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Paediatric Cardiology Flashcards

1
Q

Which congenital heart lesions cause a left to right shunt?

A

VSD
PDA
ASD

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2
Q

Which congenital heart conditions cause a right to left shunt ?

A

Tetralogy of fallout

Transposition of the great arteries

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3
Q

Which congenital heart condition causes common mixing of blood?

A

AVSD

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4
Q

Which congenital heart conditions will cause outflow obstruction in a ell child (asymptomatic murmur)?

A

Pulmonary stenosis

Aortic stenosis

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5
Q

Which congenital heart condition causes outflow obstruction, which will present as a sick neonate - collapsed with shock?

A

Coarctation of the aorta

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6
Q

How may congenital heart diseases be divided?

A

Acyanotic and Cyanotic

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7
Q

What is the mechanism which causes acyanotic heart disease?

A

L to R shunt

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8
Q

What is the mechanism that causes cyanotic heart disease?

A

R to L shunt

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9
Q

Causes of acyanotic?

A

ASD
PDA
VSD

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10
Q

Causes of cyanotic?

A 
ALL THE T's:
tetralogy of fallot
Transposition of thereat arteries
Truncal arteriosus
Tricuspid atresia
also AVSD
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11
Q

What are the causes of cyanosis (in a child)?

A 
Foreign body
Asthma
Effusions
Epiglottitis
Pneumonia 
Heart failure 
Tetralogy of fallout
Transposition of the great arteries
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12
Q

What is the management of cyanosis?

A

Warm up
Consider whether responsibility or cardiac cause
Pulse oximetry
give 100% O2 to see whether sats improve

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13
Q

How may central cyanosis present?

A

Blue mucous membranes and lips

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14
Q

How may peripheral cyanosis present?

A

Cold peripheries
Poor CO state –> sluggish circulation
Present with mottled skin

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15
Q

How do foetal RBCs get oxygen?

A

Transfer from mother’s RBCs as foetal RBCs have a higher affinity for oxygen due to the increased number of Hb molecules

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16
Q

How does the blood enter the foetus?

A

Umbilical vein

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17
Q

How saturated is the oxygenated blood to the foetus?

A

~80% oxygenated

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18
Q

How I the blood carried away from the foetus?

A

2 umbilical arteries

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19
Q

What are the adaptation sin the foetal circulation?

A 
Umbilical Vein
Ductus venosus
Foramen ovale
Ductus arteriosus
Two umbilical arteries
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20
Q

What does the ductus venous do?

A

Shunts blood away from the liver
Regulates the flow of blood through the liver sinusoids
Has a sphincter mechanism, which closes when a uterine contraction renders the venous return too high
Prevents sudden overloading of the heart

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21
Q

What is the role of the foramen ovale?

A

Shunts blood from the right atrium to the left atrium, aided by the IVC valve to bypass the lungs. (however, crista dividend prevents a little from going so some goes into RV)

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22
Q

What is the role of the ductus arteriosus?

A

Connects the pulmonary artery to the aorta so blood bypasses the lungs

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23
Q

After 10 minutes of being born what structures close in the foetal circulation?

A

Foramen ovale

Ductus arteriosus

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24
Q

When is a baby classed to have a ‘patent ductus arteriosus’?

A

when the ductus arteriosus has failed to close after 1 months of age (or 1 month after EDD)

