulmonary now). Other contrlbutlng factors lnclude upper alrway obstructlon
and an lncreased lncldence of lntrlnslc lung dlsease (pulmonary hypoplasla). when
congenltal cardlac defects are present, long-standlng lncreased pulmonary vascular
reslstance due to a rlght-to-left shunt may lead to reversal of now across the shunt and
Llsenmenger syndrome.
1.17 7ransposition of the great arteries
Answet 8: 7tonsµos|t|on ol the çteot ottet|es |s the most common couse ol cyonot|c conçen|tol
heott J|seose |n the neonotol µet|oJ
Transposltlon of the great arterles (TGA) represents about 6% of all CHD. |t ls the most
common cause of cyanotlc CHD ln the neonatal perlod. |t ls more common ln boys than
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glrls (3:l). |t ls not usually assoclated wlth syndromes. Left untreated lt ls lethal ln 90% of
cases by age l year.
|n TGA the aorta arlses fromthe rlght ventrlcle and carrles deoxygenated blood to the body,
and the pulmonary artery arlses fromthe left ventrlcle and carrles oxygenated blood to the
lungs. Chlldren presentlng ln the neonatal perlod wlth thls condltlon have hlgh pulmonary
blood nowand are severely cyanosed unless a leslon that mlxes the two clrculatlons ls
present (examples lnclude PDA, ASD, vSD). The chlld ls usually cyanotlc fromor shortly after
blrth. The leslon ls duct dependent and the lnfant's condltlon deterlorates when the duct
closes. There ls usually a metabollc acldosls at presentatlon. |f adequate mlxlng ls posslble
due to one of the defects llsted above, the chlld may present later ln lnfancy wlth poor
feedlng and congestlve cardlac fallure rather than collapse, acldosls and hypoxla on duct
closure ln the neonatal perlod. Arrhythmlas of all types are common. LCGshows a rlght-slded
axls and rlght ventrlcular hypertrophy. The chest radlograph shows cardlomegaly and
lncreased pulmonary vascularlty. Antenatal dlagnosls on anomaly ultrasound scannlng
remalns dlmcult and so the vast ma[orlty of cases are dlagnosed postnatally.
5urgicnl mnnngemenr eI 7CA
x Palllatlve - atrlal septostomy
x Physlologlcal repalr (permanent palllatlon) - mustard, Sennlng, these procedures are
assoclated wlth long-term compllcatlons lncludlng systemlc ventrlcular fallure, baMe
leaks and stenosls, and hlgh rates of arrhythmlas lncludlng sudden death
x Anatomlcal:
- 1atene arterlal swltch (correctlve) performed at less than 2 weeks of age, mortallty
rate >5%
- Pastelll condult (needs replacement).
1.18 7etraIogy of FaIIot
Answet 8: Cyonot|c sµells ossoc|oteJ w|th tettoloçy ol lollot usuolly oeç|n otounJ 4-6 months
ol oçe
Thls accounts for l0% of congenltal heart dlsease. Components of tetralogy of Pallot:
x ventrlcular septal defect
x Plght ventrlcular outnow obstructlon
x Plght ventrlcular hypertrophy
x Overrldlng aorta.
The severlty of the rlght ventrlcular outnow obstructlon wlll determlne the cllnlcal plcture.
Most cases have falterlng growth and breathlessness on feedlng.
x Mlld obstructlon: plnk tetralogy of Pallot - left-to-rlght shunt across the vDS -
murmur e[ectlon systollc (pulmonary stenosls) - cyanotlc later as the shunt reverses.
x Moderate obstructlon: presents wlth cyanosls - rlght-to-left shunt across vSD -
murmur ls e[ectlon systollc due to pulmonary stenosls, vSD sllent.
x Severe obstructlon: duct dependent, presentlng wlth cyanosls ln the neonatal
perlod.
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LCG shows rlght ventrlcular hypertrophy and rlght axls devlatlon ln cyanotlc tetralogy of
Pallot. The LCG ln acyanotlc tetralogy of Pallot shows rlght ventrlcular hypertrophy because
the rlght ventrlcular pressure ls hlgh. Cyanotlc spells usually begln around 4-6 months of
age. They are due to functlonal lnfundlbular spasm and potentlally fatal. Peatures lnclude
worsenlng cyanosls and a reductlon ln the lntenslty of the murmur. Assoclated ASDs do
occur and are referred to as the quatralogy of Pallot.
Treatment of cyanotlc spells ls as follows:
x 8rlng baby's knees to chest (reduces venous return by lncreaslng perlpheral vascular
reslstance, decreases left-to-rlght shunt across the vSD)
x E 8ockers
x Morphlne
x Sodlum blcarbonate lf acldotlc
x vasoconstrlctors.
Surglcal management of tetralogy of Pallot ls elther palllatlve (systemlc-to-pulmonary
shunt) or complete. Total correctlon, lf technlcally posslble, ls the preferred optlon. Thls ls
usually done at around 6 months.
Compllcatlons of tetralogy of Pallot lnclude:
x Polycythaemla
x Subacute bacterlal endocardltls
x Cerebral abscess, cerebral thrombosls
x Petardatlon of growth and development
x Clubblng - usually appears after age l year.
