Ten ‘not to be missed’ paediatric ECGs

Cite this article as:
Megan Thomas, Jordan Evans, Amos Wong and Jeff Morgan. Ten ‘not to be missed’ paediatric ECGs, Don't Forget the Bubbles, 2020. Available at:
https://doi.org/10.31440/DFTB.29306

To refresh your memory on how to read paediatric ECGs take a look at Anna McCorquodale’s fantastic article: Approaching the paediatric ECG.

Here we review ten ‘not to be missed’ abnormal ECGs that may be encountered in acute paediatrics. 

#1 Supraventricular Tachycardia (SVT)

What is it?

SVT is a narrow complex tachycardia, the electrical activity originates above the ventricles (‘supraventricular’). SVT is classified based on whether it originates from the atrium or from the AV node. Finding where the position of the P wave is (with respect to the QRS complex) during tachycardia (‘P wave hunting’) is essential for the diagnosis of SVT.

Why does it happen?

It usually occurs due to one of the following mechanisms:

  • An accessory pathway linking the ventricle to the atrium, which impulses can travel along returning into the atria (AVRT, Atrioventricular Re-entrant Tachycardia)
  • A micro re-entrant circuit in the AV node itself (AVNRT, AV node re-entrant tachycardia)
  • An enhanced automatic focus in the atrium which fires impulses out 

These all lead to excessive impulses being conducted to the ventricles.

So what do we see on ECG?

  • A fast narrow complex tachycardia (approx. 150-220 bpm)
  • SVT – The hunt for the missing P wave:  It is a common misconception  in SVT that there are no P waves. Whilst this may appear to be the case, this is because the P wave is in fact hidden elsewhere.  The location of the missing P wave will depend on the type of SVT.
  • Lack of beat to beat variability i.e. you will see on the monitor that the rate stays pretty much constant

Atrioventricular Re-entrant Tachycardia (AVRT)

This is when there is an accessory electrical pathway connecting the ventricles and the atria. This creates a re-entrant circuit, with impulses either being conducted down the AV node and then back up the accessory pathway (orthodromic) or vice versa (antidromic).  You may see a retrograde P wave at the end of the QRS complex. (See #2 for further info on Wolff-Parkinson-White Syndrome, a classic type of AVRT).

Atrioventricular Nodal Re-entrant Tachycardia (AVNRT)

A micro re-entrant circuit forms in, or adjacent to, the AV node itself.  Here, P waves are very hard to find as they are usually buried in the QRS complex. The circuit often stimulates both the atria and ventricles and therefore the P-wave is hidden, buried within the QRS complex. 

AVNRT: N for No P waves!

Permanent junctional reciprocating tachycardia (PJRT)

This is a type of orthodromic AVRT where the concealed accessory pathway is near the coronary sinus. This means it can conduct at a relatively slow rate for a tachycardia. The characteristic of PJRT is Long RP tachycardia where the P wave is inverted in the inferior leads (hence NOT in sinus rhythm!) and the RP interval is longer than PR interval.

PJRT is commonly misdiagnosed as sinus tachycardia.  If PJRT is suspected seek cardiology input as adenosine is often ineffective and therefore needing multiple anti-arrhythmic therapy.

#2 Wolff-Parkinson-White Syndrome (WPW)

What is it?

Wolff-Parkinson-White is a conduction abnormality, where there is an accessory pathway connecting the atria and the ventricles. If this accessory pathway conducts from the atria to the ventricles (anterogradely) then it can be seen on the ECG as ‘pre-excitation’, as the impulse will travel faster down the accessory pathway than the rate-limited AV node.  WPW can lead to SVT (AVRT type).

What do we see on ECG?

A short PR interval (<120ms) is seen.

The most distinguishing feature is a delta wave which appears as a slow upslope between the Q wave and the R wave – with the Q wave being much earlier than usual. This means that the QRS is wide (>100ms).  The delta wave reflects fusion between the accessory pathway and the normal QRS as conducted via the AV node.

#3 Complete Heart Block

What is it?

