102433730-Ern-Mrcpch

Essential Revision Notes

in Paediatrics

for theMRCPCH

Secondedition

Contents

Contributors vii

Preface to the Second edition xi

Preface to the First edition xii

1. Cardiology 1Robert Tulloh

2. Child Development, Child Psychiatry and Community Paediatrics 65Joanne Philpot and Ruth Charlton

3. Clinical Governance 131Robert Wheeler

4. Clinical Pharmacology and Toxicology 143Steve Tomlin and Michael Capra

5. Dermatology 163Helen M Goodyear

6. Emergency Paediatrics 195Serena Cottrell

7. Endocrinology 229Heather Mitchell and Vasanta Nanduri

8. Ethics and Law 281Vic Larcher and Robert Wheeler

9. Gastroenterology and Nutrition 307R M Beattie

10. Genetics 353Louise Wilson

11. Haematology and Oncology 379Michael Capra

12. Hepatology 427Nancy Tan and Anil Dhawan

v

13. Immunology 469Waseem Qasim and Bobby Gaspar

14. Infectious Diseases 497Katy Fidler, Nigel Klein and Karyn Moshal

15. Metabolic Medicine 559Michael P Champion

16. Neonatology 601Grenville F Fox

17. Nephrology 659Christopher J D Reid

18. Neurology 711Neil H Thomas

19. Ophthalmology 769William H Moore and Ken K Nischal

20. Orthopaedics 821Vel K Sakthivel and N M P Clarke

21. Respiratory 843Jane C Davies

22. Rheumatology 889Nathan Hasson

23. Statistics 919Angie Wade

24. Surgery 937Merrill McHoney, Vivien McNamara and Robert Wheeler

Picture permissions 975

Index 977

vi

Contents

Chapter 1

CardiologyRobert Tulloh

CONTENTS

1. Diagnosis of congenital heart disease 51.1 Fetal cardiology1.2 Epidemiology1.3 Cardiac anatomy1.4 Nomenclature for sequential segmental arrangement1.5 Examination technique1.6 Innocent murmurs

2. Basic cardiac physiology 152.1 Physiology of adaptation to extrauterine life2.2 Physiology of congenital heart disease2.3 Physiology of heart muscle and heart rate

3. Left to right shunt 173.1 Atrial septal defect (ASD)3.2 Ventricular septal defect (VSD)3.3 Persistent ductus arteriosus (PDA)3.4 Aortopulmonary window3.5 Others

4. Right to left shunt 224.1 Tetralogy of Fallot4.2 Transposition of the great arteries4.3 Pulmonary atresia4.4 Ebstein anomaly4.5 Eisenmenger

5. Mixed shunt 265.1 Complete atrioventricular septal defects5.2 Tricuspid atresia5.3 Others

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6. Obstruction in the well child 286.1 Aortic stenosis6.2 Pulmonary stenosis6.3 Adult-type coarctation of the aorta6.4 Vascular rings and slings

7. Obstruction in the sick newborn 307.1 Coarctation of the aorta7.2 Hypoplastic left heart syndrome7.3 Critical aortic stenosis7.4 Interruption of the aortic arch7.5 Total anomalous pulmonary venous connection

8. Non-bypass surgery for congenital heart disease 358.1 Shunt operation8.2 Coarctation of the aorta repair8.3 Pulmonary artery band8.4 Arterial duct ligation

9. Bypass surgery for congenital heart disease 369.1 Switch operation9.2 Fontan9.3 Norwood9.4 Rastelli9.5 Other operations

10. Syndromes in congenital heart disease 3810.1 Isomerism10.2 Trisomy10.3 William syndrome10.4 Noonan syndrome10.5 Di George syndrome10.6 Alagille syndrome10.7 Turner syndrome10.8 VACTERL10.9 Holt–Oram/Thrombocytopenia and Absent Radius (TAR)/Fanconi syndromes10.10 CHARGE10.11 Pentalogy of Cantrell10.12 Dextrocardia10.13 Other syndromes

