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Heart Disease in Children Flashcards

1
Q

How does a patent ductus arteriousis sound on auscultation

A

Continuous machine like murmur
Best heard at 2nd intercostal space, left sternal edge​

Large shunts may have a more mid diastolic mitral valve murmur​

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2
Q

What is the most common heart murmur picked up at the newborn baby check

A

Innocent flow murmur is most common

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3
Q

Which condition leads to weak or absent femoral pulses in children

A

Coarctation of the aorta

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4
Q

How does a VSD sound on auscultation

A

Pansystolic murmur

Heard loudest at left lower sternal edge

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5
Q

How does an ASD sound on auscultation

A

A fixed split second heart sound is typically heard

Can also have a systolic ejection murmur

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6
Q

Some children with congenital cardiac lesions will not have an audible murmur in the 1st few days of life, why is that

A

Foetal circulation has high pulmonary vascular pressure so right heart pressure is high = right to left shunt
At birth they inflate their lungs and there is a fall in pulmonary vascular resistance. The pressures in the right side of the heart slowly fall over the next few days to weeks
In this period the pressures equalise and so movement across a VSD is limited and may be hard to hear

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7
Q

How does heart failure present in babies

A

Increased work of breathing
Poor feeding and poor weight gain
Hepatomegaly

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8
Q

Which type of heart condition can lead to heart failure in babies

A

Seen in left to right shunts as blood returns to pulmonary circulation and causes oedmea

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9
Q

Which type of heart condition can lead to cyanosis in babies

A

Cyanosis is seen in right to left shunting as blood bypasses the lungs so less oxygenation

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10
Q

Which type of VSDs do not need treatment

A

Small VSDs may not need surgery if not haemodynamically significant
Some close on their own

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11
Q

How do you treat a significant VSD

A

Medicate with diuretics to reduce CHF if present
This is to give them time to grow for surgery

If this doesn’t work, a band can be applied around the pulmonary artery which reduces the blood flow to the lungs to improve symptoms.
This gives the patient a chance to grow and surgery to correct the defect can be performed at a later date
Surgical closure of a VSD is required if medical management is not enough to control symptoms and allow adequate weight gain

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12
Q

What causes Eisenmenger Syndrome

A

Chronic pulmonary overcirculation in the setting of a large left to right shunt.
The muscles in pulmonary vessels change/grow to adapt to this overcircualtion and increases resistance
This increases right heart pressure until it becomes higher than the left and the shunt reverses from right to left
Has a poor prognosis

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13
Q

How does Eisenmenger Syndrome present

A

Leads to cyanosis and poor exercise tolerance

Has a poor prognosis

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14
Q

Congenital Heart Disease is associated with which trisomies

A

Trisomy 21 – VSD and AVSD
Trisomy 18 (Edwards) – VSD, DORV
T13 (Patau) – VSD, DORV

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15
Q

Which heart defects are common seen in those with Turner’s syndrome

A

Coarctation of the aorta

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16
Q

Which heart defects are common seen in those with DiGeorge syndrome

A

Truncus Arteriosus
Interrupted Aortic Arch
Tetralogy of Fallot
VSD

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17
Q

In children the vast majority of heart disease is congenital - true or false

A

True

In adults it is mostly acquired

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18
Q

List some risk factors for congenital heart disease

A

Maternal diabetes
Prematurity
Other anomalies or a syndrome
Family history of Congenital Heart Disease

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19
Q

List symptoms of cardiac disease in babies

A

Breathlessness
Difficulty feeding or poor weight gain
Sweating with feeding
Cyanosis

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20
Q

Where would you find the apex beat in children

A

Apex can be in 4th ICS and more medial in children

Should be in normal place by age 8

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21
Q

What is perioral blueness

A

It is a blue area around the mouth, upper lip and nose
It is normal in a baby
Can be confused with central cyanosis although the rest of the baby will be pink - check tongue and mouth to be sure

