Essential Revision Notes in Paediatrics for the MRCPCH Second edition

Contents
Contributors Preface to the Second edition Preface to the First edition 1. 2. 3. 4. 5. 6. 7. 8. 9. Cardiology Robert Tulloh Child Development, Child Psychiatry and Community Paediatrics Joanne Philpot and Ruth Charlton Clinical Governance Robert Wheeler Clinical Pharmacology and Toxicology Steve Tomlin and Michael Capra Dermatology Helen M Goodyear Emergency Paediatrics Serena Cottrell Endocrinology Heather Mitchell and Vasanta Nanduri Ethics and Law Vic Larcher and Robert Wheeler Gastroenterology and Nutrition R M Beattie vii xi xii 1 65 131 143 163 195 229 281 307 353 379 427

10. Genetics Louise Wilson 11. Haematology and Oncology Michael Capra 12. Hepatology Nancy Tan and Anil Dhawan

Contents

13. Immunology Waseem Qasim and Bobby Gaspar 14. Infectious Diseases Katy Fidler, Nigel Klein and Karyn Moshal 15. Metabolic Medicine Michael P Champion 16. Neonatology Grenville F Fox 17. Nephrology Christopher J D Reid 18. Neurology Neil H Thomas 19. Ophthalmology William H Moore and Ken K Nischal 20. Orthopaedics Vel K Sakthivel and N M P Clarke 21. Respiratory Jane C Davies 22. Rheumatology Nathan Hasson 23. Statistics Angie Wade 24. Surgery Merrill McHoney, Vivien McNamara and Robert Wheeler Picture permissions Index

469 497 559 601 659 711 769 821 843 889 919 937 975 977

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Chapter 1 Cardiology
Robert Tulloh

CONTENTS
1. Diagnosis of congenital heart disease 1.1 Fetal cardiology 1.2 Epidemiology 1.3 Cardiac anatomy 1.4 Nomenclature for sequential segmental arrangement 1.5 Examination technique 1.6 Innocent murmurs Basic 2.1 2.2 2.3 cardiac physiology Physiology of adaptation to extrauterine life Physiology of congenital heart disease Physiology of heart muscle and heart rate 5

2.

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3.

Left to right shunt 3.1 Atrial septal defect (ASD) 3.2 Ventricular septal defect (VSD) 3.3 Persistent ductus arteriosus (PDA) 3.4 Aortopulmonary window 3.5 Others Right 4.1 4.2 4.3 4.4 4.5 to left shunt Tetralogy of Fallot Transposition of the great arteries Pulmonary atresia Ebstein anomaly Eisenmenger

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4.

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5.

Mixed shunt 5.1 Complete atrioventricular septal defects 5.2 Tricuspid atresia 5.3 Others

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Essential Revision Notes in Paediatrics for the MRCPCH 2nd Edition

6.

Obstruction in the well child 6.1 Aortic stenosis 6.2 Pulmonary stenosis 6.3 Adult-type coarctation of the aorta 6.4 Vascular rings and slings Obstruction in the sick newborn 7.1 Coarctation of the aorta 7.2 Hypoplastic left heart syndrome 7.3 Critical aortic stenosis 7.4 Interruption of the aortic arch 7.5 Total anomalous pulmonary venous connection Non-bypass surgery for congenital heart disease 8.1 Shunt operation 8.2 Coarctation of the aorta repair 8.3 Pulmonary artery band 8.4 Arterial duct ligation Bypass surgery for congenital heart disease 9.1 Switch operation 9.2 Fontan 9.3 Norwood 9.4 Rastelli 9.5 Other operations

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7.

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8.

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9.