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25
What occurs regarding blood flow with a PDA?
Flows from aorta into the pulmonary artery (L to R shunt)
26
What is the usual cause for the ductus arteriosus remaining patent?
Defect in the constrictor mechanism of the duct
27
What are the Sx of PDA?
Usually asymptomatic.. • apnoea • bradycardia • Increased oxygen requirement
28
Signs of PDA?
* continuous machinery murmur beneath the left clavicle * collapsing or bounding pulse * pulmonary HTN
29
What Ix are done for PDA?
CXR, ECG, ECHO* * best to diagnose
30
Mx of PDA?
* Ibuprofen or Indomethacin can cause closure of PDA through prostaglandin E2 Synthase inhibition * surgical closure
31
Which drugs can keep PDA open?
Prostaglandins
32
What is an ASD?
Is a hole between two atria - the foramen ovale remains patent allows for a L-R shunt
33
How is ASD classified?
Primary and secondary
34
What is primary ASD?
defect at the valce
35
What is secondary ASD?
simple ASD
36
What is the presentation usually?
Asymptomatic
37
What symptoms may be present for ASD?
recurrent chest infections or wheeze | Arrhythmias from the 4th decade onwards
38
What are signs of ASD?
Ejection systolic murmur on the upper left sternal edge Shunt from L-R Hear turbulence at the pulmonary valve Hear a split S2
39
Ix for ASD?
CXR, ECG, ECHO**, Cardiac catheter ** gold standard
40
What may be seen on a CXR for ASD?
cardiomegaly enlarged pulmonary arteries increased pulmonary vascular markings
41
What may be seen on an ECG for ASD?
right axis deviation and RBBB
42
What is the Mx of ASD?
Corrective surgery
43
How are VSDs categorised?
By size - small and large
44
What is meant by a small VSD?
smaller in diameter than the aortic valve so up to 3mm
45
What is meant by a large VSD?
larger in diameter than aortic valve so greater than 3mm
46
What are the symptoms of VSD?
Usually asymptomatic when small • heart failure symptoms - breathlessness, failure to thrive • recurrent chest infections with a large VSD
47
What are signs of VSD?
*  tachypnoea *  tachycardia * hepatomegaly * pansystolic murmur at LLSE - (volume depends on size - smaller is louder as more turbulence) *  quiet P2 *  large VSD may result in an apical mid-diastolic murmur
48
Ix of VSD?
* CXR * ESD * ECHO*
49
What might be seen on CXR for VSD?
Cardiomegaly, enlarged pulmonary arteries, increased pulmonary vascular markings, pulmonary oedema
50
What might be seen on ECG for VSD?
When large --> biventricular hypertrophy
51
Mx of VSDs?
Expect small ones to close spontaneously * Good dental hygiene to prevent bacterial endocarditis * Heart failure drugs - ACE inhibitor (captopril) and diuretics * Diet: increase calorie intake * Surgical correction
52
What can occur in children with a large VSD?
They have pulmonary HTN which can lead to irreversible damage of the pulmonary capillary vascular bed --> Eisenmenger syndrome.
53
When is surgery usually performed on a VSD?
3-6 months
54
Why is VSD performed when it is?
To manage HF and thrive | Prevent permanent lung damage form pulmonary HTN and high blood flow
55
What is coarctation of the aorta (CoA)?
A narrowing in the aorta
56
Where are 70% of the coarctations?
Preductal - so proximal to the insertion of the ductus arteriosus
57
What is the pathophysiology of CoA?
Due to arterial duct tissue encircling the aorta just at the point of the duct insertion, so when the PDA closes, the aorta constricts and causes severe obstruction to LV outflow
58
Who is CoA more common in?
females with turner's syndrome
59
What are the physical signs with CoA?
Sick baby with severe HF Absent femoral pulses Severe metabolic disease May get peripheral cyanosis if v severe
60
What is the commonest cause of collapse due to LV outflow obstruction?
coarctation of the aorta
61
What may be seen on a CXR for CoA?
Cardiomegaly from heart failure and shock
62
Ix for CoA?
CXR, ECG, ECHO
63
What is the Mx of CoA?
Surgical repair ASAP
64
What syndrome is AVSD commonest in?
Down's syndome (trisomy 21)
65
What do you get in AVSD? - with regards to defects
ASD VSD Tricuspid regurgitation Mitral regurgitation
66
Why do you get MR and TR in AVSD?
As part of the septum isn't formed in the heart
67
what is a complete AVSD?
Defect in the middle of the heart with a single 5-leaflet valve between the atria and the ventricles, which stretches across the whole AV junction - tends to leak
68
what are the clinical features of AVSD?
* Usually presents on antenatal screening * Cyanosis at birth or heart failure at 2-3 weeks of life * Lesion being detected on Echo screening in a baby with Down syndrome * ECG shows a superior axis
69
What is the Mx of AVSD?
* Treat heart failure medically | * Surgical repair at 3-6 months of age.
70
What is the commonest cause of cyanotic congenital heart disease?
Tetralogy of Fallot (50-70%)
71
What are the 4 cardinal signs of Tetralogy of Fallot?
*  A large VSD *  Pulmonary Stenosis *  Aorta overrides septal defect (allowing RV and LV to eject into it) *  RV hypertrophy
72
When does tetralogy of fallot present with cyanosis at birth?
