Cardiology and Haematology Flashcards
(158 cards)
1
Q
L-R shunts
A
Breathless
VSD
PDA
ASD
2
Q
R-L Shunts
A
Blue
Tetralogy of Fallot
Transposition of the great arteries
3
Q
Common mixing
A
Breathless and blue
Complete atrioventricular septal defect
4
Q
Outflow obstruction in well child
A
Pulm stenosis
Aortic stenosis
5
Q
Outflow obstruction in sick neonate
A
Coarctation of the aorta
6
Q
Ductus arteriosus
A
Ligamentum arteriosum
7
Q
Ductus venosus
A
Ligamentum venosum
8
Q
Foramen ovale
A
Fossa ovalis
9
Q
Umbilical arteries and abdo ligaments
A
Medial umbilical ligaments
Superior vesicular artery to bladder
10
Q
Umbilical vein
A
Ligamentum teres
11
Q
Rubella and CnHD
A
Peripheral pulmonary stenosis
PDA
12
Q
Warfarin
A
Pulmonary valve stenosis
PDA
13
Q
Foetal alcohol syndrome
A
ASD
VSD
Tetralogy of Fallot
14
Q
Down’s syndrome
A
AVSD
VSD
15
Q
Turner’s syndrome
A
Aortic valve stenosis
Aortic coarctation
16
Q
Characteristics innocent murmur
A
4S aSymptomatic pt Soft blowing murmur Systolic only left Sternal edge
17
Q
Still’s murmur
A
Early-mid systolic murmur
Best heard between left sternal edge and apex
Louder on lying down
18
Q
Venous hum
A
Continuous systolic and diastolic murmur
Best heard below clavicles
Abolished by compression of the jugular vein
Abolished by child lying down
19
Q
Neck bruits
A
Systolic murmur
Maximal above clavicle
Need to distinguish from mild aortic stenosis
20
Q
Symptoms HF
A
Breathlessness
Sweating
Poor feeding
Recurrent chest infections
21
Q
Signs HF
A
Poor wt gain/ faltering growth Tachypnoea Tachycardia Heart murmur, gallop rhythm Cardiomegaly Hepatomegaly Cool peripheries
22
Q
Causes HF Neonates
A
Hypoplastic LH syndrome
Critical aortic valve stenosis
Severe aortic coarctation
Interruption of the aortic arch
23
Q
Causes HF infants
A
VSD
AVSD
Large PDA
24
Q
Causes HF Older children/ adolescents
A
Eisenmenger syndrome
Rheumatic HD
Cardiomyopathy
25
Eisenmenger syndrome
Irreversibly raised pulmonary vascular resistance due to chronically increased pulmonary arterial pressure and flow
26
Peripheral cyanosis
Blue hands and feet
Child cold or unwell from any cause
Polycythaemia
27
Central cyanosis
Slate blue tongue
| ? CnHD
28
Ix for ?CnHD
CXR, ECG: rarely diagnostic but can help establish that abnormality is present, baseline for future changes
Echo and Doppler US: diagnostic
29
ASD Classification
Partial (primum) ASD: defect in anteroinferior aspect of septum, a form of AVSD
Secondum ASD: (80%) defect in the fossa ovalis
30
Sx ASD
Commonly ASYMPTOMATIC
Recurrent chest infections/ wheeze
Arrythmias (4th decade onwards)
31
Signs ASD
ESM (best heard at upper left sternal edge - increased flow across pulmonary valve)
Fixed split S2
Apical pansystolic murmur (primum only)
32
Ix ASD
CXR: cardiomegaly, enlarged pulmonary arteries, increased pulmonary vascular markings
ECG primum: superior QRS axis
ECG secundum: partial RBBB, R axis deviation
ECHO
33
Mx ASD
If ASD large enough to cause RV dilatation, it requires treatment
Secundum: catheterisation with insertion of occlusion device
Primum: surgical correction
Tx usually done age 3-5 (prevent RHF and arrhythmias later in life) ++++
34
VSD Classification
Small: smaller than aortic valve in diameter (=3mm)
35
Small VSD Sx and signs
Asymptomatic
Loud pansystolic murmur LLSE
Quiet pulmonary second sound
36
Ix Small VSDs
ECG + CXR: normal
| ECHO
37
Mx small VSDs
Lesions often close spontaneously (ass w disappearance of murmur, normal ECG + echo)
While VSD present, need good dental hygiene to prevent endocarditis
38
Sx large VSDs
HF (R+L) with breathlessness
Failure to thrive after 1 wk
Recurrent chest infections
39
Signs large VSDs
```
Active precordium
Tachypnoea, tachycardia, enlarged liver from HF
Soft pansystolic/ no murmur
Apical mid-diastolic murmur
Loud P2
```
40
ECG large VSD
Biventricular hypertrophy from age 2
| Upright T wave in V1
41
CXR Large VSD
Cardiomegaly
Enlarged pulmonary arteries
Pulmonary oedema
Increased pulmonary vascular markings
42
Mx Large VSD
Med: Diuretics +/- captopril (ACEi)
Additional calorie input
Surgical: surgery at 3-6 months to prevent HF and permanent lung damage
Usually intra-cardiac (while under cardiopulm bypass), can be transcatheter
Slowed growth should catch up after 1-2 years, excellent LT prognosis
43
Definition PDA
DA that has failed to close 1 month after EXPECTED DATE of delivery (flow across PDA is from aorta to pulmonary artery). In preterm infant PDA from CnHD but prematurity.
