Congenital Heart disease Flashcards
(132 cards)
1
Q
Why does a baby have shunts?
A
Blood doesnt pass through the pulmonary circulation as lungs not functional with gas exchange in mother
2
Q
What are the three foetal shunts?
A
Ductus venosus
Foramen ovale
Ductus arteriosis
3
Q
Where is the ductus venosus?
A
Umbilical vein to IVC
4
Q
What does ductus venosus allow?
A
Blood to bypass RV and pulmonary circulation
5
Q
Where is the foramen ovale? What does it allow to bypass?
A
RA and LA - allows bypass RV and pulmonary circulation
6
Q
Where is the ductus arteriosis and what does it allow to bypass?
A
Pulmonary artery with aorta, bypasses pulmonary circulation
7
Q
How does the foramen ovale ckose after birth?
A
First breath expands alveoli decreasing pulmonary vascular resistance -> decreased pressure in RA, which means LA has higher pressure, squashing atrial septum and closes foramen ovale
8
Q
What does the foramen ovale become a few weeks after birth>
A
Foramen ovalis - weeks (when structurally shut)
9
Q
What keeps the ductus arteriosus open?
A
Prostaglandins
10
Q
What causes the ductus arteriosis to close?
A
Increased blood oxygenation -> drop in circulating prostaglandins, causing closure of ductus arteriosus
11
Q
What does the ductus arteriosus become?
A
Ligamentum arteriosum
12
Q
Why does the ductus venossu shut immediately after birth?
A
Umbilical cord clamped, no flow in umbilical veins
Structura;y closese in dyas -> ligamentum venosum
13
Q
What to remember about presentation of PDA?
A
Pink and stable - breathless
14
Q
When does the ductus arteriosus stop functioning vs structurally close?
A
Stop functioning 2-3 days after birth
Completely closes 2-3 weeks
15
Q
What causes PDA?
A
Unclear
Prematurity high risk
Maternal infections? eg rubella
16
Q
When is PDA asymptomatic?
A
Small - no functional problems, spontaneous closure
17
Q
When do adults present with heart failure after PDA?
A
Undiagnosed PDA because asymptomatic
18
Q
What direction does the blood flow through the PDA?
A
Left to right shunt
Aorta to pulmonary vessels
19
Q
How does PDA lead to HF?
A
Increased pressure in pulmonary vessels as blood continues passing from the aorta into them
Pulmonary hypertensin
Rs heart strain
-> RV hypertrophy
Increased afterload -> LV hypertrophy
20
Q
Symptoms of PDA and ASD in childhood
A
SOB
difficulty feed
Poor weight gain
LRTIs
21
Q
Murmur heard with significatn PDAs
A
Continuous cresceno-decrescendo machinery murmur (may continue with second heart sound)
22
Q
How to diagnose PDA?
A
ECHO cardiogram
Size and charcteristics of shunt
23
Q
How long are patients with PDA monitored for?
A
Until 1 year of age
24
Q
When is surgery considered for PDA and why?
A
sYMPTOMATIC
Evicdence of HF as direct consequence of PDA
After one year as then highly unlikely to spontaneously close
25
What is an atrial septal defect?
Hole in septum between artria - mizing of blood between them
26
Where do defects a=occur in development to ause ASD?
Problems with septum primum and septum secundum or with them fusing together or with endocardial cushion
27
What hole should occur in the septum secundum?
Foramen ovale
28
Which direction does blood move in ASD?
L to R - increased pressure in L pushes
29
How cna RH failure be caused by ASD?
Increased flow to R -> overload and heart strain
-> pulm hypertension and RH failure
30
What can pulmonary hypertension lead to long term?
Eisenmonger syndrome
31
What is eisenmonger syndrome?
Pulmonary pressure > systemic
Shunt reverses - R to L across ASD
Blood bypasses lungs
Patient becomes cyantoics
32
What could you suspect in a patient with long term ASD where they become cyanotic?
Eisenmonger syndrome - reversla in direction of ASD shunt
33
Most to least common ASDs
Ostium secondum (primum secundum fails to close)
Patent foramen ovale
Ostium primum -> Atrioventricular septal defect
34
Complications from ASD
Stroke in ctonext of VTE
Atrial fibrillation or flutter
Pulm hypertension and RSH failure
Eisenmonger syndrome
35
What is a DVT likely to cause in a patient with ASD?
