Paediatrics- Cardio, Resp Flashcards

1
Q

What is Bronchiolitis

What is it caused by

A

Bronchiolitis

Respiratory syncytial virus (RSV)

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2
Q

Bronchiolitis is very common in winter or summer

A

winter

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3
Q

Bronchiolitis is very common in winter. Bronchiolitis is generally considered to occur in children under 1 year. It is most common in children under _ ______. It can rarely be diagnosed in children up to 2 years of age, particularly in ex-premature babies with _____ _______ ____

A

Bronchiolitis is very common in winter. Bronchiolitis is generally considered to occur in children under 1 year. It is most common in children under 6 months. It can rarely be diagnosed in children up to 2 years of age, particularly in ex-premature babies with chronic lung disease.

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4
Q

Patho of Bronchiolitis

A

When a virus affects the airways of adults, the swelling and mucus are proportionally so small that it has little noticeable effect on breathing. The airways of infants are very small to begin with, and when there is even the smallest amount of inflammation and mucus in the airway it has a significant effect on the infants ability to circulate air to the alveoli and back out. This causes the harsh breath sounds, wheeze and crackles heard on auscultation when listening to a bronchiolitic baby’s chest.

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5
Q

Presentation of Bronchiolitis

A
  • Coryzal symptoms. These are the typical symptoms of a viral upper respiratory tract infection: running or snotty nose, sneezing, mucus in throat and watery eyes.
  • Signs of respiratory distress
  • Dyspnoea (heavy laboured breathing)
  • Tachypnoea (fast breathing)
  • Poor feeding
  • Mild fever (under 39ºC)
  • Apnoeas are episodes where the child stops breathing
  • Wheeze and crackles on auscultation
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6
Q

Bronchiolitis

One of the foundations of paediatrics is being able to spot the signs of respiratory distress:

A
  • Raised respiratory rate
  • Use of accessory muscles of breathing, such as the sternocleidomastoid, abdominal and intercostal muscles
  • Intercostal and subcostal recessions
  • Nasal flaring
  • Head bobbing
  • Tracheal tugging
  • Cyanosis (due to low oxygen saturation)
  • Abnormal airway noises

learn this of by heart!!!!!

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7
Q

What do these mean

Wheeze

Grunting

Stridor

A

Wheezing is a whistling sound caused by narrowed airways, typically heard during expiration

Grunting is caused by exhaling with the glottis partially closed to increase positive end-expiratory pressure

Stridor is a high pitched inspiratory noise caused by obstruction of the upper airway, for example in croup

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8
Q

How does bronchiolitis start

Explain the progression

A

Bronchiolitis usually starts as an upper respiratory tract infection (URTI) with coryzal symptoms. From this point around half get better spontaneously. The other half develop chest symptoms over the first 1-2 days following the onset of coryzal symptoms. Symptoms are generally at their worst on day 3 or 4. Symptoms usually last 7 to 10 days total and most patients fully recover within 2 – 3 weeks. Children who have had bronchiolitis as infants are more likely to have viral induced wheeze during childhood.

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9
Q

Bronchiolitis

Most infants can be managed at home with advice about when to seek further medical attention. Reasons for admission include:

A
  • Aged under 3 months or any pre-existing condition such as prematurity, Downs syndrome or cystic fibrosis
  • 50 – 75% or less of their normal intake of milk
  • Clinical dehydration
  • Respiratory rate above 70
  • Oxygen saturations below 92%
  • Moderate to severe respiratory distress, such as deep recessions or head bobbing
  • Apnoeas
  • Parents not confident in their ability to manage at home or difficulty accessing medical help from home
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10
Q

Bronchiolitis Management

A

Typically patients only require supportive management. This involves:

  • Ensuring adequate intake. This could be orally, via NG tube or IV fluids depending on the severity. It is important to avoid overfeeding as a full stomach will restrict breathing. Start with small frequent feeds and gradually increase them as tolerated.
  • Saline nasal drops and nasal suctioning can help clear nasal secretions, particularly prior to feeding
  • Supplementary oxygen if the oxygen saturations remain below 92%
  • Ventilatory support if required

There is little evidence for treatments such as nebulised saline, bronchodilators, steroids and antibiotics.

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11
Q

Bronchiolitis

As breathing gets harder, the child gets more tired and less able to adequately ventilate themselves. They may require ventilatory support to maintain their breathing. This is stepped up until they are adequately ventilated:

Explain how they do this

A
  1. High-flow humidified oxygen via tight nasal cannula (i.e. “Airvo” or “Optiflow”). This delivers air and oxygen continuously with some added pressure, helping to oxygenate the lungs and prevent the airways from collapsing. It adds “positive end-expiratory pressure” (PEEP) to maintain the airway at the end of expiration.
  2. Continuous positive airway pressure (CPAP). This involves using a sealed nasal cannula that performs in a similar way to Airvo or Optiflow, but can deliver much higher and more controlled pressures.
  3. Intubation and ventilation. This involves inserting an endotracheal tube into the trachea to fully control ventilation.
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12
Q

Bronchiolitis

How do you assess ventilation

A

Capillary blood gases are useful in severe respiratory distress and in monitoring children who are having ventilatory support.

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13
Q

The most helpful signs of poor ventilation are:

A
  • Rising pCO2, showing that the airways have collapsed and can’t clear waste carbon dioxide.
  • Falling pH, showing that CO2 is building up and they are not able to buffer the acidosis this creates. This is a respiratory acidosis. If they are also hypoxic, this is classed as type 2 respiratory failure.
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14
Q

Palivizumab is a _______ _____ that targets the respiratory syncytial virus. A _____ _______ is given as prevention against bronchiolitis caused by RSV. It is given to high risk babies, such as ex-premature and those with ________ ______ _______

A

Palivizumab is a monoclonal antibody that targets the respiratory syncytial virus. A monthly injection is given as prevention against bronchiolitis caused by RSV. It is given to high risk babies, such as ex-premature and those with congenital heart disease.

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15
Q

Palivizumab _______ protection

A

passive

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16
Q

Is Palivizumab a true vaccine

A

It is not a true vaccine as it does not stimulate the infant’s immune system. It provides passive protection by circulating the body until the virus is encountered, as which point it works as an antibody against the virus, activating the immune system to fight the virus. The levels of circulating antibodies decrease over time, which is why a monthly injection is required.

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17
Q

What is viral induced wheeze

A

Viral-induced wheeze describes is an acute wheezy illness caused by a viral infection. Small children (typically under 3 years) have small airways. When these small airways encounter a virus (commonly RSV or rhinovirus) they develop a small amount of inflammation and oedema, swelling the walls of the airways and restricting the space for air to flow. This inflammation also triggers the smooth muscles of the airways to constrict, further narrowing the space in the airway.

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18
Q

What is Poiseuille’s law

A

This swelling and constriction of the airway caused by a virus has little noticeable effect on the larger airways of an older child or adult, however due to the small diameter of a child’s airway, t

states that flow rate is proportional to the radius of the tube to the power of four. Therefore, halving the diameter of the tube decreases flow rate by 16 fold.

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19
Q

Air flowing through these narrow airways causes a ______, and the restricted ventilation leads to ________ _____

A

Air flowing through these narrow airways causes a wheeze, and the restricted ventilation leads to respiratory distress.

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20
Q

How to distinguish between Viral Induced Wheeze and Asthma?

A

The distinction between a viral-induced wheeze and asthma is not definitive. Generally, typical features of viral-induced wheeze (as opposed to asthma) are:

  • Presenting before 3 years of age
  • No atopic history
  • Only occurs during viral infections

Asthma can also be triggered by viral or bacterial infections, however it also has other triggers, such as exercise, cold weather, dust and strong emotions. Asthma is historically a clinical diagnosis, and the diagnosis is based on the presence of typical signs and symptoms along with variable and reversible airflow obstruction.

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21
Q

Presentation of Viral Induced Wheeze

A

Evidence of a viral illness (fever, cough and coryzal symptoms) for 1-2 days preceding the onset of:

  • Shortness of breath
  • Signs of respiratory distress
  • Expiratory wheeze throughout the chest
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22
Q

TRUE OR FALSE

viral-induced wheeze or asthma cause a focal wheeze.