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22
Q

What is acrocyanosis

A

Blue hands and feet

Common in young babies - not a sign of cardiac disease

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23
Q

Intermittent central cyanosis can be a feature of which condition

A

Tetralogy of Fallot

Will have a murmur as well

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24
Q

Most cyanotic heart disease makes children blue all the time - true or false

A

True

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25
How do you grade a heart murmur
``` 1 – slightest possible, often missed 2- slight 3- moderate murmur, no thrill 4- loud with thrill 5 – very loud with thrill 6 – can hear away from chest ```
26
How does the infants age at the time of murmur detection help diagnosis
Within 24 hours the risk of CHD is 1 in 10 This is because your circulation is changing so murmurs in are very common When 3-6 months old the risk of CHD is 1 in 4 By twelve months the risk is 1 in 50 This is because murmurs are even more common around 1 year but not due to CHD, innocent murmur
27
List the features of an innocent murmur
Usually soft and short systolic murmurs Tend to come and go, vary with position and be more noticeable at times of increased cardiac output e.g. Tachycardia/fever Some kids will get them when they are unwell due to the tach and temp Are never diastolic Are never associated with a thrill and should not be pansystolic Are not associated with cardiac symptoms - asymptomatic Often loudest at the Left sternal edge
28
New murmur and a temperature in an child is diagnostic of endocarditis - true or false
False In children it is much more likely to be an innocent murmur These often become more noticeable when a child is unwell It is diagnostic of endocarditis in adults however
29
Describe how foetal circulation changes to adult
Babies have right to left shunts to bypass the lungs – via the foramen ovale In womb, the right side does more work – pressure is higher Once born, the left side needs to take over as peripheral vascular pressure increases Right pressure falls as the pulmonary pressure drops - due to baby breathing The pressure increase in the left shuts the foramen ovale
30
Which congenital heart defects are considered acyantoic
``` VSD ASD Pulmonary stenosis AVSD PDA Aortic stenosis Coarctation of the aorta ```
31
Which congenital heart defects are considered cyanotic
Tetralogy of Fallot | Transposition of the great arteries
32
What is a systemic duct dependent lesion
A heart defect which causes severe obstruction of blood out of the left side of the heart This means the systemic circulation is dependent on a patent ductus arteriosus
33
List examples of systemic duct dependent lesions
Hypoplastic Left Heart Syndrome Critical Aortic Stenosis Interrupted Aortic Arch (or severe coarctation)
34
How do you manage a systemic duct dependent lesion
Give prostaglandins to keep the duct open until palliative or curative procedure is performed
35
What is a cyanotic duct dependent lesion
When there is obstruction to pulmonary blood flow OR lack of oxygenation of systemic blood Patient is reliant on the PDA to maintain oxygenation
36
List examples of a cyanotic duct dependent lesion
Transposition of the great arteries | Pulmonary Atresia with Intact Septum
37
How do you manage a cyanotic duct-dependent lesion
Aim is to keep Duct open until adequate mixing is secured – atrial septostomy
38
Which defects are seen in tetralogy of fallot
Right ventricle outflow tract obstruction Right ventricular hypertrophy VSD Overriding aorta - sits above the VSD Result of anterior malalignment of aorticopulmonary system​
39
What is transposition of the great arteries
The aorta and pulmonary artery are the wrong way around Aorta arises from right ventricle meaning unoxygenated blood is delivered to the body Pulmonary artery arises from the left ventricle so well oxygenated blood is delivered back to the lungs
40
What is the most common congenital heart defect
VSD
41
What are the 4 main types of ASD
Ostium secundum defect Ostium primum defect Sinus venosus defect Coronary sinus defect​
42
What is an ASD
Atrial septal defect | A more common type of congenital heart disease that allows communication between the atria.​
43
During embryonic development when does the atria split into two chambers
Around week 4-5 division begins | Goes from a common chamber to 2 distinct ones
44
How do ASDs usually present
Patients usually asymptomatic in first and second decade of life but usually develop fatigue, dyspnoea and exercise intolerance Some may experience cardiac failure or failure to thrive if severe
45
Which type of shunt is seen in ASDs
Larger ones can have L-R shunts Magnitude of shunting depends on size of defect, relative compliance of the 2 ventricles and pulmonary and systemic vascular resistance
46
List some risk factors for ASD
Female sex - 2:1 Maternal alcohol consumption Positive family history
47
Untreated ASDs can have which consequences
Right atrial enlargement Arrhythmias Heart failure
48
What are the indications for treatment in those with ASD
Haemodynamically significant - right sided and/or pulmonary overload Evidence of embolic activity
49
What are the 2 main methods used for ASD treatment
Open surgical closure | Transcatheter closure - better short-term outcomes
50
Explain transcatheter closure of an ASD
