Paeds CARDIO Flashcards
Recall 2 differentials for a right atrium anomaly in children
Tricuspid atresia: requires ASD + VSD to remain patent to allow shunt
Ebstein’s anomaly: less severe as not reliant on shunting
Recall 3 differentials for a right ventricle anomaly in children
Pulmonary stenosis
Pulmonary atresia
Tetralogy of Fallot
(This is what it says in Ludley’s notes - bit confused about how pulmonary valve abnormalities are ventricular issues rather than atrial)
What is the tetralogy of fallot?
A cyanotic heart defect that is characterised by four features:
VSD
Overarching aorta
Right outflow tract obstruction
RV hypertrophy
Recall 2 differentials for a left atrium anomaly in children
Mitral stenosis
Mitral atresia
Recall 4 differentials for a left ventricle anomaly in children
Hypoplastic left heart
Coarctation of aorta
Interrupted arch
Aortic stenosis
What is ToGA?
Transposition of the Great Arteries - a congenital heart defect in which the aorta leaves the right ventricle and the pulmonary trunk leaves the left ventricle, resulting in the complete separation of the systemic and pulmonary circulations
When does ToGA present?
When ductus arteriosus closes at 2-4 days old
How can ToGA be managed?
Give prostaglandin infusion to keep DA open
Need urgent surgical readjustment
How quickly after birth does AVSD present?
First few hours of life
Where is the ductus arteriosus?
Between the aorta and pulmonary artery
When do aortic problems (coarctation or stenosis) present?
First few weeks of life (but not within days)
What test is used to diagnose heart disease in a cyanosed neonate, and how is it done?
Nitrogen washout test
Give 100% oxygen for 10 mins
Measure right radial artery blood gas oxygen
If it stays low (<15kPa) = positive for CHD
What is a ‘blue baby’ presentation a red flag for?
R-L shunt
What is a ‘breathless baby’ presentation indicative of?
L-R shunt
Recall the 3 types of L-R shunt
VSD, ASD, PDA
What are the differentials for cardiac outflow obstruction and how can you clinically differentiate between them
If child is otherwise well: P or A stenosis
If child is also in CV collapse + shock: coarctation
What does ‘cyanotic heart disease’ refer to?
Right to left shunt
What are the types of ASD and which is more common?
Secundum (more common): defect in atrial septum (failure of closure of foramen ovale)
Partial (AVSD): defect of AV septum
What are the signs and symptoms of ASD?
Asymptomatic
May have recurrent chest infections/ wheeze
What murmur is associated with ASD?
Ejection systolic murmum at ULSE
Fixed wide splitting of S2
How will the different types of ASD appear on ECG?
Secundum: RBBB + RAD
Partial: superior QRS axis
How should the different types of ASD be managed?
Secundum: cardiac catheterisation + insertion of occlusive device
Partial: surgical correction
What investigation is diagnostic of ASD?
Echo
How are VSDs classified?
By size:
small <3mm
large >3mm
What are the signs and symptoms of VSD?
Small: May be asymptomatic, may have breathless 3m old with normal sats, poor feeding with tiredness, LOUD murmur
Large: Heart failure, SOB, recurrent chest infections, hepatomgaly
Describe the murmur in VSD?
Small: LOUD, Pan-systolic, LLSE
Large: SOFT pan-systolic murmur
How shouls small VSDs be managed?
Self-limiting: they close over time
What does a small VSD increase the risk of?
Endocarditis
What does large VSD increase the risk of?
Eisenmenger syndrome - “the process in which a long-standing left-to-right cardiac shunt caused by a congenital heart defect causes pulmonary HTN + eventual reversal of the shunt into a cyanotic right-to-left shunt”
How should a large VSD be managed?
CDC
Calories (additional calorie input)
Diuretics
Captopril
Surgery is usually performed at 3-6 months to prevent permanent lung damage from pulmonary HTN + high blood flow
By what time should the DA usually close?
1 month postpartum
Describe the murmur in PDA
Continuous ‘machine-like’ at ULSE
What would be found OE in PDA?
Left sub-clavicular thrill
Heaving apex beat
Wide pulse pressure
Bounding, collapsing pulses
Resp symptoms from increased work
Recall the medical and surgical management of PDA
Medical: Indomethacin (NSAID): to prompt duct closure
Surgical: at 1 year
How can cyanosis be tested for?
Hyperoxia nitrogen washout test
How should cyanosis be immediately managed?
ABCs
Prostaglandin infusion (to maintain PDA patency)
Recall the timeline of presentation of the different types of cyanotic heart disease after birth
<10 mins: Tricuspid atresia
Few hours: ToGA
Up to 3 weeks: AVSD
Any age (at a few days, often): ToF
10-15 years: Eisenmenger
Which 2 types of cyanotic HD produce an ESM at the left sternal edge?