1.19 AtriaI septaI defects
Answet l: A µulmonoty to system|c howtot|o ol mote thon 2:1 |s on |nJ|cot|on lot sutç|col ot
mote commonly µetcutoneous ttonscothetet Jev|ce closute
|solated ASDs account for 8% of congenltal heart dlsease. The defect ls more common ln
glrls and there are three types:
l. Ostlum secundum (most common)
2. Ostlum prlmum
3. Slnus venosus.
Ostlum secundum defects are usually asymptomatlc, wlth pulmonary hypertenslon and
rlght ventrlcular fallure occurrlng ln the thlrd and fourth decades. Atrlal arrhythmlas occur
ln adulthood but are rare ln chlldhood. An ostlum prlmum defect ls llkely to present earller,
usually as a component of an endocardlal cushlon defect. |solated ASDs are not consldered
to be at hlgh rlsk of developlng bacterlal endocardltls. Lven ln complex ASDs prophylactlc
antlblotlcs are no longer routlnely recommended for lnterventlonal procedures ln hlgh-rlsk
patlents due thelr potentlal for harm and the lack of evldence for beneñt.
LCG appearance of atrlal septal defects:
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x ostlum prlmum - rlght bundle-branch block, left axls devlatlon
x ostlum secundum - rlght bundle-branch block, rlght axls devlatlon.
The presence of rlght bundle-branch block ls not dlagnostlc but lts absence makes the
dlagnosls unllkely. A hlgh proportlon of ostlum secundum ASDs close spontaneously by
the age of 5 years. The rlsk of pulmonary hypertenslon and lts sequelae lncreases wlth
shunt slze. Surglcal repalr ls lndlcated lf the pulmonary-to-systemlc now ratlo ls >2:l. Hlgh
pulmonary vascular reslstance ls a contralndlcatlon to surgery.
Plxed spllttlng of the second heart sound ls characterlstlc of an ASD. Thls ls due to the
defect produclng a constantly lncreased rlght ventrlcular volume and prolonglng e[ectlon
tlme. The murmur of an ASD ls not due to now across the defect but to lncreased now
across the pulmonary valve as a consequence of the shunt.
1.20 VentricuIar septaI defects
Answet 0: v50s ote ossoc|oteJ w|th h|çhet oxyçen content |n the olooJ ol the t|çht ventt|cle
thon the t|çht ott|um
vSDs are the most common congenltal heart defect. The defect can occur ln the
membranous or muscular part of the septum, although defects ln the membranous septum
are more common and usually slngle. Defects ln the muscular part of the septumare usually
multlple. The slgns and symptoms depend on the haemodynamlcs of the defect, whlch
depends on the slze of the defect and the pulmonary vascular reslstance. wlth a small defect,
the murmur ls rarely present at blrth but appears as the pulmonary vascular reslstance falls:
x |nfectlve endocardltls occurs ln less than 2% of cases.
x |t ls the left and not the rlght ventrlcle that ls volume overloaded. The shunt occurs
malnly durlng systole when the rlght ventrlcle ls contractlng, and so the shunted
blood enters the pulmonary clrculatlon.
x The rlght atrlum contalns deoxygenated blood, the rlght ventrlcle contalns
deoxygenated blood from the rlght atrlum and oxygenated blood from the left
ventrlcle.
x At cardlac catheterlsatlon, the oxygen content of blood ln the rlght ventrlcle ls
greater than that ln the rlght atrlum.
x Some 30-60% close spontaneously ln the ñrst 6 months and many more subsequent
to that. Surgery ls requlred lf the left-to-rlght shunt ls such (usually quoted as > 2:l)
that pulmonary hypertenslon has or ls llkely to develop. Surglcal closure for large
defects ls usually performed at around 3-5 months. The second heart sound ls loud lf
pulmonary hypertenslon ls present. Untreated, a large shunt wlll result ln hlgh
pulmonary now and can progress to pulmonary hypertenslon and Llsenmenger
syndrome.
1.21 £isenmenger syndrome
Answet 0: Cottect|on ol the v50oelote the Jeveloµment ol µulmonoty hyµettens|on
Llsenmenger syndrome occurs as a consequence of a long-standlng uncorrected
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congenltal heart defect wlth lntracardlac communlcatlons, leadlng to pulmonary
hypertenslon wlth a reversed or bldlrectlonal shunt at the atrlal, ventrlcular or
aortopulmonary level and assoclated cyanosls. Cardlac defects assoclated wlth the
development of Llsenmenger syndrome lnclude the followlng:
x |ncreased pulmonary arterlal now:
- ASD
- systemlc Av ñstulae
- TAPvD (total anomalous pulmonary venous dralnage)
x |ncreased pulmonary artery pressure and now:
- large vSD
- AvSD
- PDA
- aortopulmonary wlndow
- truncus arterlosus
- transposltlon of the great arterles wlth a vSD.
Symptoms of pulmonary vascular dlsease do not usually develop untll the second or thlrd
decade, typlcally presentlng between l5 and 20 years of age. Cyanosls leads to a
compensatory polycythaemla whlch ln turn causes hypervlscoslty. Progresslon of the
process often leads to early death ln the second or thlrd decade of llfe.
The followlng are rlsk factors for the earller development of pulmonary hypertenslon:
x Cardlac defect wlth large shunt
x Perlnatal asphyxla
x Pecurrent chest lnfectlons
x Chronlc upper alrway obstructlon
x Down syndrome
x 8lrth at or llvlng at hlgh altltude.
Symptoms and slgns of Llsenmenger syndrome:
x Dyspnoea
x Syncope
x Arrhythmlas
x Clubblng
x Palsed [ugular venous pressure
x Plght ventrlcular heave
x Loud P2
x Hypervlscoslty may lead to:
- thromboembollc events
- gout
- pulmonary lnfarctlon wlth chest paln and haemoptysls.
Heart-lung transplantatlon ls the only surglcal optlon. Management wlth dluretlcs and
antlcoagulants may be lndlcated. Phlebotomy may relleve the symptoms of hypervlscoslty.