Complete heart block (also known as ‘Third Degree’ heart block) occurs when an impulse isn’t conducted from the atria to the ventricles, usually due to AV node pathology. This means that whilst the atrial rate is determined by the SA node, the ventricular rate is a ventricular escape rhythm –which is much slower than the rate of the SA node. This means that the ventricles and atria, therefore, contract completely independent of one another. In AVN block a narrow QRS is seen, whereas, in an infranodal block, a wide QRS is seen. The former is more stable as the pacemaker site is more proximal (Bundle of His) so asystole is less likely.

Why does it happen?

Heart block can occur for a variety of reasons in children, but often it is congenital- secondary to either structural disease (i.e congenitally corrected transposition of the great arteries) or maternal antibodies, as seen in neonatal lupus. Congenital heart block associated with underlying structural heart disease has a poorer prognosis.

So what do we see on ECG?

There are regular P waves and regular QRS complexes, but these are completely unrelated to one another.

But what about the other blocks?

#4 Myocarditis

What is it?

As suggested in the name, myocarditis is inflammation of the myocardium. This can occur due to infection (viruses, bacteria, spirochetes, fungi, and other organisms) having a direct toxic effect on the myocardium.  It is important to consider the diagnosis in children who have recently suffered a systemic illness (esp. Coxsackie). Certain drugs may also be responsible (anthracycline chemotherapy and alcohol).  Myocarditis may occur alongside pericarditis. The inflamed myocardium is unable to contract and conduct as well as usual resulting in poor function of the heart.

So what do we see on ECG?

Usually, myocarditis presents with sinus tachycardia and non-specific T-wave and ST-segment changes (e.g. T wave inversion). We may also see:

  • QRS/QT prolongation
  • Low voltage QRS (<5mm in precordial leads)
  • Pathological Q waves
  • Ventricular arrhythmias (can be ectopics or VT)
  • AV block 

#5 Dilated Cardiomyopathy (DCM)

What is it?

Dilated Cardiomyopathy (DCM) is characterized by weak and floppy myocardium.  It may be inherited or develop as a result of myocarditis secondary to infection or drugs.

So what do we see on ECG?

Cardiomyopathies show similar features to myocarditis.  Pathologically it’s a spectrum, the inflammation in myocarditis is the ‘active phase’ leading to muscle damage present in cardiomyopathy. Changes may include:

  • QRS/QT prolongation
  • Low voltage QRS (<5mm in precordial leads)
  • T wave / ST segment changes
  • Pathological Q waves
  • Ventricular arrhythmias (can be ectopics or VT)
  • AV block 

#6 Hypertrophic Cardiomyopathy (HOCM)

What is it?

HOCM is a genetic condition that affects the sarcomeres in the heart causing left ventricular hypertrophy (LVH), which cannot be explained by other causes. It is very important as it’s the most common cause of sudden cardiac death in those <35, and those who have HOCM may require an internal cardiac defibrillator.

So how do we figure out if there is HOCM?

There are many criteria that can be used to describe HOCM, however no one criteria has been determined to be the most reliable (especially in children).

So what do we see on ECG?

Whereas in DCM there are small QRS complexes, in HOCM they are large due to the hypertrophied muscle.  As above with myocarditis and dilated cardiomyopathy: T wave inversion and ST changes indicate unhealthy myocardium. Pathological Q waves may also be seen.

#7 Long QT

What is it?

As the name suggests, this is when the QT interval is prolonged. In order to determine if the QT is prolonged then we need to determine the QTc using Bazetts formula.

Source: litfl.com/bazett-formula/
  • In boys, a prolonged QTc is >450ms
  • In girls a prolonged QTc is >460ms.

The most important tool in trying to determine the cause of a prolonged QT interval is history! Certain features make a congenital cause of a prolonged QT interval much more likely:

  • Syncope (+/- stress)
  • Congenital deafness (suggests LQT5)
  • FHx of sudden cardiac death <30 yr in immediate family
  • FHx of Long QT syndrome
  • Certain medications

What causes it?

