PAEDS - CARDIOVASCULAR Flashcards
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
2
Q
FOETAL CIRCULATION
What are the foetal shunts called when they are closed?
A
- Ductus venosus -> ligamentum venosus
- Foramen ovale -> fossa ovalis
- Ductus arteriosus -> ligamentum arteriosus
3
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
4
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
5
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
6
Q
FOETAL CIRCULATION
What happens over the next few hours/days?
A
- Ductus arteriosus will close (issue if duct-dependent CHD)
7
Q
FOETAL CIRCULATION
What are physiological (innocent flow) murmurs?
A
4S’s –
- Soft blowing murmur
- Symptomless
- left Sternal edge
- Systolic murmur only
8
Q
FOETAL CIRCULATION
When are physiological (innocent flow) murmurs seen?
A
Common during febrile illness or anaemia as CO increases
9
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
10
Q
FOETAL CIRCULATION
When would you investigate an innocent murmur?
A
Louder than 2/6, diastolic,
louder on standing
11
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
12
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
13
Q
FOETAL CIRCULATION
What are the main cyanotic heart diseases?
A
4Ts –
- ToF
- TGA
- Tricuspid atresia
- Truncus arteriosus
(Complete AVSD too)
14
Q
FOETAL CIRCULATION
How can you determine if cyanosis is cardiac or respiratory?
A
- Hyperoxic test, better = respiratory, still cyanosed = cardiac
15
Q
FOETAL CIRCULATION
What is a complication of SLE?
A
Complete heart block
16
Q
ATRIAL SEPTAL DEFECT
What is atrial septal defect (ASD)?
A
- Hole in septum connecting atria as failure of septal tissue to form
17
Q
ATRIAL SEPTAL DEFECT
What is the pathophysiology?
A
L>R shunt as LAp>RAp so increased flow into R heart + lungs
- Trisomy 21, foetal
18
Q
ATRIAL SEPTAL DEFECT
What is it common in?
A
Trisomy 21,
foetal alcohol syndrome
19
Q
ATRIAL SEPTAL DEFECT
What are the 3 main types of ASD?
A
- Ostium primum (group with AVSD)
- Ostium secundum (80%)
- Partial AVSD
20
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
21
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)
22
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
23
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
24
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)
25
ATRIAL SEPTAL DEFECT
What is the management of ASD?
- Small + asymptomatic = watchful waiting
| - Large = transvenous catheter closure via femoral vein or open heart surgery
26
VSD
What is a ventricular septal defect (VSD)?
- 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
27
VSD
What are some conditions associated to VSD?
- Trisomy 13, 18 + 21
28
VSD
What is the clinical presentation of VSD?
- Small = ?asymptomatic
- Large = heart failure with dyspnoea, failure to thrive, recurrent infections
- Harsh pansystolic murmur
29
VSD
What are the features of the pansystolic murmur in VSD?
- Left lower sternal edge
- Loud murmur = smaller VSD (larger = quieter)
- May have systolic thrill on palpation
30
VSD
What are the investigations in VSD?
- 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
31
VSD
What are some complications of VSD?
- Increased risk of infective endocarditis > Abx prophylaxis during surgery
- AR, Eisenmenger's syndrome + right heart failure
32
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
33
HEART FAILURE
What are the causes of heart failure in neonates?
Obstructed or duct-dependent systemic circulation (HLHS, severe coarctation)
34
HEART FAILURE
What are the causes of heart failure in infants?
High pulmonary blood flow (VSD, AVSD, large PDA)
35
HEART FAILURE
What are the causes of heart failure in older children?
Eisenmenger's syndrome (RHF),
rheumatic disease,
cardiomyopathy
36
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
37
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)
38
HEART FAILURE
What is the management of heart failure?
- Furosemide (loop diuretic)
- Captopril (ACEi)
- Increased calories
39
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
40
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
41
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
42
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
43
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
44
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
45
PDA
What are some risk factors of PDA?
Prematurity is key + association with maternal rubella
46
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
47
PDA
What are the signs of PDA?
- Collapsing or bounding pulse as increased pulse pressure
- Continuous 'machinery' murmur heard loudest beneath the L clavicle
48
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)
49
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
50
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)
51
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
52
TOF
What is TOF associated with?
- Trisomy 21 + 22q deletions
53
TOF
What are some risk factors?
- Rubella,
- maternal age >40,
- alcohol in pregnancy,
- maternal DM
54
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
55
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
56
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
57
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
58
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
59
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
60
TGA
What is it associated with?
Duct dependent lesion, associated with PDA, ASD + VSD
61
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
62
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
63
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
64
TRICUSPID ATRESIA
What is tricuspid atresia?
- Only LV is effective, RV is small, non-functional + complete absence of tricuspid valve
65
TRICUSPID ATRESIA
How does tricuspid atresia present?
- 'Common mixing' of systemic + pulmonary venous return in LA = cyanosis + dyspnoea
66
TRICUSPID ATRESIA
How is it managed?