Stroke
36
Why do patients with ASD have a stroke from a VTE rather than a PE?
Embolus from DVT -> RA -> LA across ASD
Clot -> LV -> aorta -> brain -> stroke
37
What is heard with an ASD?
Mid-systolic, crescendo-decrescendo murmur loudest at L sternal border with fized slit second heart sound
38
What is a fixed split S2?
Split does not change with inspriation or expiration (normally get normal split on inspiration)
39
Why is their a fixed split heard in ASD?
RV has more blood to empty due to shunt before pulmonary valve can close
40
How can ASDs present in al=dulthood?
Dyspnoea
Heart failure
Stroke
41
How can PDAs be treated surgically
Transcatheter
Surgical valve closure
42
How can ASDs be surgically fixed
Transvenous catheter closure via femoral vein
Open heart surgery
43
Management for ASD options
Watch and wait - small and asymtpomatic
Anticoagulatns - reduce risk clots in adults
Surgery
44
What colour do patients present with eisenmenger syndrome?
Blue, unstable
45
What underlying lesions can cause eisenmenger syndrome?
ASD
Ventricular septal defect
PDA
46
How long does eisenmenger syndrome take to develop large vs small shunts?
Large - 1-2 years
Small - Adulthood
47
When monitor for eisenmenger syndrome in pregnant women?
Develops more quickly in pregancy
history of hole in heart
ECHO
Close cardiologist monitoring
48
Main causes of death with eisenmengers
HF
Infection
VTE
haemorrhage
49
Main causes of death with eisenmengers
HF
Infection
VTE
haemorrhage
50
Mortality in eisenmengers
20 year reduced life expectancy
50% mortality in pregnancy
51
What shunts cause deoxygenation vs not
L to R shunt does NOT cause cyanosis 0 blood stull travels to lungs and is oxygenated
R to L shunt - blood is removed from pulmonary circultation, not enough gets oxygenated to provide for body -> cyanosis
52
Mechanism allowing chagning direction of shunt in eisenmengers?
Extra blood into R system causing pulmonary HPTN -> eventually succeeds systemic pressure, changes blood flow R to L
Allows deoxygenated blood back into the body circulation.
53
What does chronically loe oxygen saturations lead to?
Bone marrow responds by producing more RBCs and haemoglogbin to increase oxygen carrying capacity of blood
Leads to polycythaemia
54
What is polycythameia?
High conc of heamoglobin in the blood
55
What complexion does polycythameia give patients?
Plethoric (red)
56
What does polycythaemia increase the risk of?
VTE
57
What examination findings are linked to pulmonary hypertension?
RV heave
loud S2
Raised JVP
Peripheral oedema
58
What examination findings are linked to underlying atrial septal defect?
Mid systolic
crescendo-decrescendo
@ upper L sternal border
59
What examination findings are linked to underlying ventruclar septal defect?
Pan systolic murmur loudest @ L lower sternal border
60
Examination findings linked to R to L shunt and chronic hypoxia?
Cyanosis
Clubbing
Dysonoea
Plethoric complexion
61
Management of eisenmengers
Prevent development of increased pulmonary pressure and therefore swithcing of shunt direction
Cannot reverse once blood has changed direction at a certain pulmonary pressure
Definitive treatment - heart-lung transplant
62
Why don't always offer heart lung transplant for eisenmengers
High mortality
63
Medical management for eisenmenger syndrome?
Oxygen - manage symptoms
Treat pulmonary HPTN eg with sildenafil
Treat arrhythmias
Treatment of polycythaemia with venesection
Prevention and treatment of thrombosis with venesection
Prevention and treatment of thrombosis with anticoagulation
Prevention of infective endocarditis using prophylactic antibiotics
64
What is a ventricular septal defect?
Congenital hole in septum between two ventricles - tiny to entire septum (one large ventricle)
65
What are VSDs often linked to
Underlying genetic condition
Commonly ass with Downs syndrome and Turners syndrome
66
When can VSDs be picked up?
Antenatal scans
Murmur heard during newborn baby check
67
Typical time for presentation of VSD
6-8 weeks
68
Typical symptoms of VSD
Poor feeding
Dyspnoea
Tachypnoea
FTT
69
What examination signs do patients with VSD have?