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varlous medlcatlons lncludlng long-term prostacycllns bosentan and slldenañl, currently
used to treat ldlopathlc pulmonary hypertenslon, are belng lnvestlgated for thelr potentlal
to treat the underlylng pulmonary hypertenslon seen ln Llsenmenger syndrome wlth
promlslng lnltlal results.
Cardlac abnormalltles presentlng wlth cyanosls and reduced pulmonary now are protected
from the development of pulmonary vascular dlsease. These condltlons lnclude pulmonary
atresla, pulmonary stenosls and tetralogy of Pallot.
1.22 Patent ductus arteriosus
Answet l: 7he |nc|Jence ol P0A |s |ncteoseJ |n µtetetm|nlonts onJ those w|th µet|notol hyµox|o
onJ/ot J|sttess
PDA ls present ln 40-50% of preterm lnfants born welghlng <l750 g, 30% of whom have a
slgnlñcant ductus wlth congestlve cardlac fallure. The reason for the hlgher lncldence ln
preterm lnfants ls that the responslveness of ductal smooth muscle ls gestatlon
dependent. Perlnatal dlstress and/or hypoxla can delay the closure of the duct. |f not
assoclated wlth predlsposlng factors PDA ls more common ln glrls (ratlo 2:l). |n term lnfants
functlonal closure occurs wlthln l0-l5 hours of blrth wlth complete anatomlcal closure by
2-3 weeks of age. A perslstence of the duct for 3 months after the baby has reached term ls
the deñnltlon of a perslstent ductus arterlosus.
Presentatlon of PDA ln the preterm lnfant:
x Cardlac fallure
x Apnoea
x |ncreased ventllatory requlrements.
Presentatlon of PDA ln the term lnfant or chlld:
x Small shunt: asymptomatlc
x Large shunt:
- poor welght galn (up to a thlrd of chlldren wlth PDA are small for age)
- tachypnoea
- tachycardla
- cardlac murmur.
Plndlngs on examlnatlon:
x Contlnuous machlnery murmur ln left lnfraclavlcular area
x Pull pulses
x Loud P2 (can be obscured by the murmur)
x |n the preterm lnfant the murmur may be llmlted to systole.
Mnnngemenr eI PÐA
|n the preterm lnfant thls depends on symptoms. Spontaneous closure ls llkely lf the duct ls
asymptomatlc. |n symptomatlc lnfants the management ls nuld restrlctlon, dluretlcs,
malntenance of normal haemoglobln and attempted medlcal closure wlth lndometacln,
lbuprofen or surglcal llgatlon.
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|n the term lnfant lndometacln ls not helpful and the prlnclpal therapeutlc optlon ls
surglcal llgatlon. Catheter closure of the duct ls lndlcated ln chlldren aged >l year
(occaslonally younger). Spontaneous closure ls not llkely ln term lnfants.
1.23 Coarctation of the aorta
Answet l: AJult-tyµe cootctot|on ol the ootto |s ossoc|oteJ w|th olooJ µtessute J|scteµoncy
oetween the uµµet onJ lowet l|mos
Thls accounts for 5% of congenltal heart dlsease. |t ls more common ln boys (2:l). |t can
present ln lnfancy lf the coarctatlon ls slgnlñcant, maklng systemlc perfuslon duct
dependent, or ln late chlldhood or adulthood ln the non-duct-dependent leslon whlch
tends to become lncreaslngly more severe over years.
Assoclated cardlac anomalles lnclude:
x blcuspld aortlc valve (70%)
x mltral valve dlsease
x subaortlc stenosls
x vSD.
AJulr-ryµe cenrcrnrien
The coarctatlon ls usually [ust after the orlgln of the left subclavlan artery (98%) at the level
of the ductus arterlosus. |t can occur proxlmal to the orlgln of the left subclavlan artery, ln
whlch case the blood pressure ln the rlght arm wlll be hlgher than ln the left arm, the
classlc dlscrepancy belng between the upper and lower llmb blood pressure. The murmur
of coarctatlon ls systollc. Contlnuous murmurs are occaslonally heard from collaterals. Up
to l0% of chlldren wlth coarctatlon have berry aneurysms ln the cerebral clrculatlon. Plb
notchlng does not appear untll late chlldhood. There can be a classlc '3' slgn wlth a vlslble
notch on the chest radlograph ln the descendlng aorta. Treatment ls surglcal by balloon
dllatatlon, graft or a subclavlan nap. Thls ls usually carrled out soon after dlagnosls.
Ðucr-JeµenJenr cenrcrnrien
Thls leslon ls often dlagnosed antenatally on screenlng ultrasound examlnatlons. |f not the
neonate wlll present collapsed wlth absent femoral pulses at the tlme of the duct closure.
Absence of a murmur, shortness of breath, and slgns of rlght heart fallure (hepatomegaly),
breathlessness and acldosls are common at presentatlon. Pesuscltatlon and urgent
treatment wlth prostaglandln Ll or L2 ls llfe savlng ln order to support the lnfant's
clrculatlon before urgent correctlve surgery.
Assoclatlons of coarctatlon of the aorta lnclude:
x trlsomy l3
x trlsomy l8
x Turner syndrome
x valproate toxlclty.
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1.24 Hypertension
Answet C: ln |nlonts the most common couse ol hyµettens|on |s tenol µotenchymol J|seose
Chlldhood hypertenslon ls deñned as systollc or dlastollc blood pressure greater than the
95th centlle for age, recorded on three separate occaslons. There are two types: prlmary
(aetlology unknown) and secondary (aetlology known). Secondary hypertenslon ls more
common ln lnfants and younger chlldren. Prlmary hypertenslon ls more common than
secondary hypertenslon ln adolescents and young adults when there ls often a famlly
hlstory. Chlldren wlth prlmary hypertenslon are rarely symptomatlc. Obeslty ls assoclated
wlth prlmary hypertenslon, and ln 70-80% of chlldren wlth secondary hypertenslon there
ls a renal cause. |nltlal lnvestlgatlons are almed at detectlng renal parenchymal dlsease.
