Cardiovascular disease in childhood Flashcards
Acyanotic congenital heart diseases
ASD
VSD
AVSD
PDA
(increased pulmonary blood flow)
Cyanotic congenital heart diseases
tetralogy of fallot
tricuspid atresia
TGA
TAPVD (total Anomalous Pulmonary Venous Drainage
truncus (single, large blood vessel emerges from the heart instead of the usual two)
HLHS (Hypoplastic left heart syndrome)
(decreased pulmonary blood flow or mixed blood flow)
Clinical features suggestive of congenital heart disease
symptoms of heart failure
cyanosis, SaO2 <96%
abnormal pulses
hyperactive precordium
abnormal intensity/character of heart sounds
murmur >=3/6, harsh
diastolic, pansystolic or continuous murmurs
associated with ejection click
Neonatal heart disease modes of presentation
antenatal diagnosis
death
cyanosis
heart failure
abnormal neonatal examination - murmur, pulses
Signs and symptoms of heart failure in babies
poor feeding, exhaustion on feeding
tachypnoea, respiratory distress
sweating, clammy skin, poor perfusion
pallor, cyanosis
tachycardia, hyperactive precordium
gallop rhythm
oedema
hepatosplenomegaly
Name some acyanotic heart lesions
VSD
PDA
pulmonary stenosis
coarctation of aorta
ASD
aortic stenosis
AVSD
VSD clinical picture
depends on size of VSD and degree of left to right shunt
may present in heart failure
can be associated with other lesions
When is spontaneous closure of VSD more common
muscular VSDs
Types of VSD
supracristal
perimembranous
muscular
ASD clinical picture
usually asymptomatic - but may have sx of high pulmonary blood flow
widely split and often fixed 2nd heart sound
requires closure to prevent right heart failure and arrhythmias
ASD ECG
right axis deviation
mild right ventricular hypertrophy
RBBB with rsR’ pattern in V1
VSD ECG
left ventricular hypertrophy to biventricular hypertrophy
Commonest congenital heart lesion in Down’s syndrome
complete atrioventricular septal defect
Describe presentation and examination findings in AVSD
large left to right shunt –> early development of heart failure
right ventricular pressure never falls so murmur is ejection systolic across pulmonary valve
loud P2
repaired before 6 months
Coarctation of aorta presentation
neonatal collapse - circulatory collapse, resp distress, severe acidaemia
poor feeding + weight gain
SOB
hypertensive in arm
absent femoral pulses
ejection systolic murmur radiates to back
What is coarctation of aorta?
narrowing of aorta causing reduced blood flow to systemic circulation
Aortic coarctation management
PGE1 to reopen ducts
anti-failure treatment
inotropes
diuretics
needs urgent surgical resection and end-to-end anastomosis
Aortic valve stenosis presentation in newborn
cardiac failure +/- collapse
weak pulses
pale, cool skin
harsh ejection systolic murmur
ejection click with valvular AS
narrow pulse pressure
Aortic valve stenosis presentation in older child
angina
syncope
exertional dyspnoea/chest pain
mild stenosis generally worsens with time
Aortic stenosis management in children
balloon valvuloplasty
surgery
Pulmonary valve stenosis presentation
acyanotic
ejection systolic murmur radiating to axilla
often asymptomatic
TGA murmur
soft ejection or pansystolic murmur
single second heart sound
TGA xray sign
egg-shaped heart shadow
4 features of tetralogy of fallot
pulmonary stenosis
overriding aorta
ventricular septal defect
right ventricular hypertrophy
Tetralogy of fallot examination findings
harsh ejection systolic murmur and right ventricular heave
CXR tetralogy of fallot
boot-shaped heart
decreased pulmonary markings
Tet spell cause
(hypercyanotic spell)
muscle spasm below pulmonary valve leads to marked reduction in pulmonary blood flow
Tet spell presentation
cry
rapid and deep breathing
irritability
pallor
cyanosed
murmur disappears
Tet spell management
knees to chest or over your shoulder
supplemental oxygen and morphine (0.2mg/kg) to suppress resp centre and abolish hypercapnia
acidosis treatment with sodium bicarbonate
Down syndrome karyotype
47XY or 47XX
Down’s syndrome features
mental retardation/learning difficulties
developmental delay
floppiness
GI anomalies
conductive deafness
duodenal atresia
Hirschsprung’s
leukaemia
cardiac lesions - VSD, AVSD, ASD
Turner’s syndrome features
lymphoedema in infants
short stature
primary amenorrhoea
webbed neck
low hairline
low ears
dystrophic nails
horseshoes kidney
increased carrying angle (cubitus valgus)
Noonan syndrome heart defect
pulmonary valve stenosis
hypertrophic cardiomyopathy
Williams syndrome heart defect
supravalvular aortic stenosis
Digeorge syndrome heart defects
VSD, ASD, tetralogy of fallot
CHARGE syndrome features
Coloboma
heart defect
atresia (choanal)
retardation (mental)
genital
ear
ASD, VSD, mitral valve anomalies
VACTERL association features
Vertebral defects
Anal atresia
Cardiac abnormalities
Tracheo-
Esophageal fistula
Renal
Limb abnormalities
VSD, PDA, Tetralogy, TGA
Fetal alcohol syndrome features
IUGR
developmental delay
microcephaly
small maxilla
up-turned nose
smooth philtrum and upper lip
small eyes
prominent epicanthic folds
limb abnormalities
VSD or ASD
What is eisenmenger syndrome?
arises from a congenital heart defect, leading to pulmonary hypertension and a reversal of blood flow through the heart, ultimately resulting in cyanosis (bluish skin) and other symptoms
What is ebstein’s anomaly?
arises from a congenital heart defect, leading to pulmonary hypertension and a reversal of blood flow through the heart, ultimately resulting in cyanosis (bluish skin) and other symptoms
atrialisation of right ventricle
Where are pre-ductal sats measured in a neonate?
right hand
Where are post-ductal sats measured in a neonate?
either foot
What is normal for pre and post ductal sats in a neonate?
both pre and post >95% and less than or equal to 3% between readings
Why are pre and post ductal sats measured in a neonate?
as a screen for congenital heart disease
What pre and post ductal sats in a neonate would be abnormal and what should be done?
<95% in pre or post or more than 3% between pre and post readings
repeat in 1 hour
cardiovascular examination
arrange echo if still abnormal
if either reading <90% at any point –> admit to NICU
