Cardiovascular disease in childhood

Question 1

Acyanotic congenital heart diseases

Answer 1

ASD
VSD
AVSD
PDA

(increased pulmonary blood flow)

Question 2

Cyanotic congenital heart diseases

Answer 2

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)

Question 3

Clinical features suggestive of congenital heart disease

Answer 3

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

Question 4

Neonatal heart disease modes of presentation

Answer 4

antenatal diagnosis
death
cyanosis
heart failure
abnormal neonatal examination - murmur, pulses

Question 5

Signs and symptoms of heart failure in babies

Answer 5

poor feeding, exhaustion on feeding
tachypnoea, respiratory distress
sweating, clammy skin, poor perfusion
pallor, cyanosis
tachycardia, hyperactive precordium
gallop rhythm
oedema
hepatosplenomegaly

Question 6

Name some acyanotic heart lesions

Answer 6

VSD
PDA
pulmonary stenosis
coarctation of aorta
ASD
aortic stenosis
AVSD

Question 7

VSD clinical picture

Answer 7

depends on size of VSD and degree of left to right shunt
may present in heart failure
can be associated with other lesions

Question 8

When is spontaneous closure of VSD more common

Answer 8

muscular VSDs

Question 9

Types of VSD

Answer 9

supracristal
perimembranous
muscular

Question 10

ASD clinical picture

Answer 10

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

Question 11

ASD ECG

Answer 11

right axis deviation
mild right ventricular hypertrophy
RBBB with rsR’ pattern in V1

Question 12

VSD ECG

Answer 12

left ventricular hypertrophy to biventricular hypertrophy

Question 13

Commonest congenital heart lesion in Down’s syndrome

Answer 13

complete atrioventricular septal defect

Question 14

Describe presentation and examination findings in AVSD

Answer 14

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

Question 15

Coarctation of aorta presentation

Answer 15

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

Question 16

What is coarctation of aorta?

Answer 16

narrowing of aorta causing reduced blood flow to systemic circulation

Question 17

Aortic coarctation management

Answer 17

PGE1 to reopen ducts
anti-failure treatment
inotropes
diuretics
needs urgent surgical resection and end-to-end anastomosis

Question 18

Aortic valve stenosis presentation in newborn

Answer 18

cardiac failure +/- collapse
weak pulses
pale, cool skin
harsh ejection systolic murmur
ejection click with valvular AS
narrow pulse pressure

Question 19

Aortic valve stenosis presentation in older child

Answer 19

angina
syncope
exertional dyspnoea/chest pain
mild stenosis generally worsens with time

Question 20

Aortic stenosis management in children

Answer 20

balloon valvuloplasty
surgery

Question 21

Pulmonary valve stenosis presentation

Answer 21

acyanotic
ejection systolic murmur radiating to axilla
often asymptomatic

Question 22

TGA murmur

Answer 22

soft ejection or pansystolic murmur
single second heart sound

Question 23

TGA xray sign

Answer 23

egg-shaped heart shadow

Question 24

4 features of tetralogy of fallot

Answer 24

pulmonary stenosis
overriding aorta
ventricular septal defect
right ventricular hypertrophy

Question 25

Tetralogy of fallot examination findings

Answer 25

harsh ejection systolic murmur and right ventricular heave

Question 26

CXR tetralogy of fallot

Answer 26

boot-shaped heart decreased pulmonary markings

Question 27

Tet spell cause

Answer 27

(hypercyanotic spell) muscle spasm below pulmonary valve leads to marked reduction in pulmonary blood flow

Question 28

Tet spell presentation

Answer 28

cry rapid and deep breathing irritability pallor cyanosed murmur disappears

Question 29

Tet spell management

Answer 29

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

Question 30

Down syndrome karyotype

Answer 30

47XY or 47XX

Question 31

Down's syndrome features

Answer 31

mental retardation/learning difficulties developmental delay floppiness GI anomalies conductive deafness duodenal atresia Hirschsprung's leukaemia cardiac lesions - VSD, AVSD, ASD

Question 32

Turner's syndrome features

Answer 32

lymphoedema in infants short stature primary amenorrhoea webbed neck low hairline low ears dystrophic nails horseshoes kidney increased carrying angle (cubitus valgus)

Question 33

Noonan syndrome heart defect

Answer 33

pulmonary valve stenosis hypertrophic cardiomyopathy

Question 34

Williams syndrome heart defect

Answer 34

supravalvular aortic stenosis

Question 35

Digeorge syndrome heart defects

Answer 35

VSD, ASD, tetralogy of fallot

Question 36

CHARGE syndrome features

Answer 36

Coloboma heart defect atresia (choanal) retardation (mental) genital ear ASD, VSD, mitral valve anomalies

Question 37

VACTERL association features

Answer 37

Vertebral defects Anal atresia Cardiac abnormalities Tracheo- Esophageal fistula Renal Limb abnormalities VSD, PDA, Tetralogy, TGA

Question 38

Fetal alcohol syndrome features

Answer 38

IUGR developmental delay microcephaly small maxilla up-turned nose smooth philtrum and upper lip small eyes prominent epicanthic folds limb abnormalities VSD or ASD

Question 39

What is eisenmenger syndrome?

Answer 39

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

Question 40

What is ebstein's anomaly?

Answer 40

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

Question 41

Where are pre-ductal sats measured in a neonate?

Answer 41

right hand

Question 42

Where are post-ductal sats measured in a neonate?

Answer 42

either foot

Question 43

What is normal for pre and post ductal sats in a neonate?

Answer 43

both pre and post >95% and less than or equal to 3% between readings

Question 44

Why are pre and post ductal sats measured in a neonate?

Answer 44

as a screen for congenital heart disease

Question 45

What pre and post ductal sats in a neonate would be abnormal and what should be done?

Answer 45

<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