Causes of hypertenslon ln chlldhood lnclude:
x Penal parenchymal dlsease:
- renal scarrlng/renux nephropathy
- acute glomerulonephrltls
- chronlc glomerulonephrltls
- chronlc renal fallure
- acute renal fallure
- nephrotlc syndrome
- polycystlc renal dlsease
x vascular dlsease and renovascular dlsease:
- coarctatlon of the aorta
- renal artery stenosls
- renal artery or renal veln thrombosls
x Penal tumours: wllms tumour
x Catecholamlnes:
- phaeochromocytoma
- neuroblastoma
x Obstructlve uropathy
x Cortlcosterolds:
- latrogenlc
- Cushlng syndrome
x Neurologlcal causes:
- ralsed |CP (lntracranlal pressure)
- selzures
- splnal cord ln[ury
x Drugs:
- canelne
- alcohol
x Lssentlal hypertenslon: slgnlñcantly assoclated wlth obeslty.
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1.25 Infective endocarditis
Answet A: lnlect|ve enJocotJ|t|s |s ossoc|oteJ w|th o 13-20¬mottol|ty tote
|nfectlve endocardltls (|L) ls deñned as an lnfectlon of the llnlng of the heart, partlcularly
anectlng the heart valves.
x Assoclated wlth 20% mortallty rate and conslderable morbldlty
x Most common organlsm ls 5tteµtococcus v|t|J|ons
x Others lnclude 5toµhylococcus outeus, lntetococcus sp. and 5tteµtococcus eµ|Jetm|J|s
(on prosthetlc valves).
x Leslons are usually left slded (except ln lntravenous drug abusers)
x Most cases have elther congenltal or acqulred heart defects
x |ncreased rlsk wlth lndwelllng central llnes and lntravenous drug abuse
x Almost all congenltal heart defects lncrease the rlsk of |L
x Leslons consldered of no lncreased rlsk lnclude:
- ostlum secundum ASD
- fully repalred PDA or vSD.
The cllnlcal manlfestatlons are often dlmcult and non-speclñc:
x Pever ln >80%
x Non-speclñc symptoms:
- fatlgue
- myalgla
- arthralgla
- welght loss
x 50% have skln manlfestatlons (caused by clrculatlng antlbody/antlgen complexes):
- 1aneway leslons are palnless haemorrhages on the soles and palms
- Osler nodes - palnful nodules ln the pads of the ñngers and toes
- spllnter haemorrhages ln nalls
x 50% have embollc phenomena, haematurla ls common
x Plnger clubblng ln chronlc cases
x Cardlac fallure from valve destructlon
x Murmur ls unlversal - elther a new murmur or change ln character of an exlstlng
murmur.
Dlagnosls ls on cllnlcal susplclon, posltlve blood culture (whlch may need to be done
repeatedly - three blood cultures have a 95% plck-up rate) and by the demonstratlon of
valvular vegetatlons on echocardlography. Treatment ls wlth long-term (4-6 weeks)
antlblotlcs. Surgery ls occaslonally requlred.
Prevenrien
x Good dental hyglene ls essentlal.
x N|CL (Natlonal |nstltute for Health and Cllnlcal Lxcellence) no longer recommends
prophylactlc antlblotlcs for routlne procedures.
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x Antlblotlcs to cover |L organlsms are recommended for a patlent already recelvlng
antlblotlc for a gastrolntestlnal or genltourlnary procedure who ls at rlsk of |L.
1.26 Cardiomyopathy
Answet 8: 0|loteJ cotJ|omyoµothy |s ossoc|oteJ w|th Joxotuo|c|n tox|c|ty |n ch|lJten
x Common lndlcatlon for cardlac transplantatlon
x 20% are lnherlted
x Peak lncldence ln lnfancy
x Second peak ln adolescence (neuromuscular dlsorders and autosomal domlnant
hypertrophlc cardlomyopathy or HCM).
Presenrnrien
x |ncreaslng enort lntolerance (feedlng/exerclse)
x |mpalred growth
x More rarely chest paln and palpltatlons
x Slgns lnclude dyspnoea, sweatlng, compromlsed perfuslon, tachycardla, tachypnoea,
hepatomegaly r a murmur
Ryµerrreµhic cnrJiemyeµnrhy (40%evernll}
x Symmetrlcal/asymmetrlcal ventrlcular hypertrophy wlthout underlylng structural
reasons
x Atrla are often enlarged.
x 60% are autosomal domlnant
x Translent form seen ln lnfants of mothers wlth dlabetes and lnfants on sterolds
x Syndromlc assoclatlons lnclude 8eckwlth-weldemann and Noonan syndromes
x Sudden death occurs ln 4-6% of cases/year
x Treatment wlth selectlve µ blockers
x Antl-arrhythmlcs and lmplantable deñbrlllators are used ln older chlldren.
ÐilnreJ cnrJiemyeµnrhy (ÐCM- 50%evernll}
x Large, globular heart wlth blventrlcular and blatrlal dllatatlon
x Mostly ldlopathlc
x Parer X-llnked adolescent presentatlon
x Other causes lnclude: rate related (due to prolonged tachycardla |SvT/vT|,
doxorublcln toxlclty, phaeochromocytoma, mltochondrlal dlsease, vltamln D
deñclency and carnltlne deñclency)
x Treatment wlth dluretlcs, ACL lnhlbltors, non-selectlve µ blockers and dlgoxln
x Lndocardlal ñbroelastosls ls a form rarely seen ln chlldhood.
