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PAEDS CARDIOLOGY Flashcards

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1
Q

FOETAL CIRCULATION
What 3 foetal shunts are there?

A
  • Ductus venosus = connects umbilical vein + IVC so blood bypasses liver
  • Foramen ovale = connects RA+LA so bypass RV + pulmonary circulation
  • Ductus arteriosus = connects pulm. artery + aorta so blood can bypass pulm. circulation
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2
Q

FOETAL CIRCULATION
What is the flow of foetal blood?

A
  • Oxygenated + nutrients at placenta for rest of body (umbilical vein) + disposes waste like CO2 + lactate (umbilical artery)
  • Umbilical vein > ductus venosus > RA > foramen ovale > LA > LV > rest of body > umbilical artery
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3
Q

FOETAL CIRCULATION
What are the pressures like within the foetal heart?

A
  • LA pressure low as relatively little blood returns from lungs
  • RA>LA pressure as RA receives all systemic venous return + blood from placenta
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4
Q

FOETAL CIRCULATION
What happens in the first few breaths in the foetus?

A
  • Resistance to pulmonary blood flow falls as alveoli expand + volume of blood flowing through lungs massively increases so increased LA pressure.
  • Volume of blood returning to RA falls as placenta removed
  • LA > RAp = foramen ovale closes
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5
Q

FOETAL CIRCULATION

What happens over the next few hours/days?

A
  • Ductus arteriosus will close (issue if duct-dependent CHD)
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6
Q

FOETAL CIRCULATION
What are physiological (innocent flow) murmurs?

A

4S’s –
- Soft blowing murmur
- Symptomless
- left Sternal edge
- Systolic murmur only

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7
Q

FOETAL CIRCULATION
What other features of innocent murmurs are there?

A
  • Normal heart sounds (none added),
  • no parasternal thrill or radiation,
  • may vary with posture
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8
Q

FOETAL CIRCULATION
What are the 5 main types of congenital heart lesions?

A
  • L>R shunt (breathless) = ASD, VSD, PDA
  • R>L shunt (cyanotic) = ToF, TGA
  • Common mixing (breathless + blue) = complete AVSD, complex CHD (tricuspid atresia)
  • Outflow obstruction in well child = AS, PS
  • Outflow obstruction in sick neonate = coarctation, HLHS
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9
Q

FOETAL CIRCULATION
What is Eisenmenger’s syndrome?

A
  • L>R shunt as systemic pressure is higher than pulmonary pressure
  • Over time, pulmonary pressure may increase beyond the systemic pressure
  • This is due to pulmonary HTN > increasing RH pressures + so RVH leading to shunt reversal (R>L) + so cyanosis
  • May have plethoric complexion due to compensatory polycythaemia
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10
Q

FOETAL CIRCULATION
What are the main cyanotic heart diseases?

A

4Ts –
- ToF
- TGA
- Tricuspid atresia
- Truncus arteriosus
(Complete AVSD too)

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11
Q

FOETAL CIRCULATION
How can you determine if cyanosis is cardiac or respiratory?

A
  • Hyperoxic test, better = respiratory, still cyanosed = cardiac
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12
Q

ATRIAL SEPTAL DEFECT
What is atrial septal defect (ASD)?

A
  • Hole in septum connecting atria as failure of septal tissue to form
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13
Q

ATRIAL SEPTAL DEFECT
What are the 3 main types of ASD?

A
  • Ostium primum (group with AVSD)
  • Ostium secundum (80%)
  • Partial AVSD
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14
Q

ATRIAL SEPTAL DEFECT
What is a partial AVSD?

A
  • Inter-atrial communication between bottom end of atrial septum + AV valves
  • Abnormal AV valves with a left AV valve which has 3 leaflets + tends to leak
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15
Q

ATRIAL SEPTAL DEFECT
What is the clinical presentation of ASD?

A
  • Dyspnoea, difficulty feeding, failure to thrive, recurrent chest infections
  • Arrhythmia in adulthood (may need VTE prophylaxis)
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16
Q

ATRIAL SEPTAL DEFECT
What signs would you find on clinical examination in ASD?

A
  • Fixed + widely split S2 (split does not change with inspiration/expiration)
  • ES murmur at upper L sternal edge (pulmonary) as increased flow across pulmonary valve by L>R shunt
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17
Q

ATRIAL SEPTAL DEFECT
What are some investigations for ASD?