Pan-systolic murmur
L lower sternal border
3rd + 4th intercostal spaces
Systolic thrill on palpation
May be scattered wheeze
70
What murmurs can a pan systolic murmur be?
VSD
Mitral regurgitation
Trciuspid regurgitiation
71
Treatment for VSD?
Pulmonary HPTN or HF -> transcatherter closure via femoral vein
Open heart surgery
Or close spontaneously
72
What condition do you have an increased risk with VSD?
Infective endocarditis
73
Why offer prophylactic antibiotics in patients with VSD undergoing surgical procedures?
Against infective endocarditis as more likely to get
74
What condition is Turners syndrome often particuarly related to (CHD)?
cOARCTATION of the aorta
75
What is coarctation of the aorta?
Narrowing of the aortic arch, usually around the ducturs arteriosis
76
What does coarctation of the aorta cause?
Reduces pressure of blood flow to distal arteries
Increases pressure in proximal areas to narrowing eg heart and first three branches of aorta
77
What does weak femoral pulses in a neonate indicate?
Coarctation of the aorta
78
What do you perform to check for coarctation of the aorta in a newborn?
Four limb blood pressure
79
What is the result from 4 limb blood pressure if coarctation of the aorta?
Will reveal high BO in limbs supplied by arteries before narrowinf
Low BP in limbs supplied by arteries after the narrowing
80
Murmur with coarctation of the aorta?
Systolic murmur below left clavicle (left infraclavicular area) and L scapula
Coarctation may have other signs in infancy
81
Signs of coarctation in infancy
Tachypnoea + increased work of breathing
Weak femoral pulses
Poor feeding
Grey and floppy baby
82
Additiona signs with coarctation of aorta?
LV heave due to LV hypertrophy
Underdeveloped L arm where reduced flow to L subclavian artery
Underdevelopment of the legs
Collapse at 7 dyas old (when ductus arteriosus closes)
Higher BP in arms than legs
83
Management of coarctation of the aorta
Mild - symptom free until adulthood
Severe - emergency surgery shortly agter birth
Prostaglandin E - crticial coarctation
84
What use in critical coarctation of the aorta?
Prostaglandin E
85
What does Prostaglandin E allow in coarctation of the aorta?
Keep ductus arteriosus open while waiting for surgery - allows some blood flow through DA into systemic circulation distal to the coarctation
Surgery - correct coarctation and ligate ductus arteriosus
86
Which type of shunt in coarctation are more dangerous>
Post ductal because DA can be kept open in preductal to allow some systemic supply, wont really help in post ductal case
Post ductal much more likely to be fatal
87
What is tetralogy of fallot?
Congenital condition 4 coexisting pathologies:
-Ventricular septal defect
-Overriding aorta
-Pulmonary valve stenosis
-Right ventricular hypertrophy
88
What is an overriding aorta?
Aortic valve - entrance - placed further R than normal above the VSD
89
What happens to blood flow in overriding aorta/ how does it cause cyanosis?
RV contracts, blood sent upwards towards aorta. Blood that is sent up is from VSD area therefore mixed between ventricles, greater proportion of deoxygenated blood enters aorta from R side of heart
90
What does stenosis of the pulmonary valve do>
provides greater resistance against the flow of blood from the right ventricle.
91
What causes cyanosis in tetralogy of fallot?
Overriding aorta and pulmonary stenosis
92
How does pulmonary valve stenosis cause cyanosis?
Encourgaes blood to flow through VSD into aorta rather than into pulmonary vessles
93
What causes RV hypertrophy in tetralogy of fallot?
increased strain on right ventricle as it pump blood against the resistance of the left ventricle and pulmonary stenosis
94
Why are patients with tetralogy of fallot blue
Cardiac abnormalities cause R to L cardiac shunt
Blood bypasses childs lungs, stays deoxygenated when enters systemic circulation causing cyanosis
95
What is severity of cyanosis in tetralogy of fallot related to?
Degree of severity of pulmonary stenosis
96
Risk factors for tetralogy of fallto
Rubella infection
Increased age of mother >40
Alcohol in pregnancy
Diabetic mother
22q1 deletion - Di George syndrome
97
What CHD is 22q1 deletion - Di George syndrome ass with
Tetralogy of fallot
98
What will a CXR of tetraolgy of fallot show
Boot shaped heart due to R ventrular thickeing
99
How to investigate CHD
1st line is always ECHO
100
What can signal TOF in a newborn baby check?