Resrricrive (RCM- 3%evernll}
x Pare ln chlldhood
x Myocardlum unable to relax
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x Symptoms from venous congestlon ln the systemlc and pulmonary clrculatlons and
low cardlac output
x Medlan age of presentatlon of 36 months
x Commonly ldlopathlc
x Parer assoclatlons lnclude sarcoldosls, amyloldosls, haemochromatosls, Pabry dlsease
and LoeMer syndrome.
x Larly llstlng for cardlac transplantatlon ls necessary to avold lrreverslble pulmonary
artery damage.
Arrhyrhmegenic righr venrriculnr cnrJiemyeµnrhy
x Thls ls the least common type and ls exceptlonally rare ln young chlldren, usually
presentlng ln teenagers and young adults
x Plght ventrlcular dysfunctlon (reglonal or global) + ñbrofatty lnñltratlon of the rlght
ventrlcular myocardlum + LCG abnormalltles
x Cause of sudden death ln teenagers and young adults
x A genetlc cause has been ldentlñed ln some cases.
1.27 Rheumatic fever
Answet C: 7he most commonly occutt|nç mojot ct|tet|on lot J|oçnos|nç theumot|c levet |s
µolyottht|t|s
Pheumatlc fever develops secondary to lnfectlon wlth group A µ-haemolytlc 5tteµtococcus
sp. |t ls rare ln the developed world but ls estlmated to anect l.3 per l000 of all school-
aged chlldren ln developlng countrles. |t ls more common ln lower socloeconomlc classes
and the peak lncldence ls between the ages of 5 and l5.
Dlagnosls ls by the Duckett-1ones crlterla:
x two ma[or OP
x one ma[or and two mlnor crlterla
x PLUS evldence of recent streptococcal lnfectlon (ralsed ASO |antlstreptolysln O|
tltres, antl-deoxyrlbonuclease 8 or posltlve throat swab).
Ma[or crlterla:
x Cardltls: 50% (pancardltls anectlng the perlcardlum, myocardlum and endocardlum)
x Chorea: l5% (a late manlfestatlon - purposeless and lnvoluntary movements and
emotlonal lablllty, lasts >6 months but has no long-term neurologlcal sequelae)
x Polyarthrltls: 70% (mlgratory, lnvolves large [olnts, translent wlth no long-term
sequelae)
x Lrythema marglnatum: l0% (plnk rlngs on trunk and extensor surfaces of llmbs)
x Subcutaneous nodules: l%.
Mlnor crlterla:
x Arthralgla
x Pever
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x Prolonged PP lnterval on LCG
x Palsed erythrocyte sedlmentatlon rate (LSP) and C-reactlve proteln (CPP).
Pancardltls occurs ln 50%. As a consequence of thls l2.4%develop dysrhythmlas, and 6%
develop heart block, tachycardla, cardlomegaly, congestlve cardlac fallure and valve dlsease. |ts
sequelae lnclude mltral regurgltatlon, mltral stenosls, aortlc regurgltatlon and trlcuspld
regurgltatlon. Asplrln ls lndlcated ln the acute phase, lnltlally at l20 mg/kg for l4 days and then
70 mg/kg untll the fever settles. Prednlsolone ls lndlcated ln severe cardltls. Al0-day course of
penlclllln ls glven for eradlcatlon of streptococcl ln the ñrst lnstance and antlblotlc prophylaxls
should be contlnued long termas a 3-4 weekly lntramuscular dose of benzylpenlclllln. |t should
contlnue for l0 years ln those wlth cardltls or untll aged 2lyears (whlchever ls longer).
1.28 Innocent murmurs
Answet l: keossutonce thot the mutmut |s |nnocent onJ J|schotçe ltomlollow-uµ
|t ls essentlal that a paedlatrlclan be famlllar wlth the features of an lnnocent cardlac
murmur. |t ls not approprlate for all lnnocent murmurs to be seen ln the cardlology cllnlc
because up to 70% of chlldren may experlence lnnocent murmurs at some tlme ln
chlldhood. Cardlac referral and echocardlogram may aggravate parental anxlety even lf lt
ls reported as normal. However, lf the cllnlclan ls not conñdent of the dlagnosls of an
lnnocent murmur a referral to a cardlologlst ls essentlal.
Peatures of an lnnocent murmur:
x Locallsed
x Poorly conductlng
x Muslcal/vlbratory
x Soft grade l-2/6`
x Systollc`
x varles wlth posture
x Present ln hlgh-output states, e.g. febrlle lllness, durlng exerclse
x Cardlac examlnatlon otherwlse normal
x Chest radlograph, LCG normal.
`Lxcept venous hum.
A5rill´s murmur
Thls ls an early systollc murmur most commonly heard ln chlldren aged 2-6 years,
resolvlng towards adolescence. Grades l-3 present ln early systole, heart sounds normal.
Maxlmum lntenslty ls at lower left sternal edge. The murmur ls vlbratory and best heard
wlth the patlent nat, reduclng ln lntenslty when he or she slts up.
veneus hum
Thls ls a contlnuous murmur most commonly heard ln chlldren aged 2-6 years. The
dlastollc component ls usually loudest. |t ls best heard over the supraclavlcular fossa on the
rlght, wlth the head turned to the other slde. |t may radlate and ls often heard on both
sldes. |t dlsappears on lylng nat or lf the neck velns are compressed.
