Cardiology Flashcards
1
Q
What is the first thing to change from neonatal to foetal circulation?
A
PFO closure- within minutes
2
Q
What are the 5 changes seen in transition from neonatal to foetal circulation
A
- Umbilical vein closure
- PFO closure
- Ductus venosus closure (in liver)
- DA closure
- Umbilical artery closure
3
Q
What is the valve from RA to RV?
A
Tricuspid
4
Q
What is the valve from RV to pulmonay artery?
A
pulmonary
5
Q
What is the valve from LA to LV?
A
mitral
6
Q
What is the valve from LV to aorta?
A
Aortic valuve
7
Q
What valves are responsible for S1?
A
Mitral and tricuspid- shut at the end of diastole
8
Q
What valves are responsible for S2?
A
Pulmonary and aortic closure at end of systolic
9
Q
What causes FIXED wide splitting of S2?
A
ASD. Think: continuous flow back from LV to RV during diastole meaning there is increased PULMONARY volume.
Also pulmonary stenosis, Ebstein anomaly, total anomalous pulmonary venous return, and right bundle branch block.
10
Q
What causes narrow splitting of S2?
A
pulmonary hypertension.
Note splitting increasing during inspiration (think filling) and reduces during expiration.
11
Q
What causes a pansystolic murmur?
A
VSD, mitral regurg and tricuspid regurg
12
Q
What causes an ejection ssytolic murmur?
A
aortic stenosis, pulmonary stenosis
13
Q
What are the causes of a diastolic murmur?
A
Aortic regurg, pulmonary regurg, mitral stenosis, tricuspid stenosis
14
Q
What causes a continous murmur?
A
BT shunt, PDA
15
Q
Which conditions require endocarditis prophylaxis?
A
Prosthetic heart valve, prev endocarditis, rheumatic heart disease, unrepaired CYANOTIC congenital heart disease and surgical fixation of congenital heart disease within 6 months
16
Q
What is the incidence of congenital heart disease in the normal population? What does it increase to if there is a previous child with CHD or parent is affected?
A
0.8% in the ppn
Increase to 2-4% if sibling has or parent has
17
Q
What is the score used for hypermobility in the context of Marfans?
A
Beighton score.
Anything >4 = hypermobile
18
Q
What layer of the mesoderm forms the heart? And when does the cardiogenic field start developing?
A
Splanchnic mesoderm
Cardiogenic field starts in week 3
19
Q
What cells contribute to cushion formation in the heart
A
Neural crest cells from the hindbrain
20
Q
Direction of blood flow in PFO?
A
R to left.
So if right atrial pressures remain high vs lsft, PFO wont close
21
Q
What medications can keep the ductus arteriosus open
A
Prostaglandin.
Prostaglandin for PATENT ductus arteriosus
22
Q
Why is splitting normal during inspiration
A
During inspiration, lots of blood being pushed to RV and hence pulmonary artery= longer time to close pulmonary valve. Physiological splitting S2
23
Q
Generally, what causes S3
A
Fluid overload
24
Q
Generally, what causes S4
A
Increased pressure
25
Which valves have 3 leaflet
mitral and tricuspid
26
What percentage of CHD are due to genetic causes
20%
27
What is the most common congenital heart lesion
VSD 32%
28
What % of kids with Trisomy 13 (patau) have CHD and what are the common lesions
80%
VSD
PDA
dextrocardia
29
What % of kids with Trisomy 18 have CHD and what are they?
90%
VSD
PDA
PS
30
What percentage of kids with Downs have CHD? What are the common defects
40-50%
VSD/AVSD
Endocardial cusion defects
31
What % of Turner's syndrome have CHD and what is the common finding
35%
MOST HAVE BICUSPID AORTIC VALVE
- some have coarctation of aorta
32
What proportion of DiGeorge have CHD and what is the most common defect
40%
Conotruncal ie truncus arteriosus
33
What % of patients with Crit du Chat have heart problems? What are they?
This syndrome has cat like cry, low BW, craniofacial dysmorphia. 25% have CHD
anything from VSD, ASD, TOF..
34
What % of patients with Williams syndrome have CHD? Most common lesion?
This is the cocktail personality.
66% have supravalvular aortic stenosis.