A

FALSE

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23
Q

What causes focal wheeze

A

focal airway obstruction such as an inhaled foreign body or tumour. These patients will require an urgent senior review.

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24
Q

Management of viral induced wheeze

A

Management of viral-induced wheeze is the same as acute asthma in children.

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25
Q
A
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26
Q

Presentation of acute asthma

A
  • Progressively worsening shortness of breath
  • Signs of respiratory distress
  • Fast respiratory rate (tachypnoea)
  • Expiratory wheeze on auscultation heard throughout the chest
  • The chest can sound “tight” on auscultation, with reduced air entry

A silent chest is an ominous sign. This is where the airways are so tight it is not possible for the child to move enough air through the airways to create a wheeze. This might be associated with reduce respiratory effort due to fatigue. A less experienced practitioner may think because there is no respiratory distress and no wheeze the child is not as unwell, however in reality this a silent chest is life threatening.

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27
Q

Explain the severity of moderate severe and life threatening asthma

A
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28
Q

Management

Staples of management in acute viral induced wheeze or asthma are:

A
  • Supplementary oxygen if required (i.e. oxygen saturations less than 94% or working hard)
  • Bronchodilators (e.g. salbutamol, ipratropium and magnesium sulphate)
  • Steroids to reduce airway inflammation: prednisone (orally) or hydrocortisone (intravenous)
  • Antibiotics only if a bacterial cause is suspected (e.g. amoxicillin or erythromycin)

Bronchodilators are stepped up as required:

  • Inhaled or nebulised salbutamol (a beta-2 agonist)
  • Inhaled or nebulised ipratropium bromide (an anti-muscarinic)
  • IV magnesium sulphate
  • IV aminophylline
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29
Q

Asthma Management

Mild cases can be managed as an outpatient with regular_________ ______ via a spacer (e.g. 4-6 puffs every 4 hours).

A

Mild cases can be managed as an outpatient with regular salbutamol inhalers via a spacer (e.g. 4-6 puffs every 4 hours).

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30
Q

Asthma Management

Moderate to severe cases require a stepwise approach working upwards until control is achieved:

A
  1. Salbutamol inhalers via a spacer device: starting with 10 puffs every 2 hours
  2. Nebulisers with salbutamol / ipratropium bromide
  3. Oral prednisone (e.g. 1mg per kg of body weight once a day for 3 days)
  4. IV hydrocortisone
  5. IV magnesium sulphate
  6. IV salbutamol
  7. IV aminophylline

If you haven’t got control by this point the situation is very serious. Call an anaesthetist and the intensive care unit. They may need intubation and ventilation. This call should be made earlier to give the best chance of successfully intubating them before the airway becomes too constricted.

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31
Q

Management of Asthma

A

Once control is established: you can gradually work your way back down the ladder as they get better:

  • Review the child prior to the next dose of their bronchodilator.
  • Look for evidence of cyanosis (central or peripheral), tracheal tug, subcostal recessions, hypoxia, tachypnoea or wheeze on auscultation.
  • If they look well, consider stepping down the number and frequency of the intervention.
  • A typical step down regime of inhaled salbutamol is 10 puffs 2 hourly then 10 puffs 4 hourly then 6 puffs 4 hourly then 4 puffs 6 hourly.

Consider monitoring the serum potassium when on high doses of salbutamol as it causes potassium to be absorbed from the blood into the cells.

It is also worth noting that salbutamol causes tachycardia and a tremor.

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32
Q

When do you discharge a child with acute asthma

A

Generally, discharge can be considered when the child well on 6 puffs 4 hourly of salbutamol. They can be prescribed a reducing regime of salbutamol to continue at home, for example 6 puffs 4 hourly for 48 hours then 4 puffs 6 hourly for 48 hours then 2-4 puffs as required.

A few other steps to consider:

  • Finish the course of steroids if these were started (typically 3 days total)
  • Provide safety-net information about when to return to hospital or seek help
  • Provide an individualised written asthma action plan
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33
Q

Presentation Suggesting a Diagnosis of Asthma

A
  • Episodic symptoms with intermittent exacerbations
  • Diurnal variability, typically worse at night and early morning
  • Dry cough with wheeze and shortness of breath
  • Typical triggers
  • A history of other atopic conditions such as eczema, hayfever and food allergies
  • Family history of asthma or atopy
  • Bilateral widespread “polyphonic” wheeze heard by a healthcare professional
  • Symptoms improve with bronchodilators
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34
Q

Presentation Indicating a Diagnosis Other Than Asthma

A
  • Wheeze only related to coughs and colds, more suggestive of viral induced wheeze
  • Isolated or productive cough
  • Normal investigations
  • No response to treatment
  • Unilateral wheeze suggesting a focal lesion, inhaled foreign body or infection
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35
Q

Typical Triggers of chronic asthma

A
  • Dust (house dust mites)
  • Animals
  • Cold air
  • Exercise
  • Smoke
  • Food allergens (e.g. peanuts, shellfish or eggs)
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36
Q

Diagnosis of Asthma

A

There is no gold standard test or diagnostic criteria for asthma. A diagnosis is made clinically based on a typical history and examination. Children are usually not diagnosed with asthma until they are at least 2 to 3 years old. When there is a low probability of asthma and the child is symptomatic, consider referral to a specialist for diagnosis.

When there is an intermediate or high probability of asthma, a trial of treatment can be implemented and if the treatment improves symptoms a diagnosis can be made.

There are investigations that can be used where there is an intermediate probability of asthma or diagnostic doubt:

  • Spirometry with reversibility testing (in children aged over 5 years)
  • Direct bronchial challenge test with histamine or methacholine
  • Fractional exhaled nitric oxide (FeNO)
  • Peak flow variability measured by keeping a diary of peak flow measurements several times a day for 2 to 4 weeks
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37
Q

Chronic Asthma

Medical Therapy in Under 5 Years

A
  1. Start a short-acting beta-2 agonist inhaler (e.g. salbutamol) as required
  2. Add a low dose corticosteroid inhaler or a leukotriene antagonist (i.e. oral montelukast)
  3. Add the other option from step 2.
  4. Refer to a specialist.
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38
Q

Chronic Asthma

Medical Therapy Aged 5 – 12 Years

A

Medical Therapy Aged 5 – 12 Years

  • Start a short-acting beta-2 agonist inhaler (e.g. salbutamol) as required
  • Add a regular low dose corticosteroid inhaler
  • Add a long-acting beta-2 agonist inhaler (e.g. salmeterol). Continue salmeterol only if the patient has a good response.
  • Titrate up the corticosteroid inhaler to a medium dose. Consider adding:
  • Oral leukotriene receptor antagonist (e.g. montelukast)
  • Oral theophylline
  • Increase the dose of the inhaled corticosteroid to a high dose.
  • Referral to a specialist. They may require daily oral steroids.
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39
Q

Chronic Asthma

Medical Therapy Aged Over 12 Years (Same as Adults)

A
  1. Start a short-acting beta 2 agonist inhaler (e.g. salbutamol) as required
  2. Add a regular low dose corticosteroid inhaler
  3. Add a long-acting beta-2 agonist inhaler (e.g. salmeterol). Continue salmeterol only if the patient has a good response.
  4. Titrate up the corticosteroid inhaler to a medium dose. Consider a trial of an oral leukotriene receptor antagonist (i.e. montelukast), oral theophylline or an inhaled LAMA (i.e. tiotropium).
  5. Titrate the inhaled corticosteroid up to a high dose. Combine additional treatments from step 4, including the option of an oral beta 2 agonist (i.e. oral salbutamol). Refer to specialist.
  6. Add oral steroids at the lowest dose possible to achieve good control under specialist guidance.
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40
Q

Inhaled Corticosteroids in Children

does it slow growth?

A

There is evidence that inhaled steroids can slightly reduce growth velocity and can cause a small reduction in final adult height of up to 1cm when used long term (for more than 12 months). This effect was dose-dependent, meaning it was less of a problem with smaller doses.

It is worth putting this in context for the parent by explaining that these are effective medications that work to prevent poorly controlled asthma and asthma attacks that could lead to higher doses of oral steroids being given. Poorly controlled asthma can lead to a more significant impact on growth and development. The child will also have regular asthma reviews to ensure they are growing well and on the minimal dose required to effectively control symptoms.