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Pulmennry ßewmurmur
Thls ls a very common murmur. Characterlstlcally lt ls brlef and ln mldsystole. |t ls loudest
wlth the patlent suplne and durlng explratlon. Occurs ln chlldren and adolescents of all
ages and ls louder durlng hyperdynamlc states such as fever and post exerclse. |nnocent
murmurs are commonly heard ln the neonatal perlod.
1.29 Cardiac emergencies
Answet 0: 5ynchtonous shocl ol 1 1/lç
5uµrnvenrriculnr rnchycnrJin
The most common cardlac arrhythmla ln the paedlatrlc age group ls SvT. |n lnfants the rate
ls usually >220 beats/mln but can be lower ln older chlldren. The QPS complex ls narrow.
Classlcally the onset ls abrupt. |t can last for a few mlnutes or several days, and ls tolerated
well by most chlldren, although the ma[orlty wlll develop cardlac fallure lf the arrhythmla
perslsts.
|f haemodynamlcally stable:
l. vagal stlmulatlon - faclal lmmerslon/unllateral carotld massage
2. Adenoslne lnto proximaI vein with ñush:
- ñrst dose, l00 Pg/kg
- second dose after 2 mln 200 Pg/kg
- thlrd dose 300 Pg/kg after 2 mln
- conslder 400-500 Pg/kg (>l month of age)
3. Synchronous DC shock or amlodarone or procalnamlde.
|f shock present:
l. vagal manoeuvres lf no delays
2. |f vascular access lmmedlately avallable glve adenoslne
3. |f not glve synchronous DC shock l 1/kg
4. Purther synchronlsed DC shock 2 1/kg
5. Conslder amlodarone
venrriculnr rnchycnrJin
|f haemodynamlcally stable: note do not delay therapeutlc lnterventlon for long
as vT can deterlorate lnto pulseless vT or vP.
Conslder underlylng causes:
x Congenltal heart dlsease and surgery
x Polsonlng: trlcycllc antldepressants, procalnamlde, macrollde antlblotlcs
x Penal dlsease or other causes of hyperkalaemla
x Long QT syndrome
x Urgent LCG analysls by senlor paedlatrlclan wlth experlence ln cardlology and seek
further advlce
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x Conslder amlodarone 5 mg/kg over 30 mln
x Conslder synchronous DC shock.
Lmergency treatment lf shock present:
x Assess lf pulse ls present, lf not treat as vP
x |f pulse present:
- synchronous DC shock, ln the ñrst lnstance l 1/kg
- further shocks should be asynchronous at 2 1/kg
- conslder a dose of amlodarone 5 mg/kg over a few mlnutes ln vT lf a chlld ls ln
severe shock.
1.30 WoIñ-Parkinson-White syndrome
Answet 0: koJ|oltequency oolot|on ol the occessoty µothwoy |s µotent|olly cutoole
Patlents wlth thls syndrome are prone to SvT from pre-excltatlon due to an anomalous
atrloventrlcular conductlon pathway bypasslng the [unctlonal tlssue. The accessory
pathway allows a clrcult to be formed, whlch facllltates a re-entry tachycardla.
The LCG characterlstlcs lnclude:
x shortened PP lnterval due to rapld anterograde conductlon
x prolonged QPS caused by premature actlon of the ventrlcle through the accessory
pathway, followed by normal depolarlsatlon through the Av node and the bundle of
Hls
x delta wave - slurrlng of the upstroke to the QPS complex.
woln-Parklnson-whlte syndrome (wPw) syndrome ls usually assoclated wlth a
structurally normal heart (70-80%). |t ls more common ln men (60-70%). There ls a
recognlsed assoclatlon wlth Lbsteln anomaly, corrected transposltlon and cardlomyopathy.
There ls a rlsk of sudden death of approxlmately l-4%.
Patlents may present wlth attacks of tachycardla leadlng to a spectrum of symptoms from
mlld chest dlscomfort or palpltatlons, sometlmes assoclated wlth syncope, to severe shock
assoclated wlth the SvT or cardlac arrest. Most patlents present wlth tachycardla at a rate
>250 beats/mln and hypotenslon. Cllnlcally crepltatlons ln lung bases may coexlst
secondary to pulmonary vascular congestlon due to rate assoclated cardlac compromlse.
Occaslonally patlents can present lncldentally when characterlstlc LCG appearances are
detected.
when a patlent ls known to have wPw syndrome, adenoslne should be used only wlth
extreme cautlon, havlng the means to perform cardloverslon/deñbrlllatlon lmmedlately
avallable due to the rlsk of deterloratlon of the rhythm to vP as a result of blockage of the
Av node and the posslblllty of a partlal or no enect on the accessory pathway. Dlgoxln ls
contralndlcated for the same reason. Approprlate antl-arrhythmlcs lnclude verapamll,
necalnlde and procalnamlde. Padlofrequency ablatlon of the accessory pathway ls a
potentlally curatlve procedure for patlents wlth wPw syndrome.
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1.31 Paediatric £CC
Answet A: 7he Pk |ntetvol |s µtolonçeJ |n hyµetloloem|o
The paedlatrlc LCG ls recorded at a rate of 25 mm/s.
7he axis of the QPS complex ls calculated by looklng at the total denectlon ln lead l and
AvP. The normal axls for a paedlatrlc LCG changes wlth age:
x Newborn: 90-l80º
x 2-5 years: 45-l35º
x > 5 years: l0-l00º.
Causes of a superlor axls lnclude:
x AvSD
x trlcuspld atresla
x Lbsteln anomaly
x Noonan syndrome
x wPw syndrome
x <l% of normal lndlvlduals.