Think, very bubbly-, above the rest- supravalvular aortic stenosis
35
What is the CHD in Alagile and what percentage of kids have it
Peripheral pulmonary artery stenosis, 85%
36
What syndrome has mitten hands and CHD
Carpenter syndrome
Think- cant work as a carpenter cos have mitten hands and a bad heart
37
What % of kids with Carpenter syndrome have CHD
50%
1 in 2 carpenters have bad hearts
38
What proportion of cornealia de lange have CHD
30%
39
CHD in Cornelia de Lange
VSD
40
What is the CHD lesion in EHlers Danlos
MV prolapse
Aortic root dilatation
41
CHD with Pompe's disease? (GLYCOGEN storage disorder)
Hypertrophic cardiomyopathy
42
What syndrome has cardiac defect and absent thumb/radius
Holt-Oram
43
Which syndrome has pulmonary stenosis, deafness, abnormal genetalia
LEOPARD syndrome
Leopards have weird genitals, can't hear (they move so quietly) and have pulmonary stenosis
44
What is the CHD in Noonans
Pulmonary Stenosis - in 40% if patkients.
45
Heart defect in Smith-Lemli-Opitz
VSD/PDA
SMITH is a man who loves his cholesterol. 7-dehydrocholesterol (7-DHC) reductase issue
46
Congenital heart defect in Marfans
Aortic root dilatation
47
Name the 5 CYANOTIC congenital heart lesions
Use your palm
1. Truncus Arteriosus
2. Transporition of great arteries
3. Tricuspid atresia (3 fingers)
4. Tetralogy of fallot
5. TAPVR
Also hypoplastic left heart
48
Where does the murmur for TOF come from
Pulmonary stenosis.
Large VSD so no VSD murmur.
49
Name the 4 acyanotic heart lesions
VSD, ASD, PDA, Coarctation
50
What is Eisemenger syndrome
Prev L to R shunt
Increasing flow through pulmonary artery
Change in structure/dysfn= turbulent flow and vascular remodelling
Increased pulmonary pressures
Inverted R to L shunt
Treat with prostaglandin to reduce pressure.
51
What is the most common ASD
Ostium Secundum defect
52
What is heard on auscultation for ASD
Widely split second heart sound and can have ejection systolic murmur from increased flow into PA.
Due to prolonged RV emptying
53
ECG changes associated with ASD
RBBB.
54
What syndrome has AVSD
Downs syndrome
55
What is the name of the defect in AVSD
Ostium primum
(secondum most common in jsut normal ASD)
56
Murmur with VSD
Loud, pansystolic murmur.
57
Complication associated with VSD
Aortic valve regurg
due to blood rushing back from LV to RV, valve pulled down and so more floppy
58
At what age do most small VSDs close
Age 4
59
What is the basic pathophys for TAPVR
Essentially problem in pulmonary venous drainage so NO return to LA. PV goes to SVC or IVC and systemic flow from open VSD/ASD.
60
CXR sign for TAPVR
Snowman sign- for RA, RV and PA enlargement
61
Where does the fluid for a chylothorax come from
Chyle from thoracic duct/lymph into the thoracic system
62
Apart from cardiac surgery, what other things are associated with chylothorax
H type TOF
Chest injury
Lymphoma
Lymphangectasia
63
What is the diet for chylothorax
Medium chain trigluceride diet.
High protein, low fat.
64
What is the indication for surgery for a chylothorax (when chest drain already in)
If output >50ml/kg/day
65
Complications from Chylothorax (2 main)
Malnutrition
Agammaglobunaemia and abnormal cell mediated response. T cell more affected.
66
Possible murmurs with ASD? Think of the flow
1. Increased flow LA--> RA= diastolic murmur LLSE
2. Increased RV volume so increased flow into the pulmonary artery so ejection systolic CLICK + systolic murmur
67
ECG for ASD?
RBBB
68
Murmurs in AVSD- think flow
Increased LA--> RA: Diastolic murmur
Increased RA volume : Ejection systolic murmur (into pulm artery)
Increased LV -> RV flow = pansystolic murmur
69
ECG in AVSD- think size of ventricules
Severe LAD due to biventricular hypertrophy
70
Most common VSD
Membranous
25-30% of CHD
71
Murmur with VSD
Harsh, pansystolic murmur LLSE. Often heart with a SMALL VSD
72
Symptoms/presentation of large VSD
Usually not cyanotic but
FTT
Poor feeding
Dyspnoea
recurrent chest infections
Tachycardia
Tachypnoeea
73
As pulmonary pressures increase, what happens to murmur and heart sound in VSD
Think: low pulm pressure= reduced pressure in RV so increased backwards flow.
Increasing pressure = reduced flow so murmur more quiet but still loud S2
74
Complication of VSD
Aortic regurg and increased pulmonary pressures
75
What % of VSDs close spontaneously and when
80% of muscular VSDs, within 4 years
76
Do prostaglandins work for TAPVR?
