Heart Disease in Children Flashcards

1
Q

How to infants present with heart disease?

A
  • Antenatal diagnosis based on ultrasound scan • Routine foetal scan between 18-20 weeks
  • Foetus at risk
  • Heart murmur
  • Most common presentation
  • Most have an innocent murmur
  • Hallmarks of ‘innoSent murmur’
  • Murmur during febrile illness or anaemia – repeat examination when well • If murmur considered significant – refer
  • Many neonates may not have a murmur initially
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2
Q

Symptoms of heart failure in children?

A
  • Breathlessness (particularly on feeding or exertion) • Sweating
  • Poor feeding
  • Recurrent chest infections
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3
Q

Signs of heart failure in children?

A
  • Poor weight gain • Heart signs
  • Hepatomegaly
  • Cool peripheries
  • Frank signs of heart failure not commonly seen
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4
Q

What is the differential diagnosis for heart failure that presents in the first week of life?

A
  • Obstructed duct dependant lesion

* Hypoplastic left heart, critical aortic stenosis, severe coaction of aorta

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5
Q

What is the differential diagnosis for heart failure that presents in infancy?

A

(high pulmonary blood flow)

• VSD, ASD, large PDA

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6
Q

What is the differential diagnosis for heart failure that presents in older children?

A

Eisenmenger syndrome, cardiomyopathy

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7
Q

Cyanosis in a newborn infant differential.

A
  • Cardiac disorders – cyanotic
  • Respiratory disorders, persistent pulmonary hypertension
  • Infections – septicaemia
  • Metabolic diseases
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8
Q

Genetic causes of heart failure in infants

A
  • Down’s ( VSD, AVSD)
  • DiGeorge (Interrupted aortic arch, Truncus arteriosus, VSD) • CHARGE (Tetralogy of Fallot, truncus arteriosus)
  • Long QT
  • Noonan’s (Pulmonary stenosis)
  • Turner’s (Coarctation of aorta)
  • Williams (Supravalvular aortic stenosis, PA stenosis)
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9
Q

Maternal risk factors for child cardiac disorders

A
  • Familial conditions
  • Drugs – Amphetamines, phenytoin, lithium, valproate, retinoic acid, warfarin, progesterone/estrogen, alcohol
  • Infections – Rubella, HIV, other viruses
  • Medical history – Diabetes, SLE
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10
Q

Investigations for suspected cardiac abnormalities in children

A
  • Antenatal USS
  • ECG – different to adults (at least till ~10yrs of age) • Faster heart rate
  • All durations & intervals (PR, QRS, QT) are shorter
  • RV dominance in neonates & infants
  • RAD, Tall R waves in right precordial leads, deep S waves in left precordial leads • T wave is inverted in V1 in infants and young children
  • CXR – heart size, pulmonary vascular markings • Echocardiograhy
  • MRI/CT
  • Hyperoxia test
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11
Q

List examples of conditions and symptoms expected in children with cardiac abnormalities resulting in a left to right shunt.

A

Breathless or asymptomatic

ASD, VSD. PDA

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12
Q

List examples of conditions and symptoms expected in children with cardiac abnormalities resulting in a right to left shunt.

A

Blue

Tetralogy of Fallot, TGA

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13
Q

List examples of conditions and symptoms expected in children with cardiac abnormalities resulting in common mixing of blood.

A

Breathless and blue

AVSD. Complex congenital heart disease

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14
Q

List examples of conditions and symptoms expected in children with cardiac abnormalities resulting in well children with no obstruction.

A

asymptomatic

AS, PS, adult type CoA

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15
Q

List examples of conditions and symptoms expected in children with cardiac abnormalities resulting in sick neonates with obstruction.

A

Collapsed with shock,

Coarctation, Hypoplastic left heart

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16
Q

What are the subtypes of VSD?

A

muscular, membranous, endocardial, sub-arterial

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17
Q

What are the signs and symptoms of VSD?

