Paediatric Cardiology Flashcards by Ross Tulloch

What is the incidence of congenital heart disease?

Affects 8/1000 live births

8 common lesions account for 80% of CHD

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What are the eight common lesions in congenital heart disease?

Ventricular septal defect (VSD)
Patent ductus arteriosus (PDA)
Atrial septal defect (ASD)
Pulmonary stenosis
Aortic stenosis
Coarctation of the aorta
Transposition of the great arteries
Tetralogy of Fallot

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What is the most useful investigation in congenital heart disease?

Echocardiogram

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Why does congenital heart disease occur?

Due to genetic susceptibility and a teratogenic insult 18-60 days post conception

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What are the environmental factors that can contribute to congenital heart disease development?

Drugs:
-Alcohol
-Amphetamines
-Cocaine
-Ecstasy 
-Phenytoin
-Lithium 
Infection:
-TORCH (toxoplasmosis, rubella, CMV, HSV) and others
Maternal:
-Diabetes mellitus
-Systemic lupus erythematous

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What is the link between chromosomal abnormalities and congenital heart disease?

Up to 10% of CHD have underlying chromosomal abnormalities and 30% of people with chromosomal abnormalities have CHD

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What congenital heart disease anomaly is associated with trisomy 21?

Usually atrioventricular septal defect

Atrial or ventricular septal defects also more common

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What congenital heart disease anomaly is associated with Turner’s syndrome?

Coarctation of the aorta

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What congenital heart disease anomaly is associated with Noonan syndrome?

Pulmonary stenosis

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What congenital heart disease anomaly is associated with Williams syndrome?

Supraclavicular aortic stenosis

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What triad is associated with paediatric heart failure?

Tachycardia
Tachypnoea
Hepatosplenomegaly

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What is a common presentation of infant heart failure?

Failure to thrive due to improper feeds as the baby becomes breathless during feeding.

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How are murmurs characterised?

Timing in cardiac cycle- systole, diastole, continuous
Duration- early/mid/late, ejection, holo- or pan-systolic
Pitch- harsh/mixed frequency, soft/indeterminate, vibratory/pure frequency

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What are the four types of innocent murmur?

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

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What are the characteristics of Still’s murmur?

Age: 2-7
Soft, systolic, vibratory murmur
Present in the apex and left sternal border
Increases in supine position and with exercise

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What are the characteristics of a pulmonary outflow murmur?

Age: 8-10
Soft systolic, vibratory murmur
Well localised to the upper left sternal border
Increased in supine position and with exercise
Often in slim children with narrow chests

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What are the characteristics of a carotid/braciocephalic arterial bruit?

Age: 2-10
Harsh systolic murmur
Supraclavicular and radiates to the neck
Increases with exercise
Decreases with turning head or extending neck

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What are the characteristics of a venous hum?

Age: 3-8
Soft and indistinct
Continuous murmur sometimes with diastolic accentuation
Supraclavicular
Only in upright position, disappears on lying down or when turning head

What is the most common cardiac defect?

Ventricular septal defects

What are the three types of ventricular septal defect?

Subaortic
Perimembranous
Muscular

What kind of shunt is a VSD associated with and why?

A left to right shunt as pressure is higher in the left ventricle than in the right

When are VSD symptoms most severe?

Symptoms increase with size of the hole and proximity to the valves

What is the clinical presentation of VSD?

Pansystolic murmur at the lower left sternal edge, sometimes with a thrill
In very small VSDs- early systolic murmur
In very large VSDs- diastolic rumble due to relative mitral stenosis
Signs of cardiac failure in large VSDs, leading to biventricular hypertrophy and pulmonary hypertension (Eisenmenger syndrome)

How can VSD closure be done?

Amplatzer or another trans-catheter occlusion device or with open heart surgery and patch closure

What are the characteristics of an atrial septal defect?

Associated with few clinical signs in early childhood Good chance of spontaneous closure. Less severe than VSD as the pressure gradient between the atria is less severe than between the ventricles. Sometimes picked up in adulthood with atrial fibrillation, heart failure or pulmonary hypertension Can be associated with a wide fixed splitting of S2 (pulmonary flow murmur).

What is an atrioventricular septal defect?

Single atrioventricular valve High VSD Ostium primum (low ASD)

What is the most common valvular defect in children?

Pulmonary stenosis

What are the symptoms of pulmonary stenosis?

Asymptomatic in mild stenosis | In moderate-severe stenosis exertional dyspnoea and fatigue can present.

What murmur is associated with pulmonary stenosis?

An ejection systolic murmur at the upper left sternal border with radiation to the back

How is pulmonary stenosis fixed?

Can be fixed with a balloon valvoplasty or a valve replacement (should not be done pre-puberty as child will outgrow prosthetic valve)

What are the symptoms of aortic stenosis?

Aortic stenosis is usually asymptomatic but if it is severe then reduced exercise tolerance, exertional chest pain and syncope can develop

What murmur is associated with aortic stenosis?

An ejection systolic murmur in the upper right sternal border with radiation into the carotids

How is aortic stenosis fixed?

Balloon aortic valvuloplasty

What changes occur in the fetal circulation at birth?

``` Pulmonary Vascular Resistance Falls Pulmonary Blood Flow Rises Systemic Vascular Resistance is increased Ductus Arteriosus Closes Foramen Ovale Closes Ductus Venosus Closes ```

When is a patent ductus arteriosus most common?

In preterm babies

How is patent ductus arteriosus treated?

``` Fluid restriction Diuretics Prostaglandin inhibitors (indomethacin or ibuprofen) Surgical ligation Closed with umbrella device ```

What is coarctation of the aorta?

A kink in the aortic wall- usually opposite where the ductus arteriosus enters the aorta

How is coarctation of the aorta imaged?

MRI or 3D MRI

How is coarctation of the aorta managed?

Reopen ductus arteriosus with prostaglandin E1 or E2 Resection with end-to-end anastomosis Subclavian patch repair Balloon aortoplasty

What are the cyanotic heart conditions?

Transposition of the great arteries | Tetralogy of Fallot

What shunt are the cyanotic heart conditions associated with?

Right to left shunt

How can transposition of the great arteries be fixed?

Rashkind’s atrial septostomy | Switch procedure for transposition

What is implicated in tetralogy of Fallot?

Pulmonary valve stenosis Right ventricular hypertrophy Ventricular septal defect Overriding aorta

How is tetralogy of Fallot fixed?

``` Open heart surgery with the following aims: •Valvotomy •Closure of VSD •Put the aorta in the correct position •Myotomy ```