ToGA + ToF
What is Ebstein’s abnormality?
Malformation of tricuspid valve leading to severe tricuspid regurgitation
What maternal medication is associated with Ebstein’s abnormality?
Lithium
What would be heard on auscultation in Ebstein’s abnormality?
split 1st + 2nd heart sounds
How should Ebstein’s be managed?
Prostaglandin - cone repair of tricuspid valve
What is tricuspid atresia?
Complete absence of tricuspid valve: so LV is the only effective ventricle (right too small)
Describe the presentation of tricuspid atresia
Cyanosis + SOB within first 10 mins of life
What is the murmur in tricuspid atresia?
ESM at LSE due to VSD
Recall in detail the management of tricuspid atresia
1st need to maintain a secure supply of blood to the lungs
Option 1 is a Blalock-Taussig shunt insertion
Option 2 is pulmonary banding operation to reduce pulmonary blood flow
COMPLETE CORRECTIVE SURGERY NOT POSSIBLE IN MOST CASES
Why is ToGA not instantly fatal?
Usually found alongside ASDs/ VSDs/PDAs etc which aid mixing
What is heard upon auscultation in ToGA?
Loud S2 but no murmur
What investigations should be done in suspected ToGA?
CXR - cardiomegaly, ‘egg-on-a-string’ appearance
Echo - diagnostic
What would a CXR show in ToGA??
Narrow upper mediastinum (‘egg on side’)
Recall the management of ToGA
Immediate prostaglandin infusion to maintain PDA patency
Balloon atrial septostomy (this tears atrial septum down to allow mixing)
Arterial surgery to switch the vessels
What is the most common association with AVSD?
Down’s syndrome
How does AVSD present?
Cyanosis at 2-3w of life
How should AVSD be managed?
Treat heart failure medically + surgery at 3 months
What are the 4 characteristics of the tetralogy of fallot?
VSD
Overriding aorta (compression of PA –> )
Pulmonary stenosis (back pressure –> )
RVH
What would be heard on auscultation in tetralogy of fallot?
Murmur from pulmonary stenosis = loud ESM at left lower sternal border
What investigations should be done in suspected tetralogy of fallot?
CXR
Echo
What does CXR show in tetralogy of fallot?
Boot-shaped, small heart due to RVH
How is tetralogy of fallot managed?
1st medical, then surgery at 6 months old
Medical: PG/ alprostadil to maintain PDA + reverse severe cyanosis
Severe/ prolonged cyanosis should be managed using a BT shunt from subclavian-pulmonary artery or balloon dilatation of RV outflow
What is Eisenmenger syndrome?
Irreversibly raised pulmonary vascular resistance from chronically raised pulmonary arterial pressure + flow (ie from a large VSD or chronic PDA) which leads to formation of a right to left shunt
Recall the pathophysiology of Eisenmenger syndrome
High pulmonary flow from large L-to-R shunt untreated
Artery wall thickens
Resistance increases
Eventually shunt decreases + child becomes less symptomatic
At 10-15y the shunt reverses as a teenager + they become blue + cyanotic
How should Eisenmenger be managed?
Early intervention for pulmonary blood flow
Heart transplantation not easy but can be done
What is the cause of congenital aortic/ pulmonary stenosis?
Partial fusion of valve leaflets
What are the most likely co-existent conditions with aortic/pulmonary stenosis?
Coarctation of aorta + mitral valve stenosis
What are the signs and symptoms of aortic/pulmonary stenosis?
NO CYANOSIS
AS: carotid thrill (ESM)
PS: no carotid thrill, harsh heart murmur at LSE (ESM)
How should a/p stenosis be managed?
Transcatheter balloon dilatation
When does coarctation of the aorta present?
3 days - a few weeks of life
Recall some signs and symptoms of coarctation of the aorta
Sx: asymptomatic
Signs:
1. ESM
2. High BP in arms, low BP in legs
How should coarctation be managed?
If sick infant: follow ABC + PG infusion guidelines
If well child –> surgical repair OR balloon angioplasty + stenting
Recall the signs and symptoms of hypoplastic left heart syndrome
Often the sickest of all the left-outflow presentations
Will be cyanotic
How should hypoplastic left heart syndrome be managed?
1st = ABCs + PG
2nd = Blalock-Taussig (BK) shunt or Norwood Stage 1
3rd = BK shunt removal –> Glenn/ hemi-Fontan –> Fontan/ Total Cavo-Pulmonary Connection
What is a BK shunt?
Arterficial ductus arteriosus
What HR is expected in SVT?
250-300bpm
Recall the main symptom of SVT in neonates
Hydrops fetalis
What would be seen on an ECG in SVT?