7he PR intervaI varles wlth age, normally two to four small squares (0.08-0.l6 s):
x Upper llmlt of normal ln lnfants - 0.l4 s
x Upper llmlt of normal ln older chlldren - 0.l6 s.
Causes of a prolonged PP lnterval lnclude:
x AvSD
x atrloventrlcular canal defect
x Lbsteln anomaly
x myocardltls
x lschaemla
x hypothermla
x hyperkalaemla
x Duchenne muscular dystrophy
x dlgoxln
x qulnldlne.
Ç7 measurement ls made from the start of the Q wave to the end of the T wave. The QT ls
corrected for the rate as lt decreases wlth lncreased heart rate. QT corrected for rate (QTc) ¬
QT/ — PP ln seconds. The normal range ls consldered to be 0.35-0.43 s (8.75-l0.75 small
squares).
ÇR5 cemµlex
x Delta wave ln wPw syndrome ls an upslurrlng of P wave
x PS progresslon varles wlth age
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- neonate domlnant P ln vl, Domlnant s ln v6
- lnfant (l-l8 months) - domlnant P ln vl, domlnant P ln v6
- adult (>l8 months) - domlnant S ln vl, domlnant P ln v6
x Plght ventrlcular hypertrophy (PvH):
- uprlght T waves ln vl (abnormal from week l to l6 years)
- Q ln vl
- P waves >20 mm ln vl
x Left ventrlcular hypertrophy (LvH):
- lnverted T ln v6
- Q ln v6
- LAD
- P waves >20 mm ln v6.
1.32 Causes of Ç7 proIongation
Answet 8: ltythtomyc|n shoulJ oe ovo|JeJ |n µot|ents w|th µtolonçeJ Ç7 synJtome
x Prolonged QT (PQT) can be a congenltal or an acqulred phenomenon.
x QT prolongatlon can preclpltate vT and vP, whlch may lead to syncope, cardlac arrest
and sudden death.
x Torsades de polntes: a dlstlnctlve form of the ventrlcular tachyarrhythmla that ls
assoclated wlth PQT, lt can degenerate lnto vP. Many genetlc defects have been
ldentlñed lncludlng:
- autosomal domlnant - Pomano-ward syndrome
- autosomal recesslve - 1ervell-Lange-Nlelsen syndrome (assoclated wlth
congenltal deafness)
although only 50% of cases have a recognlsed genetlc abnormallty.
x Up to 30% of cases of sudden death secondary to PQT have no precedlng symptoms.
x The vast ma[orlty of patlents present ln lnfancy, chlldhood or early adult llfe.
x An unexplalned famlly death at <30 years of age may be ellclted ln the hlstory
x Cardlac events may be trlggered by exerclse, swlmmlng or emotlonal events but can
occur durlng sleep (genetlcs alter the predlsposltlon to certaln trlggers).
x QT (ln seconds) ls measured from the start of the Q wave untll the end of the T wave.
x Corrected QT: measured QT/—PP.
x >0.44 ls abnormal.
x Longer QT lntervals have been reported ln normal lnfants.
x A normal QTc ln a patlent's LCG does not exclude the dlagnosls.
x Pour to ñve per cent of cardlac events are fatal.
Management:
x µ 8lockers
x Left cardlac sympathetlc denervatlon
x Occaslonally pacemaker or lmplantable cardloverter-deñbrlllator devlce
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x Avoldance of drugs that prolong QT or decrease potasslum or magneslum levels
x Advlce to avold competltlve sports.
Sltuatlons that prolong the QT:
x hypocalcaemla
x hypomagnesaemla
x myocardltls
x central nervous system trauma
x drugs: clsaprlde, qulnldlne, terfenadlne, astemlzole, amlodarone, amltrlptyllne,
phenothlazlnes, antlmalarlals, erythromycln and trlmethoprlm.
1.33 Without fever there is no diagnosis
Answet 8: l|çh-çtoJe levet lot > 5 Joys
Kawasakl dlsease ls an acute self-llmltlng systemlc vasculltls wlth a predllectlon for the
coronary arterles. The dlagnosls ls cllnlcal. |t ls the leadlng cause of coronary vessel dlsease
ln chlldren ln the UK. Up to 25% (lf untreated) wlll progress to develop coronary artery
aneurysms, 20% of those wlll develop coronary artery stenosls and myocardlal lschaemla,
and may have a coronary thrombosls or dle suddenly (mortallty peaks 6 weeks post-fever
onset). Most common between 6 months and 5 years (peak 9-ll months).
Deñnltlon: fever of 5 days' duratlon wlth no obvlous underlylng cause plus at least four of
the followlng ñve (or fever, coronary aneurysms and three of the ñve):
x 8llateral con[unctlvltls wlthout exudate
x Oral changes (strawberry tongue, ñssurlng of the llps)
x Perlpheral extremlty changes (erythema of palms or soles, swelllng, desquamatlon of
ñngers or toes |l-2 weeks after the onset of fever|)
x Cervlcal adenopathy >l.5 cm - uncommon and may be unllateral
x Pleomorphlc rash (not veslcular) generallsed.
Systemlc lnvolvement ls common wlth lrrltablllty, neurologlcal compllcatlons, abdomlnal
paln, vomltlng, and small and large [olnt lnvolvement, l5% develop hydrops of the
gallbladder wlthln the ñrst 2 weeks.
|nnammatory markers (CPP and LSP) are ralsed and may take 6 weeks to normallse. A ralsed
w8C ln the acute phase (hlgh neutrophlls) ls common. The platelet count often rlses some
2 weeks lnto the lllness. Derangement of serum transamlnases occurs ln approxlmately
40% and mlld hyperblllrublnaemla ln l0%. There ls a sterlle pyurla ln l0% (urethrltls).