A
  • Often antenatal Dx
  • CXR = cardiomegaly, enlarged pulmonary arteries + increased pulmonary vascular markings
  • Primum ECG = RBBB + LAD
  • Secundum ASD = RBBB + RAD
  • ECHO is diagnostic
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18
Q

ATRIAL SEPTAL DEFECT
What are the complications of ASD?

A
  • Eisenmenger syndrome = shunt switch = cyanotic

- Stroke risk in context of VTE (can be from AF or atrial flutter)

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19
Q

ATRIAL SEPTAL DEFECT
What is the management of ASD?

A
  • Small + asymptomatic = watchful waiting

- Large = transvenous catheter closure via femoral vein or open heart surgery

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20
Q

VSD
What is a ventricular septal defect (VSD)?

A
  • Hole in the septum between the 2 ventricles, most common heart defect
  • L>R shunt as LVp>RVp so increased flow to R heart + lungs
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21
Q

VSD
What are some conditions associated to VSD?

A
  • Trisomy 13, 18 + 21
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22
Q

VSD
What is the clinical presentation of VSD?

A
  • Small = ?asymptomatic
  • Large = heart failure with dyspnoea, failure to thrive, recurrent infections
  • Harsh pansystolic murmur
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23
Q

VSD
What are the features of the pansystolic murmur in VSD?

A
  • Left lower sternal edge
  • Loud murmur = smaller VSD (larger = quieter)
  • May have systolic thrill on palpation
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24
Q

VSD
What are the investigations in VSD?