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10. Syndromes in congenital heart disease 38 10.1 Isomerism 10.2 Trisomy 10.3 William syndrome 10.4 Noonan syndrome 10.5 Di George syndrome 10.6 Alagille syndrome 10.7 Turner syndrome 10.8 VACTERL 10.9 HoltOram/Thrombocytopenia and Absent Radius (TAR)/Fanconi syndromes 10.10 CHARGE 10.11 Pentalogy of Cantrell 10.12 Dextrocardia 10.13 Other syndromes 11. Syncope in childhood 12. Pulmonary hypertension 12.1 Persistent pulmonary hypertension of the newborn 12.2 Increased pulmonary blood ow 12.3 Chronic hypoxia 12.4 Pulmonary venous hypertension 44 45

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13. Drug 13.1 13.2 13.3 13.4

therapy for congenital heart disease Heart failure Anticoagulation Pulmonary hypertension Antiarrhythmia

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14. Acquired heart disease 14.1 Kawasaki disease 14.2 Dilated cardiomyopathy 14.3 Hypertrophic cardiomyopathy 14.4 Suspected bacterial endocarditis 14.5 Rheumatic fever 14.6 Pericarditis 15. ECG 15.1 The ECG and how to read it 15.2 Tachycardias 15.3 Bradycardias 16. Chest X-rays 16.1 Cardiac outlines 17. Cardiac catheterization 17.1 Diagnostic cardiac catheterization 17.2 Interventional cardiac catheterization 18. Imaging 18.1 Echocardiography 18.2 Magnetic resonance imaging 18.3 Positron emission tomography 18.4 Radionuclear angiography 19. Further reading

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1.

DIAGNOSIS OF CONGENITAL HEART DISEASE

1.1 Fetal cardiology

Diagnosis In the south of England, most children (. 70%) who require infant surgery for congenital heart disease (CHD) are diagnosed during pregnancy at 1620 weeks gestation. This gives a signicant advantage to the parents who are counselled by specialists who can give a realistic guide to the prognosis and treatment options. A few undergo termination of pregnancy (depending on the diagnosis). Most continue with the pregnancy and can be offered delivery within the cardiac centre if there could be neonatal complications or if treatment is likely to be needed within the rst 2 days of life. Surgical intervention during fetal 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 ndings on fetal echocardiography include echodensities:

Used to be called Golf-balls Found on anterior mitral valve papillary muscle Thought to be calcication 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 conrm

normal anatomy and to conrm type of arrhythmia. Fetal electrocardiogram (ECG) is not yet a routine investigation Multiple atrial ectopics are usually not treated Supraventricular tachycardia is usually treated with maternal digoxin or ecainide

Essential Revision Notes in Paediatrics for the MRCPCH 2nd Edition

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 Persistent arterial duct Atrial septal defect Pulmonary stenosis Aortic stenosis Coarctation of the aorta Tetralogy of Fallot Transposition of the great arteries Atrioventricular septal defect

30% 12% 7% 7% 5% 5% 5% 5% 2%

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

Previous sibling with CHD Two siblings with CHD Father with CHD Mother with CHD

2% 4% 3% 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

Aorta Pulmonary Artery PV Right Atrium TV Left Atrium

PV SVC TV IVC

5 5 5 5

Pulmonary valve Superior vena cava Tricuspid valve Inferior vena cava

SVC

Pulmonary Veins

IVC Right Ventricle Left Ventricle

Normal heart

1.4

Nomenclature for sequential segmental arrangement

The European (as opposed to American) system for complete heart diagnosis is referred to as sequential segmental arrangement. The advantage is that it is no longer necessary to remember the pattern of an eponymous syndrome. The disadvantage is that it is quite longwinded. 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)

Essential Revision Notes in Paediatrics for the MRCPCH 2nd Edition

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 Associated malformations

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 the candidate examines the child, listens to the heart and then tries to make a diagnosis, this will prove difcult. 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 hands and 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 mothers knee For a baby at 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.