44
Sx PDA
Usually asymptomatic
| If duct v large --> pulmonary blood flow --> pulm HTN and HF
45
Signs PDA
Continuous murmur behind L clavicle
| Bounding pulse
46
Ix PDA
ECG + CXR: usually normal
If v large, as for VSD
Echo: distinguish btwn PDA and VSD
47
Mx PDA
Surgical closure recommended (abolish lifelong risk bac endocarditis and pulm vasc disease). Closure via coil/ occlusion device introduced via cardiac catheter at 1 yr
48
Cyanotic CnHD
```
5Ts
Truncus arteriosus
Transposition great arteries
Tricuspid atresia
Tetralogy of Fallot
Total anomalous pulm venous return
```
49
Hyperoxia (N2 washout) test
Determine presence HD in cyanosed infant
Put infant in 100% O2 for 10 mins
If R radial pAO2 still low ( dx Cyanotic CnHD
50
Mx Cyanotic CnHD
```
ABC
Prostagladin Infusion (PGE, 5ng/kg/min) to maintain PDA
```
51
Tetralogy of Fallot defects
(Most common cause cyanotic CnHD)
1) Large VSD
2) Overriding aorta
3) Sub pulm art stenosis
4) RV hypertrophy as a result
52
TOF Sx
Some dx antenatally
Some dx after identifying murmur in 1st 2 months (+/- cyanosis)
Classical triad: severe cyanosis, hypercyanotic spells, squatting on exercise
53
TOF Signs
Clubbing fingers/ toes
| Loud, harsh ESM at L sternal edge
54
TOF CXR
Small heart, 'boot-shaped' (RV hypertrophy)
Pulm artery 'bay' (concavity on L heart border
Reduced pulm vascular markings
55
Mx TOF
Initially med mx, definitive surgery 6 months (close VSD, relieve RV outflow tract obstruction)
V cyanosed neonates may require shunt
56
Transposition great arteries definition
Aorta -> RV, pulm artery -> LV
Deoxy blood returned to body, oxy blood returned to lungs
Often co-morbid abnormalities -> mixing, e.g. ASD, VSD, PDA
57
Sx transposition great arteries
Cyanosis (profound and life-threatening)
| Usually day 2 when duct closes)
58
Signs transposition great arteries
Cyanosis (always present)
Loud and single S2
Usually no murmur
59
Transposition great arteries Ix
```
CXR: Classically narrow upper mediastinum
'Egg on side' appearance cardiac shadow
Increased pulm vascular markings
ECG: normal
ECHO
```
60
Mx transposition great arteries
```
Sick cyanosed infant, key = improve mixing
Prostaglandin infusion (keep PDA open)
Balloon atrial septostomy (tears fossa ovalis to enable mixing)
Arterial switch surgery during neonatal period
```
61
Aortic Stenosis Comorbidities
Mitral valve stenosis
| Aortic coarctation
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Aortic stenosis symptoms
Most ASYMPTOMATIC
Severe: reduced exercise tolerance
Chest pain on exertion/ syncope
Critical: severe HF -> shock
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Aortic stenosis signs
```
Slow rising pulse
Carotid thrill
ESM
Delayed, soft S2
Apical ejection click
```
64
Aortic stenosis Ix
CXR: normal or
Prominent LV with post-stenotic dilatation of asc aorta
ECG: LV hypertrophy
65
Mx aortic stenosis
```
Reg clin + echo assessment (assess when to intervene)
Balloon valvotomy (children w sx on exercise, resting pressure gradient >64mmHg)
```
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Pulm stenosis sx
Most ASYMPTOMATIC
| Critical: present in 1st few days life w cyanosis
67
Pulm stenosis signs
ESM
Ejection click
RV heave
68
Pulm stenosis Ix
CXR: normal or post-stenotic dilatation of pulm artery
ECG: RV hypertrophy
69
Pulm stenosis mx
Pressure gradient >64mmHg -> intervene
| Transcatheter balloon dilatation
70
Adult-type aortic coarctation definition
Constricted aortic segment DISTAL to ductus arteriosus
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Infantile aortic coarctation definition
Constricted segment proximal to ductus arteriosus
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Adult-type aortic coarctation sx and signs