A
  • Small VSDs (smaller than aortic valve, less than 3 mm)
  • Asymptomatic
  • Signs
  • Loud murmur, quiet pulmonary second sound
  • Moderate to large – pulmonary overload, heart failure
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18
Q

How would you investigate and manage a vsd?

A
  • Investigations
  • Echocardiography, Chest Xray and ECG normal
  • Management
  • Close spontaneously
  • Prevention of bacterial endocarditis
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19
Q

What are the symptoms and signs of A large VSD?

A
  • Symptoms
  • Heart failure, breathless, failure to thrive after 1 week of age • Recurrent chest infections
  • Signs
  • Tachypnoea, tachycardia, enlarged liver • Active precordium
  • Soft murmur or no murmur
  • Apical mid-diastolic murmur
  • Loud P2
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20
Q

Management os large VSD?

A
• Investigations
• Chest XRay
• ECG
• Echocardiography
Large VSDs
• Management
• Drugs for heart failure – diuretics and captopril
• Nutrition – additional calories
• Prevent lung damage and reduce pulmonary hypertension • Surgery by 3-6 months of age
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21
Q

What are the two types of atrial septal defect?

A
  • Two main types
  • Secundum ASD - common – 80%
  • Defect in the centre of atrial septum involving foramen ovale
  • Partial atrioventricluar septal defect (primum ASD, pAVSD) • Inter-atrial communication
  • Abnormal atrioventricular valves
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22
Q

what are the symptoms of an atrial septal defect?

A
  • Symptoms • None
  • Recurrent chest infections /wheeze
  • Arrhythmia from fourth decade onwards
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23
Q

signs of ASD?

A
  • Signs
  • Ejection systolic murmur
  • Fixed and widely split second heart sound • pAVSD – apical pansystolic murmur
24
Q

investigations in ASD?

A
  • Investigations • Chest Xray
  • ECG
  • secundum ASD – partial right bundle branch block, RAD • pAVSD – superior QRS axis Symptoms
  • Echo
25
Q

Management of ASD?

A
  • Management
  • Significant ASD
  • Occlusion device • pAVSD
  • Surgical correction
  • Usually 3-5 years of age
26
Q

What is the anatomy of a persistent ductus arteriosis?

A
  • Normally closes after birth, if failed to close by 1 month abnormal • Blood flow from aorta to pulmonary artery
  • Preterm baby - separate entity
27
Q

Clinical features of PDA?

A
  • Symptoms unusual
  • Can develop heart failure and pulmonary hypertension • Continuous murmur beneath left clavicle
  • Bounding pulse
28
Q

Describe coarctation of the aorta

A

• Narrowing of the aortic lumen due to failure of ductus arteriosus to develop correctly

29
Q

clinical presentation of coarctation of the aorta

A
  • Clinicalpresentation
  • Poor feeding
  • Respiratory distress
  • Shock
  • Weak femoral pulses
  • Murmurmay/maynotbepresent
  • Gallop rhythm, heart failure (1-4 weeks)
30
Q

what is the treatment for coarctation?

A
  • Treatment
  • Resuscitation + Prostaglandin
  • Surgical repair
31
Q

How is a right to left shunt investigated and treated?

A
tetralogy of Fallot, transposition of great arteries
• Cyanosis in first week of life
• Hyperoxia test
• 100% oxygen for 10 min
• Right radial PaO2 <15 kPA
• Immediate treatment
• ABC with ventilation
• Prostaglandin infusion (PGE)
• Maintenance of ductal patency – key to early survival
32
Q

Anatomical features of TOF?

A

• Large VSD
Tetralogy of Fallot
• Overriding of aorta with respect to ventricular septum
• Sub pulmonary stenosis causing right ventricular outflow tract
obstruction
• Right ventricular hypertrophy

33
Q

Symptoms and signs of TOF?

A

• Antenatal diagnosis • Murmur or cyanosis

  • Signs
  • Clubbing
  • Loud ejection systolic murmur
34
Q

Investigations for TOF?

