Paediatric Cardiology Flashcards
Examples of diseases acquired in paediatric cardiology
Kawasaki
Rheumatic fever
Bacterial endocarditis
8 commonest lesions for congenital heart disease
Ventricular septal defect (VSD) Patent ductus arteriosus (PDA) Atrial septal defect (ASD) Pulmonary stenosis Aortic stenosis Coarctation of aorta Transposition of great arteries Teratology of fallot
Most common congenital heart disease
Ventricular septal defect
Causes of congenital heart disease
Genetic susceptibility (environmental hazard)
Teratogenic insult
- 18 - 20 days post conception
Environmental factors causing congenital heart disease
Drugs - alcohol - amphetamines - cocaine - ecstasy - Phenytoin - Lithium (anti depressants) Infections - Toxoplasma - Rubella - CMV - herpes Maternal DM - trophic cardiomyopathy SLE - antibodies can cross placenta and specifically attack the conduction system of the heart and destroy the his bundle and end up with congenital heart block
Most common environmental cause of congenital heart disease
Maternal DM
How many of all CHD have an underlying chromosomal problem?
6-10%
How many of chromosomal abnormalities have CHD?
30%
How many of trisomy 18 have CHD?
80%
What is trisomy 18 associated with?
VSD
PDA
What is trisomy 21 associated with?
AVSD
How many of trisomy 21 has CHD?
40%
What do all children with trisomys get screened for?
Heart disease
What is Turners syndrome associated with?
Co arctation of the aorta
Bicuspid aortic valve
What is noonans syndrome associated with?
Pulmonary stenosis
What is Williams syndrome associated with?
Supravalvular AS
Presentation of congenital heart disease
Cyanosis Tachypnoea Dyspnoea Exercise tolerance Chest pain / stitches Syncope Palpitation Joint problems
What is a presenting sign of coarctation of aorta?
Weak femoral pulse
What is a late presenting sign of coarctation of aorta, i.e. found in teenagers?
Radial femoral delay
Investigations for congenital heart defects
BP O2 sats, arterial BGA ECG CXR Echo Angiography Catheter MRI / A Exercise testing (ECG, sO2)
What is dextrocardia?
The heart is on the wrong side of the body
Palliative procedures used in congenital heart diseases
BT shunt
Balloon valvopathy
Prostaglandin infusion
Pulmonary banding
Different timings in cardiac cycles of murmurs
Systole
Diastole
Continous
Different duration in cardiac cycles of murmurs
Early Mid Late Ejection Holo Pan systolic
Different pitch / quality of murmurs
Harsh or mixed frequency (turbulence)
Soft or indeterminate
Vibratory / pure frequency (laminar flow)
What % of murmurs are innocent murmurs?
70-80%
What is an innocent murmur?
Nothing wrong with the heart, it is just making a noise
4 types of innocent murmurs
Still’s murmur (LV outflow murmur)
Primary outflow murmur
Carotid / Brachiocephalic arterial bruits
Venous Hum
Common features of innocent murmurs
Systolic murmur (exception - continuous in venous hum) No other signs of cardiac disease Quiet, soft murmur, grade 1/6 or 2/6 Vibratory, musical Localised Varies with - respiration - position - exercise
Grades of murmurs
1/6 / 2/6 = quiet and soft
3/6 = loud
5/6 = loud and thrill
6/6 = can hear from EOB without stethoscope
Innocent murmurs vary with…..
Position
respiration
exercise
What age gets still’s murmur?
2 - 5 y /o
Features of stills murmur
Vibratory
Musical
“Twangy”
Where is stills murmur found?
Apex, left sternal border
What does Stills murmur increase with?
Supine position
Exercise
What age gets primary outflow murmur?
8 - 10 years
Features of primary outflow murmur
Soft systolic
Vibratory
Where can you hear a primary outflow murmur?
Upper left sternal border
Well localised
Not radiating to back
Who is a primary outflow murmur often heard in?
Children with a narrow chest
What does primary outflow murmurs increase in….
Supine position
Exercise
What age gets carotid / brachiocephalic arterial bruits?
2-10 years
Features of carotid / brachiocephalic arterial bruits
1/6 - 2/6 systolic
harsh
Where can you hear carotid / brachiocephalic arterial bruits?
Supraclavicular
Radiates to neck
Carotid / brachiocephalic arterial bruits increase with….
Exercise
Carotid / brachiocephalic arterial bruits decrease with……
Turning head
Extending neck
What age do you get venous hums?
3 - 8 years
Features of venous hums
Soft
Indistinct
Continuous murmur
Sometimes with diastolic accentuation
Where can you hear venous hums?
