Paediatric Cardiology

Question 1

Describe the aetiology of congenital heart disease

Answer 1

Combination of genetic susceptibility plus environmental insult, mainly from drugs, infections and/or maternal disease

Question 2

What drugs are associated with increased risk of congenital heart disease?

Answer 2

Alcohol Amphetamines Cocaine Ecstasy Phenytoin Lithium

Question 3

Which infections are associated with increased risk of congenital heart disease?

Answer 3

Toxoplasmosis HepB Rubella CMV HSV

Question 4

Give two maternal illnesses that are associated with increased risk of CHD

Answer 4

Diabetes Mellitus SLE

Question 5

Which chromosomal abnormalities are associated with CHD? Which CHDs are they associated with?

Answer 5

Trisomy 13 (Patau) - VSD, ASD Trisomy 18 (Edward’s) - VSD, PDA Trisomy 21 (Down’s) - AVSD Turner - Coarctation of aorta Noonan - Pulmonary stenosis Williams - supravalvular aortic stenosis 22q11 deletion syndrome

Question 6

List the 8 most common congenital heart defects

Answer 6

• Ventricular Septal Defect (VSD) • Patent Ductus Arteriosus (PDA) • Atrial Septal Defect (ASD) • Pulmonary Stenosis • Aortic Stenosis • Coarctation of the Aorta • Transposition of Great Arteries • Tetralogy of Fallot

Question 7

What triad of symptoms is commonly present in paediatric heart failure patients?

Answer 7

Tachypnoea/dyspnoea Tachycardia Hepatomegaly

Question 8

What is the main sign on heart failure from the parent’s perspective?

Answer 8

Poor feeding - breathlessness, sweating, not feeding for very long - subsequent failure to thrive

Question 9

Which pulses should be felt when examining babies?

Answer 9

Brachial Femoral

Question 10

What are the main treatment options for congenital heart disease?

Answer 10

Medication Palliative procedures - BT shunt - balloon valvoplasty - Prostaglandin infusion - pulmonary banding Heart transplant

Question 11

What proportion of paediatric murmurs are innocent? Are they systolic or diastolic?

Answer 11

70 - 80% Systolic - diastolic murmurs are pathological

Question 12

Give four types of innocent heart murmurs

Answer 12

Still’s murmur - LV outflow murmur Pulmonary outflow murmur Carotid/brachiocephalic arterial bruits Venous hum

Question 13

Describe the thought processes involved in distinguishing between innocent and pathological paediatric murmurs

Answer 13

Are there any symptoms and signs of heart disease? Are there any predisposing medical conditions? What are the characteristics of the murmur?

Question 14

What cardinal features indicate that a murmur is likely to be pathological?

Answer 14

Diastolic murmur (always pathological) If systolic: - Holosystolic (pansystolic) murmur - Harsh murmur - Abnormal heart sounds - Early or mid-systolic click - Grade 3 murmur or greater - Heard over upper left sternal border

Question 15

Give seven features of innocent heart murmurs

Answer 15

Sensitive (changes with child’s position or with respiration) Short duration (not holosystolic) Single (no associated clicks or gallops) Small (murmur limited to a small area and not radiating) Soft (low amplitude) Sweet (not harsh-sounding) Systolic (occurs and is limited to systole)

Question 16

Describe the four components of Tetralogy of Fallot

Answer 16

1. stenosis of RV outflow tract 2. right ventricular hypertrophy 3. ventricular septal defect 4. overriding aorta

Question 17

Which components of ToF require treatment?

Answer 17

Stenosis of RV outflow tract and ventricular septal defect; treatment of these will resolve the other two problems

Question 18

What behaviour in older children is characteristic of a right to left shunt?

Answer 18

Squatting to rest when exercising; kinks the femoral arteries, increasing pressure in the LV outflow tract and reducing the right to left shunt.

Question 19

What type of murmur is caused by ASD?

Answer 19

Systolic murmur with a split S2

Question 20

What type of murmur is caused by VSD?

Answer 20

Pansystolic murmur heard at the left lower sternal border

Question 21

What type of cyanosis can be caused by PDA and/or coarctation of the aorta?

Answer 21

Cyanosis of the lower extremities

Question 22

Describe the management options for VSD

Answer 22

Closure of VSD - Amplatzer device - patch closure If severe, may require heart AND lung transplant

Question 23

What is Eisenmenger’s syndrome?

Answer 23

Development of a right to left shunt due to pulmonary hypertension

Question 24

What drug can be given to a PDA patient to help the ductus arteriosus to close?

Answer 24

Indomethacin (prostaglandin E inhibitor)

Question 25

What are the management options for coarctation of the aorta?

Answer 25

Balloon dilatation Surgical removal of the coarctation

Question 26

What is the main surgical treatment option for PDA?

Answer 26

Surgical ligation of the ductus arteriosus

Question 27

Describe the difference between cyanotic and acyanotic congenital heart disease

Answer 27

Acyanotic: blood is still able to get to the lungs. Typically a left to right shunt. Cyanotic: blood cannot properly get to the lungs. Typically a right to left shunt.

Question 28

Which congenital heart diseases are acyanotic?

Answer 28

• Left-to-right shunt:
  
  • ventricular septal defect
  • atrial septal defect
  • persistent ductus arteriosus
• Outflow obstruction
  
  • coarctation of the aorta
  • pulmonary stenosis
  • aortic stenosis

Question 29

Which congenital heart conditions are cyanotic?

Answer 29

• Transposition of great arteries
• Tetralogy of Fallot
• Truncus arteriosus
• (Eisenmenger syndrome)