Narrow complex tachycardia + T wave inversion due to ischaemia
How should SVT be managed?
- Circulatory + respiratory support (correct any tissue acidosis)
- Vagal stimulating manoevres - 80% success
- IV adenosine
- Electrical cardioversion with synchronised DC shock if adenosine fails
What is the common cause of rheumatic fever?
Group A beta-haemolytic strep
What age child can get rheumatic fever?
5-15 y/o
What is the long term risk of rheumatic fever?
Mitral stenosis
Describe the typical presentation of rheumatic fever
Latent interval of 2-6w after pharyngeal infection: polyarthritis
Pericarditis
Erythema marginatum (map-like outlines)
What are the diagnostic criteria for rheumatic fever?
Jones criteria
2 majors / 1 major + 2 minors
Major = CASES
Carditis, Arthritis, SC nodules, Erythema marginatum, Sydenham’s chorea
Minor = FRAPP: fever, raised ESR/CRP, Arthralgia, Prolonged PR, Previous RF
How should rheumatic fever be managed?
Anti-inflammatories
High dose aspirin (suppresses inflammatory responses in heart + joints)
Abx (if evidence of present infection)
Corticosteroids (if not resolved rapidly)
What should be done following resolution of rheumatic fever?
Prophylatic monthly injections of benzyl penicillin until age 21
May need surgical valve repair
Recall the signs and symptoms of infective endocarditis in a child
Necrotic skin lesions: from infected emboli
Splinter haemorrhages
Changing cardiac signs
Fever, aneamia, pallor
Splenomegaly
Arthritis/ arthralgia
Clubbing
How is infective endocarditis diagnosed in children?
Multiple blood cultures (before ABx) + echocardiography to identify vegetations
What is the most common pathogen implicated in paediatric IE?
Streptococcus viridians
How should infective endocarditis be managed in children?
6 weeks IV Abx
Strep viridians (native/prosthetic valve) = amoxicillin + gentamicin/vancomycin
Staph aureus (native valve): Amoxicillin/ vancomycin/ daptomycin
Staph aureus (prosthetic valve): nafcillin/ oxacillin
How is the liver affected by cardiac failure?
May see hepatomegaly
How is the respiratory system affected by cardiac failure?
Increased RR
Recurrent chest infections
What basic investigations are necessary in cardiac failure?
O2 sats
BP
FBC
U+Es
calcium
BNP/ANP
Recall systematically the management of paediatric cardiac failure
Decrease preload: diuretics (furosemide)/ GTN
Enhance contractility: eg digoxin, dopamine, dobutamine
Reduce afterload: ACE inhibitors
Improve oxygen delivery: B-blockers (eg carvedilol)
How should cyanosis be managed?
Prostaglandin infusion
How would tetralogy of fallot present?
Cyanosis – possibly at rest depending on severity
Dyspnoea on feeding / crying / exertion
Failure to thrive
Murmur – usually harsh, and at the left sternal edge, grade 3-6.
- Due to pulmonary outflow obstruction rather than VSD
- Systolic thrill may also be present
What is the most common congenital heart defect?
Tetralogy of Fallot
What is a ‘tet spell’?
- Sudden onset dyspnoea / cyanosis
- Typically triggered by an event that slightly reduces O2 concentration (e.g. crying, defecating, feeding, distress)
- Can be a vicious cycle, as the tet spell can make the child more distressed
- Sometimes preceded by rapid, deep breathing
- This increases venous return to the right ventricle, whose output is mainly via the aorta – and thus this is part of the vicious cycle
Severe spells can lead to death.
Who do tet spells tend to affect?
Babies aged between 2 and 4 months old
How may a toddler react to a tet spell?
Some toddlers may adopt a squatting position – which reduces venous return to the heart, and may help increase blood O2
What are some risk factors for ToGA?
Maternal diabetes
Maternal smoking
Advanced maternal age
Why is ToGA a ‘shunt-dependent’ defect?
Because infants survive only if a shunt between the two circulations exist to mix oxygenated blood into the systemic circulation such as:
- Patent ductus arteriosus (PDA)
- Ventricular septal defect (VSD)
- Atrial septal defect (ASD)
- Patent foramen ovale (PFO)
What the normal HR ranges for a) under 1, b) 1-2, c) 2-5, d) 5-12 and e) >12?
<1: 110-160
1-2: 100-150
2-5: 95-140
5-12: 80-120
>12: 60-100 (normal adult range)
What the normal RR ranges for a) under 1, b) 1-2, c) 2-5, d) 5-12 and e) >12?
<1: 30-40
1-2: 25-35
2-5: 25-30
5-12: 20-25
>12: 15-20