No.
Because issue with no return to LA and hence LV!
77
What is the one main triggering reason for Eisenmenger
Pulmonary HTN
78
How does large PDA lead to Eisengmenger
++ flow from aorta to pulmonary artery = increased flow to lungs.
Eventually this causing pulmonary HTN = now R to left shunting = deoxygenated blood to the pierpheries
79
Murmur associated with PDA
Machinery continous murmur
80
Treatment for PDA
Paracetamol/NSAID/Indomethacin
81
What is the location for 98% of coarctation of aorta?
Origin of ductus arteriosus
82
Syndrome commonly associated with coarctation of aorta
Turners.
Also remember bicuspid aortic value
Also PHACES
83
Heart changes as a result of a coarct aorta
LV hypertrophy
84
Examination findings for coarct aorta
Systemic HTN
BP changes UL vs LL
No murmur
lower O2 sats in LL
85
When is mild vs severe coarct generally detected
Mild usually later in childhood with leg weakness or incidental HTN
86
Treatment for coarct in neonate
Prostaglandin E1 to keep DA open while awaiting surgery.
87
What is a common post-op complication of aortic stenosis
Postoperative mesenteric arteritis
Rebound HTN
THink increased blood flow to these organs again= readjustment
88
What are the 4 features to a tetralogy of fallot
1. RVOT (pulmonary stenosis)
2. VSD
3. RV hypertrophy
4. Overriding aorta
89
What is the name of the lesion of aorta overides VSD by 50%?
double outlet right ventricle
90
Why does tetralogy of fallot cause cyanosis
Essentially:
RVOT causes increased pressure in RV so blood blows (deoxygenated) across the VSD into the LV.
Overiding aorta also means that the aorta is JUST under the VSD so blood from RV essentially flows directly into the aorta.
91
When do Tetralogy of fallot patients start to exhibit signs of cyanosis and why
In first few months of life due to increasing RV hypertrophy
92
Pathophys of tet spells
Drop in systemic pressures (ie waking) OR increased pulmonary pressures
Crying/upon awakening = drop in systemic BP --> increased deoxy blood in circulation --> drop PaO2--> increased oxygen demand so increased systemic blood flow (of deoxygenated blood) --> cyanosis --> increased resp drive and getting wrose.
93
Treatment of tet spells
Keep baby calm
Knee to chest: this kinks femoral artery and increasing systemic pressure.so increased LV pressure which means blood will go THROUGH pulmonary circulation to come to aorta rather than through VSD = improved oxygenation.
94
Why does murmur reduce during tet spells
All the flow is via VSD which is large.
Remember the TOF murmur is due to RVOT but in tet spells with drop in systemic BP/high pulmonary pressures, all blood going through VSD into aorta
95
Most common syndrome associated with duodenal atresia
Down's syndrome.
D-duodenal, D - downs
96
CXR for TOF
Boot shaped from RV enlargement
97
ECG for TOF
RAD, tall peaked p waves
98
Long-term complications to tetralogy of fallot if left unrepaired
Cerebral thromboses (from polycythaemia and dehydration), brain abscess, endocarditis
99
What syndrome is associated with a TOF and pulmonary atresia
DiGeorge
100
Inheritance of CHD? General and if sibling affected
The incidence of congenital heart disease in the normal population is approximately 0.8%, and this incidence increases to 1–4% for a second pregnancy after the birth of a child with congenital heart disease or if a parent is affected.
101
What is doublet outlet RV?
Both main vessels priginating from RV with a VSD (flow from LV to RV)
102
What is Ebstein's anomaly associated with
Maternal lithium use in pregnancy. These kids also get WPW
103
Main pathology in Ebsteins
Atrialisation of tricuspid valve- WEAK tricuspid valve and RA
104
CXR for Ebsteins
wall-wall heart- RVH and LVH
105
What % of d-TGA patients have VSD
50%
106
What are risk factors for development of TGA?
Maternal diabetes and being male.
107
If there are other cardiac defects ie pulmonary stenosis or R sided aortic arch, what is a syndrome associated with TGA?
DiGeorge
108
Pathophys for TGA
abnormal arteries. So pulmonary artery from left ventricle and aorta from right ventricle. Issue is the rest remains the same so essentially two SEPARATE circuits which aren't compatible with life without PDA or VSD etc.
109
Management of TGA?
Prostaglandin to keep duct open
Can do atrial septostomy to keep foremen ovale open.