There are many causes of a prolonged QT interval, including:

Acquired prolonged QT: This is when a child has a prolonged QT interval secondary to an underlying cause such as:

  • Drugs: antibiotics, antidepressants, antipsychotics, antihistamines, antiarrythmics, antifungals
  • Electrolyte disturbances
  • Hypothermia

Congenital long QT syndrome: This is an inherited channelopathy, where the child has a prolonged QT interval present either at baseline or unmasked by a stimulus.

There are 17 different forms of long QT syndrome (and counting!), which each have a different genetic mutation.

Three main types of Long QT syndrome (LQTS):

NameGeneTriggersFrequencyT waves
LQT1KCNQ1Peak exercise40%Early onset broad based.
LQT2KCNH2Sudden loud noises, swimming, emotion, stress30%Low voltage double bumped ‘bifid’ T wave with notching
LQT3SCN5ARest, sleep10%Late onset T wave  

ECG changes may be seen at rest or the child may need to go through exercise tolerance testing or an adrenaline challenge in order to unmask the prolonged QT interval.

So what do we see on ECG?

Nice one, Sherlock, you guessed it!  The main feature is a prolonged QTc interval.      

Why do we care so much about a prolonged QT interval?

Those who have a prolonged QT interval are at a higher risk of developing VT or Torsades de Pointes and therefore sudden cardiac death. This means it is important to identify these children so that they can receive medical therapy or an ICD if deemed necessary.

In paediatric cardiology, Schwartz criteria is used to determine the likelihood of Long QT syndrome. Whilst we do not reach an ultimate diagnosis in the ED, it is useful to note the risky features.

#8 Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT)


What is it?

As the name suggests, it is a polymorphic (i.e. of multiple morphologies) ventricular tachycardia that is stimulated by catecholamines such as adrenaline. It is an inherited condition, which affects ion channels causing altered calcium flux leading to delayed after-depolarisations causing VT. Whilst baseline ECG may be normal, states in which there is a high adrenaline surge can unmask the CPVT (i.e. heavy exercise). If detected, these children need to be sent for further investigation for medical management or potentially an ICD.

So what do we see on ECG?

A polymorphic ventricular tachycardia. There are two types:

  • Normal baseline ECG!
  • Typical polymorphic / bidirectional VT – where both QRS complexes and T wave change in axis.

Differential for bidirectional VT = CPVT, LQTS Type 7 and Digoxin toxicity.

Do not shock CPVT! This may induce ‘electrical storm’ perpetuate the catecholamine release.  Seek advice from your local Paediatric Cardiologist.

#9 Brugada Syndrome

What is it?

This is an inherited channelopathy which affects impulse conduction, causing ventricular tachyarrhythmias and potentially sudden cardiac death. It is more common in males and commonly seen in carbohydrates consuming nations e.g. rice in South East Asia and pasta in the Western population. Interestingly, the same gene that causes LQT3 (see above) causes Brugada (SCN5A), however in the former there is a gain in function, whereas in Brugada there is a loss of function.

#10 Anomalous Left Coronary Artery arising from the Pulmonary Artery (ALCAPA)

What is it?

This is when the left coronary artery is connected to the pulmonary artery instead of the aorta. This means that instead of receiving oxygenated blood, the left side of the myocardium will receive deoxygenated blood. This can lead to myocardial ischaemia, which is initially transient occurring only in periods of increased myocardial demand (feeding, crying). However, as oxygen demand increases, infarction of the anterolateral left ventricular wall can occur.

So what ECG changes do we see?

  • Pathological Q waves (esp. in leads I, aVL and V6) – 50% of kids with Q waves in aVL have ALCAPA!
  • Ischaemic / T wave changes in inferolateral leads (II, III, aVF, V5-6).  Note on ECG below T waves in V5 and V6 are flattened

Top Tips for Paediatric Cardiology

Cite this article as:
Ana Waddington. Top Tips for Paediatric Cardiology, Don't Forget the Bubbles, 2020. Available at:
https://doi.org/10.31440/DFTB.28999

Here is our next fabulous little treasure trove of tips on Paediatric Cardiology from Helen Ormrod and Anna Mcquorquodale…