Ejection systolic murmur caused by pulmonary stenosis
101
When are most cases of TOF picke up
Antenatal cns
102
What will severe TOF cause bfore 1?
HF
103
What suspect in older child with symptoms of HF
Mild tetralogy of fallit
104
Signs and symptoms of TOF
* Cyanosis (blue discolouration of the skin due to low oxygen saturations) – “goes blue while crying”
* Clubbing
* Poor feeding
* Poor weight gain
* Ejection systolic murmur heard loudest in the pulmonary area (second intercostal space, left sternal border)
* “Tet spells”
105
What is a tet spell?
Intermittent symptomatic periods where RL shunt worse
Precipitates cyanotic episode
106
What do children do when a tet spell occurs?
Older - squat
Ypunger - knees to chest
Encourages blood to enter the pulmonary vessels
107
How to medically manage a tet spell?
Supplementary oxygen
Beta blockers
IV fluids
Morphine
Na bicarbonate
Phenylephrine infusion
108
What can improve blood flow in pulmonary vessels in a tet spell?
Beta blockers (relax RV)
IV fluids (increase pre-load)
109
What can be used to treat metabolic acidosis in a tet spell?
Sodium bicarbonate
110
How does morphine help in a tet spell?
Decrease respiratory drive -> more effective breathing - decrease ulmonary reistance
111
What does phenylephrine infusion do in a tet spell?
Increase systemic vascular resistance
112
Emergency reaction to tet spell
Place in knee to chest position
Oxygen
Calm the child
Morphine
Sedation
113
What colour and stability will transposition of the great vessels present with?
Blue and unstable
114
What is the prognosis for tetralogy of fallot?
90% survival with surgery
Depends on severity
115
What is transposition of the great vessels?
Attachments of aorta and pulmonary trunk to heart are swapped -
RV -> aorta
LV -> pulmonary vessels
116
What conditions can transposition of the great vessels be ass with?
Ventricular septal defect
Coarctation of the aorta
Pumonary stenosis
117
What does immediate survival in transposition of the great vessels
A shunt - PDA, ASD, VSD
118
Prognosis of transposition of great vessels
Incompatible with life
119
When is transposition of the great vessels often picked up?
Antenatal US scans
120
Presntation of transposition of the great vessles
Cyanosis at birth
PDA or VSD - compensates - allows blood to mix between systemic and pulmonary
after few weeks - respiratory distress, tachycardia, poor feeding, poor weight gain and sweating
121
How does transposition of great vessels appear on an Xray?
Egg or potato on a string
122
What is initial management of transposition of the great vessles?
resus with colour, tone, breathing, keep them walm and call the paeds reg.
123
How is an atrial septal defect created in transposition of the great vessels?
Balloon sepostomy
Insert catheter into foramen ovale via umbilicus, inflate balloon and create large ASD
124
What is definitive management for transposition of great vessels?
Open heart surgery
Cardiopulmonary bypass machine perform arterial switch within few days birth
125
Conservative general management for CHDs
High calorie feeding
upright nursing
Supplemental oxygen
126
Medical management of CHDs
Diuretics
ACEis
PGE2 inhibtors - maintain PDA
Prostaglandin inhibitors - close DA (NSAIDs)
127
Palliative care for CHDs
Surgical Transcather
Home O2
128
Curative treatment for CHD
Heart transplant
Surgical
Transcatheter
128
Curative treatment for CHD
Heart transplant
Surgical
Transcatheter
129
Causes of HF in paediatrics
CHD
myocarditis
Cardiomyopathy - hyperthrophic or dilated
Endocarditis
Tacharrhtyhmias
IHD - kawasaki disease
HPTN - renal disease
High output - anaemia/thyrotoxicosis
130
Symptoms of HF in paeds
SOB
Sweat
coguh
poor feed
Recurrent chest infections
Palpitations
Chest pain
Lethargy
131
Signs of HF in paeds
Tachypnoea
Tachycardia
Heart murmur
Poor weight gain
Faltering growth
Hepatomegaly
Cool peripheries
Radio femoral delay
Thrills
Cyanosis