Treatment:
x Hlgh-dose asplrln untll fever subsldes, low dose for 6 weeks (or no evldence of
aneurysms on echo)
x |mmunoglobulln - reduces aneurysms lf glven ln the ñrst l0 days
x Ten per cent do not respond to |v|G and methyl prednlsolone or lncreaslngly
lnnlxlmab ls used
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1.34 Subacute bacteriaI endocarditis (Sߣ)
Answet A: 5µl|ntet hoemotthoçes
S8L ls a serlous systemlc lnfectlon wlth a cardlac focus. Underlylng cardlac abnormalltles
(of valves or septa) predlspose to, but are not an lmperatlve part of, the aetlology of thls
condltlon. Organlsms lnclude 5tteµtococcus v|t|Jons and 5toµhylococcus outeus. Cllnlcal
slgns lnclude spllnters, retlnal haemorrhages and changlng cardlac murmurs. Dlagnosls
rests on a posltlve blood culture (ln practlce at least three should be taken) and showlng
vegetatlons on an echo (not l00% sensltlve). Treatment conslsts of prolonged antlblotlc
course (6 weeks standard), valve replacement ln cases of lrreverslble damage and
(arguably) llfelong antlblotlc prophylaxls, although the N|CL guldellnes publlshed ln 2008
refute the value of glvlng routlne prophylaxls to any patlent wlth a condltlon that
predlsposes to S8L.
1.35 SV7
Answet 0: Oxyçen
Thls baby has SvT and ls compromlsed cardlovascularly. Thls ls more llkely to be due to a
re-entry phenomenon than a structural defect. As ln any cardloresplratory emergency, an
A8C approach ls the prlorlty followed by management to termlnate the dysrhythmla. vagal
manoeuvres such as lce packs, carotld slnus massage (hard ln a baby) and ocular pressure
may help to termlnate the dysrhythmla. Parenteral adenoslne glven rapldly ln escalatlng
doses ls the drug treatment of cholce lf vagal methods fall, followed by synchronous DC
shock. Pecurrent SvT may requlre longer-term antl-arrhythmlc treatment (dlgoxln,
necalnlde, propranolol or amlodarone). |n more severe cases, pathway ablatlon ls a ñnal,
usually successful, optlon.
1.36 Cyanotic congenitaI heart disease
Answet l: 7tonsµos|t|on ol the çteot ottet|es
Thls baby cllnlcally has cyanotlc congenltal heart dlsease. The key features are deep
cyanosls wlthout ma[or resplratory dlstress, lndlcatlve of a physlologlcal rlght-to-left shunt.
The most llkely dlagnosls ls TGA. Untll the patent ductus closes there may be no symptoms
or slgns, but, as the pulmonary-systemlc connectlon reaches crltlcal polnt, cyanosls
becomes overt. Tetralogy of Pallot, pulmonary atresla, trlcuspld atresla, anomalous
pulmonary venous dralnage and truncus arterlosus all glve rlse to a slmllar plcture but are
much less common. A vSD ls unllkely to cause symptoms at thls age and would manlfest as
heart fallure wlthout cyanosls. Perslstent fetal clrculatlon and dlaphragmatlc hernlas
present wlth a much 'slcker' baby who has resplratory slgns and wlthln the ñrst few hours,
lf not sooner.
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1.37 ßariumswaIIow
Answet 8: 8ot|umswollow
Although 'slmple' laryngomalacla ls by far the most common cause of early strldor (and
almost always self-resolvlng), the plcture ln thls case ls dlnerent. Thls chlld ls falllng to
thrlve (always slgnlñcant) and has a murmur suggestlng a vascular/aortlc rlng whlch ls best
elucldated by a barlum swallow because lt wlll compress the oesophagus. ¥ou would, of
course, when presented wlth thls cllnlcal scenarlo, requlre an expert cardlology revlew and
echocardlogram to evaluate the underlylng cardlac slgns. |n addltlon a resplratory or LNT
bronchoscoplc evaluatlon of the upper alrway may be helpful ln due course.
Causes of strldor:
x |ntrlnslc, e.g.
- laryngomalacla
- cord nodules/polyps
- haemanglomas of the cords
- cord palsles
- laryngeal nerve palsy
- subglottlc stenosls after prolonged lntubatlon
- laryngeal web
x Lxtrlnslc, e.g.
- vascular rlng
- rlght atrlal enlargement
- cystlc hygroma (obvlous)
- thyrold enlargement.
1.38 kawasaki disease
Answet C: l|çh-Jose osµ|t|n onJ µotentetol |mmunoçlooul|n
Thls chlld fulñls the crlterla for Kawasakl dlsease. The rash ls often confused wlth measles
(whlch was rare ln the UK but became more common agaln wlth the reductlon ln MMP
vacclne uptake). However, the extreme mlsery, desquamatlon and cervlcal nodes polnt ln
another dlrectlon. Once suspected, treatment should be started wlthout delay due to the
rlsk of a coronary artery aneurysm developlng or enlarglng wlth the lncreased duratlon of
the febrlle phase of the lllness. Meta-analyses suggest that hlgh-dose asplrln (50 mg/kg per
day ln dlvlded doses) untll the fever subsldes and a slngle dose of lmmunoglobulln (2 g
over l2 h l.v.) ls the best management pendlng echocardlography, and thls reglmen ls now
accepted practlce.
vltamln A at presentatlon ls routlne management of measles ln developlng country
settlngs and reduces mortallty by up to 50%.

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