  1. In SVT – use a 3 way tap because the adenosine half life is so small that even if you use a bio-connector, the medication will get lost and you don’t want to be giving more adenosine than you need to.
  2. When performing Pre and Post ductal sats, use the right hand and right leg – as a rule of thumb, looking for a gradient of more than 5 (95% vs 90%). We do this because we are worried about any cardiac lesion that is affecting systemic circulation such as cortication of the aorta
  3. Four limb BP is only useful within the first 3-4 months of life because of the conditions that we are looking for. The gradient has to be 20 or more. Don’t worry if there is a tiny discrepancy.
  4. When following up a patient with a known congenital heart disease, try and find out through their letter or consultant where they are with their surgery. If they now have an anatomically normal heart, we don’t need to be as concerned about their cardiac disease. For example, a PDA ligation 3 years ago.
  5. It’s important to be aware that CHD kids, especially post repair, are more likely to have arrhythmias (even if they have reached a stage where their heart is structurally normal). Arrhythmias that can be quite benign in general kids can have a significantly more detrimental effect on those with CHD (even if repaired).
  6. Fluids are part of a delicate balance. These patients need to remain hydrated in order to help their cardiac function but for the same reason they should not be overloaded. Strict input and output balances are required.
    • If the patient has a cardiac shunt (BT Shunt etc) and are dehydrated, this is a life threatening emergency and can become a cardiac arrest very quickly. Move to resus!
  7. If a child has an uncorrected TOFF and have come in with pyrexia or discomfort need to be managed in HDU bay as they can become sick very quickly. Apply cardiac monitoring and saturation.
  8. If a cardiac baby is septic, there is no reason not to give treatment as they need it to help their cardiac function.

What are some of your top tips? Feel free to share them in the comments below!

For your convenience, the top tips are summarised in an A4 poster format (infographic design by Kat Priddis @kls_kat & Grace Leo @gracie_leo):

Paediatric Murmurs: Ari Horton at DFTB18

Cite this article as:
Team DFTB. Paediatric Murmurs: Ari Horton at DFTB18, Don't Forget the Bubbles, 2019. Available at:
https://doi.org/10.31440/DFTB.18861

Ari Horton is many things – an advocate for kindness, a Cordon Bleu trained pastry chef and, just very occasionally, a paediatric cardiologist. Andrew Tagg remembers the day Ari found his calling. Working as a paediatric ED resident in Melbourne’s inner west he came to present a patient. He could barely sit still and his grin threatened to infect the fishbowl as he announced, “I found a murmur!”

We may not all be as acoustically gifted as Ari but that thing we wield around our necks is not just for listening for wheezes or for distracting toddlers.

At 3:30am in emergency overnight,
You got a seriously worrisome fright.
That harsh sound whooshing through the chest,
Try hide your concern, you say “It’s for the best”.
Is it innocent or the beginning of the end,
Go back to the basics they’re your best friend.

Horton’s distraught, his heart is fraught.
Stress fills his tired mind, luckily he left his steth behind.
Numbers and statistics running through his head,
But he stood still watching the child from the end of his bed.
By 9am poor old Horton, more dead than alive,
Had picked, searched and listened to more than 9005

Examination is a dynamic process they say,
Watching the kid run this and that way.
See them feed, sleep, run, jump and cry,
Do some special tests before you say goodbye.
A person’s a person no matter how small

It’s the real story that captures us all.
A murmur’s just a murmur no matter how loud
I’ve learnt my lessons and I’m so proud.
This child is healthy and safe because we cared
Cardiac fellowship awaits because I dared.

Horton Hears A What? Ari Horton (2018)

 

 

 

Here is a little sketchnote by @gracie_leo of the talk:

 

This talk was recorded live at DFTB18 in Melbourne, Australia. With the theme of ‘Science and Story‘ we pushed our speakers to step out of their comfort zones and consider why we do what we do. Caring for children is not just about acquiring the scientific knowhow but also about taking a look beyond a diagnosis or clinical conundrum at the patient and their families.

DFTB19 has just a handful of main conference tickets left but there are still spots for some of the pre-conference workshops.