The catheter is placed in the femoral artery and tracks up the IVC to enter the right atrium. From here it is inserted into the septal defect and expanded blocking the defect and preventing blood flow between the left and right atria. Over time tissue will grow over the devise creating a tight and permanent seal. 
51
Explain open surgical closure of an ASD
They visualise the heart with a median sternotomy and isolate it using a cardiopulmonary bypass. The defect is sutured close or in the case of a larger defect a flap can be used to properly cover the hole. Some modern methods use thorascopy and robotics to reduce the recovery time
52
What is a VSD
Ventricular septal defect A congenital hole in the septum between the two ventricles ​ This can vary in size from tiny to the entire septem, forming one large ventricle ​
53
What kind of shunt is seen in VSD
Left to right - due to higher pressure in left ventricle However, a large left to right shunt leads to right sided overload, right heart failure and increased flow into the pulmonary vessels - can switch it to R-L (Eisenmengers)
54
Why are patients with VSD acyanotic
Blood shunts from L-R Blood is still flowing around the lungs before entering the rest of the body, therefore the patient remains acyanotic because their blood is properly oxygenated
55
VSDs are associated with which other conditions
Commonly associated with Down’s Syndrome and Turner’s Syndrome ​
56
What is the definition of a small VSD
Less than the diameter of the aortic valve (≤3mm) ​
57
How do small VSD's present
Often asymptomatic, are found incidentally​
58
What causes a small VSD
During development the ventricular septum does not fuse properly​ Account for 80-90% of VSDs​
59
How do you manage a small VSD
Review by paediatrician until murmur is no longer heard and ECG remains normal​ Maintenance of good dental hygiene, to prevent endocarditis​ Majority close before the age of 10​
60
What is the definition of a large VSD
They are the same size or larger than the aortic valve (21-23mm)​
61
How do large VSDs present
Heart failure​ Shortness of breath​ Faltering growth after 1 week old​ Recurrent chest infection​ Tachypnoea​ Tachycardia​ Hepatomegaly​ Hyperactive precordium Soft pansystolic murmur OR no murmur​ Apical mid-diastolic murmur​ Loud pulmonary second sound (P2))​
62
How might a large VSD present on CXR
Cardiomegaly​ Enlarged pulmonary arteries​ Increased pulmonary vascular markings​ Pulmonary oedema​
63
How might a large VSD present on ECG
Biventricular hypertrophy by 2 months of age​ Katz Wachtel Phenomenon​
64
How do you manage a large VSD
Diuretics​ Captopril​ Additional calorie input​ - aids growth Surgery at 3-6 months​ - to manage heart failure, faltering growth and prevention of Eisenmenger Syndrome​
65
What is a AVSD
Atrial Ventricular Septal defect Defect with the atrioventricular septa of the heart​ Can be Partial or Complete
66
What is a partial AVSD
Defect of septum separating atria​ | No communication between ventricles
67
What is a complete AVSD
Effective absence of internal divisions between heart chambers​ Specifically large defect between lower portion of atria and the upper portion of atria, allowing blood between all chambers of the heart​
68
AVSDs are associated with which other conditions
Prominent association of Down Syndrome​ - increased likelihood of complete AVSD Noonan Syndrome - typically partial
69
List risk factors for AVSD
Maternal alcohol use during pregnancy​ Poor control of maternal diabetes during gestation​ Family history of heart defects​
70
How do those with a complete AVSD present
``` Respiratory issues Pounding heart​ Weak pulse​ Pallor or Cyanosis (central)​ Poor feeding, slow weight gain​ Tiring easily​ Swelling of the legs or abdomen ```
71
All AVSDs present with a heart murmur immediately - true or false
False A heart murmur is often picked up at the first baby check.​ However, not all murmurs are picked up immediately and some do not present until later.​
71
All AVSDs present with a heart murmur immediately - true or false
False A heart murmur is often picked up at the first baby check.​ However, not all murmurs are picked up immediately and some do not present until later.​
72
AVSD’s can be fatal if not treated.​ - true or false
True
73
How do partial AVSDs present
May be asymptomatic for several year | Present later with arrhythmias, congestive heart failure, pulmonary hypertension
74
What is the gold standard for diagnosis of AVSD
Echo
74
How might AVSD present on CXR
Enlarged heart
75
How do you diagnose an AVSD
A combination of both clinical and medical investigative diagnosis:​ - Heart Murmur Present ​ - Poor Weight Gain ​ - Echocardiogram, CXR and Oxygen saturations
76
How do you manage an AVSD
Initial pharmacological intervention to treat congestive heart failure including diuretics (Furosemide) and ACEi (Enalapril)​ Surgical intervention for full within 3-6 months ​ Surgical intervention for partial within 6-18 months​ Surgery involves closure of hole with patches → Challenging aspect is dividing common AV valve
77
What is meant by an overriding aorta in ToF
Aortic valve enlarged and seems to open from both ventricles This enlarged aortic valve sits directly on top of the ventricular septal defect​
78
At what age does tetralogy of Fallot typically present
In the first 1-2 months of life
79
What is the most common cause of cyanotic congenital heart disease​
