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
2
Q
Symptoms of heart failure in children?
A
- Breathlessness (particularly on feeding or exertion) • Sweating
- Poor feeding
- Recurrent chest infections
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
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
5
Q
What is the differential diagnosis for heart failure that presents in infancy?
A
(high pulmonary blood flow)
• VSD, ASD, large PDA
6
Q
What is the differential diagnosis for heart failure that presents in older children?
A
Eisenmenger syndrome, cardiomyopathy
7
Q
Cyanosis in a newborn infant differential.
A
- Cardiac disorders – cyanotic
- Respiratory disorders, persistent pulmonary hypertension
- Infections – septicaemia
- Metabolic diseases
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)
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
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
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
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
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
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
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
16
Q
What are the subtypes of VSD?
A
muscular, membranous, endocardial, sub-arterial
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
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
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
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
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
22
Q
what are the symptoms of an atrial septal defect?
A
- Symptoms • None
- Recurrent chest infections /wheeze
- Arrhythmia from fourth decade onwards
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
Management of ASD?
* Management
* Significant ASD
* Occlusion device • pAVSD
* Surgical correction
* Usually 3-5 years of age
26
What is the anatomy of a persistent ductus arteriosis?
* Normally closes after birth, if failed to close by 1 month abnormal • Blood flow from aorta to pulmonary artery
* Preterm baby - separate entity
27
Clinical features of PDA?
* Symptoms unusual
* Can develop heart failure and pulmonary hypertension • Continuous murmur beneath left clavicle
* Bounding pulse
28
Describe coarctation of the aorta
• Narrowing of the aortic lumen due to failure of ductus arteriosus to develop correctly
29
clinical presentation of coarctation of the aorta
* Clinicalpresentation
* Poor feeding
* Respiratory distress
* Shock
* Weak femoral pulses
* Murmurmay/maynotbepresent
* Gallop rhythm, heart failure (1-4 weeks)
30
what is the treatment for coarctation?
* Treatment
* Resuscitation + Prostaglandin
* Surgical repair
31
How is a right to left shunt investigated and treated?
```
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
Anatomical features of TOF?
• 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
Symptoms and signs of TOF?
• Antenatal diagnosis • Murmur or cyanosis
* Signs
* Clubbing
* Loud ejection systolic murmur
34
Investigations for TOF?
* Chest Xray
* Relatively small heart (boot shaped) • Pulmonary artery bay
* Decreased pulmonary markings
* ECG
* RVH when older
* Echo
35
Management of TOF?
```
• 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
Anatomy of TGA?
• 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
Clinical features of TGA?
* 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
Investigations for TGA?
* 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
Describe Eisenmenger syndrome
* 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
What are the clinical features of conditions that result in common mixing of blood in the heart?
* 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
What causes outflow obstruction in a well child?
* 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
What cause outflow obstruction in a sick infant?
* 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
List cyanotic heart conditions
* <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
List cyanotic heart conditions
• <1week
• Hypoplastic left heart • Severeaorticstenosis
•
>1 week
• Ventricular septal defect
• Patent Ductus Arteriosus
• Total Anomalous Pulmonary Venous Drainage
45
list duct dependent lesions
* 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
list non -duct dependent lesions
* Non duct dependant lesions
| * VSD, ASD, PDA, myocardial dysfunction, arrhythmias
47
How would you manage SVT in a child?
* 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
What is the pathophysiology of rheumatic fever?
* 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
What is the clinical presentation of rheumatic fever?
* Clinical presentation
* h/o ‘strep throat’ or tonsillitis 2-6 weeks prior
* Jones major criteria • Pancarditis
* Polyarthritis
* Sydenham chorea
* Erythema marginatum • Subcutaneous nodules
50
What is the treatment for rheumatic fever?
* 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
What is the pathophysiology of cardiomyopathy?
* Dilated cardiomyopathy
* Secondary to viral infection of the heart • Secondary to metabolic disease
* Inherited condition
* Heart failure with enlarged heart
52
what is the treatment for cardiomyopathy?
* Treatment
* Symptomatic with diuretics, ACE inhibitors
* Heart transplantation
53
what are the symptoms of infective endocarditis in children? What is the treatment?
* 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
pathology of Kawasaki disease?
• 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
Clinical features of Kawasaki
* 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
Treatment of Kawasaki
* Treatment
* Immunoglobulins
* Aspirin
* Referral to cardiologist
