Paediatric Cardio Conditions

Question 1

What are the causes of cyanotic congenital heart disease?

Answer 1

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• Truncus arteriosus
• Transposition of the Great Arteries
• Tricuspid / Pulmonary Atresia
• Tetralogy of Fallot
• Total anomolous pulmonary venous return

Question 2

What are the two classes of acyanotic congenital heart disease?

Answer 2

Obstructive and L-R shunt

Question 3

What are the causes of obstructive heart disease?

Answer 3

• Hypoplastic L heart syndrome
• AS
• Coarctation of the aorta
• Interruption of the aortic arch

Question 4

As a general rule, which group of congenital heart defects are ALWAYS duct dependent? How is this treated in the early stages?

Answer 4

Obstructive congenital heart defects (hypoplastic L heart syndrome, AS, interruption of the aortic arch and aortic coarctation). They are treated by PGE1 in the short term, to keep the duct open

Question 5

As a general rule, which group of congenital heart defects are USUALLY duct dependent? Is it harmful to treat with PGE1, while still in the diagnostic process?

Answer 5

R–>L shunts (1T2T3T4T5T) but this time you depend on the duct being open for blood to flow into the PULMONARY circulation

Question 6

How and when do obstructive congenital heart defects present?

Answer 6

A shocked neonate. Often on D2 of life when PDA closes. Present as a dusky / grey coloured neonate with weak / absent pulses and a low BP, with a metabolic acidosis.

Question 7

What are the defects of tetralogy of fallot?

Answer 7

1. PULMONARY STENOSIS 2. RVH 3. VSD 4. Overriding aortic arch

Question 8

When does tetralogy of fallot tend to present and how?

Answer 8

6-12 months of life. “Tet spells” or “hypoxic spells” - cyanosis or LOC / going floppy on feeding, crying or exertion

Question 9

What is the surgical correction of transposition of the great arteries?

Answer 9

balloon atrial septotomy

Question 10

What is the surgical correction of hypoplastic L heart syndrome?

Answer 10

Norwood operation

Question 11

Which two congenital heart defects are associated with DiGeorge syndrome? Which chromosomal abnormality is this and what are the other associated features?

Answer 11

TOF and transpos are both associated with microdeletion of the long arm of chromosome 22. The other associated features are ‘CATCH 22’ Cardiac (trasnpos, TOF) Abnormal (long) facies Thymic hypoplasia Cleft palate Hypocalcaemia / hypoparathyroidism

Question 12

With which CHD is Turner’s syndrome associated?

Answer 12

Aortic coarctation

Question 13

Compare the timing of onset of transposition of the great arteries and TOF?

Answer 13

Transpos always presents as cyanosis in the newborn period. TOF presents 6-12 months of life as cyanosis.

Question 14

What is the most common cause of ACQUIRED heart disease in paediatrics?

Answer 14

Kawasaki disease

Question 15

What are the diagnostic features of Kawasaki disease?

Answer 15

Fever persisting 5 days or more (usually high, >39 degrees)

AND

4 of the following 5 features

Important note: you can also diagnose Kawasakis with only 3 of the 5 features if you get a positive echo.

1. bilateral nonpurulent conjunctivitis
   * ie. redness without exudate

1. red fissured lips, strawberry tongue, erythema of oropharynx (without tonsillitis or evidence of URTI)

1. changes of the peripheral extremities

• acute phase: erythema, edema of hands and feet, groin peeling. May manifest as refusal to weight bear.
• subacute phase: peeling from tips of fingers and toes

1. polymorphous rash

• usually begins in the nappy area (where there may be desquamation early in the disease) and spreads to involve the trunk, extremities and face.
• Rash may be maculopapular, annular or scarlatiniform.

1. cervical lymphadenopathy > 1.5 cm in diameter

Question 16

What is the treatment of Kawasaki disease

Answer 16

• Intravenous immunoglobulin (2 g/kg over 10 hours; preferably within the first 10 days of the illness.)
• Aspirin 3 - 5 mg/kg once a day for at least 6 to 8 weeks

Question 17

What should be organised before a patient treated for Kawasaki disease is discharged?

Answer 17

Follow-up echocardiogram in 6-8 weeks

Question 18

Kawasaki disease is primarily a clincial diagnosis, but what Ix would you perform (and what might you expect to see)?