Tetralogy of Fallot
80
Tetralogy of fallot is more common in females - true or false
False | More common in males
81
What type of shunt is seen in tertralogy of fallot
right-left | Blood therefore bypasses the lungs and leads to cyanosis
82
ToF is associated with which genetic conditions
Down syndrome DiGeorge syndrome (chromosome 22 deletions)​ Specific genetic associations: JAG1, VEGF, NOTCH1, FLT4, etc.​
83
ToF is associated with which extra-cardiac abnormalities
Cleft lip/palate | Skeletal abnormalities
84
Most cases of ToF are genetic - true or false
False | Sporadic and unknown in most cases​
85
How does ToF present
Cyanosis/collapse in first month of life, hypercyanotic spells - worse when breathing deeply/fast Poor feeding, dyspnoea, agitation, low birth weight, finger clubbing​ Ejection systolic murmur (best heard at second intercostal space, left sternal edge)​ - due to pulmonary stenosis​ Right parasternal heave ​- due to right ventricular hypertrophy​ Tet spells - Intermittent symptomatic periods leading to cyanotic episode, child may squat to recover
86
How do you diagnose ToF
Pulse oximetry ​- low CXR​ - ‘Boot-shaped’ heart​ ECG​ - Right axis deviation due to right ventricular hypertrophy​ Echo ​
87
How do you manage ToF
Surgical repair is the mainstay of treatment (typically within the first year of life)​ In some cases a shunt operation/valvuloplasty procedure is required prior to complete repair ​ Valve replacement surgery will likely be required later in life (and replaced every 10-15 years) ​
88
List risk factors for coarctation of the aorta
Male gender​ Young age​ Family history​ Genetic syndrome eg. Turner’s ​
88
List risk factors for coarctation of the aorta
Male gender​ Young age​ Family history​ Genetic syndrome eg. Turner’s ​
89
Which other cardiac defects are commonly seen alongside coarctation of the aorta
Bicuspid aortic valve​ Aortic stenosis​ Ventricular septal defect​ Patent ductus arteriosus​
90
How do babies present with coarctation of the aorta
``` Pale skin​ Irritability ​ Heavy sweating ​ Difficulty breathing​ Difficulty feeding ​ ```
91
How do infants/toddlers with coarctation of the aorta present
``` Headaches​ Muscle weakness​ Leg cramps or cold feet​ Nosebleeds​ Chest pain ```
92
What is coarctation of the aorta
A narrowing of the aorta
93
Which examination findings may be present in a child with coarctation of the aorta
Pulse in lower limbs weaker than upper limbs​ Murmur ​- varied reports of ejection, continuous or late murmur heard best infraclavicularly or below left scapula​ Hypertension​
94
How do you diagnose coarctation of the aorta
Physical exam - murmur, weak limb pulse etc CXR Transthoracic echo - diagnostic
95
How might coarctation of the aorta present on CXR
Leftward convexity of the descending aorta​ Enlargement of the left subclavian artery ​ A figure-3 sign (formed by prestenotic and poststenotic dilatation of the descending aorta)
96
How is coarctation of the aorta treated
Open surgery with end-to-end anastomosis is the treatment of choice in neonates, infants and young children. Prosthetic patch is not offered due to the high risks of aneurysm and rupture,​ Balloon angioplasty is an option after 3-6 months however stenting will be not be included as the aorta will grow and widen. A deliberate tear in the intima will be performed to reduce the chance of recoil of the coarctation back to its original width, however, ​ Stent is included in children >25kg and adults if it has gone undiagnosed or re-occurred ​
97
List some risk factors for patent ductus arteriosus
``` Trisomy 21​ Holt-Oram syndrome Foetal exposure to valproic acid​ Rubella infection​ High altitude birth​ More common in girls (2:1) ```
98
What is the physiological purpose of the ductus arteriousus
It connects the pulmonary artery to the aorta​ bypassing the lungs as blood is oxygenated by mum via placenta In utero pulmonary pressure > aortic so blood pumped away from lungs in a right to left shunt​
99
How does the ductus areteriosus normally close
Following delivery, the lungs open and so pulm pressure < aortic pressure so duct should close
100
Which type of shunt is seen in PDA
left to right shunt | Sends excess blood to the lungs leading to overflow
101
How does patent ductus arteriosus present
``` Murmur Growth retardation​ Signs of heart failure​ - sweating, breathless, tachypnoea, lung creps, hepatomegaly, cardiomegaly, tachycardia​ Poor feeding​ Wide pulse pressure​ Collapsing peripheral pulses​ ```
102
How do you diagnose a PDA
ECG may show ventricular hypertrophy​ CXR may show cardiomegaly​ Echocardiography is best investigation​ - Assess PDA size​ - Assess degree of shunt
103
What are the conservative management options for PDA
Small PDA’s often close without treatment.​ | The baby’s heart can be monitored to see if the vessel is closing on its own. ​
104
What are the medical management options for PDA
Indomethacin or Ibuprofen ​- NSAIDs inhibit cyclo-oxigenase and decreasing prostaglandin synthesis which can close the duct Paracetamol​ - directly inhibits prostaglandin synthase Effective in 50% of prem babies, 80% of mature infants
105
What are the surgical management options for PDA
Catheter PDA Closure ​ Surgical Ligation ​- incision at 3rd/4th intercostal space, mid-axiallary and duct is ligated or clamped

Child Health (14 decks)

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