 

If you want our podcasts delivered straight to your listening device then subscribe to our iTunes feed or check out the RSS feed. If you are more a fan of the visual medium then subscribe to our YouTube channel. Please embrace the spirit of FOAMed and spread the word.

 

iTunes Button

 

Daily JA, Bolin E, Eble BK. Teaching pediatric cardiology with meaning and sense. Congenital heart disease. 2018 Jan;13(1):154-6.
Haney I, Ipp M, Feldman W, McCrindle BW. Accuracy of clinical assessment of heart murmurs by office based (general practice) paediatricians. Archives of disease in childhood. 1999 Nov 1;81(5):409-12.
Keren R, Tereschuk M, Luan X. Evaluation of a novel method for grading heart murmur intensity. Archives of pediatrics & adolescent medicine. 2005 Apr 1;159(4):329-34.
Lefort B, Cheyssac E, Soulé N, Poinsot J, Vaillant MC, Nassimi A, Chantepie A. Auscultation While Standing: A Basic and Reliable Method to Rule Out a Pathologic Heart Murmur in Children. The Annals of Family Medicine. 2017 Nov 1;15(6):523-8.
Mahnke CB, Nowalk A, Hofkosh D, Zuberbuhler JR, Law YM. Comparison of two educational interventions on pediatric resident auscultation skills. Pediatrics. 2004 May 1;113(5):1331-5.
Noponen AL, Lukkarinen S, Angerla A, Sepponen R. Phono-spectrographic analysis of heart murmur in children. BMC pediatrics. 2007 Dec;7(1):23.

[/toggle

Josh Francis: Paediatrics in East Timor at DFTB17

Cite this article as:
Team DFTB. Josh Francis: Paediatrics in East Timor at DFTB17, Don't Forget the Bubbles, 2018. Available at:
https://doi.org/10.31440/DFTB.16238

And so we come to the final talk of the final day of DFTB17 in Brisbane.

You can check out any of our other conference talks on our YouTube channel.

Kawasaki’s Disease

Cite this article as:
Alyssa Courtney. Kawasaki’s Disease, Don't Forget the Bubbles, 2017. Available at:
https://doi.org/10.31440/DFTB.12282
A four-year-old Japanese boy was brought into Emergency with 5 days of fevers, non-exudative bilaterally injected sclerae, erythematous pharynx and irritability.

Wondering about the possibility of Kawasaki Disease, I turned to check the 2017 update of the American Heart Association Scientific Statement, focusing on considering a diagnosis of Incomplete Kawasaki Disease.

 

Bottom line:

  • Unchanged diagnostic criteria of complete Kawasaki Disease (KD)
  • Refined algorithm for evaluation of suspected incomplete KD (15-20% of cases)
  • Recommended ECHO at diagnosis, and repeated at 1-2 weeks and 4-6 weeks after treatment
  • Unchanged acute management– Intravenous immunoglobulin (IVIG) single dose 2g/kg over 10-12 hours. Ideally prior to day 10. Some countries continue to use high dose aspirin for varying durations.
  • Additional therapeutic options are outlined for the 10-20% with persistent or recurrent fever
  • New model of KD vasculopathy

 

What is Kawasaki Disease?

An acute, self-limited febrile illness of unknown cause, predominantly in children <5 years. It is the most common cause of acquired heart disease in developed countries. Without pathognomonic tests, we need to detect it clinically!

 

Epidemiology

  • Most common in Japan (where it was first described) with an annual incidence of 264.8 per 100 000 children in 2012. The estimated incidence in North America is 25 cases per 100 000 children <5 years of age per year. Australia has one of the lowest reported rates (3.7 per 100 000 <5 years of age), equivalent to 50–60 cases Australia-wide per year. It is likely that the current Australian incidence is higher.
  • Highest relative risk is in Asian children, especially of Japanese ancestry
  • The ratio of males to females is 1.5:1
  • Predominantly affects children 6 months to 4 years
  • Predisposing factors have been reported inconsistently
  • In Japan, the recurrence rate is 3%, and the relative risk in siblings is ten-fold higher

 

What is the aetiology?

