Congenital Heart Conditions

Question 1

What are the most common causes of Acyanotic Congenital heart disease

Answer 1

ventricular septal defects (VSD) - most common, accounts for 30%
atrial septal defect (ASD)
patent ductus arteriosus (PDA)
coarctation of the aorta
aortic valve stenosis

Question 2

VSDs are more common than ASDs

Answer 2

true

However, in adult patients ASDs are the more common new diagnosis as they generally presents later.

Question 3

What are the most common causes of cyanotic Congenital heart disease

Answer 3

tetralogy of Fallot
transposition of the great arteries (TGA)
tricuspid atresia

Question 4

When does fallot’s present?

Answer 4

Fallot’s generally presenting at around 1-2 months

Fallot’s is more common than TGA. However, at birth TGA is the more common lesion as patients with Fallot’s generally presenting at around 1-2 months

Question 5

The presence of cyanosis in pulmonary valve stenosis depends very much on the severity and any other coexistent defects.

Answer 5

true

Question 6

Peripheral cyanosis, for example of the feet and hands, is very common in the first 24 hours of life

Answer 6

true

may occur when the child is crying or unwell from any cause

Question 7

Central cyanosis can be recognised clinically when the concentration of reduced haemoglobin in the blood exceeds

Answer 7

5g/dl

Question 8

In Cyanosis in the neonatal period what test differentiates cardiac from non-cardiac causes?

Answer 8

nitrogen washout test
nfant is given 100% oxygen for ten minutes after which arterial blood gases are taken. A pO2 of less than 15 kPa indicates cyanotic congenital heart disease

Question 9

Initial management of suspected cyanotic congenital heart disease

Answer 9

supportive care
prostaglandin E1
used to maintain a patent ductus arteriosus in ductal-dependent congenital heart defect

Question 10

What is acrocyanosis

Answer 10

refers to the peripheral cyanosis around the mouth and the extremities (hands and feet)
often seen in healthy newborns

Question 11

What causes acrocyanosis?

Answer 11

It is caused by benign vasomotor changes that result in peripheral vasoconstriction and increased tissue oxygen extraction and is a benign condition

Question 12

How can acrocynaosis be differentiated from other causes of peripheral cyanosis with significant pathology?

Answer 12

it occurs immediately after birth in healthy infants. It is a common finding and may persist for 24 to 48 hours.

Question 13

What is the most common cause of cyanotic congenital heart disease?

Answer 13

Tetralogy of Fallot (TOF)

however, at birth transposition of the great arteries is the more common lesion as patients with TOF generally present at around 1-2 months

Question 14

When does TOF present?

Answer 14

It typically presents at around 1-2 months, although may not be picked up until the baby is 6 months old

Question 15

What are the features of TOF?

Answer 15

Ventricular septal defect (VSD)
Right ventricular hypertrophy & outflow tract obstruction
Pulmonary stenosis
Overriding aorta

Question 16

TOF is a result of what malalignment?

Answer 16

anterior malalignment of the aorticopulmonary septum

The severity of the right ventricular outflow tract obstruction determines the degree of cyanosis and clinical severity

Question 17

What are the clinical features of TOF?

Answer 17

cyanosis
causes a right-to-left shunt
ejection systolic murmur due to pulmonary stenosis
ECG shows right ventricular hypertrophy
a right-sided aortic arch is seen in 25% of patients

Question 18

What does CXR show inTOF?

Answer 18

chest x-ray shows a ‘boot-shaped’ heart,

Question 19

What is the management of TOF?

Answer 19

surgical repair is often undertaken in two parts

cyanotic episodes may be helped by beta-blockers to reduce infundibular spasm

Question 20

What causes Transposition of the great arteries?

Answer 20

caused by the failure of the aorticopulmonary septum to spiral during septation

Question 21

Who is most at increased risk of TGA?

Answer 21

Children of diabetic mothers are at an increased risk of TGA.

Question 22

What are the anatomic changes in TGA?

Answer 22

aorta leaves the right ventricle

pulmonary trunk leaves the left ventricle

Question 23

What are the features of TGA?

Answer 23

cyanosis
tachypnoea
loud single S2
prominent right ventricular impulse

Question 24

What does CXR show in TGA?

Answer 24

‘egg-on-side’ appearance on chest x-ray

Question 25

What is the management of TGA

Answer 25

maintenance of the ductus arteriosus with prostaglandins

surgical correction is the definite treatment.

Question 26

What is the most common cause of congenital heart disease?

Answer 26

Ventricular septal defect

Question 27

VSDs close spontaneously in around 50% of cases.

Answer 27

true

Question 28

What is the aetiology of VSD?

Answer 28

congenital VSDs are often association with chromosomal disorders: Down’s syndrome, Edward’s syndrome, Patau syndrome, cri-du-chat syndrome

congenital infections

post-myocardial infarction

Question 29

VSDs may be detected in utero during which scan

Answer 29

the routine 20 week

Question 30

Post-natal presentations of VSD include

Answer 30

failure to thrive
hepatomegaly
features of heart failure
tachypnoea, tachycardia, pallor

classically a pan-systolic murmur which is louder in smaller defects

Question 31

What is the management of VSD?

Answer 31

small VSDs which are asymptomatic often close spontaneously are simply require monitoring

moderate to large VSDs usually result in a degree of heart failure in the first few months - surgical closure of the defect?

Otherwise nutritional support, medication for heart failure e.g. diuretics

Question 32

What are the complications of VSD?

Answer 32

infective endocarditis
right heart failure

aortic regurgitation: due to a poorly supported right coronary cusp resulting in cusp prolapse

Eisenmenger’s complex: due to prolonged pulmonary hypertension from the left-to-right shunt results in right ventricular hypertrophy and increased right ventricular pressure. This eventually exceeds the left centricular pressure resulting in reversal of blood flow this is turn results in cyanosis and clubbing Eisenmenger’s complex is an indication for a heart-lung transplant

pulmonary hypertension: pregnancy is contraindicated in women with pulmonary hypertension as it carries a 30-50% risk of mortality

Question 33

Patent ductus arteriosis (PDA) can only be acyanotic

Answer 33

False
generally classed as ‘acyanotic’. However, uncorrected can eventually result in late cyanosis in the lower extremities, termed differential cynaosis

Question 34

What is PDA?

Answer 34

connection between the pulmonary trunk and descending aorta

Question 35

PDA is more common in?

Answer 35

premature babies, born at high altitude or maternal rubella infection in the first trimester

Question 36

How does ductus arteriosus close in first breaths?

Answer 36

increased pulmonary flow which enhances prostaglandins clearance

Question 37

Features of PDA?

Answer 37

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

Question 38

Management of PDA?

Answer 38

indomethacin or ibupofen - inhibits prostaglandin synthesis & closes the connection in the majority of cases
if associated with another congenital heart defect amenable to surgery then prostaglandin E1 is useful to keep the duct open until after surgical repair

Question 39

Coarctation of the aorta is associated with which syndrome?

Answer 39

Turner’s syndrome

HOWEVER it is more common in males overall

Question 40

What are the features of Coarctation of the aorta?

Answer 40

infancy: heart failure

adult:
hypertension
radio-femoral delay, mid systolic murmur, maximal over back, apical click from the aortic valve

notching of the inferior border of the ribs (due to collateral vessels) is not seen in young children

Question 41

What is coarctation of the aorta associated with?

Answer 41

bicuspid aortic valve
berry aneurysms
neurofibromatosis