A
  • Often antenatal Dx or murmur on NIPE
  • CXR + ECG often normal in small VSDs
  • CXR in large = cardiomegaly, increased pulmonary vascular markings ± pulmonary oedema
  • ECHO is diagnostic
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25
VSD What are some complications of VSD?
- Increased risk of infective endocarditis > Abx prophylaxis during surgery - AR, Eisenmenger's syndrome + right heart failure
26
VSD What is the management of VSD?
- Small VSDs with no signs of pulmonary HTN or heart failure may watch + wait as may close spontaneously - Transvenous catheter closure via femoral vein or open-heart surgery
27
HEART FAILURE What are the causes of heart failure in neonates?
Obstructed or duct-dependent systemic circulation (HLHS, severe coarctation)
28
HEART FAILURE What is the clinical presentation of left heart failure?
- SOB (esp. on feeding or exertion), sweating, recurrent chest infections - Poor weight gain, gallop rhythm - Cardiomegaly, cool peripheries - As a rule = increased HR, RR + creps
29
HEART FAILURE What is the clinical presentation of right heart failure? What are the causes?
- Peripheral oedema - Hepatomegaly - TR, PS, large VSD (anything making fluid overload backwards > IVC)
30
HEART FAILURE What is the management of heart failure?
- Furosemide (loop diuretic) - Captopril (ACEi) - Increased calories
31
COMPLETE AVSD What is a complete atrioventricular septal defect (AVSD)?
- Defect in middle of heart with single 5-leaflet valve between atria + ventricles which stretches across entire AV junction + tends to leak - Large defect means pulmonary HTN too
32
COMPLETE AVSD What condition is complete AVSD commonly seen in? How does it present?
- Down's syndrome (need routine ECHO) - Cyanosis at birth with heart failure 2–3w of life, no murmur
33
COMPLETE AVSD What is the management of complete AVSD?
- May have antenatal Dx or on routine ECHO in Down's baby - Medical Tx for heart failure + surgical repair at 3-6m
34
PDA What is a patent ductus arteriosus (PDA)?
- DA normally stops functioning within 1–3d - If patent, pressure in aorta higher than in pulmonary vessels so blood flows L>R
35
PDA What can happen due to increased blood flowing through pulmonary vessels?
- More blood returning to left side of heart so can lead to LVH
36
PDA What are the symptoms of PDA?
- Small may be asymptomatic or present in adulthood with heart failure - May present with SOB, difficulty feeding, failure to thrive + LRTIs
37
PDA What are the signs of PDA?
- Collapsing or bounding pulse as increased pulse pressure - Continuous 'machinery' murmur heard loudest beneath the L clavicle
38
PDA What are some investigations for PDA?
- ECHO to confirm Dx - Doppler flow studies during ECHO can assess size + characteristics - Can also assess effects of PDA on heart (LVH/RVH)
39
PDA What is the management of PDA?
- Monitor until 1y with ECHOs (treat early if Sx or heart failure) - NSAIDs (indomethacin) facilitates closure of PDA as inhibits prostaglandins - After 1y unlikely to resolve so trans-catheter or surgical closure to reduce IE risk
40
TOF What abnormalities are described in tetralogy of fallot (TOF)?
- Large VSD - Pulmonary stenosis (RV outflow obstruction) - RVH - Overriding aorta (If ASD present too = pentad of Fallot)
41
TOF What is the pathophysiology of TOF?
- Pulmonary stenosis leads to RVp>LVp so R>L shunt and cyanosis - Cyanosis presents later than in TGA, about 1–2m
42
TOF What is TOF associated with?
- Trisomy 21 + 22q deletions
43
TOF What is the clinical presentation of TOF?
- Severe cyanosis with exertional hyper-cyanotic 'tet' spells - Loud harsh ES murmur at left sternal edge (from PS) - Digital clubbing - Squatting on exercise = increased peripheral vascular resistance + decreased degree of R>L shunt so improved Sx
44
TOF What happens during a hyper-cyanotic tet spell?
- Rapid increase in cyanosis, irritability + dyspnoea - If severe can lead to reduced GCS, seizures + potential death
45
TOF What are the investigations for TOF?
- May be Dx antenatally or murmur on NIPE - CXR = 'boot shaped' heart due to RVH - ECHO ± cardiac catheterisation to show anatomy + degree of stenosis
46
TOF What is the management of a hyper-cyanotic tet spell in TOF?
- Morphine for sedation + pain relief - IV propranolol as peripheral vasoconstrictor - IV fluids, sodium bicarbonate if acidotic
47
TOF What is the management of TOF?
- Neonates = prostaglandin infusion to maintain ductus arteriosus to allow blood to flow from aorta > pulmonary arteries - Early surgical repair with closure of VSD + correction of pulmonary stenosis at 6m
48
TGA What is transposition of the great vessels (TGA)?
- Aorta is connected to the RV, pulmonary artery is connected to LV meaning deoxygenated blood is pumped around body = cyanosis
49
TGA What is the clinical presentation of TGA?
- Cyanosis at birth/day 1–2 as duct closes > life-threatening - Less severe if PDA, ASD or VSD to allow mixing - No murmur but S2 usually loud + singular - Prominent RV impulse on palpation
50
TGA What are the investigations for TGA?
- May be Dx antenatally, pre (R arm) + post duct (foot) sats - CXR may show narrow mediastinum with 'egg on its side' appearance - ECHO confirms Dx