Supraclavicular
When can you hear venous hums?
Only in upright position
When do venous hums disappear?
Lying down
Turning head
3 main types of VSD
Subaortic
Perimembranous
Muscular
What is a VSD?
Left to right shunt
Presentation of VSD
Pansystolic murmur lower left sternal edge (4 / 5th IC) sometimes with thrill
Very small VSDs = early systolic murmur
Very large VSDs = diastolic rumble due to relative mitral stenosis
Signs of cardiac failure / HF in large VSDs - eventually leading to biventricular hypertrophy and Pulmonary HTN
What is Eisenmenger syndrome?
Right to left shunt
What do you become with Eisenmenger syndrome?
Cyanotic
How do you close a VSD?
Amplatzer device
- if VSD small and in good position
- occlusion device - plug the hole and endocardium grows over it
Patch closure
- too big or in a position where cant fiddle around with catheter
- open heart surgery with cardiopulmonary bypass
What is there a good chance of with atrial septal defects?
Spontaneous closure
What can ASD present with in adulthood?
AF
HF
Pulmonary HTN
What do you hear with a ASD?
wide fixed splitting of 2nd heart sound
Pulmonary flow murmur
Treatment of ASD
Atrial septa defect occlusion device in situ
Atrioventricular septal defect or endocardial cushions defect or AV canal defect are associated with what?
Trisomy 21
Presentation of pulmonary stenosis
Mild = asymptomatic
Moderate / severe = exertional dyspnoea and fatigue
What do you hear in pulmonary stenosis?
Ejection systolic murmur upper left sternal border
Radiation to back
Loud then quiet again
Treatment of pulmonary stenosis
Balloon valvuloplasty
What is there a risk of when treating pulmonary stenosis with a balloon valvuloplasty?
Pulmonary regurgitation
Presentation of aortic stenosis
Mostly asymptomatic If severe - reduced exercise tolerance - exertional chest pain - syncope
What can you hear in aortic stenosis?
Ejection systolic murmur
Upper right sternal border
Radiation to carotids
Treatment of aortic stenosis
Balloon aortic valvuloplasty
What happens in a bicuspid aortic valve?
There are two parts to the valve instead of 3
Changes at birth to the foetal circulation
Pulmonary vascular resistance falls Pulmonary blood flow rises Systemic vascular resistance is increased Ductus arteriosus closes Foramen ovale closes Ductus venosus closes
In the foetus, what do veins carry?
Blood TOWARDS the heart
Who is patent ductus arteriosus common in?
Pre term infants
Treatment of patent ductus arteriosus
Fluid restriction / diuretics
Prostaglandin inhibitors - Indomethacin
Surgical ligation
In who is there a chance of spontaneous closure of PDA?
Term babies
What is coarctation of aorta?
Congenital narrowing of the aorta
Where is coarctation of the aorta usually found?
In the descending part of the arch
Treatment of coarctation of the aorta
Re-open PDA with prostaglandin E1 or E2
Resection with end to end anastomoses
- resection of the bit of the aorta that is kinked
Subclavian patch repair
Balloon aortoplasty
- balloon dilatation of the coarctated area
Pathology of transposition of the great arteries
Aorta comes out of your right ventricle and the pulmonary artery comes out of the left ventricle
Treatment of transposition of the great arteries
A shunt
If known antenatally - umbilical venous catheter to keep duct open
Rashkinds atrial septostomy
Features of fallot’s teratology
Pulmonary stenosis
Right ventricular hypertrophy
High VSD
Overriding aorta
What does fallot’s teratology lead to?
Cyanosis
Treatment of fallot’s teratology
Palliative shunt
Presentation of HF in children
Tachypnoea - cant feed / failure to thrive - cold sweat Tachycardia Hepatomegaly
Signs of PDA
Left subclavicular thrill Continuous 'machinery' murmur Large volume, bounding and collapsing pulse Wide pulse pressure Heaving apex beat
Example of the a prostaglandin used in the management of ductus arteriosus for duct dependent congenital heart disease
Alprostadil
Cyanotic CHD presenting within the first days of life indicates what?
Transposition of great arteries
Cyanotic CHD presenting at 1 - 2 months of age indicates what?
Teratology of fallot
What can Ebsteins anomaly be described as?
‘Atrialisation’ of the ventricle
Features of Ebsteins anomaly
Low insertion of tricuspid valve resulting in a large right atrium and a small right ventricle causing tricuspid incontinence
Associations of Ebsteins anomaly
Tricuspid incontinence
WPW syndrome
Exposure to what may cause Ebsteins anomaly?
Lithium in utero
What does a VSD increase the risk of?
Endocarditis