```
ASYMPTOMATIC
Systemic HTN in R arn
ESM at upper sternal edge
Collaterals heard w continuous murmur at back
Radiofemoral delay
```
73
Adult-type aortic coarctation Ix
CXR: 'Rib-notching'
'3 sign' visible notch in desc aorta
ECG: LV hypertrophy
74
Mx
Severe (as assessed by echo): stent via catheter
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Infantile-type coarctation of aorta clin features
```
Normal on 1st day life
Acute circulatory collapse at 2 days
Sick baby with severe HF
Absent femoral pulses
Severe metabolic acidosis
```
76
Infantile-type coarctation of aorta Ix
CXR: cardiomeg from HF and shock
ECG: normal
77
Mx Infantile-type coarctation of aorta
ABC
Prostaglandin ASAP
Surgical repair soon after dx
78
Rheumatic fever definition
Abnormal immune response to preceding infection with group A strep mainly in children aged 5-15
79
Rheumatic fever major manifestations
```
Pancarditis (endocarditis, myocarditis, pericarditis)
Polyarthritis
Syndenham chorea
Erythema marginatum
Subcut nodules
```
80
Rheumatic fever clin features
Pharyngeal infection -> latent interval 2-6 weeks -> polyarthritis, mild fever, malaise
81
Rheumatic fever minor manifestations
```
Fever
Polyarthralgia
Hx RF
Raised inflammatory markers
Prolonged PR interval on ECG
```
82
Jones criteria for the diagnosis of RF
2 major OR 1 major and 2 minor criteria and supportive evidence of preceding group A strep infection
(Markedly increased ASO titre/ other strep Abs/ group A strep on throat culture)
83
Chronic RF complications
Mitral stenosis
84
Mx acute Rheumatic fever
Bed-rest and NSAIDs
Steroids if fever and inflamm don't resolve quickly
Symptomatic HF -> diuretics and ACEis
Monthly injections BENZATHINE PENICILLIN for 18-21 yrs, possibly life to prevent recurrence
85
RFs infective endocarditis
```
Usually occurs w CnHD
- small VSDs
- PDAs
- mitral regurg
- aortic regurg
- severely restricted outflow tract obstruction
- aorto-pulmonary shunts
Prosthetic valves
Risk NOT increased in isolated secondum ASD
```
86
Microorganisms causing infective endocarditis
Commonest = viridans strep
87
Symptoms infective endocarditis
Recurrent fever, arthralgia, malaise
Splenomegaly
Uncommon signs: splinter haemorrhages, petechial haemorrhagic Janeway lesions, Osler nodes, retinal Roth's spots
88
Dx infective endocarditis
multiple blood cultures before giving abx
| echo can confirm but not exclude dx
89
Tx infective endocarditis
High dose penicillin + an aminoglycoside
| 6 wks high-dose therapy
90
Prevention infective endocarditis
Good dental hygiene - encourage in all pts w CnHD
Dental/ surgical interventions non-infected areas -> no abx
Procedure in actively infected area -> prophylactic abx
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Embryonic Hb production
4-8 weeks gestation
92
Foetal Hb structure
2 alpha chains 2 gamma chains
| Increased affinity for O2
93
Foetal Hb production
After 8 wks gestation
| Main hb during foetal life
94
Hb types at birth
HbF
HbA (adult Hb)
HbA2 (normal variant HbA, may be increased in beta-thalassaemia)
95
Hb types at 1 yr
V low percentage HbF
| Increased HbF sensitive indicator haemoglobinopathies
96
Hb values at birth
Term infants: 14-21.5 g/dl
| High as low O2 conc in foetus
97
Hb values 2 weeks
13.4-19.8 g/dl
98
Hb values 2 months
9.4-13 g/dl
99
Hb values 1yr
11.3- 14.1
100
Hb values 2-6yrs
11.5-13.5
101
Hb values 6-12yrs
11.5-15.5
102
Hb values males 12-18
13-16
103
Hb values females 12-18
12-16
104
Anaemia values (neonate/ 1-12 months/ 1-12yrs)
Neonate:
105
Commonest cause anaemia
Iron deficiency
106
Causes anaemia
Impaired red cell production
Increased red cell destruction (haemolysis)
Blood loss
107