Essential Revision Notes in Paediatrics for the MRCPCH 2nd Edition

Heart failure The delivery of oxygen to the peripheral vascular bed is insufcient to meet the metabolic demands 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 rst 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 the
conjunctivae (not appropriate in a baby)

Cyanosis the tongue should be examined for central cyanosis. If in doubt ask the
child to stick out their tongue and ask the mother to do the same. This will detect oxygen saturations of , 85% Jugular venous pressure the head is turned towards the candidate so that the other side 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 pressure should 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 ( open heart surgery see Section 9) Lateral thoracotomy scar (BlalockTaussig (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 nger is lifted by the
impulse of the heart. Place ngers along the fth 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 not important for the candidate to comment on the nature of the rst heart sound. Second heart sound, however, is more important, created by closure of rst the aortic and then 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 sternal edge in expiration Atrial septal defect Right bundle-branch block Single second sound in transposition of great arteries (TGA), pulmonary atresia, or hypoplastic left heart syndrome Quiet second sound may occur in pulmonary valve stenosis or pulmonary artery band Additional sounds Added sounds present may be a normal third or fourth heart sound heard in the neonate or these sounds can be pathological, for example in a 4-year-old with a dilated cardiomyopathy and heart failure. An ejection click is heard at aortic valve opening, after the rst heart sound, and is caused by a bicuspid aortic valve in most cases.

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Essential Revision Notes in Paediatrics for the MRCPCH 2nd Edition

Murmurs Before listening for any murmurs, the candidate should have a good idea of the type of congenital heart disease, which is being dealt with. The candidate should know whether the child is blue (and therefore likely to have tetralogy of Fallot) or is breathless (likely to have a left to right shunt) or has no positive physical ndings before auscultation of the murmurs (and therefore more likely to either be normal, have a small left to right shunt or mild obstruction). By the time the murmurs are auscultated, there should only be two or three diseases to choose between, with the stethoscope being used to perform the ne tuning. It is best to start at the apex with the bell, and move to the lower left sternal edge with the diaphragm. Then on to the upper left sternal edge and upper right sternal edge both with the diaphragm. Additional areas can be auscultated, but provide little additional information. Murmurs are graded out of 6 for systolic, 1 very soft, 2 soft, 3 moderate, 4 loud with a thrill, 5 heard with a stethoscope off the chest, 6 heard as you enter the room. Murmurs are out of 4 for diastolic, again 2, 3 and 4. Ejection systolic murmur Upper sternal edge implies outow tract obstruction. Right or left ventricular outow tract obstruction can occur at valvar (+ ejection click), subvalvar or supravalvar level.

Upper right sternal edge (carotid thrill) Upper left sternal edge (no carotid thrill) Mid/lower left sternal edge Long harsh systolic murmur + cyanosis
Pansystolic murmur

Aortic stenosis Pulmonary stenosis or atrial septal defect (ASD) Innocent murmur (see below) Tetralogy of Fallot

Left lower sternal edge (+/ thrill ) Apex (much less common) Rare at left lower sternal edge (+/ cyanosis)
Continuous murmur

VSD Mitral regurgitation Tricuspid regurgitation (Ebstein anomaly)

Left infraclavicular (+/ collapsing pulse) Infraclavicular (+ cyanosis + lateral thoracotomy) Any site (lungs, shoulder, head, hind-quarter)
Diastolic murmurs

Persistent arterial duct BT shunt Arteriovenous stula

Unusual in childhood Left sternal edge/apex (+/ carotid thrill or VSD) Aortic regurgitation Median sternotomy (+/ PS (pulmonary stenosis) murmur) Apical (+/ VSD)
Tetralogy of Fallot, repaired Mitral ow/(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 the examination is complete. The candidate should stand, look the examiner in the eye, put hands behind his/her back and present. The important positives and negatives should be stated quickly and succinctly with no umms or errrs. It is important to judge the mood of the examiner, if he/she is looking bored, then go faster. Practise with a tape recorder or video-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 ndings are consistent with the diagnosis of . . ..