A
  • Chest Xray
  • Relatively small heart (boot shaped) • Pulmonary artery bay
  • Decreased pulmonary markings
  • ECG
  • RVH when older
  • Echo
35
Q

Management of TOF?

A
• Stable infant
• Initial medical
• Surgery at 6 months
• Close VSD, relieve RVOT, sometimes artificial patch used
• Severe cyanosis
• Shunt to increase pulmonary blood flow (modified Blalock-Taussig
shunt)
• Balloon dilatation of RVOT
  • Hypercyanotic spells
  • Rare but important to recognise
  • rapid increase in cyanosis, irritability or inconsolable crying • Breathlessness and pallor
  • Short murmur
  • Management
  • Self limiting
  • If prolonged
  • Sedation and pain relief with morphine • Further treatment in hospital
36
Q

Anatomy of TGA?

A

• Anatomy
• Aorta connected to right ventricle, pulmonary artery connected to left
ventricle
• 2 parallel circulations and unless there is mixing of blood, it is incompatible with life
• Usually there are naturally occurring anomalies to help mixing

37
Q

Clinical features of TGA?

A
  • Cyanosis – profound and life threatening on D2
  • Can be less severe and delayed if associated anomalies • Second heart sound loud and single
  • Usually no murmur
38
Q

Investigations for TGA?

A
  • Chest x-ray
  • Narrow mediastinum with egg on side appearance • Increased pulmonary vascular markings
  • ECG normal
  • Management of sick cyanosed infant
  • Maintain patency of the duct with Prostaglandin infusion
  • Balloon atrial septostomy
  • Surgery in neonatal period – arterial switch Clinical features
39
Q

Describe Eisenmenger syndrome

A
  • If high pulmonary blood flow due to a large left to right shunt or common mixing is not treated early, pulmonary arteries become thick walled and shunt decreases and eventually reverses by about 10-15 years
  • Treatment is prevention
40
Q

What are the clinical features of conditions that result in common mixing of blood in the heart?

A
  • Atrioventricular septal defect (complete)
  • Downs syndrome
  • Defect in middle of heart with a single five-leaflet valve and pulmonary hypertension
  • Features
  • Antenatal screening
  • Cyanosis at birth
  • Routine echo in baby with Down’s syndrome
  • Superior axis on ECG
  • Treat heart failure medically and surgery at 3-6 months
41
Q

What causes outflow obstruction in a well child?

A
  • Aortic stenosis
  • Asymptomatic murmur
  • Critical aortic stenosis in neonatal period – heart failure and shock
  • Pulmonary stenosis
  • Asymptomatic
  • Critical pulmonary stenosis in neonatal period - cyanosis
  • Adult type coarctation of aorta
  • Becomes severe over time
  • Asymptomatic
  • Systemic hypertension in right arm • Brachio-femoral delay
42
Q

What cause outflow obstruction in a sick infant?

A
  • Coarctation of aorta
  • Acute collapse at 2 days of age • Heart failure
  • Absent femoral pulses
  • Severe metabolic acidosis
  • Hypoplastic left heart • Maintain ABC
  • Prostaglandin infusion • Surgery
43
Q

List cyanotic heart conditions

A
  • <48hrs
  • Persistent Pulmonary Hypertension of the newborn (PPHN)
  • Transposition of the great arteries (TGA)
  • Tricuspidatresia
  • Within first week
  • Ebstein’s anomaly • AVcanaldefect
  • After first week
  • Tetralogy of Fallot
  • Total Anomalous Pulmonary Venous Drainage
44
Q

List cyanotic heart conditions

A

• <1week
• Hypoplastic left heart • Severeaorticstenosis

>1 week
• Ventricular septal defect
• Patent Ductus Arteriosus
• Total Anomalous Pulmonary Venous Drainage

45
Q

list duct dependent lesions

A
  • Duct dependant lesions
  • Transposition of great arteries
  • Duct dependant pulmonary circulation (cyanotic) • Pulmonary atresia
  • Tricuspid atresia
  • Critical pulmonary stenosis
  • Ebstein’s anomaly
  • Duct dependant systemic circulation • Coarctation of aorta
  • Hypoplastic left heart syndrome
  • Critical aortic stenosis
46
Q

list non -duct dependent lesions

A
  • Non duct dependant lesions

* VSD, ASD, PDA, myocardial dysfunction, arrhythmias

47
Q

How would you manage SVT in a child?