51
TGA What is the management of TGA?
- Neonates = prostaglandin E1 infusion to maintain ductus arteriosus - Balloon atrial septostomy to create hole between 2 atria for mixing - Arterial switch procedure = open heart surgery, definitive Mx
52
TRICUSPID ATRESIA What is tricuspid atresia?
- Only LV is effective, RV is small, non-functional + complete absence of tricuspid valve
53
TRICUSPID ATRESIA How does tricuspid atresia present?
- 'Common mixing' of systemic + pulmonary venous return in LA = cyanosis + dyspnoea
54
COARCTATION OF AORTA What is coarctation of the aorta?
- Arterial duct tissue encircling the aorta at the level of the ductus arteriosus which causes constriction + narrowing when the duct closes
55
COARCTATION OF AORTA What is a consequence of coarctation of aorta?
- Collateral circulation forms to increase flow to the lower part of the body leading to the intercostal arteries becoming dilated + tortuous
56
COARCTATION OF AORTA What is the clinical presentation of coarctation of aorta? How may it present if severe?
- Weak femoral pulses + radiofemoral delay - Systolic murmur between scapulas or below L clavicle - Heart failure, tachypnoea, poor feeding, floppy - LV heave (LVH) - Acute circulatory collapse at 2d as duct closes (duct dependent)
57
COARCTATION OF AORTA What are the investigations for coarctation of the aorta?
- 4 limb BP (R arm > L arm), pre + post-duct sats - CXR may show cardiomegaly + rib notching (often teens + adults)
58
COARCTATION OF AORTA What is the management of coarctation of aorta?
- ABCDE if collapse - Prostaglandin E1 infusion if critical - Stent insertion or surgical repair
59
HYPOPLASTIC LEFT HEART What is hypoplastic left heart syndrome (HLHS)?
- Under development of entire left side of heart - Mitral valve is small or atretic, LV is diminutive + usually aortic valve atresia - Ascending aorta very small + almost invariably coarctation of aorta
60
HYPOPLASTIC LEFT HEART What is the clinical presentation of HLHS?
- Sickest neonates with duct-dependent circulation - No L side flow so ductal constriction > profound acidosis + rapid CV collapse - Weakness or absence of all peripheral pulses
61
HYPOPLASTIC LEFT HEART What is the management of HLHS?
- ABCDE - Prostaglandin E1 infusion to prevent duct closure - Surgical management
62
EBSTEIN'S ANOMALY What is Ebstein's anomaly?
- Low insertion of tricuspid valve on right leading to larger atrium + smaller ventricle - Leads to poor RA>RV flow + so poor flow to pulmonary vessels - Associated with R>L shunt via ASD + so blood can bypass lungs > cyanosis
63
EBSTEIN'S ANOMALY What is Ebstein's anomaly associated with?
- Wolff-Parkinson-White syndrome + lithium in pregnancy
64
EBSTEIN'S ANOMALY What is the clinical presentation of Ebstein's anomaly?
- Evidence of heart failure - SOB, tachypnoea, poor feeding, collapse or cardiac arrest - Gallop rhythm with S3 + S4 - Cyanosis few days after birth if ASD when ductus arteriosus closes
65
EBSTEIN'S ANOMALY What are the investigations for Ebstein's anomaly?
- ECG = arrhythmias, RA enlargement (P pulmonale), LAD + RBBB - CXR = cardiomegaly + RA enlargement - ECHO diagnostic
66
EBSTEIN'S ANOMALY What is the management of Ebstein's anomaly?
- Prophylactic Abx to prevent infective endocarditis - Definitive Mx = surgical correction
67
AORTIC STENOSIS What is aortic stenosis?
- Aortic valve leaflets partly fused together giving restrictive exit from LV
68
AORTIC STENOSIS What is aortic stenosis associated with?
- Bicuspid aortic valve + William's syndrome (supravalvular) - Also may be mitral stenosis + coarctation of aorta too
69
AORTIC STENOSIS What is the normal clinical presentation of aortic stenosis?
- Most asymptomatic with ejection-systolic murmur at upper right sternal edge (aortic area) radiating to neck (carotid thrill) - Ejection click before murmur - Palpable systolic thrill - Slow rising pulses + narrow pulse pressure
70
AORTIC STENOSIS How does more severe aortic stenosis present?
- Severe = syncope, chest pain + dyspnoea on exertion - Critical = severe heart failure + shock
71
AORTIC STENOSIS What are the investigations for aortic stenosis?
- CXR (prominent LV), - regular ECHO to assess need for interventions
72
AORTIC STENOSIS What is the management of aortic stenosis?
- Balloon valvotomy/dilatation if symptomatic - May need surgical aortic valve replacement
73
PULMONARY STENOSIS What is pulmonary stenosis? What is it associated with?
- Pulmonary valve leaflets are partly fused together giving a restrictive exit from RV - ToF, Noonan syndrome + congenital rubella syndrome
74
PULMONARY STENOSIS What is the clinical presentation of pulmonary stenosis?
- Ejection systolic murmur at upper left sternal edge with ejection click - ?RV heave due to RVH - Critical PS = duct-dependent pulmonary circulation so cyanosis in first few days of life
75
PULMONARY STENOSIS What are the investigations for pulmonary stenosis?
- CXR normal or post-stenotic dilatation of the pulmonary artery - ECG may show RVH (upright T wave in V1) - ECHO Dx
76
PULMONARY STENOSIS What is the management of pulmonary stenosis?