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Essential Revision Notes in Paediatrics for the MRCPCH 2nd Edition

Patient Cyanosed Acyanosed Femoral Pulses No Coarctation No other pulses Critical Aortic Stenosis HLHS Absent Left Carotid Interrupted Aortic Arch Carotid thrill No ?Kartagener with normal heart ?Abnormal heart Murmur Yes Fallot No TGA Yes PSM Lower S.E. ESM Upper S.E. No Apex in left chest Yes Precordial thrill No Murmur No Yes Yes Yes

KEY 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

AS

VSD or PS

Normal Heart 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 Ejection click No ejection click Pulmonary sound

Loud

Algorithm for clinical examination The patient with surgical scars:

Left lateral thoracotomy

PA band Coarctation Shunt PDA No signs

Thrill + ejection systolic murmur at upper left sternal edge +/ left brachial pulse Blue + continuous murmur

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Cardiology

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 heart murmur (40% of all children). They are often discovered in children with an intercurrent infection or with anaemia. These all relate to a structurally normal heart but can cause great concern within the family. There are several different types depending on the possible site of their origin. It is clearly important to make a positive diagnosis of a normal heart. The murmur 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 ow across branch pulmonary artery this is frequently seen in preterm

neonates, is a physiological nding and resolves as the pulmonary arteries grow. The murmur disappears after a few weeks of age, and never causes symptoms Stills murmur this is vibratory in nature and is found at the mid-left sternal edge. It may be caused by turbulence around a muscle band in the left ventricle Venous hum it may be easy to hear the venous blood ow returning to the heart, especially at the upper sternal edge. This characteristically occurs in both systole and diastole and disappears on lying the child at

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 rst few breaths, but this
continues until 3 months of age

A resultant fall in the pulmonary arterial pressure Loss of the placenta from the circulation
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Essential Revision Notes in Paediatrics for the MRCPCH 2nd Edition

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 ductdependent 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 rst 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 in expiration. Can be very marked in children

Cardiac output is increased by Adrenergic stimulus Increased stretch (Starlings curve) Increased preload Reduced afterload

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3.

LEFT TO RIGHT SHUNT

(Pink +/ breathless) General principles No signs or symptoms on rst 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

Secundum ASD 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 (difcult to hear)

ECG

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

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Essential Revision Notes in Paediatrics for the MRCPCH 2nd Edition

Chest X-ray

Increased pulmonary vascular markings

Management

Closure at 35 years (ideally) 90% undergo device closure in catheter laboratory 10% undergo surgical closure (too large or personal preference)
Partial atrioventricular septal defect (Primum ASD) A defect in the lower atrial septum, involving the left atrioventricular valve which has three leaets 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 (difcult to hear)

ECG

Partial right bundle-branch block (90%) Right ventricle hypertrophy Superior axis
Chest X-ray

Increased pulmonary vascular markings

Management

Closure at 35 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 drain directly into the SVC or right atrium adding to the left to right shunt. Clinical features

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

Cardiology

ECG

Partial right bundle-branch block Right ventricle hypertrophy

Chest X-ray

Increased pulmonary vascular markings Cardiomegaly

Management

Closure at 15 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 or outlet). Restrictive defects are smaller than the aortic valve. There is no pulmonary hypertension. Clinical features

Asymptomatic (8090%) 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 the aortic valve. There is always pulmonary hypertension.

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Essential Revision Notes in Paediatrics for the MRCPCH 2nd Edition

Clinical features

Symptomatic with heart failure after age 1 week 1020% 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 35 months 3.3 Persistent ductus arteriosus (PDA)

There is persistence of the duct beyond 1 month after the date the baby should have been born. 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 13 months
NB The presence of an arterial duct in a preterm baby is not congenital heart disease. If there is a clinical problem, with difculty 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 13 months 3.5 Others

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

Summary Disease
ASD VSD

Symptoms
Minimal None Moderate Severe

Treatment
Surgery/catheter device at 35 years None (in 8090% of cases) Diuretics/captopril/added calories then review early Surgery at 35 months (1020% cases) Coil occlusion at cardiac catheter (at 1 year old) Surgery, especially in preterm babies Surgery at 34 months

PDA

None Mod/severe

Others rare (A-P window, etc.)

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