110
What is the procedure called for permanent repair of TGA
Arterial switch- done at 2 weeks of age
95% success rate, complications only 2-5%
111
Complications post TGA repair
RBBB, arrythmias, heart failure (in adulthood)
112
Main pathology in hypoplastic left heart?
NO LV. and as a result, hypoplastic aortic arch
113
in Hypoplastic LH, what are the other heart lesions required to keep the heart working?
ASD and PDA
114
What are the three procedures for Hypoplastic left heart, the order of operation and age when they are done?
Nor wood at one week
Gleen at 4-6 months
Fontan at 4-5 yo
End result is a two chamber heart
115
What is the main pathology in truncus arteriosus
COMBINED aorta and pulmonary artery connected to a VSD. Occurs due a failed truncus septum formation.
4 types.
116
What is specific about heart sounds in truncus arteriosus?
SINGLE loud S2
117
Which congenital heart lesions will be present day 1 of life?
(Think of the ones that dont need a PDA)
Critical aortic stenosis
Tricuspid or pulmonary atresia
Hypoplastic left heart
118
Which CHD present in days? (think of all the ones that need PDA)
TGA
TAPVR
Truncus arteriosus
PDA
119
TGA XR?
Egg on a string. Large ventricles trying to make everything work
120
2 viruses associated with foetal endocardial fibroelastosis
Mumps and cocksackie
Essentially viral myocarditis leading to endocardial thickening.
COCKsackie - CARDIAL fibroelastosis
121
One maternal cause of foetal HYPERTROPHIC cardiomyopathy?
Diabetes
122
What is the most common cause of death in an atheletic adolescent?
HYPERTROPHIC CARDIOMYOPATHY
Think atheletic, lots of muscles
123
Syndromic causes of HYPERTROPHIC CDM
Noonan syndrome, LEOPARD syndrome, Danon syndrome, Fabry disease, Wolff-Parkinson-White syndrome, Friedreich ataxia, MERRF, MELAS
Pompe's as well. All the storage disorders.
124
ECG for hypertrophic cardiomyopathy
left ventricular enlargement
Some ST and T wave anomalies
125
What is the most common form of cardiomyopathy
Dilated CDM
126
What is the most common cause of dilated CDM
50% genetic
Usually muscular dystrophies, mitochondrial or fatty acid oxidation defects.
127
Medications leading to dilated CDM
DOXORUBICIN
128
Genetic syndromes associated with restrictive CDM
Gaucher disease, hemochromatosis, Fabry disease, familial amyloidosis; mucopolysaccharidoses, Noonan syndrome
129
Pathophys of restrictive CDM
normal ventricle size, muscle size and contraction but POOR contraction
130
How to differentiate between myocarditis and DCM with onset of new LV dysfn
Myocarditis= short onset, raised trop/CRP, normal heart size, lymphocytes on biopsy
DCM= long onset, normal inflamma markers, thin LV, no lymphocytes but scarring ntoed
131
What criteria are used for diagnosis of ARF
Jones criteria
Need 2 major and 1 minor OR
1 minor and evidence of prev GAS
132
Major manifestations in ARF criteria?
J: Joint (arthritis)
O: Think heart, pancarditis
N: nodules, subcutaneous
E: erythema marginatum
S: sydneham chorea
133
Minor crtieria for ARF
FRAPP
Fever
Raised ESRCRP
Arthalgia
Prev RF
Prolonged PR interval.
NOte this is diff for low and high risk ppn
134
First and second most common presenting major criteria for rheumatic fever
Arthritis
Pancarditis
135
Classic examination findings for sydenham chorea
milkmaid grip on finger squeeze, pronation when arms extended, tongue darting when protruded
136
Treatment for acute ARF
10 days Pen V for acute infection.
Monthly Bezathine Penicillin until 21 yo or for 10 years whichever is longer
137
2 of the most common valves involved in RHD
mitral and aortic
138
2 most common causes of infective endocarditis in paeds
Strep viridans and staph aureus
139
signs of endocarditis (peripheral)
Embolic phenomenoa
Roth spots, petechaie, splinter nail bed, haemorrhages, osler's nodes, janeway lesions
140
Prevention of endocarditis
Prophylactic antibiotics for:
1. RHD
2. prosthetic cardiac valves
3. Unrepaired CHD
Cephalexin prior to dental procedures
141
Most common virus assocaited with myocarditis
coxsackie
142
Which 2 medications can you not give in SVT in a patient with WPW
Verapimil and Digoxin
143
Treatment for atrial flutter in neonates
cardioversion
144
Long term therapy for atrial flutter in neonates
Digoxin, propranolol, or sotalol for 6-12 mo
145
Which drug binds to and inhibits the sodium/potassium-ATPase (sodium pump) within the plasma membrane of cardiac myocytes.