11. Syncope in childhood 44

12. Pulmonary hypertension 4512.1 Persistent pulmonary hypertension of the newborn12.2 Increased pulmonary blood flow12.3 Chronic hypoxia12.4 Pulmonary venous hypertension

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Essential Revision Notes in Paediatrics for the MRCPCH 2nd Edition

13. Drug therapy for congenital heart disease 4813.1 Heart failure13.2 Anticoagulation13.3 Pulmonary hypertension13.4 Antiarrhythmia

14. Acquired heart disease 4914.1 Kawasaki disease14.2 Dilated cardiomyopathy14.3 Hypertrophic cardiomyopathy14.4 Suspected bacterial endocarditis14.5 Rheumatic fever14.6 Pericarditis

15. ECG 5515.1 The ECG and how to read it15.2 Tachycardias15.3 Bradycardias

16. Chest X-rays 6016.1 Cardiac outlines

17. Cardiac catheterization 6117.1 Diagnostic cardiac catheterization17.2 Interventional cardiac catheterization

18. Imaging 6318.1 Echocardiography18.2 Magnetic resonance imaging18.3 Positron emission tomography18.4 Radionuclear angiography

19. Further reading 63

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Cardiology

Cardiology

1. DIAGNOSIS OF CONGENITAL HEART DISEASE

1.1 Fetal cardiology

Diagnosis

In the south of England, most children (. 70%) who require infant surgery for congenitalheart disease (CHD) are diagnosed during pregnancy at 16–20 weeks’ gestation. This givesa significant advantage to the parents who are counselled by specialists who can give arealistic guide to the prognosis and treatment options. A few undergo termination ofpregnancy (depending on the diagnosis). Most continue with the pregnancy and can beoffered delivery within the cardiac centre if there could be neonatal complications or iftreatment is likely to be needed within the first 2 days of life. Surgical intervention duringfetal life is not yet routinely available.

Screening (by a fetal cardiologist) is offered to those with:

• Abnormal four-chamber view on routine-booking, antenatal-anomaly ultrasound scan• Increased nuchal translucency (thickness at back of the neck), which also increases the

risk of Down syndrome• Previous child with or other family history of CHD• Maternal risk factors, such as phenylketonuria or diabetes• Suspected Down, or other, syndrome

Important normal findings on fetal echocardiography include echodensities:

• Used to be called ‘Golf-balls’• Found on anterior mitral valve papillary muscle• Thought to be calcification during development• No importance for CHD• Positive association with Down syndrome• Do not need echocardiogram after delivery

Arrhythmias

• Diagnosed at any time during pregnancy: an echocardiogram is required to confirmnormal anatomy and to confirm type of arrhythmia. Fetal electrocardiogram (ECG) is notyet a routine investigation

• Multiple atrial ectopics are usually not treated• Supraventricular tachycardia is usually treated with maternal digoxin or flecainide

5

• Heart block may be treated with maternal isoprenaline or salbutamol• Presence of hydrops is a poor prognostic sign

1.2 Epidemiology of congenital heart disease

Eight per 1,000 live births have CHD, of which the commonest are:

• Ventricular septal defect 30%• Persistent arterial duct 12%• Atrial septal defect 7%• Pulmonary stenosis 7%• Aortic stenosis 5%• Coarctation of the aorta 5%• Tetralogy of Fallot 5%• Transposition of the great arteries 5%• Atrioventricular septal defect 2%

Incidence is increased by a positive family history, so the proportion of live births with CHDwill be:

• Previous sibling with CHD 2%• Two siblings with CHD 4%• Father with CHD 3%• Mother with CHD 6%

Incidence also increased by

• Presence of other anomaly or syndrome• Parents with an abnormal genotype• Maternal ingestion of lithium (Ebstein anomaly)• Third-trimester enterovirus or coxsackievirus infection (myocarditis, dilated

cardiomyopathy)• Maternal systemic lupus erythematosus (anti-ro, anti-la antibodies leading to congenital

heart block)