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41
Q

Good inhaler technique will reduce …

A

oral thrush

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42
Q

is salbutamol dry powder inhaler or metered dosed inhaler

A

metered dosed inhaler

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43
Q

MDI technique without a spacer:

Explain the instruction

A
  • Remove the cap
  • Shake the inhaler (depending on the type)
  • Sit or stand up straight
  • Lift the chin slightly
  • Fully exhale
  • Make a tight seal around the inhaler between the lips
  • Take a steady breath in whilst pressing the canister
  • Continue breathing for 3 – 4 seconds after pressing the canister
  • Hold the breath for 10 seconds or as long as comfortably possible
  • Wait 30 seconds before giving a further dose
  • Rinse the mouth after using a steroid inhaler
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44
Q

MDI technique with a spacer:

A
  • Assemble the spacer
  • Shake the inhaler (depending on the type)
  • Attach the inhaler to the correct end
  • Sit or stand up straight
  • Lift the chin slightly
  • Make a seal around the spacer mouthpiece or place the mask over the face
  • Spray the dose into the spacer
  • Take steady breaths in and out 5 times until the mist is fully inhaled

Alternatively exhale fully before putting making a seal with the spacer, spray the dose and take one deep breath in to inhale the mist in one breath before holding for 10 seconds.

Spacers should be cleaned once a month. Avoid scrubbing the inside and allow them to air dry to avoid creating static. Static can interact with the mist and prevent the medication being inhaled.

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45
Q

Bacterial causes of Pneumonia?

A
  • Streptococcus pneumonia is most common
  • Group A strep (e.g. Streptococcus pyogenes)
  • Group B strep occurs in pre-vaccinated infants, often contracted during birth as it often colonises the vagina.
  • Staphylococcus aureus. This causes typical chest xray findings of pneumatocoeles (round air filled cavities) and consolidations in multiple lobes.
  • Haemophilus influenza particularly affects pre-vaccinated or unvaccinated children.
  • Mycoplasma pneumonia, an atypical bacteria with extra-pulmonary manifestations (e.g. erythema multiforme).
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46
Q

Viral causes of pneumonia

A
  • Respiratory syncytial virus (RSV) is the most common viral cause
  • Parainfluenza virus
  • Influenza virus
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47
Q

Investigations for Pneumonia

A

A chest xray is the investigation of choice for diagnosing pneumonia. It is not routinely required, but can be useful if there is diagnostic doubt or in severe or complicated cases.

Sending sputum cultures and throat swabs for bacterial cultures and viral PCR can establish the causative organism and guide treatment. All patients with sepsis should have blood cultures. Capillary blood gas analysis can be helpful in assessing or monitoring respiratory or metabolic acidosis and the blood lactate level in unwell patients.

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48
Q

Pneumonia management paediatric

A

Pneumonia should be treated with antibiotics according to local guidelines.

Amoxicillin is often used first line. Adding a macrolide (erythromycin, clarithromycin or azithromycin) will cover atypical pneumonia. Macrolides can be used as monotherapy in patients with a penicillin allergy.

IV antibiotics can be used when there is sepsis or a problem with intestinal absorption.

Oxygen is used as required to maintain saturations above 92%.

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49
Q

What is CROUP

A

Croup is an acute infective respiratory disease affecting young children. It typically affects children aged 6 months to 2 years, however they can be older. It is an upper respiratory tract infection causing oedema in the larynx. The classic cause of croup that you need to spot in your exams, is parainfluenza virus. It usually improves in less than 48 hours and responds well to treatment is steroids, particularly dexamethasone.

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50
Q

The common causes for croup are:

A

Parainfluenza

Influenza

Adenovirus

Respiratory Syncytial Virus (RSV)

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51
Q

Croup used to be caused by __________ Croup caused by ______ leads to ________ and has a high mortality. Vaccination mean that this is very rare in developed countries.

A

Croup used to be caused by diphtheria. Croup caused by diphtheria leads to epiglottitis and has a high mortality. Vaccination mean that this is very rare in developed countries.

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52
Q

Presentation of Croup

A
  • Increased work of breathing
  • “Barking” cough, occurring in clusters of coughing episodes
  • Hoarse voice
  • Stridor
  • Low grade fever
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53
Q

Management of Croup

A

Most cases can be managed at home with simple supportive treatment (fluids and rest). During attacks it can help to sit the child up and comfort them. Measures should be taken to avoid spreading infection, for example hand washing and staying off school.

Oral dexamethasone is very effective. This is usually a single dose of 150 mcg/kg, which can be repeated if required after 12 hours. Prednisolone is sometimes used as an alternative where dexamethasone in not available (e.g. by GPs).

Stepwise options in severe croup to get control of symptoms:

  • Oral dexamethasone
  • Oxygen
  • Nebulised budesonide
  • Nebulised adrenalin
  • Intubation and ventilation
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54
Q

What is Epiglottitis

A

Epiglottitis is inflammation and swelling of the epiglottis caused by infection, typically with haemophilus influenza type B. The epiglottis can swell to the point of completely obscuring the airway within hours of symptoms developing. Therefore, epiglottitis is a life threatening emergency.

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55
Q

Why is epiglottis rare now in children

A

Epiglottitis is now rare due to the routine vaccination program, which vaccinates all children against haemophilus. You need to be extra cautious and have high suspicion in children that have not had vaccines. It can present in a similar way to croup, but with a more rapid onset.

In you exams keep a lookout for an unvaccinated child presenting with a fever, sore throat, difficulty swallowing that is sitting forward and drooling and suspect epiglottitis.

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56
Q

Presentation Suggesting Possible Epiglottitis

A
  • Patient presenting with a sore throat and stridor
  • Drooling
  • Tripod position, sat forward with a hand on each knee
  • High fever
  • Difficulty or painful swallowing
  • Muffled voice
  • Scared and quiet child
  • Septic and unwell appearance
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57
Q

Investigations of Epiglottis

A

If the patient is acutely unwell and epiglottitis is suspected then investigations should not be performed. Performing a lateral xray of the neck shows a characteristic “thumb sign” or “thumbprint sign”. This is a soft tissue shadow that looks like a thumb pressed into the trachea. This is caused by the oedematous and swollen epiglottis. Neck xrays are also useful for excluding a foreign body.

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58
Q

Management of Epiglottitis

A

Epiglottitis is an emergency and there is an immediate risk of the airway closing. A key point that is often talked about with epiglottitis is the importance of not distressing the patient, as this could prompt closure of the airway. If you see a child with suspected epiglottitis, leave them well alone and in their comfort zone. Don’t examine them and don’t make them upset. The most important thing is to alert the most senior paediatrician and anaesthetist available.

Management of epiglottis centres around ensuring the airway is secure. Most patients do not require intubation, however there is an ongoing risk of sudden upper airway closure, so preparations need to be made to perform intubation at any time. Intubation is often difficult and needs to be performed in a controlled environment with facilities available to do a tracheostomy (intubating through the neck) if the airway completely closes. When patients are intubated they are transferred to an intensive care unit.

Additional treatment once the airway is secure:

  • IV antibiotics (e.g. ceftriaxone)
  • Steroids (i.e. dexamethasone)
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59
Q

Prognosis of Epiglottitis

A

Most children recover without requiring intubation. Most patients that are intubated can be extubated after a few days and also make a full recovery. Death can occur in severe cases or if it is not diagnosed and managed in time.

A common complication to be aware of is the development of an epiglottic abscess, which is a collection of pus around the epiglottis. This also threatens the airway, making it a life threatening emergency. Treatment is similar to epiglottitis.

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60
Q

What is Laryngomalacia

A

Laryngomalacia is a condition affecting infants, where the part of the larynx above the vocal cords (the supraglottic larynx) is structured in a way that allows it to cause partial airway obstruction. This leads to a chronic stridor on inhalation, when the larynx flops across the airway as the infant breathes in. Stridor is a harsh whistling sound caused by air being forced through an obstruction of the upper airway.

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61
Q

What are Structural Change of sLaryngomalacia

A

There are two aryepiglottic folds at the entrance of the larynx. They run between the epiglottis and the arytenoid cartilages. They are either side of the airway and their role is to constrict the opening of the airway to prevent food or fluids entering the larynx and trachea. In laryngomalacia the aryepiglottic folds are shortened, which pulls on the epiglottis and changes it shape to a characteristic “omega” shape.