Shunt between subclavian + pulmonary artery with surgery later
67
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
68
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
69
COARCTATION OF AORTA
What is it associated with?
Turner's syndrome
70
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)
71
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)
72
COARCTATION OF AORTA
What is the management of coarctation of aorta?
- ABCDE if collapse
- Prostaglandin E1 infusion if critical
- Stent insertion or surgical repair
73
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
74
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
75
HYPOPLASTIC LEFT HEART
What is the management of HLHS?
- ABCDE
- Prostaglandin E1 infusion to prevent duct closure
- Surgical management
76
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
77
EBSTEIN'S ANOMALY
What is Ebstein's anomaly associated with?
- Wolff-Parkinson-White syndrome + lithium in pregnancy
78
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
79
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
80
EBSTEIN'S ANOMALY
What is the management of Ebstein's anomaly?
- Prophylactic Abx to prevent infective endocarditis
- Definitive Mx = surgical correction
81
AORTIC STENOSIS
What is aortic stenosis?
- Aortic valve leaflets partly fused together giving restrictive exit from LV
82
AORTIC STENOSIS
What is aortic stenosis associated with?
- Bicuspid aortic valve + William's syndrome (supravalvular)
- Also may be mitral stenosis + coarctation of aorta too
83
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
84
AORTIC STENOSIS
How does more severe aortic stenosis present?
- Severe = syncope, chest pain + dyspnoea on exertion
- Critical = severe heart failure + shock
85
AORTIC STENOSIS
What are the investigations for aortic stenosis?
- CXR (prominent LV),
- regular ECHO to assess need for interventions
86
AORTIC STENOSIS
What are some complications?
LV outflow tract obstruction,
heart failure
ventricular arrhythmias
87
AORTIC STENOSIS
What is the management of aortic stenosis?
- Balloon valvotomy/dilatation if symptomatic
- May need surgical aortic valve replacement
88
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
89
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
90
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
91
PULMONARY STENOSIS
What is the management of pulmonary stenosis?
- Most asymptomatic so wait until pressure gradient across pulmonary valve increases > trans-catheter balloon dilatation
92
INFECTIVE ENDOCARDITIS
Who are at risk of infective endocarditis?
- All children with CHD (except secundum ASD)
- Highest risk where turbulent blood flow = VSD, coarctation of aorta + PDA
93
INFECTIVE ENDOCARDITIS
What is the pathophysiology of infective endocarditis?
- Infection of heart valves, septal defects or endocardial lined structures consisting of vegetations which are masses of fibrin, platelets + infectious organisms
94
INFECTIVE ENDOCARDITIS
What are some risk factors for infective endocarditis?
- IVDU,
- prosthetics,
- structural heart defects
95
INFECTIVE ENDOCARDITIS
What is the most common causative pathogen for infective endocarditis?
Staph aureus (previous Strep Viridians)
96
INFECTIVE ENDOCARDITIS
What are the septic and systemic signs of infective endocarditis?
- Fever, malaise, night sweats, arthralgia/myalgia
- Systemic emboli from L sided vegetations may result in brain abscess + stroke
97
INFECTIVE ENDOCARDITIS
What are the clinical signs of infective endocarditis?
- Splinter haemorrhage under nail beds
- Retinal infarcts (Roth's spots)
- Tender nodules on fingers + toes (Osler's nodes)
- Painless erythematous palms + soles (Janeway lesions)
- Microscopic haematuria
98
INFECTIVE ENDOCARDITIS
What are the investigations for infective endocarditis?
- FBC (WCC raised), CRP/ESR raised
- Blood cultures before Abx started
- Echo to look for valve vegetations
99
INFECTIVE ENDOCARDITIS
What is the management?
High dose IV Abx (penicillin with aminoglycoside like vancomycin) for 6w
100
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
101
RHEUMATIC FEVER
How is rheumatic fever diagnosed?
Jones criteria –
- Evidence of recent strep infection plus 2 major or 1 major + 2 minor criteria
102
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
103
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
104
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
105
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
106
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)
107
DILATED CARDIOMYOPATHY
What is it?
Large, poorly contracting heart can be inherited or secondary to metabolic disease or direct viral infection of myocardium (myocarditis)
108
DILATED CARDIOMYOPATHY
When would you suspect dilated cardiomyopathy?
- Any child with enlarged heart + heart failure who was previously well
109
DILATED CARDIOMYOPATHY
What is the management of dilated cardiomyopathy?
- Dx by ECHO
- Sx treatment with diuretics, ACEi + carvedilol (beta blocker)
110
DILATED CARDIOMYOPATHY
What is the management of myocarditis?
Myocarditis usually improves spontaneously but some may need heart transplant
111
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
112
SUPRAVENTRICLAR TACHYCARDIA
How does a supraventricular tachycardia present?
- Sx of heart failure in young child,
palpitations,
dyspnoea
113
SUPRAVENTRICLAR TACHYCARDIA
What is the ECG like?
- Narrow complex tachycardia (250-300bpm)
- WPW = delta wave (slurred upstroke to QRS) with a short PR interval
114
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