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1.3 Cardiac anatomy

Right

Atrium

Left

Atrium

PV

TV

IVC

Right

Ventricle

Left

Ventricle

SVCPulmonary

ArteryPulmonary

Veins

Aorta

PV 5 Pulmonary valve

SVC 5 Superior vena cava

TV 5 Tricuspid valve

IVC 5 Inferior vena cava

Normal heart

1.4 Nomenclature for sequential segmental arrangement

The European (as opposed to American) system for complete heart diagnosis is referred toas ‘sequential segmental arrangement’. The advantage is that it is no longer necessary toremember the pattern of an eponymous syndrome. The disadvantage is that it is quite long-winded. The idea is that each component is described in turn:

Atrial arrangement (atrial situs)

• Usual (solitus)• Mirror image (inversus)• Right isomerism (asplenia syndrome)• Left isomerism (polysplenia syndrome)

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Cardiology

Atrioventricular (AV) connection

Type of atrioventricular connection

• Biventricular• Concordant• Discordant• Ambiguous (with atrial isomerism)

• Univentricular• Absent left AV connection• Absent right AV connection• Double inlet AV connection

Mode of atrioventricular connection

• Two AV valves• Common AV valve• Straddling right or left AV valve• Imperforate right or left AV valve• Overriding right or left AV valve

Ventricular topology

• Right-hand (normal) or left-hand topology

Ventriculoarterial connection

Type of ventriculoarterial connection

• Concordant• Discordant• Double outlet• Single outlet:

• Common arterial trunk• Solitary arterial trunk• With pulmonary atresia• With aortic atresia

Mode of ventriculoarterial connection

• Two perforate valves• Left or right imperforate valve

Infundibular morphology

Arterial relationships

Associatedmalformations

• Position of heart in the chest — left, right or middle• Systemic and pulmonary veins

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• Atrial septum• Atrioventricular valves• Ventricular septum• Semilunar valves• Anomalies of great arteries (e.g. double aortic arch)

Surgical or interventional procedures

Acquired or iatrogenic lesions

1.5 Examination technique

To many candidates the diagnosis of congenital heart disease is daunting. Certainly, if thecandidate examines the child, listens to the heart and then tries to make a diagnosis, thiswill prove difficult. The following system should be used instead.

History

The history-taking is short and to the point. The candidate needs to know:

• Was the child born preterm?• Are there any cardiac symptoms of:

• Heart failure (breathlessness, poor feeding, faltering growth, cold handsand feet)

• Cyanosis• Neonatal collapse

• Is it an asymptomatic heart murmur found on routine examination?• Is there a syndrome such as Down syndrome?• Is there any family history of congenital heart disease?• Did the mother have any illnesses or take any medication during pregnancy?

Examination

• Introduce yourself to mother and patient. Ask if you can examine the child.• Position child according to age:

• For a 6-year-old — at an angle of 45 degrees• For a toddler — upright on mother’s knee• For a baby — flat on the bed

• Remove clothes from chest• Stand back and look for:

• Dysmorphism• Intravenous infusion cannula• Obvious cyanosis or scars

The following examinations sbould be performed.

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Cardiology

Heart failure

The delivery of oxygen to the peripheral vascular bed is insufficient to meet the metabolicdemands of the child. Usually because of left to right shunt with good heart pump function.

• A thin, malnourished child (Faltering growth)• Excessive sweating around the forehead• Tachycardia• Breathlessness +/– subcostal or intercostal recession• Poor peripheral perfusion with cold hands and feet• A large liver• Never found with ventricular septal defect (VSD) or other left to right shunt in first week

of life• An emergency if found up to 7 days of age. Implies a duct-dependent lesion, e.g.

hypoplastic left heart syndrome or coarctation

Cyanosis

• Mild cyanosis is not visible — use the pulse oximeter

Clubbing

• Visible after 6 months old• First apparent in the thumbs or toes• Best demonstrated by holding thumbs together, back to back to demonstrate loss of

normal nail-bed curvature• Disappears a few years after corrective surgery

Pulse

• Rate (count for 6 seconds 3 10)• Rhythm (only ‘regular’ or ‘irregular’, need ECG for ‘sinus rhythm’)• Character at the antecubital fossa with the elbows straight, using the thumbs — on both

arms together

Head and neck

• Anaemia — for older children only — ask the patient to look up and examine theconjunctivae (not appropriate in a baby)