A
  • Commonest arrhythmia with HR between 250-300/min • Poor cardia output and pulmonary oedema
  • In utero – hydrops fetalis or intrauterine death
  • ECG – narrow complex tachycardia
  • Wolf-Parkinson-White syndrome
  • Management in sick child
  • Restoration of sinus rhythm
  • Vagal stimulation manouevres
  • Carotid sinus massage, cold ice pack to face
  • Intravenous Adenosine • Electrical cardioversion
48
Q

What is the pathophysiology of rheumatic fever?

A
  • Due to abnormal immune response to preceding infection with group A β haemolytic streptococcal infection of pharynx (but not skin)
  • Affects children 5 –15 years
49
Q

What is the clinical presentation of rheumatic fever?

A
  • Clinical presentation
  • h/o ‘strep throat’ or tonsillitis 2-6 weeks prior
  • Jones major criteria • Pancarditis
  • Polyarthritis
  • Sydenham chorea
  • Erythema marginatum • Subcutaneous nodules
50
Q

What is the treatment for rheumatic fever?

A
  • Minor criteria
  • Fever, polyarthralgia, raised CRP, ESR
  • Treatment
  • Bed rest and anti-inflammatory agents – aspirin or prednisolone • Penicillin
  • Heart failure – diuretics and ACE inhibitors
  • Chronic rheumatic heart disease
  • Long term damage and scarring causes mitral stenosis • Other valves also affected
51
Q

What is the pathophysiology of cardiomyopathy?

A
  • Dilated cardiomyopathy
  • Secondary to viral infection of the heart • Secondary to metabolic disease
  • Inherited condition
  • Heart failure with enlarged heart
52
Q

what is the treatment for cardiomyopathy?

A
  • Treatment
  • Symptomatic with diuretics, ACE inhibitors
  • Heart transplantation
53
Q

what are the symptoms of infective endocarditis in children? What is the treatment?

A
  • Children of any age can be affected
  • Secundum ASD only CHD not affected
  • Symptoms
  • Persistent fever, malaise,
  • Peripheral stigmata of IE
  • Diagnosis
  • Blood cultures, echo
  • Treatment
  • Intravenous antibiotics for 6 weeks • Rarely surgical removal
  • Prophylaxis - good dental hygiene
54
Q

pathology of Kawasaki disease?

A

• Etiology - Unknown
• Demographics – 80% are < 4yrs, cases in >8 yrs and <3 months
are rare
• Pathology
• First 10 days – multisystem vasculitis with predilection for coronary arteries
• 1-3 weeks – coronary artery aneurysm may develop, pancarditis may be present
• Late changes – healing and fibrosis with thrombus formation, stenosis in postaneurysmal segment

55
Q

Clinical features of Kawasaki

A
  • Clinical features (essential criteria)
  • Fever >390 > 5 days
  • 4 of the following:
  • Erythema of palms & soles (acute), Peeling of fingers & toes (subacute)
  • Polymorphous exanthema(rash)
  • Bilateral bulbar conjunctivitis without exudate
  • Changes in lips & oral cavity – lip cracking, strawberry tongue
  • Cervical lymphadenopathy (>1.5 cm), usually unilateral
  • Beware of the ‘atypical’ presentations
56
Q

Treatment of Kawasaki

A
  • Treatment
  • Immunoglobulins
  • Aspirin
  • Referral to cardiologist