When there is severe pulmonary stenosis
73
When is tetralogy of fallot usually diagnosed?
• antenatally OR • following identification of a murmur in first 2 months of life
74
What are the symptoms of Tetralogy of Fallot?
Cyanosis • of lips • fingertips •hyper cyanotic spells (but these are super rare now)
75
What are hyper cyanotic spells?
Rapid increase of cyanosis usually associated with irritability and inconsolable crying due to severe hypoxia There is breathlessness and pallor due to tissue acidosis. These spells are accompanied by a very short murmur
76
What are signs of tetralogy of fallot
* clubbing in older children * loud, harsh, ejection systolic murmur at LSE present from day 1 of life *  failure to thrive * failure to develop normally * failure to gain weight *  cyanosis *  feeding difficulties
77
What investigations would be done for tetralogy of fallot?
CXR, ECG, ECHO, cardiac catheterisation
78
What would be seen on CXR for tetralogy of fallot ?
*  relatively small heart with untitled apex (boot shape) due to RV hypertrophy *  decreased pulmonary vascular markings
79
What would ECG of tetralogy of fallot show?
Normal at birth, RV hypertrophy in older children
80
What is gold standard Ix for diagnosis?
ECHO
81
What is the management plan for tetralogy of fallot?
Treat medically initially then definitive surgery around 3-6 months
82
What medical management is given?
* prostaglandin to maintain PDA (IV 5ng/kg per min) *  oxygen therapy in very severely cyanosed newborns *  unless severely ill infants - prevent dehydration and iron deficiency
83
What is the treatment for hypercyanotic attacks 'test spells'?
* initially place child over parents shoulder or on abdomen *  try keep child calm to abort the attack *  prom treatment needed if lasts > 15 min * oxygen, morphine, bicarbonate, fluids, sedation, IV propanolol
84
What surgical management is offered?
Initially can have palliative surgical procedure if not old enough for primary repair to either shunt from subclavian to pulmonary artery or to dilate the RV outflow tract. Primary repair is then doe when 3-6 months of age - which closes the septal defect and the enlarges/dilates the RV outflow tract
85
What is transposition the great arteries?
Where the aorta is attached to the RV and the pulmonary artery is attached to the LV. ( aorta and PA switch)
86
Why is transposition of the great arteries a cyanotic congenital heart defect?
as deoxygenated blood enters from the IVC and then gets pumped to the body via the aorta, no interaction with oxygenated blood. Oxygenated blood from the lungs goes to LV - then back into PA and all oxygenated. NO COMMUNICATION BETWEEN PULMONARY AND SYSTEMIC CIRCULATIONS
87
What must be present in order for an infant to survive with transposition of the great arteries?
ASD or PDA or VSD
88
How is transposition of the great arteries usually diagnosed?
Antenatally on anomaly scan
89
Symptoms of transposition of the great arteries?
Cyanosis - usually presents on day 2 when the ductus arteriosus closes
90
Signs of transposition of the great arteries?
*  cyanosis *  second heart sound * usually no murmur
91
What Ix for transposition of the great arteries?
CXR,ECG, ECHO
92
What would be seen on a CXR for Transposition of the great arteries?
*  narrow upper mediastinum with an 'egg on the side' appearance of cardiac shadow *  increased pulmonary vascular markings
93
What is the management of Transposition of the great arteries?
* first step keep ductus arteriosus patent | * then operate and switch the arteries when achieved 6kg in weight and 6 months old
94
What techniques are used to improve mixing of oxygenated and deoxygenated blood in Transposition of the great arteries?
*  maintain PDA patency with IV prostaglandin | * cardiac catheterisation to perform an atrial septostomy
95
What is Eisenmenger Syndrome?
Occurs when there is high pulmonary blood flow due to a L - R shunt. The pulmonary arteries become thick walled and resistant to flow increases. Gradually, the shunt decreases until it reverses and the child becomes cyanosed (usually after 10-15 years) So L- R shunt turns into R-L shunt due to pulmonary vascular disease.
96
What is the presentation of eisenmenger syndrome?
*  primarily cyanosis * dyspnoea, fatigue, syncope, * chest pain *  haemoptysis
97
What are signs of eisenmenger syndrome?
* cyanosis * clubbing * plethora *  RV heave with palpable, loud pulmonary component of the second heart sound * ejection systolic murmur audible along left sternal border *  loud second heart sound with a narrow split * Graham Steel murmur
98
What is the treatment of eisenmenger syndrome?
HEART TRANSPLANT IS THE DEFINITIVE Tx Treat heart failure, and arrhythmias Mainly Tx aims to prevent eisenmenger syndrome
99
What is the preventative treatment for eisenmenger syndrome?
* early intervention for high pulmonary blood flow | *  prevent infective endocarditis
100
What is hypoplastic left heart syndrome?