• Cyanosis — the tongue should be examined for central cyanosis. If in doubt ask thechild to stick out their tongue and ask the mother to do the same. This will detectoxygen saturations of , 85%

• Jugular venous pressure — the head is turned towards the candidate so that the otherside of the neck (the left side) can be seen with the jugular venous pressure visible,outlined against the pillows. In a child who is under 4 years, the jugular venous pressureshould not be assessed

• Carotid thrill — essential part of the examination, midway up the left side of the neck,felt with the thumb, proof of the presence of aortic stenosis

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Precordium

Inspection

• Respiratory rate• Median sternotomy scar (¼ open heart surgery — see Section 9)• Lateral thoracotomy scar (Blalock–Taussig (BT) shunt, patent ductus arteriosus (PDA)

ligation, pulmonary artery (PA) band, coarctation repair)• Additional scars, e.g. on the abdomen

Palpation

• Apex beat ‘the most inferior and lateral position where the index finger is lifted by theimpulse of the heart’. Place fingers along the fifth intercostal space of both sides of chest(for dextrocardia) and count down apex position only if patient is lying at 45 degrees

• Left ventricular heave• Right ventricular heave at the left parasternal border• Thrills at upper or lower left sternal edge

Auscultation

• Heart sounds and their character• Additional sounds• Murmurs, their character, intensity and where they are best heard

Heart sounds

First heart sound is created by closure of the mitral and then tricuspid valves. It is notimportant for the candidate to comment on the nature of the first heart sound.

Second heart sound, however, is more important, created by closure of first the aortic andthen the pulmonary valves.

• Loud pulmonary sound — pulmonary hypertension• Fixed splitting of second sound (usually with inspiration the sounds separate and then

come together during expiration). Listen when patient is sitting up, at the mid-left sternaledge in expiration• Atrial septal defect• Right bundle-branch block

• Single second sound in transposition of great arteries (TGA), pulmonary atresia, orhypoplastic left heart syndrome

• Quiet second sound may occur in pulmonary valve stenosis or pulmonary artery band

Additional soundsAdded sounds present may be a normal third or fourth heart sound heard in the neonate orthese sounds can be pathological, for example in a 4-year-old with a dilated cardiomyo-pathy and heart failure. An ejection click is heard at aortic valve opening, after the firstheart sound, and is caused by a bicuspid aortic valve in most cases.

11

Cardiology

Murmurs

Before listening for any murmurs, the candidate should have a good idea of the type ofcongenital heart disease, which is being dealt with. The candidate should know whetherthe child is blue (and therefore likely to have tetralogy of Fallot) or is breathless (likely tohave a left to right shunt) or has no positive physical findings before auscultation of themurmurs (and therefore more likely to either be normal, have a small left to right shunt ormild obstruction). By the time the murmurs are auscultated, there should only be two orthree diseases to choose between, with the stethoscope being used to perform the finetuning. It is best to start at the apex with the bell, and move to the lower left sternal edgewith the diaphragm. Then on to the upper left sternal edge and upper right sternal edgeboth with the diaphragm. Additional areas can be auscultated, but provide little additionalinformation. Murmurs are graded out of 6 for systolic, 1 ¼ very soft, 2 ¼ soft, 3 ¼ moderate,4 ¼ loud with a thrill, 5 ¼ heard with a stethoscope off the chest, 6 ¼ heard as you enterthe room. Murmurs are out of 4 for diastolic, again 2, 3 and 4.

Ejection systolic murmurUpper sternal edge — implies outflow tract obstruction. Right or left ventricular outflowtract obstruction can occur at valvar (+ ejection click), subvalvar or supravalvar level.

