Paediatric cardiology

Question 1

Cardiac problems in children (5)

Answer 1

Often have signs (murmurs) or symptoms without any underlying pathology Arrythmias
Congenital or Acquired heart disease
Cardiac complications of other diseases or systemic complications of cardiac disease

Question 2

Common cardiac signs in children

Answer 2

Murmurs Chest pain
Palpitations
Breathlessness on exertion – ‘Funny turns’

Question 3

Murmurs in children

Answer 3

NOT a condition, just a sign which is usually benign

V. Common, especially in children when there is increased CO (fever, anaemia, etc)

Question 4

Innocent murmurs in children

Answer 4

Venous hum at the upper right sternal edge
Mid-systolic murmu at lower or mid left sternal edge
Soft outflow murmurs bilaterally at the upper sternal edge

Question 5

Suspicious murmurs in children

Answer 5

Pan-systolic or diastolic murmurs, or murmurs at the apex

All murmurs are suspicious if there are other clinical signs

Question 6

Clinical signs of cardiac disease in children

Answer 6

Inspect - at rest?, colour, oedema, clubbing, scars, chest wall deformity
Palpate - rate and volume of pulse, are radial and femoral the same?
Auscultate - Heart sounds and murmurs (but not in isolation)

Question 7

Prevalence of congenital heart disease

Answer 7

6-8 per 1000 live births

Question 8

Types of congenital heart disease

Answer 8

Acyanotic –> Shunts and outflow obstructions

Cyanotic –> Complex syndromes, transpositions, DDPC/DDSC, Right>left shunts

Question 9

Acyanotic congenital heart disease

Answer 9

Shunts –> VSD, ASD, PDA - rarer –> AVSD, aortopulmonary window,
Obstructions –> Pulmonary stenosis, aortic stenosis & co-arctation of the aorta - rarer –> mitral stenosis, pulmonary vein stenosis, branch PA stenosis, Cor triatriatum or interuption of the aortic arch (full coarctation)

Question 10

Cyanotic congenital heart disease (3,3)

Answer 10

Tetralogy of Fallot, Transposition of the great arteries, hypoplastic left heart syndrome - rarer –> Tricuspid or pulmonary atresia, double inlet left ventricle, common arterial trunk

Question 11

Tetralogy of Fallot

Answer 11

Most common cyanotic congenital heart condition (4%) involving four components: (1) pulmonary outflow stenosis, (2) Overriding aorta which draws from both chambers due to a (3) VSD and this leads to (4) right ventricular hypertrophy

Question 12

Ventricular septal defects (VSD)

Answer 12

Most common congenital heart defect ~33% of defects
Vary in position –> inlet, outlet, perimembranous, muscular,
Most important element is the physiological effect which largely depends on the size of the defect

Question 13

Impact of a Small VSD

Answer 13

An asymptomatic murmur (pan-systolic at the LLSE), a small left>right shunt without heart enlargement and no symptoms – does not need treatment

Question 14

Impact of a Large VSD

Answer 14

A non-specific murmur (less turbulence as bigger hole), large left>right shunt and pulmonary HTN –> this can cause reversal of the shunt as pulmonary resistance increases (Eisenmenger phenomenon), cardiac dilation and heart failure

Question 15

Treatment of VSD

Answer 15

If small they often don’t require intervention if not symptoms
Diuretics or ACEis if moderate symptoms - 60% small/moderate close spontaneously
Large defects require Diuretics, ACEis, calorie supplements and ultimately surgery to treat

Question 16

Causes of Heart failure in children

Answer 16

Pump failure (cardiomyopathy, arrythmia, pulmonary HTN) or valve disease (rheumatic) - like in adults - but also Left>Right shunts (VSD, AVSD, etc)

Question 17

Signs & Symptoms of heart failure in children (3,6)

Answer 17

Symptoms - SOB, respiratory distress, FTT
Signs - Baby will look unwell, tachycardia & tachypnoea, breathless, hepatomegaly, heaves, pulmonary but not peripheral oedema

Question 18

Atrial septal defects (3,3)

Answer 18

6-8% of congenital heart defects - often asymptomatic but can get SOB on extertion and frequent chest infections
Signs – can have a parasternal heave, pulmonary flow murmur or a split S2