The tissue surrounding the supraglottic larynx is softer and has less tone in laryngomalacia, meaning it can flop across the airway. This happens particularly during inspiration, as the air moving through the larynx to the lungs pulls the floppy tissue across the airway to partially occlude it. This partial obstruction of the airway generates the whistling sound.

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62
Q

Presentation of Laryngomalacia

A

Laryngomalacia occurs in infants, peaking at 6 months. It presents with inspiratory stridor, a harsh whistling sound when breathing in. Usually this is intermittent and become more prominent when feeding, upset, lying on their back or during upper respiratory tract infections. Infants with laryngomalacia do not usually have associated respiratory distress.

It can cause difficulties with feeding, but rarely causes complete airway obstruction or other complications.

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63
Q

Disease Course and Management of Laryngomalacia

A

The problem resolves as the larynx matures and grows and is better able to support itself, preventing it from flopping over the airway. Usually, no interventions are required and the child is left to grow out of the condition.

Rarely tracheostomy may be necessary. This involves inserting a tube through the front of the neck into the trachea, bypassing the larynx. Surgery is also an option to alter the tissue in the larynx and improve the symptoms.

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64
Q

What is a Whooping cough

A

Whooping cough is an upper respiratory tract infection caused by Bordetella pertussis (a gram negative bacteria). It is called “whooping cough”, because the coughing fits are so severe that the child is unable to take in any air between coughs and subsequently makes a loud whooping sound as they forcefully suck in air after the coughing finishes.

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65
Q

Whats another name of Whooping cough

A

pertussis

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66
Q

Children and pregnant women are vaccinated against ______. The vaccine becomes less effective a few years after each dose

A

Children and pregnant women are vaccinated against pertussis. The vaccine becomes less effective a few years after each dose

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67
Q

Pertussis typically starts with mild ______ symptoms, a low grade fever and possibly a mild dry cough.

A

Pertussis typically starts with mild coryzal symptoms, a low grade fever and possibly a mild dry cough.

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68
Q

Presentation of Whooping Cough

A

More severe coughing fits start after a week or more. These involve sudden and recurring attacks of coughing with cough free periods in between. This is described as a paroxysmal cough. Coughing fits are severe and keep building until the patient is completely out of breath. Patient typically produces a large, loud inspiratory whoop when the coughing ends. Patients can cough so hard they faint, vomit or even develop a pneumothorax. Bear in the mind that not all patients will “whoop” and infants with pertussis may present with apnoeas rather than a cough

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69
Q

Diagnosis Whooping Cough

A

A nasopharyngeal or nasal swab with PCR testing or bacterial culture can confirm the diagnosis within 2 to 3 weeks of the onset of symptoms.

Where the cough has been present for more than 2 weeks patients can be tested for the anti-pertussis toxin immunoglobulin G. This is tested for in the oral fluid of children aged 5 to 16 and in the blood of those aged over 17.

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70
Q

Management of whooping cough

A

Pertussis is a notifiable disease. Therefore Public Health need to be notified of each case.

Management typically involves simple supportive care. Vulnerable or acutely unwell patients, those under 6 months and patients with apnoeas, cyanosis or patients with severe coughing fits may need to be admitted. Measures to prevent spread are important, such as avoiding contact with vulnerable people, disposing of tissues and careful hand hygiene.

Macrolide antibiotics such as azithromycin, erythromycin and clarithromycin can be beneficial in the early stages (within the first 21 days) or vulnerable patients. Co-trimoxazole is an alternative to macrolides.

Close contacts with an infected patient are given prophylactic antibiotics if they are in a vulnerable group, for example pregnant women, unvaccinated infants or healthcare workers that have contact with children or pregnant women.

The symptoms typically resolve within 8 weeks, however they can last several months. It is also known as the “100-day cough” due to the potential long duration of the cough.

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71
Q

A key complication of whooping cough is __________

A

A key complication of whooping cough is bronchiectasis.

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72
Q

What is CF

A

Cystic fibrosis (CF) is an autosomal recessive genetic condition affecting mucus glands. It is caused by a genetic mutation of the cystic fibrosis transmembrane conductance regulatory gene on chromosome 7.

There are many variants of this mutation, the most common is the delta-F508 mutation. This gene codes for cellular channels, particularly a type of chloride channel. Around 1 in 25 are carriers of the mutation and 1 in 2500 children have CF.

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73
Q

The key consequences of the cystic fibrosis mutation are:

A
  • Thick pancreatic and biliary secretions that cause blockage of the ducts, resulting in a lack of digestive enzymes such as pancreatic lipase in the digestive tract
  • Low volume thick airway secretions that reduce airway clearance, resulting in bacterial colonisation and susceptibility to airway infections
  • Congenital bilateral absence of the vas deferens in males. Patients generally have healthy sperm, but the sperm have no way of getting from the testes to the ejaculate, resulting in male infertility
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74
Q

What is popular scenario for CF

A

A popular scenario is: both parents are healthy, one sibling has cystic fibrosis and a second child does not have the disease, what is the likelihood of the second child being a carrier? We know the child doesn’t have the condition, so the answer is two in three.

75
Q

Presentation Cystic fibrosis

A

Cystic fibrosis is screened for at birth with the newborn bloodspot test.

Meconium ileus is often the first sign of cystic fibrosis. The first stool that a baby passes is called meconium. This is usually black and should be passed within 24 hours of birth. In about 20% of babies with CF, the meconium is thick and sticky, causing it to get stuck and obstruct the bowel. This is called meconium ileus, and is practically pathognomonic for cystic fibrosis. This presents as not passing meconium within 24 hours, abdominal distention and vomiting.

If cystic fibrosis is not diagnosed shortly after birth it can present later in childhood with typical signs and symptoms, recurrent lower respiratory tract infections, failure to thrive or pancreatitis.

76
Q

Symptoms of CF

A
  • Chronic cough
  • Thick sputum production
  • Recurrent respiratory tract infections
  • Loose, greasy stools (steatorrhoea) due to a lack of fat digesting lipase enzymes
  • Abdominal pain and bloating
  • Parents may report the child tastes particularly salty when they kiss them, due to the concentrated salt in the sweat
  • Poor weight and height gain (failure to thrive)
77
Q

Signs of CF

A
  • Low weight or height on growth charts
  • Nasal polyps
  • Finger clubbing
  • Crackles and wheezes on auscultation
  • Abdominal distention
78
Q

Causes of Clubbing in Children

A
  • Hereditary clubbing
  • Cyanotic heart disease
  • Infective endocarditis
  • Cystic fibrosis
  • Tuberculosis
  • Inflammatory bowel disease
  • Liver cirrhosis
79
Q

Diagnosis of CF

A

There are three key methods for establishing a diagnosis that you should remember for your exams:

  • Newborn blood spot testing is performed on all children shortly after birth and picks up most cases
  • The sweat test is the gold standard for diagnosis
  • Genetic testing for CFTR gene can be performed during pregnancy by amniocentesis or chorionic villous sampling, or as a blood test after birth
80
Q

WHat is a sweat test

A

The sweat test is the key investigation to remember for cystic fibrosis. It is the gold standard for confirming the diagnosis. A patch of skin is chosen for the test, typically on the arm or leg. Pilocarpine is applied to the skin on this patch. Electrodes are placed either side of the patch and a small current is passed between the electrodes. This causes the skin to sweat. The sweat is absorbed with lab issued gauze or filter paper and sent to the lab for testing for the chloride concentration. The diagnostic chloride concentration for cystic fibrosis is more than 60mmol/l.

81
Q

Patients with cystic fibrosis struggle to clear the secretions in their airways. This creates a perfect environment with plenty of moisture and oxygen for colonies of bacteria to live and replicate. Examples of common colonisers are:

A
  • Staphylococcus aureus
  • Haemophilus influenza
  • Klebsiella pneumoniae
  • Escherichia coli
  • Burkhodheria cepacia
  • Pseudomonas aeruginosa
82
Q

CF

: The key colonisers to remember for your exams are ___ _____ and ________

A

: The key colonisers to remember for your exams are staph aureus and pseudomonas

83
Q

Pseudomonas colonisation can be treated with long term nebulised antibiotics such as _________. Oral _________ is also used.