Digoxin
146
What are some antiarrythmics used to manage cardiac arrythmia- just know these vaguely
class I agents such as procainamide or propafenone or class III agents such as amiodarone and sotalol
147
In the first week of infancy, are T waves upright or inverted?
UPRIGHT in precordial leads
148
When are T waves inverted in paeds
Can be until age 8
149
5 causes of peaked T waves
Hyperkalemia
Hypercalcaemia
RIGHT ventricular hypertrophy
volume overload
benign early repolarisation
150
5 causes of flat T waves
Hypokalemia
Hypocalcaemia
Newborn
hypothyroidism
Digoxin
Peri/myocarditis
151
Hypokalmia ECG changes
Flat T waves
U wave
ST depression
Atrial and ventricular ectopics
VF and VT
152
Hyperkalemia ECG changes
Peaked T waves
Wide QRS
Increased PR interval
(think of Jen's dance)
153
Hypocalemia ECG changes
Flat or absent T waves
U waves
Prolonged ST and QT
154
ECG changes with hypercalcemia
Tall T waves
Reduced QT
Prolonged and depressed ST
Arrythmia
155
AVRT or AVNRT- which is more common in neonates vs childhood
AVRT in neonates
AVNRT in childhood
156
Classic features for WPW on ECG
Short PR
Delta wave
157
What is WPW associated with
Ebstein's anomaly
(which is also associated with lithium use in pregnancy)
158
Dose for synchronoous cardioversion in kids
0.5-2J/kg
159
Which tachycardia ia cardioversion ineffective for
junctional ectopic tachycardia
160
Treatment for SVT - first, second line
First line: Valsava
If stable-> IV adenosine
If unstable, DC cardioversion
161
Jervell-Lange-Nielsen syndrome
What is this syndrome associated with?
Cogenital hearing loss and prolonged QT
Jervell is a QT said Lange, but Jervell couldnt hear as he is deaf.
162
Clinical presentation of long QT syndrome
The clinical manifestation of LQTS in children is most often a syncopal episode brought on by exercise, fright, or a sudden startle; some events occur during sleep (LQT3).
163
Treatment for long QT syndrome
Propranolol and nadolol may be more effective than atenolol and metoprolol.
Can require pacemaker
164
Which antipsychotics cause prolonged QT
haloperidol, risperidone, chlorpromazine
165
Inheritance for Brugada
Autosomal dominant
166
Defect in Brugada
Cardiac sodium channel genes
'Think Brugada doesnt like salt'
167
Describe first degree HB
Fixed PR prolongation al all atrial impulses--> ventricular beat
168
Describe second degree heart block- Mobitz 1
Wenkebach
Progressively longer PR interval and then one missed ventricular contraction (dropped beat)
169
Mobitz II- describe
PR prolongation but no progression. Some atrial impulses wont have a ventricular impulse.
170
3rd degree HB? Describe
complete dissociation
171
Prevention of CHB in mums with lupus?
Commencement of hydroxychloroquiine at 10 weeks
172
What is Holt -Oram syndrome
AD
Hypoplastic thumbs and radii, first degree HB
'Holtaram has no thumbs and heart block'
173
Cardiac problem in PHACES?
abberant subclavian origin and coarctation
174
Stelleate iris, long philtrum, prominent lips, supravavular aortic stenosis
Williams
175
Category of heart defects in CHARGE
conotruncal
176
Gene and chromosome for Charge
think 6,7,8
6 letters in charge
CHD 7 gene
Chromosome 8
177
Common cardiac lesion in FASD
ASD
A alco , A ASD
178
4 medications that can cause tetralogy of fallot
Na valproate
Warfarin
Thalodomide
Oestrogen/progesterone
179
What is the natural pacemaker of the heart?
SA node
180
What is the functional unit of a myocyte?
Sarcomere
181
How is contraction mediated in the heart
Calcium mediated.
181
How is contraction mediated in the heart
Calcium mediated.
182
What are the 3 long QT syndromes and a feature of each? Include which one has what ion/channel involved.
1. KCNQ1 – Hannah is bad at swimming, SLOWK (M>F)
2. KCNH2- 2 startled. FAST potassium channel. (F>M)
3. SCN5A- Sodium channel and sleep in special care nursery. (causes sleep death)