Question 19

Treatment of ASD

Answer 19

Non urgent but should be closed at some point – if untreated there is an increased risk of stroke or pulmonary HTN

Question 20

Persistant ductus arteriosis (PDA)

Answer 20

8-10% of congenital heart defects - especially in preterm babies
Can lead to heart failure symptoms and contribute to chronic lung disease
Can be heard as a continuous ‘machinery’ murmur at the left upper sternal edge

Question 21

Treatment of PDA

Answer 21

Either close surgically or by catheter occlusion (more common now)
Prostaglandins are responsible for keeping it open so NSAIDs can be used to help closure in premature infants

Question 22

Pulmonary stenosis

Answer 22

10% of congenital heart defects - mostly asymptomatic but if severe can limit exercise capacity - can present with a right ventricular heave and early systolic murmur at pulmonary area
Monitor and if symptomatic treat with balloon valvuloplasty

Question 23

Aortic stenosis

Answer 23

4% of congenital heart defects – symptoms depend on severity of obstruction
If mild - asymptomatic aortic murmur radiating to the carotids - if severe there is an inability to maintain CO and so baby has a ‘Duct dependent systemic circulation’

Question 24

Treatment of aortic stenosis

Answer 24

maintain in Duct dependent systemic circulation using prostaglandins until Balloon valvuloplasty or aortic valve replacement can be performed
Significant mortality

Question 25

Duct dependent systemic circulation (DDSC)

Answer 25

Left side of the heart is unable to maintain systemic circulation so a PDA is required so blood can enter the systemic circulation from the pulmonary artery – occurs in severe aortic stenosis, severe coarctation and hypoplastic left heart syndrome — must keep DA patent with prostaglandins

Question 26

Coarctation of the aorta

Answer 26

4% of congenital heart disease – a pinch in the aorta as it arches – when severe this leads to DDSC – Will have reduced or absent Femoral pulses, mid-systolic murmur at left mid sternal edge and between scapula and HTN
Treat with balloon, stenting or surgery

Question 27

Duct dependent Pulmonary circulation (DDPC)

Answer 27

Pulmonary atresia with or without VSD is main one

Similar treatment as DDSC

Question 28

Signs of Tetralogy of Fallot

Answer 28

Cyanosis can vary from minor to severe depending on the desaturation; often from 3-6 months.
Right ventricular heave from hypertrophy. Oligaemic lung fields from pulmonary stenosis. Early systolic murmur in pulmonary area — the bluer the child, the quiter the murmur
Associated with deletions from chr 22 and Digeorge syndrome

Question 29

Transposition of the Great arteries (TGA)

Answer 29

3-4% congenital heart defects - causes cyanosis (may be well but blue). Dextro-transposition is cyanotic as the right side pumps deoxygenated blood around the body and the left side is a closed circuit with the lungs. Must be surgically corrected within the first 2wks of life

Question 30

Hypoplastic left heart syndrome

Answer 30

2-3% of congenital heart defects – severe mitral or aortic stenosis or atresia leading to DDSC and fatal if not corrected within first few days - 20% mortality

Question 31

Causes of single ventricle circulations

Answer 31

Hypoplastic left heart syndrome, Tricuspid atresia, pulmonary atresia with intact septum –> managed for first two years and when pulmonary bed resistance is low enough the Fontan procedure may be attempted

Question 32

Acquired heart disease in children

Answer 32

Viral myocarditis (heart failure with raised trop), rheumatic fever, Infective endocarditis, Kawasaki disease (can cause coronary artery aneurysm)
1/5 mortality, 1/5 transplant rate

Question 33

Cardiac arrythmias in children (3,4)

Answer 33

Supraventricular - neonatal atrial flutter, ectopic atrial tachycardia, SVT
Ventricular - catecholamine induced VT, ARVC, Long QT syndrome
Congenital complete heart block

Question 34

Inherited cardiac diseases (3,2,2,1)

Answer 34

Cardiomyopathies - Dilated, hypertrophic, arrythmogenic
Channelopathies - long QT and Brugada syndrome
Connective tissue disorders - marfans or elos-danos syndrome
Familial hypercholesterolaemia

Question 35

Presentation of cyanotic heart conditions

Answer 35

TGA is less common but presents in the first few days while tetralogy of fallot presents in the first months.