A

Pseudomonas colonisation can be treated with long term nebulised antibiotics such as tobramycin. Oral ciprofloxacin is also used.

84
Q

Cystic fibrosis will be managed by the specialist MDT. There are many aspects to management:

A
  • Chest physiotherapy several times a day is essential to clear mucus and reduce the risk of infection and colonisation
  • Exercise improves respiratory function and reserve, and helps clear sputum
  • High calorie diet is required for malabsorption, increased respiratory effort, coughing, infections and physiotherapy
  • CREON tablets to digest fats in patients with pancreatic insufficiency (these replace the missing lipase enzymes)
  • Prophylactic flucloxacillin tablets to reduce the risk of bacterial infections (particularly staph aureus)
  • Treat chest infections when they occur
  • Bronchodilators such as salbutamol inhalers can help treat bronchoconstriction
  • Nebulised DNase (dornase alfa) is an enzyme that can break down DNA material in respiratory secretions, making secretions less viscous and easier to clear
  • Nebulised hypertonic saline
  • Vaccinations including pneumococcal, influenza and varicella
85
Q

Other Treatment Options for CF

A
  • Lung transplantation is an option in end stage respiratory failure
  • Liver transplant in liver failure
  • Fertility treatment involving testicular sperm extraction for infertile males
  • Genetic counselling
86
Q

What is Monitoring like with CF

A

Patients with cystic fibrosis are managed and followed up in specialist clinics, typically every 6 months. They require regular monitoring of their sputum for colonisation of bacteria like pseudomonas. They also need monitoring and screening for diabetes, osteoporosis, vitamin D deficiency and liver failure.

87
Q

90% of patients with CF develop __________ _______

50% of adults with CF develop cystic fibrosis-related ______ and require treatment with ______

30% of adults with CF develop_____ _____

Most males are infertile due to absent vas deferens

A

90% of patients with CF develop pancreatic insufficiency

50% of adults with CF develop cystic fibrosis-related diabetes and require treatment with insulin

30% of adults with CF develop liver disease

Most males are infertile due to absent vas deferens

88
Q
A
89
Q

fetus

In the fetus, blood needs to go via the______ to collect oxygen and nutrients and to dispose of waste products such as ____ ______ and _____(via the mother).

A

In the fetus, blood needs to go via the placenta to collect oxygen and nutrients and to dispose of waste products such as carbon dioxide and lactate (via the mother).

90
Q

As the fetal lungs are not fully developed or functional, it does not make sense for the fetal blood to pass through the pulmonary circulation. Therefore there are three _____ that allow blood to bypass the lungs.

A

As the fetal lungs are not fully developed or functional, it does not make sense for the fetal blood to pass through the pulmonary circulation. Therefore there are three shunts that allow blood to bypass the lungs.

91
Q

Name the three shunts

A

Ductus venosus

Foramen ovale

Ductus arteriosus.

92
Q

What is ductus venosus

A

Ductus venosus: This shunt connects the umbilical vein to the inferior vena cava and allows blood to bypass the liver.

93
Q

What is Foramen ovale:

A

This shunt connects the right atrium with the left atrium and allows blood to bypass the right ventricle and pulmonary circulation.

94
Q

What is Ductus arteriosus.

A

This shunt connects the pulmonary artery with the aorta and allows blood to bypass the pulmonary circulation

95
Q

The first breaths the baby takes expands the alveoli, decreasing the __________ ______ ________

A

The first breaths the baby takes expands the alveoli, decreasing the pulmonary vascular resistance.

96
Q

he decrease in pulmonary vascular resistance causes a fall in pressure in the ____ ______. At this point the left atrial pressure is greater than the right atrial pressure, which squashes the atrial septum to cause functional closure of the foramen ovale, similar to a closed valve with nothing flowing through it. This then gets sealed shut structurally after a few weeks and becomes the ____ _____

A

The decrease in pulmonary vascular resistance causes a fall in pressure in the right atrium. At this point the left atrial pressure is greater than the right atrial pressure, which squashes the atrial septum to cause functional closure of the foramen ovale, similar to a closed valve with nothing flowing through it. This then gets sealed shut structurally after a few weeks and becomes the fossa ovalis.

97
Q

____________ are required to keep the ductus arteriosus open

A

Prostaglandins are required to keep the ductus arteriosus open

98
Q

What causes the closure of ductus arteriosus

A

ncreased blood oxygenation causes a drop in circulating prostaglandins. This causes closure of the ductus arteriosus, which becomes the ligamentum arteriosum.

99
Q

Immediately after birth the ductus venosus stops functioning

Why is this

A

because the umbilical cord is clamped and there is no flow in the umbilical veins. The ductus venosus structurally closes a few days later and becomes the ligamentum venosum.

100
Q

What is an innocent murmurs

A

Innocent murmurs are also known as flow murmurs. They are very common in children. They are caused by fast blood flow through various areas of the heart during systole.

101
Q

Innocent murmurs have typical features, all beginning with S:

A
  • Soft
  • Short
  • Systolic
  • Symptomless
  • Situation dependent, particularly if the murmur gets quieter with standing or only appears when the child is unwell or feverish
102
Q

Clear innocent murmurs with no concerning features may not require any investigations. Features that would prompt further investigations and referral to a paediatric cardiologist would be:

A

Murmur louder than 2/6

Diastolic murmurs

Louder on standing

Other symptoms such as failure to thrive, feeding difficulty, cyanosis or shortness of breath

103
Q

The key investigations to establish the cause of a murmur and rule out abnormalities in a child are:

A

ECG

Chest Xray

Echocardiography

104
Q

Ventricular septal defect heard at

A

the left lower sternal border

105
Q

Hypertrophic obstructive cardiomyopathy heart at the

A

fourth intercostal space on the left sternal border

106
Q

Splitting of the Second Heart Sound

What does this mean?

A

During inspiration the chest wall and diaphragm pull the lungs open. This also pulls the heart open. This is called negative intra-thoracic pressure. This causes the right side of the heart to fill faster as it pulls in blood from the venous system. The increased volume in the right ventricle causes it to take longer for the right ventricle to empty during systole, causing a delay in the pulmonary valve closing. When the pulmonary valve closes slightly later than the aortic valve, this causes the second heart sound to be “split”.

107
Q

What type of murmur causes atrial septal defect

A

Atrial septal defects cause a mid-systolic, crescendo-decrescendo murmur loudest at the upper left sternal border,with a fixed split second heart sound.

108
Q

Patent Ductus Arteriosus causes what type of murmur

A

ore significant PDAs cause a normal first heart sound with a continuous crescendo-decrescendo “machinery” murmur that may continue during the second heart sound, making the second heart sound difficult to hear.

109
Q

The murmur in tetralogy of Fallot arises from

A

pulmonary stenosis, giving an ejection systolic murmur loudest at the pulmonary area (second intercostal space, left sternal border).

110
Q

When does cyanosis occur

A

Cyanosis occurs when deoxygenated blood enters the systemic circulation. Cyanotic heart disease occurs when blood is able to bypass the pulmonary circulation and the lungs. This occurs across a right-to-left shunt. A right-to-left shunt describes any defect that allows blood to flow from the right side of the heart (the deoxygenated blood returning from the body) to the left side of the heart (the blood exiting the heart into the systemic circulation) without travelling through the lungs to get oxygenated.

111
Q

Heart defects that can cause a right-to-left shunt, and therefore cyanotic heart disease, are:

A

Ventricular septal defect (VSD)

Atrial septal defect (ASD)

Patent ductus arteriosus (PDA)

Transposition of the great arteries

112
Q

Are VSD ASD PD cyanotic

A

no

113
Q

why are VSD, ASD or PDA not cyanoti c

A

This is because the pressure in the left side of the heart is much greater than the right side, and blood will flow from the area of high pressure to the area of low pressure.

This prevents a right-to-left shunt. If the pulmonary pressure increases beyond the systemic pressure blood will start to flow from right-to-left across the defect, causing cyanosis. This is called Eisenmenger syndrome.

114
Q

Patients with transposition of the great arteries will always have _____ because the right side of the heart pumps blood directly into the aorta and systemic circulation.

A

Patients with transposition of the great arteries will always have cyanosis because the right side of the heart pumps blood directly into the aorta and systemic circulation.

