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Child Health > Paediatric Cardiology - Cyanotic Heart Defects > Flashcards

Paediatric Cardiology - Cyanotic Heart Defects Flashcards

1
Q

What are the four major defects in tetralogy of fallot?

A

Pulmonary stenosis
Overriding aorta
Right ventricular hypertrophy
Ventricular septal defect

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2
Q

Why do you get arterial desaturation in tetralogy of fallot?

A

Blood is shunted across the VSD into the aorta

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3
Q

What determines the severity of cyanosis in Tetralogy of Fallot?

A

Degree of pulmonary stenosis

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4
Q

What are the complications associated with Tetralogy of Fallot?

A

Cerebral thrombosis and ischaemia
Brain Abscess
Bacterial endocarditis

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5
Q

How does Tetralogy of Fallot present?

A

Cyanosis
Clubbing
Heart Failure
Failure to thrive
Dyspnoea on exertion - relieved by squatting
Ejection systolic murmur and loud S2 - third left intercostal space
Boot shaped heart on CXR

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6
Q

Why is dyspnoea relieved by squatting in Tetralogy of Fallot?

A

Squatting increases peripheral vascular resistance so reduces the Right to Left shunting

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7
Q

What does an ECG show in Tetralogy of Fallot?

A

Right axis deviation

Right ventricular hypertrophy

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8
Q

How are prolonged hypercyanotic spells managed?

A

Morphine - relieve pain and abolish hyperpnoea
Sodium bicarbonate IV - correct acidosis
Propranolol - peripheral vasoconstriction and relieve infundibular. Also prevent hypoxic spells

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9
Q

What is the definitive management for Tetralogy of Fallot?

A

Corrective Surgery for full repair - 6 months old

Prostaglandin infusion

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10
Q

What does corrective surgery for Tetralogy of Fallot entail?

A

Closure of VSD
Widening of the right ventricular outflow tract
Shunt between subclavian and pulmonary arteries to increase pulmonary blood flow (initial surgery)

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11
Q

What happens in transposition of the great arteries?

A

RV connect to aorta and LV connect to pulmonary artery creating 2 unconnected parallel circuits

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12
Q

What is required for transposition of the great arteries to be compatible with life?

A

Mixing of the circuits through ASD, VSD or PDA

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13
Q

Who is transposition of the great arteries more likely to affect?

A

Males

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14
Q

How does transposition of the great arteries present?

A

Within the first hours of life, the child has worsening cyanosis and severe hypoxia

May hear heart murmur if there is a septal defect (allow for life)

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15
Q

How is transposition of the great arteries managed in the immediate term?

A

Maintain body temperature
Correct any hypoglycaemia
Prostaglandin E1 infusion

Can do emergency cardiac catheter and therapeutic balloon atrial septostomy

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16
Q

Why is it important to maintain body temperature in transposition management?

A

Don’t want to worsen metabolic acidosis

17
Q

Why do you give Prostaglandin E1 infusion in immediate management of transposition?

A

Reopen ductus arteriosus

18
Q

What long term management is used for transposition of the great arteries?

A

Corrective surgery within 2 weeks - arterial switch procedure

19
Q

What is hypoplastic left heart syndrome?

A

LV is small and non functional so RV maintains both pulmonary and systemic circulation

20
Q

How does the Right Ventricle maintain the systemic circulation in hypoplastic left heart?

A

Via ASD or retrograde flow through PDA

21
Q

How does hypoplastic left heart syndrome present?

A

Neonate becomes cyanosed with heart failure within days of birth

Blue-grey with weak pulses

22
Q

How is hypoplastic left heart managed?

A

Palliative management or heart transplant

23
Q

What is Ebstein’s anomaly?

A

Congenital heart defect characterised by low insertion of the tricuspid valve

24
Q

How does a the heart of a child with Ebstein’s anomaly appear?

A

Large atrium and small ventricle

25
Q

What is Ebstein’s anomaly associated with?

A

Tricuspid incompetence

Wolff-Parkinson White syndrome

26
Q

How would you determine a child has tricuspid incompetence?

A

Pan-systolic murmur

Giant V wave in JVP

27
Q

What can cause Ebstein’s anomaly?

A

Exposure to lithium in utero

28
Q

What is Eisenmenger’s syndrome associated with?

A

Ventricular Septal Defect, Atrial Septal Defect, Patent Ductus Arteriosus

29
Q

What are the features of Eisenmenger’s syndrome?

A 
Original murmur disappear
Clubbing
Cyanosis
RV failure
Haemoptysis
Embolism
30
Q

How is Eisenmenger’s syndrome managed?

A

Heart-Lung transplant required

Child Health (30 decks)

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