- Most asymptomatic so wait until pressure gradient across pulmonary valve increases > trans-catheter balloon dilatation
77
RHEUMATIC FEVER What is the pathophysiology of rheumatic fever?
- Multi-system disorder due to autoimmune response producing antibodies against group A beta-haemolytic strep pyogenes (after tonsillitis) that targets other tissues - T2 hypersensitivity reaction as immune system attacks cells throughout body
78
RHEUMATIC FEVER How is rheumatic fever diagnosed?
Jones criteria – - Evidence of recent strep infection plus 2 major or 1 major + 2 minor criteria
79
RHEUMATIC FEVER What are the major criteria in rheumatic fever?
JONES – - Joint arthritis (migratory as affects different joints at different times) - Organ inflammation (pancarditis > pericardial friction rub) - Nodules (subcut over extensor surfaces) - Erythema marginatum rash (pink rings of varying sizes on torso + proximal limbs) - Sydenham chorea
80
RHEUMATIC FEVER What are the minor criteria in rheumatic fever?
FEAR – - Fever - ECG changes (prolonged PR interval) without carditis - Arthralgia without arthritis - Raised CRP/ESR
81
RHEUMATIC FEVER What are the investigations for rheumatic fever
- Throat swab for MC&S - Anti-streptococcal antibodies (ASO) titres = anti-DNase B +ve indicates strep infection (repeat after 2w to check if negative) - Echo, ECG + CXR to check cardiac involvement
82
RHEUMATIC FEVER What are some complications of rheumatic fever?
- Recurrence - Valvular heart disease (mitral stenosis especially) - Chronic heart failure - Recurrent acute RF > scarring + fibrosis of heart valves > chronic RF
83
RHEUMATIC FEVER What is the management of rheumatic fever?
- Prevention by treating strep infections with 10d phenoxymethylpenicillin - Specialist Mx (NSAIDs for joint pain, aspirin + steroids for carditis) - Prophylactic 1/12 IM benzathine penicillin most effective to prevent recurrence (if not daily PO penicillin)
84
DILATED CARDIOMYOPATHY What is it?
Large, poorly contracting heart can be inherited or secondary to metabolic disease or direct viral infection of myocardium (myocarditis)
85
DILATED CARDIOMYOPATHY What is the management of dilated cardiomyopathy?
- Dx by ECHO - Sx treatment with diuretics, ACEi + carvedilol (beta blocker)
86
SUPRAVENTRICLAR TACHYCARDIA What is a supraventricular tachycardia? Give a type
- Re-entry tachycardia as circuit of conduction is set up with premature activation + excitation of the atrium via an accessory pathway - Wolff-Parkinson-White syndrome accessory pathway is Bundle of Kent
87
SUPRAVENTRICLAR TACHYCARDIA How does a supraventricular tachycardia present?
- Sx of heart failure in young child, palpitations, dyspnoea
88
SUPRAVENTRICLAR TACHYCARDIA What is the management of a supraventricular tachycardia?
- 1st line = Vagal stimulation (carotid sinus massage, cold ice pack to face) - 2nd line = IV adenosine - 3rd line = Electrical cardioversion - Long term = ablation of pathway or flecainide
89
ATRIAL SEPTAL DEFECT What is the pathophysiology?
L>R shunt as LAp>RAp so increased flow into R heart + lungs - Trisomy 21, foetal
90
ATRIAL SEPTAL DEFECT What is it common in?
Trisomy 21, foetal alcohol syndrome
91
PDA What are some risk factors of PDA?
Prematurity is key + association with maternal rubella
92
FOETAL CIRCULATION What are the foetal shunts called when they are closed?
- Ductus venosus -> ligamentum venosus - Foramen ovale -> fossa ovalis - Ductus arteriosus -> ligamentum arteriosus
93
FOETAL CIRCULATION When are physiological (innocent flow) murmurs seen?
Common during febrile illness or anaemia as CO increases
94
FOETAL CIRCULATION When would you investigate an innocent murmur?
Louder than 2/6, diastolic, louder on standing
95
FOETAL CIRCULATION What is a complication of SLE?
Complete heart block
96
HEART FAILURE What are the causes of heart failure in infants?
High pulmonary blood flow (VSD, AVSD, large PDA)
97
HEART FAILURE What are the causes of heart failure in older children?
Eisenmenger's syndrome (RHF), rheumatic disease, cardiomyopathy
98
PDA What is a consequence?
- If patent, pressure in aorta higher than in pulmonary vessels so blood flows L>R - Can lead to Eisenmenger's syndrome
99
TOF What are some risk factors?
- Rubella, - maternal age >40, - alcohol in pregnancy, - maternal DM
100
TGA What is it associated with?
Duct dependent lesion, associated with PDA, ASD + VSD
101
TRICUSPID ATRESIA How is it managed?
Shunt between subclavian + pulmonary artery with surgery later
102
COARCTATION OF AORTA What is it associated with?
Turner's syndrome
103
AORTIC STENOSIS What are some complications?
LV outflow tract obstruction, heart failure ventricular arrhythmias
104
DILATED CARDIOMYOPATHY When would you suspect dilated cardiomyopathy?
- Any child with enlarged heart + heart failure who was previously well
105
DILATED CARDIOMYOPATHY What is the management of myocarditis?
Myocarditis usually improves spontaneously but some may need heart transplant
106
SUPRAVENTRICLAR TACHYCARDIA What is the ECG like?
- Narrow complex tachycardia (250-300bpm) - WPW = delta wave (slurred upstroke to QRS) with a short PR interval

MY REVISION - ALL PHASES (36 decks)

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