115
Q

The ductus arteriosus normally stops functioning within 1-3 days of birth, and closes completely within the first 2-3 weeks of life. When it fails to close, it is called ….

A

patent ductus arteriosus” (PDA)

116
Q

Key risk factor of PDA

A

Prematurity

117
Q

Pathophysiology of PDA

A

The pressure in the aorta is higher than that in the pulmonary vessels, so blood flows from the aorta to the pulmonary artery. This creates a left to right shunt where blood from the left side of the heart crosses to the circulation from the right side. This increases the pressure in the pulmonary vessels causing pulmonary hypertension, leading to right sided heart strain as the right ventricle struggles to contract against the increased resistance. Pulmonary hypertension and right sided heart strain lead to right ventricular hypertrophy. The increased blood flowing through the pulmonary vessels and returning to the left side of the heart leads to left ventricular hypertrophy.

118
Q

A patent ductus arteriosus can be picked up during the newborn examination if a murmur is heard. It may also present with symptoms of:

A

Shortness of breath

Difficulty feeding

Poor weight gain

Lower respiratory tract infections

119
Q

The diagnosis of PDA

A

can be confirmed by echocardiogram.

The use of doppler flow studies during the echo can assess the size and characteristics of the left to right shunt.

An echo is also useful for assessing the effects of the PDA on the heart, for example demonstrating hypertrophy of the right ventricle, left ventricle or both

120
Q

Management of PDA

A

Patients are typically monitored until 1 year of age using echocardiograms. After 1 year of age it is highly unlikely that the PDA will close spontaneously and trans-catheter or surgical closure can be performed. Symptomatic patient or those with evidence of heart failure as a result of PDA are treated earlier.

121
Q

What is ASD

A

An atrial septal defect is a defect (a hole) in the septum (the wall) between the two atria. This connects the right and left atria allowing blood to flow between them.

122
Q

Patho of ASD

A

During the development of the fetus the left and right atria are connected. Two walls grow downwards from the top of the heart, then fuse together with the endocardial cushion in the middle of the heart to separate the atria. These two walls are called the septum primum and septum secondum.

Defects this these two walls lead to atrial septal defects, a hole connecting the left and right atria. There is a small hole in the septum secondum called the foramen ovale. The foramen ovale normally closes at birth.

An atrial septal defect leads to a shunt, with blood moving between the two atria. Blood moves from the left atrium to the right atrium because the pressure in the left atrium is higher than the pressure in the right atrium. This means blood continues to flow to the pulmonary vessels and lungs to get oxygenated and the patient does not become cyanotic, however the increased flow to the right side of the heart leads to right sided overload and right heart strain. This right sided overload can lead to right heart failure and pulmonary hypertension.

123
Q

What is Eisenmenger syndrome

A

. This is where the pulmonary pressure is greater than the systemic pressure, the shunt reverses and forms a right to left shunt across the ASD, blood bypasses the lungs and the patient becomes cyanotic.

124
Q
  • The types of atrial septal defect from most to least common are:
A
  • Ostium secondum, where the septum secondum fails to fully close, leaving a hole in the wall.
  • Patent foramen ovale, where the foramen ovale fails to close (although this not strictly classified as an ASD).
  • Ostium primum, where the septum primum fails to fully close, leaving a hole in the wall. This tends to lead to atrioventricular valve defects making it an atrioventricular septal defect.
125
Q

Complications of ASD

A
  • Stroke in the context of venous thromboembolism (see below)
  • Atrial fibrillation or atrial flutter
  • Pulmonary hypertension and right sided heart failure
  • Eisenmenger syndrome
126
Q

ASD Presentation

A

Atrial septal defects are often picked up through antenatal scans or newborn examinations. It may be asymptomatic in childhood and present in adulthood with dyspnoea, heart failure or stroke. Typical symptoms in childhood are:

  • Shortness of breath
  • Difficulty feeding
  • Poor weight gain
  • Lower respiratory tract infections
127
Q

Management of ASD

A

Patients with an ASD should be referred to a paediatric cardiologist for ongoing management. If the ASD is small and asymptomatic, watching and waiting can be appropriate. ASDs can be corrected surgically using a transvenous catheter closure (via the femoral vein) or open heart surgery. Anticoagulants (such as aspirin, warfarin and NOACs) are used to reduce the risk of clots and stroke in adults.

128
Q

What is VSD

What is it associated with

A

A ventricular septal defect (VSD) is a congenital hole in the septum (wall) between the two ventricles. This can vary in size from tiny to the entire septum, forming one large ventricle. VSDs can occur in isolation, however there is often an underlying genetic condition and they are commonly associated with Down’s Syndrome and Turner’s Syndrome.

129
Q

A left to right shunt leads to

A

right sided overload, right heart failure and increased flow into the pulmonary vessels.

130
Q

Often VSDs are initially symptomless and patients can present as late as adulthood. They may be picked up on antenatal scans or when a murmur is heard during the newborn baby check.

Typical symptoms include:

A

Poor feeding

Dyspnoea

Tachypnoea

Failure to thrive

131
Q

Examination Findings of VSD

A

Patients with a VSD typically have a pan-systolic murmur more prominently heard at the left lower sternal border in the third and fourth intercostal spaces. There may be a systolic thrill on palpation.

132
Q

The causes of a pan-systolic murmur are

A

ventricular septal defect, mitral regurgitation and tricuspid regurgitation.

133
Q

Treatment of VSD

A

Treatment should be coordinated by a paediatric cardiologist. Small VSDs with no symptoms or evidence of pulmonary hypertension or heart failure can be watched over time. Often they close spontaneously.

VSDs can be corrected surgically using a transvenous catheter closure via the femoral vein or open heart surgery.

There is an increased risk of infective endocarditis in patients with a VSD. Antibiotic prophylaxis should be considered during surgical procedures to reduce the risk of developing infective endocarditis.

134
Q

There are three underlying lesions that can result in Eisenmenger syndrome:

What are they

A

Atrial septal defect

Ventricular septal defect

Patent ductus arteriosus

135
Q

Eisenmenger syndrome can develop after ___ ____ with large shunts or in adulthood with small shunts. It can develop more quickly during_____so women with a history of having a “hole in the heart” need an echo and close monitoring by a cardiologist during pregnancy.

A

Eisenmenger syndrome can develop after 1-2 years with large shunts or in adulthood with small shunts. It can develop more quickly during pregnancy, so women with a history of having a “hole in the heart” need an echo and close monitoring by a cardiologist during pregnancy.

136
Q

Normal blood flow in the heart in children

A

Normally when there is a septal defect blood will flow from the left side of the heart to the right. This is because the pressure in the left side is greater than in the right. Remember, the left ventricle has to pump blood through the entire body, whereas the right ventricle simply has to fill the lungs. A left to right shunt means blood still travels to the lungs and gets oxygenated, so the patient does not become cyanotic.

137
Q

Over time the extra blood flowing into the right side of the heart and the lungs increases the pressure in the pulmonary vessels. This leads to ________ _________. When the pulmonary pressure exceeds the ________ _________, blood begins to flow from the right side of the heart to the left across the septal defect. This is a ____ __ ____ shunt. Essentially it becomes easier for the right side of the heart to pump blood across the defect into the left side of the heart compared with pumping blood into the lungs. This causes _________ blood to bypass the lungs and enter the body. This causes ________

A

Over time the extra blood flowing into the right side of the heart and the lungs increases the pressure in the pulmonary vessels. This leads to pulmonary hypertension. When the pulmonary pressure exceeds the systemic pressure, blood begins to flow from the right side of the heart to the left across the septal defect. This is a right to left shunt. Essentially it becomes easier for the right side of the heart to pump blood across the defect into the left side of the heart compared with pumping blood into the lungs. This causes deoxygenated blood to bypass the lungs and enter the body. This causes cyanosis.

138
Q

What does cyanosis mean

A

Cyanosis refers to the blue discolouration of skin relating to a low level of oxygen saturation in the blood. The bone marrow will respond to low oxygen saturations by producing more red blood cells and haemoglobin to increase the oxygen carrying capacity of the blood.

This leads to polycythaemia, which is a high concentration of haemoglobin in the blood. Polycythaemia gives patients a plethoric complexion. A high concentration of red blood cells and haemoglobin make the blood more viscous, making patients more prone to developing blood clots.