Causes impaired red cell production
1) Red cell aplasia (no red cell production)
- parovirus infection
- Diamond-Blackfan anaemia
- Transient erythroblastopenia of childhood
2) Ineffective erythropoiesis (RC production at normal rate but differentiation/ survival of cells defective)
- iron deficiency
- folic acid deficiency
- chronic inflamm (e.g. JIA)
- chronic RF
108
Causes increased haemolysis
1) Red cell memb disorders
- Hereditary spherocytosis
2) Red cell enzyme disorders
- G6PD def
3) Haemoglobinopathies
- thalaessaemias
- sickle cell disease
4) Immune
- Haemolytic disease of the newborn
- Autoimmune haemolytic anaemia
109
Causes blood loss
1) Fetomaternal bleeding
2) Chronic GI blood loss
- Meckel diverticulum
3) Inherited bleeding disorders
- von Willebrand disease
110
Blood results ineffective erythropoiesis
Normal reticulocyte count
| Abnormal MCV
111
Causes iron deficiency
Inadequate intake (common)
Malabsorption
Blood loss
112
Elemental iron requirements
1mg/kg/day
113
Sources iron
Breast milk (low iron content but 50% absorbed)
Infant formula (supplemented with iron)
Cow's milk (higher content vs breast milk but only 10% absorbed)
Solids at weaning
114
Causes inadequate iron intake
Delay in introduction mixed feeding beyond 6 months
Diet with insufficient iron-rich foods
Iron intake much increased when taken w vitamin C
115
Clin features iron-def anaemia
Children asymptomatic until
116
Dx iron-def anaemia
Microcytic, hypochromic anaemia
| Low serum ferritin
117
Causes microcytic anaemia
Iron def
b-thalassaemia trait
a-thalassaemia trait
anaemia chronic disease
118
Complications iron-def anaemia
Detrimental to behavioural and intellectual function
119
Mx iron def anaemia
Dietary advice
Oral iron supplementation (e.g. sytron or niferex - don't stain teeth)
Continue until Hb normal, and then another 3 months to replenish iron stores
Blood transfusion NEVER needed for iron def
If fail to respond, ix for malabsorption/ chronic blood loss
120
Tx iron def with normal Hb
Dietary advice to increase oral iron
| Offer parents oral iron supplements
121
Blood values red cell aplasia
Low reticulocyte count despite low Hb
Normal bilirubin
Negative direct antiglobulin test (Coombs test)
Absent red cell precursors on bone marrow exam
122
Diamond-Blackfan anaemia
FHx 20%, 80% sporadic
Mutations in ribosomal protein
Most cases present 2-3 months
Some sx anaemia, some congen abnormalities, e.g. short stature, abnormal thumbs
Tx: oral steroids, monthly blood transfusions if unresponsive
123
Transient erythroblastopenia of childhood
Triggered viral infections
Same haem features as diamond-blackfan anaemia (DBA)
Unlike DBA, TEC always recovers, usually within several weeks
124
Commonest severe inherited coagulation disorders
Haemophilia A
Haemophilia B
Both X-linked recessive
125
Haemophilia A factor deficiency
Factor 8
126
Inheritance haemophilia A and B
XLR
| B: 2/3 FHx, 1/3 sporadic
127
Freq Haemophilia A and B
1/5,000 male births (A)
| 1/30,000 male births (B)
128
Definition haemophilia
Group of hereditary blood disorders that impair the body's ability to clot blood normally
129
Haemophilia B deficiency
Factor 9
130
Von Willebrand Disease deficiency
Absence of VWFactor -> rapid degradation of factor 8
131
Grading of haemophilias
Mild/ moderate/ severe depending on Factor 8 or 9 level
Mild: >5-40%
Moderate: 1-5%
Severe:
132
Sx mild haemophilia
Bleeding after surgery
133
Sx moderate haemophilia
Bleed after minor trauma (intracranial haemorrhage, bleeding post-circumcision, oozing from heel-rpick/ venepucture sites)
134
Sx severe haemophilia
Recurrent, spontaneous joint/ muscle bleeds
Can lead to crippling arthritis if not properly treated
Present towards end of 1st year