• Upper right sternal edge (carotid thrill) ¼ Aortic stenosis• Upper left sternal edge (no carotid thrill) ¼ Pulmonary stenosis or atrial

septal defect (ASD)• Mid/lower left sternal edge ¼ Innocent murmur (see below)• Long harsh systolic murmur + cyanosis ¼ Tetralogy of Fallot

Pansystolic murmur

• Left lower sternal edge (+/– thrill ) ¼ VSD• Apex (much less common) ¼ Mitral regurgitation• Rare at left lower sternal edge (+/– cyanosis) ¼ Tricuspid regurgitation

(Ebstein anomaly)

Continuous murmur

• Left infraclavicular (+/– collapsing pulse) ¼ Persistent arterial duct• Infraclavicular (+ cyanosis + lateral thoracotomy) ¼ BT shunt• Any site (lungs, shoulder, head, hind-quarter) ¼ Arteriovenous fistula

Diastolic murmurs

• Unusual in childhood• Left sternal edge/apex (+/– carotid thrill or VSD) ¼ Aortic regurgitation• Median sternotomy (+/– PS (pulmonary stenosis) murmur)

¼ Tetralogy of Fallot, repaired• Apical (+/– VSD) ¼ Mitral flow/(rarely stenosis)

NB Listening to the back gives little diagnostic information, but is useful thinking time.

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Presentation of ¢ndings

Few candidates pay enough attention to the case presentation. This should be done after theexamination is complete. The candidate should stand, look the examiner in the eye, puthands behind his/her back and present. The important positives and negatives should bestated quickly and succinctly with no ‘umms’ or ‘errrs’. It is important to judge the mood ofthe examiner, if he/she is looking bored, then go faster. Practise with a tape recorder orvideo-recording.

To complete the examination you would:

• Measure the blood pressure• Measure the oxygen saturation• Feel the femoral pulses• Feel the liver edge

The presentation should be rounded off with the phrase ‘the findings are consistent with thediagnosis of . . .’.

13

Cardiology

Patient

Cyanosed Acyanosed

Femoral Pulses

No YesCoarctation

No other pulses

Critical Aortic StenosisHLHS

Absent Left CarotidInterrupted Aortic Arch

YesCarotid thrill AS

No

Apex in left chestNoYes

YesPrecordial thrill VSD or PS

No

Murmur

Yes No Normal Heart

?Kartagener with

normal heart

?Abnormal heart

Murmur

Yes No

Fallot TGA VSD

Mitral regurgitation

(AVSD is not visibly

cyanosed and may have

apical murmur of left AV

valve regurgitation)

Split S2 ASD

Not split PS

AS 1 Carotid thrill

Valvar AS or PS

Sub- or supra valvar

Pulmonary hypertension

Left

Apex

Left

Right

Loud

PSM Lower S.E.

ESM Upper S.E.

Ejection click

No ejection click

Pulmonary sound

HLHS

L

AS

VSD

PS

TGA

ASD

PSM

ESM

AVSD

AV

hypoplastic left heart syndrome

Left

aortic stenosis

ventricular septal defect

pulmonary stenosis

transposition of great arteries

atrial septal defect

Pan Systolic murmur

ejection systolic murmur

atrioventricular septal defect

atrioventricular

KEY

Algorithm for clinical examination

The patient with surgical scars:

• Left lateral thoracotomy• PA band Thrill + ejection systolic murmur at upper

left sternal edge• Coarctation +/– left brachial pulse• Shunt Blue + continuous murmur• PDA No signs

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• Right lateral thoracotomy• Shunt Blue + continuous murmur

• Median sternotomy• Any intracardiac operation

1.6 Innocent murmurs

The commonest murmur heard in children is the functional, innocent or physiological heartmurmur (40% of all children). They are often discovered in children with an intercurrentinfection or with anaemia. These all relate to a structurally normal heart but can cause greatconcern within the family. There are several different types depending on the possible siteof their origin. It is clearly important to make a positive diagnosis of a normal heart. Themurmur should be:

• Soft (no thrill)• Systolic• Short, never pansystolic• ASymptomatic• Left Sternal edge

It may change with posture.

Innocent murmurs do not require antibiotic prophylaxis.

Diastolic murmurs are not innocent.

An innocent murmur is not associated with abnormal or added heart sounds.