Answer 18

Bedside tests ECG - check for ischaemia / infarction Urine dipstick - negative Laboratory Tests FBE - neutrophilia, anaemia, thrombocytosis ESR/CRP - markedly raised LFTs - ALT raised Imaging Echocardiogram

Question 19

What are the clinical features of a Patent ductus arteriosus?

Answer 19

• Patients with a small PDA are usually asymptomatic
• Murmur
  
  • Continuous murmur audible at the upper left sternal border or left infraclavicular area – called a machinery murmur
  • May disappear during diastole and be mistaken for a systolic murmur, especially if the duct is large and there is associated pulmonary hypertension.
  • Radiates along the pulmonary arteries, and often well heard over the left side of the back
• With a large duct, the large left-to-right shunt causes left heart dilation
  
  • Can cause symptoms such as failure to thrive, dyspnea and recurrent chest infections.
  • Bounding pulses
  • Low diastolic BP
  • Apex may be displaced and forceful, and an apical mid-diastolic murmur may be heard due to increased flow through the mitral valve
  • A thrill may be palpable

Question 20

What is the management of a patent ductus arteriosus in preterm infants?

Answer 20

IV indomethacin

IV ibuprofen can be used in infants of larger weight

Question 21

What is the management of PDA in term infants?

Answer 21

Percutaneous catheter closure

Question 22

What is Eisenmenger syndrome?

Answer 22

Intracardiac communication with severe pulmonary vascular disease which is inoperable

Question 23

What is the most common congenital heart defect?

Answer 23

Ventricular septal defect

Question 24

What are the clinic presentation of VSDs?

Answer 24

• Small VSDs with little shunt are often asymptomatic but have a loud murmur
  
  • Loud, harsh, high pitched pansystolic murmur usually heard best at the lower left sternal border
  • There may be a thrill
• Large VSDs - pulmonary overcirculation and heart failure
  
  • Increase flow across the mitral valve causing a mid-diastolic murmur
  • Parasternal heave
  • Difficulty feeding due to tachypnea
  • Hepatomegaly
• Splitting of S2 and intensity of P2 – depending on the pulmonary artery pressure

Eisenmenger syndrome

• At rest patients may be asymptomatic, but experience exertional dyspnea, cyanosis, chest pain, syncope and hemoptysis with exercise

Question 25

What are the four structural defects of Tertalogy of Fallot?

Answer 25

Ventricular septal defect
Pulmonary stenosis
Overriding aorta
Right ventricular hypertrophy

Question 26

At what age would you expect to see cyanosis in children with tertaology of fallot?

Answer 26

6 to 12 months

Question 27

How do you treat a hypercynanotic spell or tet spell?

Answer 27

Treatment of hypercyanotic spells

Basics

ABC, oxygen, sedation and pain relief (morphine)

Place and person

Hospital, ICU

Investigate and confirm diagnosis

Text

Non-invasive management

Keep the child calm – stress will exacerbate

Definitive management

IV propranolol

• Works as a peripheral vasoconstrictor and by relieving the subpulmonary muscular obstruction that is the cause of reduced pulmonary blood flow

IV volume administration

Bicarbonate to correct acidosis

Muslce paralysis and artificial venticlation in order to reduce metabolic oxygen demand

Question 28

At what age does transposition of the great arteries usually become apparent?

Answer 28

• Presentation is usually on day 2 of life when ductal closure leads to a marked reduction in mixing of the desaturated and saturated blood.

Physical signs

• Cyanosis is always present
• Quiet tachypnea
• The second heart sound is often loud and single
• Usually no murmur, but may be a systolic murmur from increased flow or stenosis within the left ventricular outflow tract
• Metabolic acidosis may develop because of tissue hypoxia.

Question 29

What are the signs of heart failure in a child?

Answer 29

Recurrent chest infections

FTT

Difficulty feeding

Hepatomegaly

Tachycardia

Respiratory distress

Profuse sweating when feeding

Question 30

What is Eisenmenger’s Syndrome?

Answer 30

Eisenmenger syndrome refers to any untreated congenital cardiac defect with intracardiac communication that leads to pulmonary hypertension, reversal of flow, and cyanosis. The previous left-to-right shunt is converted into a right-to-left shunt secondary to elevated pulmonary artery pressures and associated pulmonary vascular disease.