Paediatric cardiology Flashcards
Cardiac problems in children (5)
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
Common cardiac signs in children
Murmurs Chest pain
Palpitations
Breathlessness on exertion – ‘Funny turns’
Murmurs in children
NOT a condition, just a sign which is usually benign
V. Common, especially in children when there is increased CO (fever, anaemia, etc)
Innocent murmurs in children
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
Suspicious murmurs in children
Pan-systolic or diastolic murmurs, or murmurs at the apex
All murmurs are suspicious if there are other clinical signs
Clinical signs of cardiac disease in children
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)
Prevalence of congenital heart disease
6-8 per 1000 live births
Types of congenital heart disease
Acyanotic –> Shunts and outflow obstructions
Cyanotic –> Complex syndromes, transpositions, DDPC/DDSC, Right>left shunts
Acyanotic congenital heart disease
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)
Cyanotic congenital heart disease (3,3)
Tetralogy of Fallot, Transposition of the great arteries, hypoplastic left heart syndrome - rarer –> Tricuspid or pulmonary atresia, double inlet left ventricle, common arterial trunk
Tetralogy of Fallot
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
Ventricular septal defects (VSD)
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
Impact of a Small VSD
An asymptomatic murmur (pan-systolic at the LLSE), a small left>right shunt without heart enlargement and no symptoms – does not need treatment
Impact of a Large VSD
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
Treatment of VSD
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
Causes of Heart failure in children
Pump failure (cardiomyopathy, arrythmia, pulmonary HTN) or valve disease (rheumatic) - like in adults - but also Left>Right shunts (VSD, AVSD, etc)
Signs & Symptoms of heart failure in children (3,6)
Symptoms - SOB, respiratory distress, FTT
Signs - Baby will look unwell, tachycardia & tachypnoea, breathless, hepatomegaly, heaves, pulmonary but not peripheral oedema
Atrial septal defects (3,3)
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
Treatment of ASD
Non urgent but should be closed at some point – if untreated there is an increased risk of stroke or pulmonary HTN
Persistant ductus arteriosis (PDA)
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
Treatment of PDA
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
Pulmonary stenosis
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
Aortic stenosis
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’
Treatment of aortic stenosis
maintain in Duct dependent systemic circulation using prostaglandins until Balloon valvuloplasty or aortic valve replacement can be performed
Significant mortality
Duct dependent systemic circulation (DDSC)
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
Coarctation of the aorta
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
Duct dependent Pulmonary circulation (DDPC)
Pulmonary atresia with or without VSD is main one
Similar treatment as DDSC
Signs of Tetralogy of Fallot
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
Transposition of the Great arteries (TGA)
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
Hypoplastic left heart syndrome
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
Causes of single ventricle circulations
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
Acquired heart disease in children
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
Cardiac arrythmias in children (3,4)
Supraventricular - neonatal atrial flutter, ectopic atrial tachycardia, SVT
Ventricular - catecholamine induced VT, ARVC, Long QT syndrome
Congenital complete heart block
Inherited cardiac diseases (3,2,2,1)
Cardiomyopathies - Dilated, hypertrophic, arrythmogenic
Channelopathies - long QT and Brugada syndrome
Connective tissue disorders - marfans or elos-danos syndrome
Familial hypercholesterolaemia
Presentation of cyanotic heart conditions
TGA is less common but presents in the first few days while tetralogy of fallot presents in the first months.
