congenital heart disease

Question 1

commonest causes of acyanotic congenital heart diseases

Answer 1

ventricular septal defects - commonest (30%)

atrial septal defects
patent ductus arteriosus
coarcation of aorta
aortic valve stenosis

Question 2

VSDs vs ASDs in kids vs adults

Answer 2

VSDs overall are more common

in adults -> ASDs more common new diagnosis, generally present later

Question 3

commonest causes of cyanotic congenital heart disease

Answer 3

tetralogy of fallot (commonest)

transposition of great arteries

tricuspid atresia

Question 4

presentation age of TGA vs tetralogy

Answer 4

TGA -> at birth

tetralogy -> 1-2months

Question 5

peripheral cyanosis in first 24hrs of life

Answer 5

v common, may occur when child is crying or unwell from any cause

Question 6

how is central cyanosis clinically recognised

Answer 6

conc of reduced Hb in blood >5g/dl

Question 7

test given to cyanotic neonates

Answer 7

nitrogen washout test (heperoxia test)
- used to differentiate between cardiac vs non-cardiac causes

given 100% o2 for 10mins, then ABGs taken
- pO2 <15 = cyanotic congen heart disease

Question 8

intial management of suspected cyanotic congenital heart disease

Answer 8

suportive care
prostaglandin E1 - elprostadil
- used to maintain patent ductus arteriosus in ductal dependant congen heart dis

(acts as holding measure until a definite diagnosis is made/surgical correction done)

Question 9

acrocyanosis

Answer 9

cyanosis around mouth + extremities - hanfs/feet
- seen in healthy newborns
- seen immediately after birth
- may persist for 25-48hrs

Question 10

what age does tetralogy of fallot typically present

Answer 10

around 1-2months

but may not be picked up until 6months

Question 11

4 characteristics of tetralogy of fallot

Answer 11

Pulmonary stenosis
Overriding Aorta
Ventricular Septal Defect (VSD)
Right ventricular hypertrophy

Question 12

which aspect of tetralogy of fallot alters the degree of clinical severity and cyanosis

Answer 12

right ventricular outflow tract obstruction (pulmonary stenosis)

Question 13

what are tet spells?

Answer 13

unrepaired tetra of fallot develop episodic hypercyanotic “tet” spells
- due to near occlusion of right ventricular outflow tract

px - tacypnoea, severe cyanosis, occasionally loss of consciousness

typically occur when upset, in pain or fever

Question 14

murmur heard in tetralogy of fallot

Answer 14

ejection systolic
- due to pulmonary stenosis

Question 15

Chest Xray + ECG of tetralogy of fallot

Answer 15

“boot-shaped” heart

ECG - ventricular hypertrophy, right axis deviation

Question 16

management of tetralogy of fallot

Answer 16

• surgical repair undertaken in 2 parts
• cyanotic episodes may be helped by beta-blockers to reduce infundibular spasm

Question 17

Transposition of the Great Arteries (TGA)

Answer 17

cyanotic congenital heart disease

failure of aorticopulmonary septum to spiral during septation

• aorta leaves right ventricle
• pulmonary trunk leaves left ventricle

Question 18

who is at increased risk of Transposition of the Great Arteries (TGA)?

Answer 18

kids of diabetic mums

Question 19

Transposition of the Great Arteries (TGA) presentation

Answer 19

cyanosis
tachypnoea
loud single S2
prominent right ventricular impulse

egg on side appearance on CXR

Question 20

management of Transposition of the Great Arteries (TGA)

Answer 20

maintenance of ductus arteriosus with prostaglandins (prostaglandin E1 - elprostadil)

definitive mx = surgical correction

Question 21

ebsteins anomaly

Answer 21

low insertion of tricuspid valve resulting in a large atrium + small ventricle

= atrialisation of right ventricle

Question 22

what can cause ebsteins anomaly and other assoc conditions

Answer 22

exposure to lithium in utero

Assoc
- patent foramen ovale or ASD in 80% -> right to left shunt
- wolff-parkinson white
- supraventricular tachycardia

Question 23

ebsteins anomaly features

Answer 23

cyanosis
prominent “a” wave in distended jugular venous pulse

tricuspid regurg - pansystolic murmur, worse on inspiration

right bundle branch block -> widely split S1 + S2

may be asymptomatic until adolescence/adult

Question 24

ebsteins anomaly investigation + management

Answer 24

ix = echo

medical = mx arrythmias + heart failure

surgical = tricuspid valve repair/replacement

Question 25

patent ductus arteriosus

Answer 25

acyanotic - if uncorrected can result in late cyanosis of lower extemeties connection between pulmonary trunk + ascending aorta - usually closes with first breaths

Question 26

which babies is PDA commoner

Answer 26

premature born at high altitude maternal rubella infection in 1st tri

Question 27

patent ductus arteriosus features

Answer 27

left subclavicular thrill continuous machinery murmur large volume, bounding, collapsing pulse wide pulse pressure heaving apex beat

Question 28

patent ductus arteriosus management in preterm infants

Answer 28

preterm infants if ventilator dependant after 1 week = pharma closure - ibuprofen, indomethacin or paracetamol --> (inhibits prostaglandin synthesis)

Question 29

indication for closure management of patent ductus arteriosus in term infants + kids

Answer 29

- moderate or large PDA - prior episode of endocarditis - small audible PDA

Question 30

management of patent ductus arteriosus in term infants + kids

Answer 30

transcatheter PDA closure (NOT pharma management, not effective in term infants)

Question 31

coarctation of aorta

Answer 31

congenital narrowing of the descending aorta - more common in males, despite assoc with Turners syndrome

Question 32

coarctation of aorta associations

Answer 32

- turners - bicuspid aortic valve - berry aneurysms - neurofibromatosis

Question 33

coarctation of aorta features

Answer 33

infancy - heart failure adults - hypertension **radio-femoral delay** **mid-systolic murmur, maximal over back** apical click from aortic valve **notching of ribs** - due to collateral vessels (NOT seen in young)`