139
Q

Examination findings associated with pulmonary hypertension

A
  • Right ventricular heave: the right ventricle contracts forcefully against increased pressure in the lungs
  • Loud P2: loud second heart sound due to forceful shutting of the pulmonary valve
  • Raised JVP
  • Peripheral oedema
140
Q

Eisenmenger Syndrome

Examination findings related to the underlying septal defect:

A

Atrial septal defect: mid-systolic, crescendo-decrescendo murmur loudest at the upper left sternal border

Ventricular septal defect: pan-systolic murmur loudest at the left lower sternal border

Patent ductus arteriosus: continuous crescendo-decrescendo “machinery” murmur

Arrhythmias

141
Q

Findings related to the right to left shunt and chronic hypoxia:

A
  • Cyanosis
  • Clubbing
  • Dyspnoea
  • Plethoric complexion (a red complexion related to polycythaemia)
142
Q

Eisenmenger syndrome Prognosis

A

Eisenmenger syndrome reduces life expectancy by around 20 years compared with healthy individuals. The main causes of death are heart failure, infection, thromboembolism and haemorrhage. The mortality can be up to 50% in pregnancy.

143
Q

Management

Eisenmenger syndrome.

A

Patients with Eisenmenger syndrome will be closely followed up by a specialist. Medical management involves:

  • Oxygen can help manage symptoms but does not affect overall outcomes
  • Treatment of pulmonary hypertension, for example using sildenafil
  • Treatment of arrhythmias
  • Treatment of polycythaemia with venesection
  • Prevention and treatment of thrombosis with anticoagulation
  • Prevention of infective endocarditis using prophylactic antibiotics
144
Q

Once the pulmonary pressure is high enough to cause the syndrome, it is not possible to medically reverse the condition. The only definitive treatment is a _____ _____ ______ however this has a high mortality.

A

Once the pulmonary pressure is high enough to cause the syndrome, it is not possible to medically reverse the condition. The only definitive treatment is a heart-lung transplant, however this has a high mortality.

145
Q

What is Coarctation of the aorta

A

Coarctation of the aorta is a congenital condition where there is narrowing of the aortic arch, usually around the ductus arteriosus.

146
Q

The severity of the coarctation (or narrowing) can vary from mild to severe. It is often associated with an underlying genetic condition, particularly _______ _______

A

The severity of the coarctation (or narrowing) can vary from mild to severe. It is often associated with an underlying genetic condition, particularly Turners syndrome.

147
Q

affect on narrowing aortia on blood pressure

A

Narrowing of the aorta reduces the pressure of blood flowing to the arteries that are distal to the narrowing. It increases the pressure in areas proximal to the narrowing, such as the heart and the first three branches of the aorta.

148
Q

Presentation

Coarctation of the Aorta

A

Often the only indication of coarctation in a neonate may be weak femoral pulses. Performing a four limb blood pressure will reveal high blood pressure in the limbs supplied from arteries that come before the narrowing, and lower blood pressure in limbs that come after the narrowing. There may be a systolic murmur heard below the left clavicle (left infraclavicular area) and below the left scapula. Coarctation may have other signs in infancy:

  • Tachypnoea and increased work of breathing
  • Poor feeding
  • Grey and floppy baby

Additional signs may develop over time:

  • Left ventricular heave due to left ventricular hypertrophy
  • Underdeveloped left arm where there is reduced flow to the left subclavian artery
  • Underdevelopment of the legs
149
Q

Coarctation of the Aorta

Management

A

The severity of the coarctation varies between patients. In mild cases patients can live symptom free until adulthood without requiring surgical input, and in severe cases patients will require emergency surgery shortly after birth.

In cases of critical coarctation where there is a risk of heart failure and death shortly after birth Prostaglandin E is used keep the ductus arteriosus open while waiting for surgery. This allows some blood flow flow through the ductus arteriosus into the systemic circulation distal to the coarctation. Surgery is then performed to correct the coarctation and to ligate the ductus arteriosus.

150
Q

Patients with aortic stenosis may have one, two, three or four _______.

A

Patients with aortic stenosis may have one, two, three or four leaflets.

151
Q

Presentation aortic stenosis

A

Mild aortic stenosis can be completely asymptomatic, discovered as an incidental murmur during a routine examination. More significant aortic stenosis can present with symptoms of fatigue, shortness of breath, dizziness and fainting. Symptoms are typically worse on exertion as the outflow from the left ventricle cannot keep up with demand. Severe aortic stenosis will present with heart failure within months of birth.

152
Q

Congenital Aortic Valve Stenosis

Signs

A

The key examination finding is an ejection systolic murmur heard loudest at the aortic area, which is the second intercostal space, right sternal border. It has a crescendo-decrescendo character and radiates to the carotids.

Other signs that may be present on examination are:

  • Ejection click just before the murmur
  • Palpable thrill during systole
  • Slow rising pulse and narrow pulse pressure
153
Q

Paediatric Aortic Stenosis

Management

A

The gold standard investigation for establishing a diagnosis is an echocardiogram.

Congenital aortic stenosis tends to be a progressive condition that worsens over time. Patients need regular follow-up under a paediatric cardiologist, with echocardiograms, ECGs and exercise testing to monitor the progression of the condition. Patient with more significant stenosis may need to restrict physical activities.

Options for treating the stenosis are:

  • Percutaneous balloon aortic valvoplasty
  • Surgical aortic valvotomy
  • Valve replacement
154
Q

Paediatric Aortic Stenosis

Complications

A
  • Left ventricular outflow tract obstruction
  • Heart failure
  • Ventricular arrhythmia
  • Bacterial endocarditis
  • Sudden death, often on exertion
155
Q

The pulmonary valve usually consists of ______ leaflets that open and close to let blood out and prevent blood from returning to the heart. These leaflets can develop abnormally, becoming thickened or fused. This results in a narrow opening between the_____ ________ and the _________ ______. This is called congenital pulmonary valve stenosis.

A

The pulmonary valve usually consists of three leaflets that open and close to let blood out and prevent blood from returning to the heart. These leaflets can develop abnormally, becoming thickened or fused. This results in a narrow opening between the right ventricle and the pulmonary artery. This is called congenital pulmonary valve stenosis.

156
Q

Associations with congenital pulmonary valve stenosis

A

Congenital pulmonary valve stenosis often occurs without any associations. It can be associated with other conditions such as:

  • Tetralogy of Fallot
  • William syndrome
  • Noonan syndrome
  • Congenital rubella syndrome
157
Q

Presentation pulmonary stenosis

A

Often pulmonary stenosis is completely asymptomatic, and it is discovered as an incidental finding of a murmur during routine baby checks. More significant pulmonary valve stenosis can present with symptoms of fatigue on exertion, shortness of breath, dizziness and fainting.

158
Q

Congenital Pulmonary Valve Stenosis

Signs

A
  • Ejection systolic murmur heard loudest at the pulmonary area (second intercostal space, left sternal border)
  • Palpable thrill in the pulmonary area
  • Right ventricular heave due to right ventricular hypertrophy
  • Raised JVP with giant a waves
159
Q

Management

Pulmonary Valve Stenosis

A
160
Q

Pulmonary Valve Stenosis

Management

A

The gold standard investigation for establishing a diagnosis is an echocardiogram.

In mild pulmonary stenosis without symptoms patients generally do not require any intervention. They are followed up by a cardiologist with a “watching and waiting” approach.

If the patient is symptomatic or the valve is more significantly stenosed, balloon valvuloplasty via a venous catheter is the treatment of choice. This involves inserting a catheter under xray guidance into the femoral vein, through the inferior vena cava and right side of the heart to the pulmonary valve, and dilating the valve by inflating a balloon. If valvuloplasty is not appropriate or fails open-heart surgery can be performed.

161
Q

Tetralogy of Fallot is a congenital condition where there are four coexisting pathologies:

A

Ventricular septal defect (VSD)

Overriding aorta

Pulmonary valve stenosis

Right ventricular hypertrophy

162
Q
A
163
Q

“overriding aorta”

WHat does this mean?

A

The VSD allows blood to flow between the ventricles.

