4. Cardiovascular pathology

Question 1

What is an innocent murmur?

Answer 1

-Increased flow in a normal heart
-Often more pronounced in minor illness (resolves when illness resolves 6-8 weeks later)
-No clinical significance and child is asymptomatic

Question 2

What are the 7 S’s of an innocent murmur?

Answer 2

-Soft
-Short
-Systolic
-(L) Sternal edge
-Symptomless
-Sensitive (changes with position)
-Sweet (in pitch)

Question 3

What features are present in a normal foetal and neonatal heart?

Answer 3

-Foramen oval (between atria)
-Ductus arteriosus (between pulmonary artery and aorta

Question 4

What are the main types of paediatric cardiac pathology and how are they classified?

Answer 4

CYANOTIC
-Tetralogy of Fallot
-Transposition of the Great Arteries
NON-CYANOTIC
-Coarctation of the aorta
-ASD/AVSD/VSD
-Patent ductus arteriosus

Question 5

What murmur can be heard at the L 2nd ICS?

Answer 5

-Pulmonary flow murmur
-Suggests turbulent flow
-Brief, high-pitched

Question 6

What murmur can be heard below the clavicles?

Answer 6

-Venous hum (rare)
-Blowing continuous murmur in systole and diastole
-Disappears on lying down

Question 7

What risk factors are associated with congenital heart disease?

Answer 7

-Family history
-Teratogens
-Maternal drug use
-Maternal diabetes

Question 8

How does CHD tend to present?

Answer 8

-If severe can be detected antenatally
-Commonly presents at birth with:
–Cyanosis
–Shock
–Breathlessness
-Some may present with:
–Difficulty feeding
–Asymptomatic murmur
–Syncope
–Chest infections
–Oedema

Question 9

How should you examine a child with ?CHD?

Answer 9

-RR, HR, pre- and post-ductal sats (legs = post-ductal)
-Lying and standing BP
-Feel pulses
–Easy to feel? Collapsing pulse?
-CRT
-?Clubbing in hands
-Auscultate chest for wheeze, crackles
-HS
-Assess for hepatomegaly + increased tone

Question 10

How would you investigate a child with ?CHD?

Answer 10

-Routine bloods (septic screen if presenting with cyanosis / SOB)
-CXR (pulmonary oedema, consolidation, heart size)
-Blood gases (high lactate)
-Echo
-ECG

Question 11

How are children with congenital heart disease managed?

Answer 11

-Many can be monitored for years and may not require surgical correction
-Medical control of heart failure pending any definitive repair

Question 12

What is the order of foetal circulation?

Answer 12

1.Maternal blood received through umbilical vein
2.Umbilical vein – IVC – RA/LA (via foramen ovale)
3. RA – RV – PA – ductus arteriosus – aorta
OR
3. LA – LV – aorta
4. Aorta – body – umbilical arteries – placenta

Question 13

What are the 4 features of Tetraology of Fallot?

Answer 13

1. Large VSD
2. Pulmonary stenosis
3. Overriding aorta
4. RV hypertrophy
   Associated with DiGeorge syndrome

Question 14

How do children with ToF present?

Answer 14

-Colouring depends on degree of pulmonary stenosis (blue = severe stenosis causing R-L shunt)
-Tet spells
= hyper-cyanotic episodes which are relieved by squatting down to reverse R-L shunt (increases LV pressure)
-Failure to thrive
-SOBOE
-Loud ESM due to pulmonary stenosis
-‘Boot-shaped heart’ on echo

Question 15

How is ToF managed?

Answer 15

-Surgery - VT shunt
-Shunt placed between subclavian and pulmonary artery
-Done before 1y/o

Question 16

What can be given to manage Tet spells?

Answer 16

-Propanolol
-Morphine

Question 17

How does ASD present and what condition is it associated with?

Answer 17

-Associated with Downs Syndrome
-Usually asymptomatic / not detected until adolescence
-SOB
-Recurrent chest infections
-R heart failure
-Soft ESM at L 2nd ICS

Question 18

How is ASD managed?

Answer 18

-Treated surgically if moderate or large
–Open heart surgery
–Catheterisation

Question 19

What complications can arise from untreated ASD?

Answer 19

-Arrhythmias
–Ostrium Primum = defect in AV septum, RBBB, LAD and prolonged PR seen on ECG
–Ostrium Secondum = defect in FO and AS, RBBB + RAD seen on ECG

Question 20

What occurs in VSD and what babies are most at risk of developing it?

Answer 20

-Hole in ventricular septal wall causing L-R shunt
–Excess blood to lungs – pulmonary oedema – SOB
-More common in:
–Trisomies
–Maternal diabetes
–Turner’s syndrome
–Foetal alcohol syndrome

Question 21

How does VSD present?

Answer 21

-Signs of L heart failure eg tachypnoea, tachycardia, hepatomegaly
-SOB and sweating on feeding in large defects
-Pansystolic murmur heard at lower L sternal border radiating across chest
-Poor growth
-Recurrent chest infections
-Cardiomegaly
-Diagnosis confirmed on echo

Question 22

How is VSD managed?

Answer 22

-Medical = diuretics, ACEis and high calorie feeds
-Surgical = wait til 3-6 months
-Aim to prevent endocarditis
-If small, often close before 2 y/o
Recognised complication = Eisenmengers

Question 23

What causes a patent ductus arteriosus?

Answer 23

-Duct shunts blood from pulmonary artery to aorta during foetal life, closes within 2-3 days
-Duct remains patent in babies who are:
–Unwell (rubella, valproate withdrawal)
–Preterm
–Hypoxic
-Cardiac failure and pulmonary oedema caused by L-R shunt

Question 24

How does PDA present?

Answer 24

-Machinery hum murmur heard over tricuspid area
-Collapsing pulse
-Chest infection
-SOB
-Eisenmenger syndrome (thickening of pulmonary arteries)

Question 25

How is PDA managed?

Answer 25

-Anti-prostaglandins eg ibuprofen
-Diuretics for HF
-Surgery to place occlusion device around pulmonary artery

Question 26

What is coarctation of the aorta and what condition is it associated with?

Answer 26

-Narrowing of the aorta usually distal to the subclavian artery
-Associated with Turner’s

Question 27

How does CoA present and how is it managed?

Answer 27

ACUTE
-Shock
-HF
-Weak femoral pulses, radio-femoral delay
-ESM at upper sternal edge
-Metabolic acidosis
IN OLDER CHILDREN
-HTN
-Higher BP in arms than legs
-Bells palsy
MANGEMENT
-IV prostaglandins to keep duct patent
-Diuretics for HF
-Stent / surgical correction

Question 28

What is transposition of the great arteries and how does it present?

Answer 28

-Aorta and pulmonary artery are transposed
-Presents soon after birth (once ductus arteriosus has closed)
–Profound cyanosis
-Acidosis

Question 29

How should ToGA be managed?

Answer 29

-Prostaglandins to maintain duct
-Emergency atrial septostomy to bide time
-Arterial switch surgery required in a couple of weeks
-Often results in learning disability and coronary artery disease

Question 30

How do paediatric ECGs differ from adult?

Answer 30

-HR much faster
-At birth, appearance of RVH on ECG due to RV being larger and thicker than LV
-Conduction intervals are shorter due to small heart size