Types of innocent murmur include:

• Increased flow across branch pulmonary artery — this is frequently seen in pretermneonates, is a physiological finding and resolves as the pulmonary arteries grow. Themurmur disappears after a few weeks of age, and never causes symptoms

• Still’s murmur — this is vibratory in nature and is found at the mid-left sternal edge. Itmay be caused by turbulence around a muscle band in the left ventricle

• Venous hum — it may be easy to hear the venous blood flow returning to the heart,especially at the upper sternal edge. This characteristically occurs in both systole anddiastole and disappears on lying the child flat

2. BASIC CARDIAC PHYSIOLOGY

2.1 Physiology of adaptation to extrauterine life

During the adaptation from fetal life there are a number of changes in the normal child:

• A fall in the pulmonary vascular resistance, rapidly in the first few breaths, but thiscontinues until 3 months of age

• A resultant fall in the pulmonary arterial pressure• Loss of the placenta from the circulation

15

Cardiology

• Closure of the ductus venosus• Closure of the ductus arteriosus• Closure of the foramen ovale

The arterial duct is kept patent with prostaglandins E1 or E2 infusion in children with duct-dependent circulation such as transposition of the great arteries, or pulmonary atresia.

2.2 Physiology of congenital heart disease

The main principles of congenital heart disease are

• The pressure on the left side of the heart is usually higher than that on the right• Any communication between atria, ventricles or great arteries leads to a left to right

shunt• Pulmonary vascular resistance falls over the first 12 weeks of life, increasing the shunt• There will only be cyanosis if the desaturated blood shunts from the right to left side• Common mixing leads to cyanosis and breathlessness• Duct-dependent conditions usually present at 2 days of life• Prostaglandin E2 or E1 can be used to reopen the duct up to about 2 weeks of life

2.3 Physiology of heart muscle and heart rate

Arterial pulse volume depends on stroke volume and arterial compliance.

• Small pulse volume in• Cardiac failure• Hypovolaemia• Vasoconstriction

• Large pulse volume in• Vasodilatation• Pyrexia• Anaemia• Aortic regurgitation• Hyperthyroid• CO2 retention

• Pulsus paradoxus• Exaggeration of normal rise and fall of blood pressure with respiration, seen in

airways obstruction, such as asthma• Sinus arrhythmia

• Variation of the normal heart rate with respiration. Faster in inspiration and slower inexpiration. Can be very marked in children

Cardiac output is increased by• Adrenergic stimulus• Increased stretch (Starling’s curve)• Increased preload• Reduced afterload

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3. LEFT TO RIGHT SHUNT

(Pink +/– breathless)

General principles

No signs or symptoms on first day of life because of the high pulmonary vascular resistance.Later, at 1 week, infant can develop symptoms and signs of heart failure.

Symptoms of heart failure

• Tachypnoea• Poor feeding, Faltering growth• Cold hands and feet• Sweating• Vomiting

Signs of heart failure

• Thin• Tachypnoea• Displaced apex• Dynamic precordium• Apical diastolic murmur• Hepatomegaly

3.1 Atrial septal defect (ASD)

Types of defect

• Secundum ASD• Primum ASD (partial atrioventricular septal defect)• Sinus venosus ASD• Other

SecundumASD

A defect in the centre of the atrial septum involving the fossa ovalis.

Clinical features

• Asymptomatic• 80% of ASDs• Soft systolic murmur at upper left sternal edge• Fixed split S2 (difficult to hear)

ECG

• Partial right bundle-branch block (90%)• Right ventricle hypertrophy

17

Cardiology

Chest X-ray

• Increased pulmonary vascular markings

Management

• Closure at 3–5 years (ideally)• 90% undergo device closure in catheter laboratory• 10% undergo surgical closure (too large or personal preference)

Partial atrioventricular septal defect (PrimumASD)

A defect in the lower atrial septum, involving the left atrioventricular valve which has threeleaflets and tends to leak.

Clinical features

• Asymptomatic• 10% of ASDs• Soft systolic murmur at upper left sternal edge• Apical pansystolic murmur (atrioventricular valve regurgitation)• Fixed split S2 (difficult to hear)

ECG

• Partial right bundle-branch block (90%)• Right ventricle hypertrophy• Superior axis

Chest X-ray

• Increased pulmonary vascular markings

Management

• Closure at 3–5 years• All require surgical closure (because of the need to repair valve)

Sinus venosus ASD

A defect at the upper end of the atrial septum, such that the superior vena cava (SVC)overrides the atrial septum. The right pulmonary veins are usually anomalous and draindirectly into the SVC or right atrium adding to the left to right shunt.