underdevelopment of the left side of the heart causing: • small or atretic mitral valve • diminutive LV • aortic valve atresia • ascending aorta v small- usually coarctation
101
How is hypoplastic left heart syndrome usually diagnosed?
antenatally
102
If hypoplastic left heart syndrome presents after birth what are the clinical features?
* present with a duct-dependent systemic circulation * sickest of all neonates * no flow through the left side of the heart - ductal construction leads to profound acidosis and rapid cardiovascular collapse *  weakness or absence of peripheral pulses
103
What is the management of hypoplastic left heart syndrome?
surgery • Norwood procedure • glenn or heme-fontal procedure around 6 months • fontan procedure at 3 years
104
What type of heart disease is aortic stenosis?
acyanotic | outflow obstruction
105
what is the pathology of aortic stenosis?
Cusps of aortic valve are partly fused - leading to restrictive exit from the left ventricle
106
What is aortic stenosis often associated with?
Mitral valve stenosis and coarctation of the aorta
107
What are the clinical features of aortic stenosis?
``` • majority - asymptomatic murmur when severe stenosis: • reduced exercise tolerance • chest pain on exertion • syncope ```
108
What are the physical signs of AS?
* small volume, slow rising pulses *  carotid thrill *  ejection systolic murmur @URSE, radiates to the neck * delayed and soft A2 sound * apical ejection click
109
What investigations are done for AS?
CXR, ECG, Echo
110
What may be seen on CXR when aorta stenosis is present?
Normal or prominent left ventricle with post-stenotic dilatation of the ascending aorta
111
What is the management of aortic stenosis ?
For younger children: - regular clinics and echo appointments to assess and evaluate when to intervene. Thenbaloon valvotomy
112
When do you interfere to do surgical management for aortic stenosis?
* symptoms on exercise | * high resting pressure gradient (>64mmHg) across the aortic valve
113
What type of heart disease is pulmonary stenosis?
Acyanotic | Outflow obstruction
114
What is pulmonary stenosis usually associated with?
patent ductus arteriosus
115
How can PDA partially compensate for pulmonary stenosis?
As PDA shunts blood from aorta into the pulmonary artery so that blood gets to the lungs
116
What is the pathology of pulmonary stenosis?
Pulmonary valve leaflets are partly fused together and leads to restrictive exit from the RV
117
What are the symptoms of pulmonary stenosis?
MOstly asymptomatic | However when severe in neonates - present cyanosed within first few days esp. if still a patent foramen ovale
118
Signs of pulmonary stenosis?
* ejection systolic at ULSE *  may have a thrill present *  prominent RV heave when severe
119
Ix of pulmonary stenosis?
CXR, ECG, ECHO
120
What may be present in ECG of pulmonary stenosis ?
Show RV hypertrophy - upright T wave in V1 lead
121
What is the management of pulmonary stenosis?
Conservative when asymptomatic until pressure gradient across pulmonary valve is > 64mmHg and then do percutaneous balloon valvuplasty
122
What is congestive cardiac failure?
+LV and RV failure | when the heart is unable to pump sufficiently to maintain blood flow to meet the body's needs
123
What are the symptoms of left heart failure?
``` • dyspnoea • poor exercise tolerance • fatigue • cold peripheries • orthopnoea • nocturnal cough •wheeze • muscle wasting • tachycardia tachypnoea ```
124
What are the causes of left heart failure?
* aortic stenosis *  mitral valve disease * large L - R shunts *  leaky mitral valve * coarctation of the aorta
125
What are the hearts compensatory mechanisms?
* increased heart rate (controlled by neural and humeral input) * pumps harder (increases ventricular contractility) * increases preload
126
What happens when the compensatory mechanisms are no lone able to cope?
heart failure
127
What are Sx of Right heart failure?
``` Hepatomegaly Peripheral oedema (pittig) ascites anorexia epistaxis raised JVP ```
128
What are the causes of Right heart failure?
Progressive pulmonary disease Defects/pathology of myocardium Epstein's anomaly
129
What are the clinical signs of CCF in infants?
* tachycardia >150 beats/min * tachypnoea >50 breaths/min * gallop rhythm * hepatomegaly * diaphoresis * poor weight gain * growth problems * oedema of face and limbs
130
What are clinical signs of CCF in older children?
*  fatigue *  poor exercise tolerance * dyspnoea * orthopnoea *  abdo pain * oedema * ascites
131
What are the major criteria of the framingham criteria?
* Paroxysmal nocturnal dyspnoea * Crepitations * Jugular vein distension * S3 Gallop * Acute pulmonary oedema * Cardiomegaly * Hepatojugular reflux * Increased central venous pressure * Weight loss (>4.5kg in 5 days in response to treatment)
132
What are the minor criteria of the framingham criteria?
* Bilateral ankle oedema * Dyspnoea (on ordinary exertion) * Nocturnal cough * Hepatomegaly * Tachycardia (>120bpm) * Pleural effusion * Decrease in vital capacity by 1/3 from maximum recorded

Paediatrics (2 decks)

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