We have no idea why…. BUT the resultant systemic inflammation leads to associated clinical findings: liver (hepatitis), lung (interstitial pneumonitis), gastrointestinal tract (abdominal pain, vomiting, diarrhoea, gallbladder hydrops), meninges (aseptic meningitis, irritability), heart (myocarditis, pericarditis, valvulitis), urinary tract (pyuria), pancreas (pancreatitis), and lymph nodes (lymphadenopathy).

A new model of Kawasaki disease vasculopathy involves three processes impacting muscular arteries. The first is a necrotising arteritis, followed by subacute/chronic vasculitis. The final process is luminal myofibroblastic proliferation.

 

How do we diagnose it in Australia?

Diagnostic Criteria

Fever for 5 days or more (typically high spiking (>39°C to 40°C) and remittent)

Plus 4/5 of:

  • polymorphous rash (usually within 5 days of fever onset)
  • bilateral (non-purulent) conjunctival injection (usually begins shortly after fever onset and often spares the limbus, an avascular zone around the iris)
  • mucous membrane changes e.g. reddened or dry cracked lips, strawberry tongue, diffuse redness of oral or pharyngeal mucosa (oral ulcers and pharyngeal exudates are not consistent with KD)
  • peripheral changes, e.g. erythema of the palms or soles, oedema of the hands or feet, and in convalescence desquamation
  • cervical lymphadenopathy (> 15 mm diameter, usually unilateral, single, non-purulent and painful in the anterior cervical chain)

AND exclusion of diseases with a similar presentation

  • Staphylococcal infection (e.g. scalded skin syndrome, toxic shock syndrome)
  • Streptococcal infection (e.g. scarlet fever, toxic shock-like syndrome not just isolation from throat)
  • Measles
  • Viral exanthems
  • Steven’s Johnson syndrome
  • Drug reactions
  • Juvenile rheumatoid arthritis.

Trickily, these children may have a concurrent viral infection, often adenovirus. Adenovirus is more likely with exudative pharyngitis and conjunctivitis and positive PCR assay. Kawasaki disease is more likely with erythema/swelling of hands and feet, a strawberry tongue, and a desquamating groin rash.

Inflammation and crusting of a recent Bacille-Calmette-Guérin (BCG) injection site may occur.

Consider an alternative diagnosis to Kawasaki Disease if there is exudative conjunctivitis, exudative pharyngitis, ulcerative intraoral lesions, bullous or vesicular rash, generalized adenopathy, or splenomegaly.

 

What is Incomplete Kawasaki Disease?

Scarily, this is so easily missed. They make up 15-20% of all cases!!
Patients with incomplete KD, particularly those <6 months of age and older children, may experience significant delays in diagnosis and these children are at high risk of developing coronary artery abnormalities.

Consider KD if:

  • Infants <6 months old with prolonged fever and irritability
  • Infants with prolonged fever and unexplained aseptic meningitis
  • Infants or children with prolonged fever and unexplained or culture-negative shock
  • Infants or children with prolonged fever and cervical lymphadenitis unresponsive to antibiotic therapy
  • Infants or children with prolonged fever and retropharyngeal oroparapharyngeal phlegmon unresponsive to antibiotic therapy

Evaluation of suspected incomplete Kawasaki disease (via McCrindle BW et al. 2017)

What can we investigate?

As suggested by RCH Melbourne guidelines, all patients should have

  • ASOT / Anti DNAase B
  • Echocardiography (at least twice: at initial presentation and, if negative, again at 6 – 8 weeks).
  • Platelet count (marked thrombocytosis common in the second week of illness)
  • Consider Mycoplasma

In addition, findings can provide support when considering Incomplete Kawasaki Disease – refer to the above algorithm.

Evolution of laboratory findings via Tremoulet et al.

