Paediatric cardiology

Question 1

What are the 8 congenital heart defects

Answer 1

• Ventricular septal defet
• Patent Ductus Arteriosus
• Atrial Septal Defect
• Aortic Stenosis
• Coarctation of the Aorta
• Transposiiton of the Great Arteries
• Tetralogy of Fallot

Question 2

Aetiology of congenital heart disease

Answer 2

• Drugs → alcohol, amphetamines, cocaine, ecstasy, phenytoin, lithium
• Infection → TORCH (toxoplasmosis, other agents, rubella, cytomegalovirus, herpes)
• Maternal → DM, SLE
• Chromosomal abnormality → trisomy 13, 18, 21

Question 3

Presentation of CHD

Answer 3

• Feeding, weight, development
• Cyanosis
• Tachypnoea, dyspnoea
• Exercise tolerance
• Chest pain
• Syncope
• Palpitations
• Joint problems
• Murmurs
• Clubbing
• Dysmorphic features
• Heart sounds (clicks, split, 3 and 4)

Question 4

Investigations for CHD

Answer 4

• BP
• O2 saturations
• ECG
• CXR
• ECHO
• Cardiac catheter
• Angiography
• MRI/
• Exercise tolerance testing

Question 5

Principles of treatment

Answer 5

• Fix it immediately
• Improve situation → balloon valvoplasty, prostaglandin infusion,
• Replace it

Question 6

Characteristics of murmurs

Answer 6

• Timing → systole, diastole, continuous
• Duration → ejection, holo, pan
• Pitch/ quality → harsh, soft, indeterminate, vibratory, pure frequency
• Site → URSB, ULSB, LLSB, apex

Question 7

Define an innocent murmur

Answer 7

• Majority of murmurs
• 4 main types
• Not a diagnosis of exclusions

Question 8

Features of innocent murmur

Answer 8

• Systolic ( continuous in venous hum)
• No signs of cardiac disease
• Sofy
• Vibratory, musical
• Localised
• Varies with position, respiration, exercise

Question 9

Define Still’s murmur

Answer 9

• Left ventricular outflow murmur
• Age 2-7
• Soft systolic
• Apex of left sternal border
• Increased in supine position and exercise

Question 10

Define Pulmonary outflow murmur

Answer 10

• Aged 8-10
• Soft systolic, vibratory
• Upper left sternal border, well localised, does not radiation
• Increased in supine position and exercise
• Often child with narrow chest

Question 11

Define carotid/ brachiocephalic arterial bruits

Answer 11

• Aged 3-20
• Systolic harsh murmur
• Supraclavicular, radiates to neck
• Increased with exercise, decreased with turning head or extending neck

Question 12

Define venous hum

Answer 12

• Aged 3-8
• Soft, indistinct
• Continuous murmur, diastolic accentuations
• Supraclavicular
• Upright position, disappears when lying down

Question 13

Define ventricular septal defects

Answer 13

• 3 types
  
  • Subaortic
  • Perimembranous
  • Muscular
• Left to right shunt
• Amplatzer or patch closure

Question 14

Clinical features of VSD

Answer 14

• Pan-systolic murmur on lower left sternal edge → sometimes thrills
• Diastolic rumble → mitral stenosis
• Cardiac failures signs → ventricular hypertrophy, pulmonary hypertension

Question 15

Features of ASD

Answer 15

• Few clinical signs
• Can spontaneously close
• Sometimes into adulthood → AF, heart failure, pulmonary hypertension
• Wide fixed splitting of 2 heart sounds, pulmonary flow murmur

Question 16

Features of pulmonary stenosis

Answer 16

• Asymptomatic in mild disease
• Exertional dyspnoea
• Ejection systolic murmur → upper left sternal border
• Radiation into back
• May require ballon valvoplasty

Question 17

Features of aortic stenosis

Answer 17

• Mostly asymptomatic
• Severe cases → reduced exercise tolerance, exertion chest pain, syncope
• May have tricuspid aortic valve

Question 18

What changes occur in foetal circulation at birth

Answer 18

• Pulmonary vascular resistance falls
• Pulmonary blood flow increased
• systemic vascular resistance increased
• Ductus arterioles closes
• Foramen Ovale closes
• Ductus venous closes

Question 19

Define Patent Ductus Arteriosus

Answer 19

• Common in pre-term babies
• Treatment with with fluid restriction/ diuretics, prostaglandins inhibitors, surgical ligation

Question 20

Define coarctation of the Aorta

Answer 20

• Narrowing of the aorta at the site of insertion of the ductus arterosus
• Associated with diffuse arteriopathy and bicuspid aortic valve
• Typically presents with upper extremity systolic hypertension or murmur

Question 21

Key diagnostic features of coarctation of aorta

Answer 21

• Hypertension young age resistant to treatment
• Diminished lower extremity pulses
• Differential BP between upper and lower extremity
• Systolic ejection murmur
• Male
• Genetic syndrome → Turner’s syndrome
• Claudication

Question 22

Management of coarctation of aorta

Answer 22

• Reopening PDA with prostanglandins
• Resection with end to end anastomoses
• Subclavian patch repair
• Ballon aortoplasty

Question 23

Define transposition of the great arteries

Answer 23

• Swapping of the aorta and pulmonary aorta in its attachments sites onto the heart
• Blood to the body is sent to the lungs, blood for the lungs is sent to the body
• Presents with cyanosis
• Often associated with pulmonary stenosis, coarctation of the aorta and VSD

Question 24

Management of transposition of the Great Arteries

Answer 24

• Reopen ductus arterioles with prostaglandins → allows blood flow from aorta to pulmonary arteries
• Ballon septostomy
• Open heart surgery for switch procedure

Question 25

Define tetralogy of fallout

Answer 25

• Syndrome of 4 CHD
  
  • Pulmonary stenosis
  • Right ventricular hypertrophy
  • Overriding aorta
  • Ventricular septal defet
• VSD and overriding aorta causes causes RV hypertrophy
• Presents with murmur, cyanosis and Tet spells

Question 26

Define Tet spell

Answer 26

• Period of worsened symptoms due to worsened right to left shunting
• Due to increased pulmonary vascular resistance and decreased systemic resistance

Question 27

Treatment of tetralogy of Fallot

Answer 27

• Surgical repair
• Intracardiac repari
• Aortopulmonary shunt → temporarily
• Treat complications → pulmonary regurgitation , RV failure, arrhythmias