Clinical features

• Asymptomatic or heart failure• 5% of ASDs• Soft systolic murmur at upper left sternal edge• Fixed split S2 (easily heard)

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ECG

• Partial right bundle-branch block• Right ventricle hypertrophy

Chest X-ray

• Increased pulmonary vascular markings• Cardiomegaly

Management

• Closure at 1–5 years• All require surgical closure and repair to the anomalous pulmonary veins

There are other rare types of ASD, which are similarly treated.

3.2 Ventricular septal defect (VSD)

Small defect

A defect anywhere in the ventricular septum (perimembranous or muscular, can be inlet oroutlet). Restrictive defects are smaller than the aortic valve. There is no pulmonary hyper-tension.

Clinical features

• Asymptomatic (80–90%)• May have a thrill at left lower sternal edge• Loud pansystolic murmur at lower left sternal edge (the louder the murmur, the smaller

the hole)• Quiet P2

ECG

• Normal

Chest X-ray

• Normal

Management

• Review with echocardiography• Spontaneous closure, but may persist to adult life

Large defect

Defects anywhere in the septum. Large defects tend to be the same size or larger than theaortic valve. There is always pulmonary hypertension.

19

Cardiology

Clinical features

• Symptomatic with heart failure after age 1 week• 10–20% of VSDs• Right ventricular heave• Soft or no systolic murmur• Apical mid-diastolic heart murmur• Loud P2

ECG

• Biventricular hypertrophy by 2 months (see Section 15 — ECG)

Chest X-ray

• Increased pulmonary vascular markings• Cardiomegaly

Management

• Initial medical therapy, diuretics +/– captopril + added calories• Surgical closure at 3–5 months

3.3 Persistent ductus arteriosus (PDA)

There is persistence of the duct beyond 1 month after the date the baby should have beenborn.

Clinical features

• Asymptomatic usually, rarely have heart failure• Continuous or systolic murmur at left infraclavicular area

ECG

• Usually normal• If large, have left ventricle volume loading (see Section 15 — ECG)

Chest X-ray

• Usually normal• If large, have increased pulmonary vascular markings

Management

• Closure in cardiac catheter laboratory with coil or plug at 1 year• If large, surgical ligation age 1–3 months

NB The presence of an arterial duct in a preterm baby is not congenital heart disease. Ifthere is a clinical problem, with difficulty getting off the ventilator, or signs of heart failure

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with bounding pulses, the problem is usually treated with indomethacin or ibuprofen(, 34 weeks). If medical management fails, surgical ligation is undertaken.

3.4 Aortopulmonary window

A defect in the wall between the aorta and pulmonary artery.

Clinical features

• Rare• Usually develop heart failure• Continuous murmur as for PDA

ECG

• If large, have left ventricle volume loading (see Section 15 — ECG)

Chest X-ray

• If large, have increased pulmonary vascular markings

Management

• If large, surgical ligation age 1–3 months

3.5 Others

There are other rare causes of significant left to right shunt, such as arteriovenousmalformation. These are all individually rare. Medical and surgical treatment is similar tothat for large ducts or VSDs.

Summary

Disease Symptoms Treatment

ASD Minimal Surgery/catheter device at 3–5 yearsVSD None None (in 80–90% of cases)

Moderate Diuretics/captopril/added caloriesthen review early

Severe Surgery at 3–5 months (10–20%cases)

PDA None Coil occlusion at cardiac catheter (at1 year old)

Mod/severe Surgery, especially in pretermbabies

Others rare (A-P window, etc.) Surgery at 3–4 months

21

Cardiology

102433730-Ern-Mrcpch

Documents

essential revision notes

sequential segmental arrangement

mrcpch 2nd edition

congenital heart disease

sinus venosus asd

ventricular septal defect

left sternal edge

require surgical closure