  • KD is unlikely if ESR, CRP, and platelet count are normal after day 7 of illness.
  • Low WBC and lymphocyte predominance suggests an alternative diagnosis
  • Leukocytosis is typical in the acute stage, with granulocyte predominance
  • Normocytic, normochromic anaemia is common during inflammation
  • CRP and ESR elevation is nearly universal, CRP normalizes more quickly with inflammation resolution. ESR is elevated by IVIG therapy.
  • Minimally elevated ESR in the setting of severe clinical disease should prompt investigation for disseminated intravascular coagulation.
  • Thrombocytosis is a characteristic feature that generally doesn’t occur until the second week, peaking in the third week, normalizing by 4 to 6 weeks
  • Thrombocytopenia can be a sign of disseminated intravascular coagulation and is a risk factor for the development of coronary artery abnormalities
  • Mild to moderate elevations in serum transaminases or gammaglutamyl transpeptidase occur in 40% to 60% of patients, and mild hyperbilirubinemia occurs in ≈10%.
  • Hypoalbuminaemia is common and associated with more severe and more prolonged acute disease
  • Urinalysis may show pyuria in up to 80% of children, non-specific for KD
  • In children who undergo lumbar puncture, ≈30% demonstrate pleocytosis with a mononuclear cell predominance, normal glucose levels, and generally normal protein levels

 

Pitfalls

Fever and pyuria in an infant or young child may be diagnosed as a urinary tract infection, with subsequent development of rash, red eyes, and red lips attributed to an antibiotic reaction. Irritability and a culture-negative pleocytosis of the cerebrospinal fluid in an infant with prolonged fever suggestive of aseptic meningitis (or if antibiotics have been given, partially treated meningitis) may cause a diagnosis of KD to be overlooked. Cervical lymphadenitis as the primary clinical manifestation can be misdiagnosed as having bacterial adenitis. Gastrointestinal symptoms are considered for surgical causes, other physical findings of KD can be overlooked.

 

What is the treatment?

We’re aiming to prevent important coronary artery abnormalities. Timely (as soon as possible, ideally within 10 days) IVIG treatment reduces the incidence of coronary artery aneurysms (defined from absolute luminal dimensions) from 25% to 4%. Studies with additional therapies to IVIG have not substantially reduced this residual risk of 4%. Adverse effects are rare but include Coomb’s positive haemolytic anaemia and aseptic meningitis. The measles, mumps, and varicella vaccine should be deferred for 11 months unless at high risk (seek advice, may need repeat vaccination). If the diagnosis is delayed, IVIG should still be given (after the tenth day of illness) IF there is presence of fever, or continued elevation of ESR or CRP>3, indicating ongoing inflammation. Aspirin is used with the theoretical rationale of reducing coronary artery aneurysms (although there is no well-established evidence for this). In Australia, a dose of 3-5mg/kg daily from diagnosis until cardiology review at 6 weeks is routine. The newly released statement advises the administration of moderate to high-dose (80–100 mg/kg/day) aspirin is reasonable until the patient is afebrile. Patients should receive a seasonal influenza vaccination.

Fever usually resolves within 36 hours after IVIG infusion has been completed; if not, the patient is considered to have resistance to IVIG. 10-20% of patients will not respond to the single IVIG treatment dose. There is minimal data to support therapeutic agents for the child with IVIG resistance. Repeating the IVIG dose, 3 days of high-dose pulsed steroids, or 2-3 weeks of tapering prednisolone are all options. There are lower levels of evidence for infliximab and cyclosporine.

 

Coronary artery abnormalities

An angiographic study of 1100 patients showed coronary artery lesions in 24%, with aneurysms in 8% and a number of patients with stenoses and occlusions. Valvular regurgitation is usually mild to moderate in severity and resolves prior to follow-up. MR can occur after the acute stage from myocardial ischaemia. Patients after KD have been shown to have functional and anatomic abnormalities of the aorta with unknown long-term implications. Myocarditis is common during the acute illness but complete resolution is expected. Risk stratification for long-term management is based primarily on maximal coronary artery luminal dimensions, normalized as Z scores, and is calibrated to both past and current involvement. Patients with aneurysms require life-long and uninterrupted cardiology follow-up.

 

What is the prognosis?