The term “overriding aorta” refers to the fact that the entrance to the aorta (the aortic valve) is placed further to the right than normal, above the VSD. This means that when the right ventricle contracts and sends blood upwards, the aorta is in the direction of travel of that blood, therefore a greater proportion of deoxygenated blood enters the aorta from the right side of the heart.

164
Q

How does Stenosis of the pulmonary valve causes cyanosis

A

Stenosis of the pulmonary valve provides greater resistance against the flow of blood from the right ventricle. This encourages blood to flow through the VSD and into the aorta rather than taking the normal route into the pulmonary vessels. Therefore, the overriding aorta and pulmonary stenosis encourage blood to be shunted from the right heart to the left, causing cyanosis.

165
Q

TOF

The increased strain on the muscular wall of the right ventricle as it attempts to pump blood against the resistance of the left ventricle and pulmonary stenosis causes _____ _________ ________, with thickening of the heart muscle.

A

The increased strain on the muscular wall of the right ventricle as it attempts to pump blood against the resistance of the left ventricle and pulmonary stenosis causes right ventricular hypertrophy, with thickening of the heart muscle.

166
Q

Does TOF cause a right to left cardiac shunt or left to right

A

right to left

167
Q

Risk Factors for TOF

A

Rubella infection

Increased age of the mother (over 40 years)

Alcohol consumption in pregnancy

Diabetic mother

168
Q

Investigations for TOF

A

As with all structural congenital cardiac abnormalities, an echocardiogram is the investigation of choice for establishing the diagnosis. During the echocardiogram, the machine can produce coloured pictures that demonstrate the direction of flow of blood. This is called doppler flow studies. This is useful in assessing the severity of the abnormality and shunt.

A chest xray may show the characteristic “boot shaped” heart due to right ventricular thickening. This not particularly useful diagnostically except during medical exams

169
Q

Presentation of TOF

A

Most cases are picked up before the child is born during the antenatal scans. Additionally, an ejection systolic murmur caused by the pulmonary stenosis may be heard on the newborn baby check.

Severe cases will present with heart failure before one year of age. In milder cases, they can present as older children once they start to develop signs and symptoms of heart failure.

170
Q

Signs and Symptoms of TOF

A
  • Cyanosis (blue discolouration of the skin due to low oxygen saturations)
  • Clubbing
  • Poor feeding
  • Poor weight gain
  • Ejection systolic murmur heard loudest in the pulmonary area (second intercostal space, left sternal border)
  • “Tet spells”
171
Q

WHat is Tet Spells

A

“Tet Spells” are intermittent symptomatic periods where the right to left shunt becomes temporarily worsened, precipitating a cyanotic episode. This happens when the pulmonary vascular resistance increases or the systemic resistance decreases. For example, if the child is physically exerting themselves they are generating a lot of carbon dioxide. Carbon dioxide is a vasodilator that causes systemic vasodilation and therefore reduces the systemic vascular resistance. Blood flow will choose the path of least resistance, so blood will be pumped from the right ventricle to the aorta rather than the pulmonary vessels, bypassing the lungs.

These episodes may be precipitated by waking, physical exertion or crying. The child will become irritable, cyanotic and short of breath. Severe spells can lead to reduced consciousness, seizures and potentially death.

172
Q

Older children may ____ when a tet spell occurs. Younger children can be positioned with their knees to their chest. Squatting increases the ________ _______ _______ This encourages blood to enter the _________ _____.

A

Older children may squat when a tet spell occurs. Younger children can be positioned with their knees to their chest. Squatting increases the systemic vascular resistance. This encourages blood to enter the pulmonary vessels.

173
Q

Any medical management of a tet spell should involve an experienced paediatrician, as they can be potentially life threatening.

A
  • Supplementary oxygen is essential in hypoxic children as hypoxia can be fatal.
  • Beta blockers can relax the right ventricle and improve flow to the pulmonary vessels.
  • IV fluids can increase pre-load, increasing the volume of blood flowing to the pulmonary vessels.
  • Morphine can decrease respiratory drive, resulting in more effective breathing.
  • Sodium bicarbonate can buffer any metabolic acidosis that occurs.
  • Phenylephrine infusion can increase systemic vascular resistance.
174
Q

Management and Prognosis of TOF

A

In neonates, a prostaglandin infusion can be used to maintain the ductus arteriosus. This allows blood to flow from the aorta back to the pulmonary arteries.

Total surgical repair by open heart surgery is the definitive treatment, however mortality from surgery is around 5%.

Prognosis depends on the severity, however it is poor without treatment. With corrective surgery, 90% of patients will live into adulthood.

175
Q

What is Transposition of the Great Arteries

A

Transposition of the great arteries is a condition where the attachments of the aorta and the pulmonary trunk to the heart are swapped (“transposed”). This means the right ventricle pumps blood into the aorta and the left ventricle pumps blood into the pulmonary vessels. In this scenario are two separate circulations that don’t mix: one travelling through the systemic system and right side of the heart and the other traveling through the pulmonary system and left side of the heart.

176
Q

Transpostion of arteries

The condition can also be associated with:

A

Ventricular septal defect

Coarctation of the aorta

Pulmonary stenosis

177
Q

Will Transposition of the Great Arteries causec cyanosis

A

Yes

During pregnancy there is normal development of the fetus. The gas and nutrient exchange happens in the placenta, therefore it is not necessary for blood to flow to the lungs. After birth the condition is immediately life threatening as there is no connection between the systemic circulation and the pulmonary circulation. The baby will be cyanosed.

178
Q

Presentation Transposition of the Great Arteries

A

The defect is often diagnosed during pregnancy with antenatal ultrasound scans. Close monitoring is necessary during the pregnancy and arrangements should be made so that the woman gives birth in a hospital capable of managing the condition after birth.

Where the defect was not detected during pregnancy it will present with cyanosis at or within a few days of birth. A patent ductus arteriosus or ventricular septal defect can initially compensate by allowing blood to mix between the systemic circulation and the lungs, however within a few weeks of life they will develop respiratory distress, tachycardia, poor feeding, poor weight gain and sweating.

179
Q

Management for Transposition of the Great Arteries

A

Where there is a ventricular septal defect, this will allow some mixing of blood between the two systems and provide some time for definitive treatment.

A prostaglandin infusion can be used to maintain the ductus arteriosus. This allow blood from the aorta to flow to the pulmonary arteries for oxygenation.

Balloon septostomy involves inserting a catheter into the foramen ovale via the umbilicus, and inflating a balloon to create a large atrial septal defect. This allows blood returning from the lungs (on the left side) to flow to the right side of the heart and out through the aorta to the body.

Open heart surgery is the definitive management. A cardiopulmonary bypass machine is used to perform an “arterial switch” procedure within a few days of birth. If present, a VSD or ASD can be corrected at the same time.

180
Q

What is Ebstein’s Anomaly

A

Ebstein’s anomaly is a congenital heart condition where the tricuspid valve is set lower in the right side of the heart (towards the apex), causing a bigger right atrium and a smaller right ventricle. This leads to poor flow from the right atrium to the right ventricle, and therefore poor flow to the pulmonary vessels. It is often associated with a right to left shunt across the atria via an atrial septal defect. When this happens blood bypasses the lungs, leading to cyanosis. It is also associated with Wolff-Parkinson-White syndrome.

181
Q

What is Ebstein’s Anomaly Presentation

A

Typical presenting features include:

  • Evidence of heart failure (e.g. oedema)
  • Gallop rhythm heard on auscultation characterised by the addition of the third and fourth heart sounds
  • Cyanosis
  • Shortness of breath and tachypnoea
  • Poor feeding
  • Collapse or cardiac arrest

Symptoms in patients with an associated atrial septal defect often present a few days after birth, when the ductus arteriosus closes. Where there is a right to left shunt across an atrial septal defect the ductus arteriosus allows blood to flow from the aorta into the pulmonary vessels to get oxygenated. This minimises the cyanosis. When the duct closes the patient becomes cyanotic and symptomatic.

182
Q

Diagnosis of Ebstein’s Anomaly

A

Echocardiogram is the investigation of choice for confirming the diagnosis and assessing the severity.

183
Q

Management of

Ebstein’s Anomaly

A

Medical management includes treating arrhythmias and heart failure. Prophylactic antibiotics may be used to prevent infective endocarditis. Definitive management is by surgical correction of the underlying defect.