135
Age of onset of bleeding disorders
Neonate: 20% haemophilias present now
Toddlers: haemophilias may present when starting to walk
Adolescent: vWF disease may present with menorrhagia
136
Mucous membrane bleeding and skin haemorrhage
Platelet disorders/ vWF disease
137
Bleeding into muscles/ joints
Haemophilias
138
Scarring and delayed haemorrhage
Disorders of connective tissue, e.g. Marfan syndrome, osteogenesis imperfecta, factor 8 deficiency
139
PT/ APTT/ Factor8:c Haemophilia A
PT: Normal
APTT: Increased (++)
Factor 8:c : Reduced (--)
140
PT/ APTT/ Factor8:c vWF disease
PT: Normal
APTT: Increased/ normal
Factor 8:c : Reduced or normal
141
Mx haemophilia
A: recombinant factor 8 concentrate
B: recombinant factor 9 concentrate
Both via IV infusion whenever there is any bleeding
Minor bleed/ simple joint bleed: raise circulating level to 30% normal
Home tx encouraged to avoid delay in tx
Major surgery/ life-threatening bleed: raise to 100%, maintain at 30-50% for up to 2 weeks to prevent 2y haemorrhage
Desmopressin can be given for mild haemophilia A to prevent use blood products.
AVOID IM injections, aspirin and NSAIDs
142
Complications of tx of haemophilia
Inhibitors (antibodies to factor 8/9): 5-20%, reduce or completely inhibit effect of tx
Transfusion-transmitted infections (e.g, HIV, Hep A/B/C)
Periph veins may be difficult to cannulate, central lines may become infected/ thrombosed
143
Prophylactic tx haemophilia
All children with severe haemophilia A/B to prevent severe joint damage. Begins age 2-3, given 2-3x week
144
Functions vWF
1) Facilitates platelet adhesion to damaged endothelium
2) Acts as carrier protein for factor 8:c, protecting it from inactivation and clearance
vWF disease: defective platelet plug formation, factor 8:c deficiency
145
Inheritance and subtypes vWF
Usually AD
But many mutations in gene/ types vWD
Commonest subtype = type 1, (60%), fairly mild, often not dx until puberty/ adulthood
146
Clinical features vWD
Bruising
Excessive, prolonged bleeding after surgery
Mucosal bleeding e.g. epistaxis, menorrhagia
Spont soft tissue bleeding, e.g. haematomas, haemarthroses uncommon
147
Mx vWD
Type 1: DDVAP (Desmopressin), increases both F8 and vWF secretion into plasma
DDVAP: use with caution in
148
Vitamin K deficiency symptoms
Increased risk of bleeding
| Prolonged PT
149
Causes vitamin K def
1) Inadequate intake
- Neonates
- LT chronic illness w inadequate intake
2) Malabsorption
- Coeliac disease
- Cystic fibrosis
- Obstructive jaundice
3) Vitamin K antagonists
- Warfarin
150
Thrombocytopaenia definition
Platelet count
151
Sx thrombocytopaenia
Bruising
Petechiae
Purpura
Mucosal bleeding (epistaxis, gums bleed when brushing teeth)
Less common: severe GI bleeding, intracranial haemorrhage, haematuria
152
Immune thrombocytopaenia
Commonest cause thrombocytopaenia of childhood
Destruction circulating platelets by anti-platelet IgG antibodies
Often after viral illness
153
Immune thrombocytopaenia mx
Most children treated at home
Usually remits spont in 6-8 weeks
If severe, tx options: oral prednisolone, IV anti-D, IVIg
154
DIC definition
Coagulation pathway activation leading to diffuse fibrin deposition in the microvasculature and consumption of coagulation factors and platelets
155
Causes DIC
Severe sepsis
Shock due to circulatory collapse
- meningococcal septicaemia
- extensive tissue damage from trauma/ burns
156
Clin features DIC
Bruising
Purpura
Haemorrhage
157
Dx DIC
```
No single diagnostic test
Thrombocytopaenia
Prolonged PT
Prolonged APTT
Low fibrinogen
Raised fibrinogen degradation products, D-dimers
```
158
Mx DIC
Tx underlying cause
| Supportive care: fresh frozen plasma, cryoprecipitate, platelets