  1. The case fatality rate is <0.1% in Japan, virtually all from cardiac sequelae.
  2. Peak mortality occurs 15 to 45 days after onset of fever, during which time well-established coronary artery vasculitis occurs concomitantly with marked elevation of the platelet count and a hypercoagulable state
  3. Coronary artery aneurysms from KD account for 5% of acute coronary syndromes (ACS) in adults <40 years of age

 

In Summary:

  • Be aware of the diagnostic criteria of complete Kawasaki Disease (KD)
  • Highest relative risk in Asian children, especially Japanese ancestry
  • Always consider incomplete KD (15-20% of cases) and refer to the algorithm if concerns, there are pitfalls!
  • Liaise with cardiology regarding an ECHO
  • Unchanged acute management– Intravenous immunoglobulin (IVIG) single dose 2g/kg over 10-12 hours. Ideally prior to day 10. Some countries continue to use high dose aspirin for varying durations.
  • Additional therapeutic options are outlined for the 10-20% with persistent or recurrent fever, minimal evidence for these
  • New model of KD vasculopathy but we are still in the dark regarding aetiology
  • Coronary artery aneurysms from KD account for approximately 5% of acute coronary syndromes (ACS) in adults <40 years of age

 

 Selected references

McCrindle BW, Rowley AH, Newburger JW, et al. Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals From the American Heart Association. Circulation2017;Mar 29

Yim D, Curtis N, Cheung M, Burgner D. Update on Kawasaki disease: Epidemiology, aetiology and pathogenesis. Journal of Paediatrics and Child Health 49 (2013) 704–708

Clinical Practice Guidelines, Royal Children’s Hospital, Melbourne

Kim DS, Kawasaki Disease. Yonsei Medical Journal. 47 (2006) (6): 759–72. PMC 2687814 PMID 17191303. doi:10.3349/ymj.2006.47.6.759

Yim D, Curtis N, Cheung M, Burgner D. An update on Kawasaki disease II: Clinical features, diagnosis, treatment and outcomes. Journal of Paediatrics and Child Health 49 (2013) 614–623

Tremoulet AH, Jain S, Chandrasekar D, Sun X, Sato Y, Burns JC. Evolution of laboratory values in patients with Kawasaki disease. Pediatr Infect Dis J. 2011;30:1022–1026

Dengler LD, Capparelli EV, Bastian JF, Bradley DJ, Glode MP, Santa S, Newburger JW, Baker AL, Matsubara T, Burns JC. Cerebrospinal fluid profile in patients with acute Kawasaki disease. Pediatr Infect Dis J. 1998;17:478–481

 

DFTB go to Berlin – #SMACCmini

Cite this article as:
Tagg, A. DFTB go to Berlin – #SMACCmini, Don't Forget the Bubbles, 2017. Available at:
https://dontforgetthebubbles.com/dftb-go-to-berlin-smaccmini/

Having flown 16,893 kilometres to visit family, a short hop over the Berlin was nothing. This year Tessa and I were honoured to be able to help out with SMACCmini – the paediatric workshop before the main event.  DasSMACC is the second-most* anticipated conference of the year and we wanted to make sure the delegates left better able to look after critically unwell children.

Transition from Fetal Physiology

Cite this article as:
Andrew Tagg. Transition from Fetal Physiology, Don't Forget the Bubbles, 2016. Available at:
https://doi.org/10.31440/DFTB.10360

The imminent arrival of another Tagglet (not to be confused with aglet*) has prompted me to go back to my textbooks and refresh my knowledge of what to expect.  One of the problems of being a medical parent is being expected to know the answers to the most random of medical questions that are thrown out there. Is it supposed to look like that? Why are her hands that colour? And the question that is really being asked is, “Are they normal?” So here begins a series of posts on what is “normal” in neonates.

PAC Conference – Pflaumer on Sudden Death in the young

Cite this article as:
Goldstein, H. PAC Conference – Pflaumer on Sudden Death in the young, Don't Forget the Bubbles, 2016. Available at:
https://dontforgetthebubbles.com/pac-conference-pflaumer-on-sudden-death-in-the-young/

We have teamed up with APLS to share the videos from their Paediatric Acute Care Conferences. These videos have never been open access before, so if you weren’t able to attend the conferences, then now’s your chance to catch up.

The PAC Conference is run each year by APLS and consists of presentations on a range of topics relevant to paediatric acute and critical care.