Transcript tet spell - Calgary Emergency Medicine

PEDIATRIC CARDIOLOGY
in the ED
Naminder Sandhu
& Roger Galbraith
October 27, 2011
OBJECTIVES
• Approach to the infant/neonate presenting with
• Shock
• CHF
• Cyanosis
• Approach to children presenting with
• Syncope
• Palpitations
• Chest pain
• Approach to the child with known CHD
• Tips for looking at CXRs and ECGs
Pediatric ECGs
• Why do we do them?
– Chest pain, suspected dysrhythmia, seizure,
syncope, drug exposure, electrical burns, abN
lytes
Normal Values by Age
RV dominance


What are normal T waves?
• Upright up to first 3 days
– If persists  sign of RVH
• Flat or inverted until pre-adolescence/adolescence
Back to basics: fetal circulation
And now for some cases...
Group 1:
Alyssa
Jen
Erik
Geoff
Group 2:
Kristen
Joe
Kip
Kasia
Sean
Group 4:
Ping
Ashlea
Michael
Adam
Group 5:
Russ
Meira
Marshall
Jeff
Group 3:
Chris
Jason
Puja
Jasmin
CASE 1
• 3 week old with dyspnea, poor feeding
• Brought to ED by mother for rapid breathing
and poor breast feeding, worsening over past
few days
• Previously well with unremarkable prenatal
history
• Becoming more lethargic (not interested in
feeding)
CASE 1
• Afebrile, HR 180, RR 80, BP 85/50, sats 88%
• What is your broad ddx for neonatal shock?
Ddx cardiogenic shock
• Myocardial dysfunction
– Myocarditis/pericarditis
– Sepsis
• Arrhythmias
– SVT
• Obstructed/impaired forward flow
– AS, Ao Coarct, HLH
• Umm… why is the infant presenting now??
When does the duct close??
• Functional closure within several hours;
anatomic closure up to 2 – 3 weeks
• Term infants:
– 50% by 24hrs
– 90% by 48hrs
• by 4 days in nearly all healthy infants prems
What are ductal dependent lesions?
• Left sided obstructive lesions = shocky
– AS, Ao Coarct, Hypoplastic left heart
• Right sided obstructive lesions = cyanotic
– Tetralogy of Fallot, pulm stenosis, TGA
Crashing Neonate
• Think ductal dependent
lesion!
• Most common lesion to
present this way =
Aortic Coarct
CASE ECG
Management of neonatal shock
• ABCs
– Feel for femorals and check BP in four limbs!!!
•
•
•
•
•
Oxygen
IV access* and fluids, investigations and ?abx
PGE1 infusion
Call cardiology/arrange transport
Also consider Sepsis, Metabolic, NAT, etc.
Alprostadil
• Indications:
– Maintenance PDA
– Severe PPHTN
• Infusion via peripheral or central line
• Dilute to concentration of 10mcg/mL (in
dextrose or NS)
• Infuse starting at 0.05mcg/kg/min – max
0.2mcg/kg/min
• Effect within minutes
Side effects?
– Apnea
– Fever
– Flushing
– Hypotension
– Seizures
CASE 2
• 3 month old girl with cyanosis this AM
• She has had this happen before but often will resolve
on own
• Today after feeding, seemed to be straining, noticed
lips were blue
• Newly immigrated from Malaysia, no previous medical
problems
• Exam: Sats 76%, RR 40, HR 166
– Irritable ++
– Deep resps with indrawing
– Difficult resp/cardiac exam
Oxygen to the
rescue!....
Cyanotic CHD
• Cyanotic heart lesions…. Do I
really need to know them???
•
•
•
•
•
•
•
ToF
10% CHD
TGA
5%
Tricuspid atresia
1-2%
TAPVR
1%
Truncus arteriosus
<1%
Pulm atresia/stenosis <1%
Ebstein’s anomaly
<1%
Age of CHD presentation by lesion
First Week
• TGA
• TAPVR
• HLH
• Tricuspid atresia
First Month (ductal dependent
lesions)
•
•
•
•
1-6 months (often lead to CHF)
• VSD
• PDA
• Coronary anomalies
• TOF
• AV canal defect
Later
Coarctation
• ASD
Severe AS
• PAPVR
Severe PS
TOF with pulmonary atresia • Valve stenosis
Is CXR useful?
• Pulmonary vascularity
• Shape/size of heart
Increased Vascularity
←
Decreased
Vascularity
→
Cardiac silhouette
Snowman = TAPVR
Egg on a string = TGA
Boot shaped = ToF
So what’s going on with this girl?
TET SPELL
Tetralogy of Fallot
• Most common
cyanotic CHD
beyond infancy
• Sx:
– Dyspnea on
exertion
– Cyanosis
– Clubbing
– Hypoxic spells
– squatting
CASE ECG
Tet spell
• Episode of severe hypoxia due to  R to L
shunting and  pulmonary blood flow
• Characteristics:
– Paroxysm of hyperpnea
– Irritability and prolonged crying
– Increasing cyanosis
– Decreased intensity murmur
• Increased R → L shunting and decreased pulmonary
blood flow b/c of:
–
–
–
–
Hyperpnea  increase in venous return to R heart
decrease in SVR (lower L heart pressures)
increase in PVR (higher R heart pressures)
increase in RV outflow tract obstruction
 R-L shunt
VICIOUS
CYCLE
 pO2
 pCO2
 pH
 Systemic
venous return
Hyperpnea
•
•
•
•
Seizures
Syncope
Stroke
DEATH
Management
• Try to calm infantavoid pokes if you
can
• Knee-chest position
• Give oxygen
 R-L shunt
 pO2
 pCO2
 pH
 Systemic
venous return
Hyperpnea
Then what??
•
•
•
•
•
IV access & fluids
Sedate with morphine
Bicarbonate
Phenylephrine
Propranolol 0.2mg/kg over 5 min
• ICU/cardiology
CASE 3
• 2 month old presenting in December with cough,
congestion, dyspnea, poor feeding x 4 days
• Hx
– Difficulty feeding, decreased u/o, lethargic
– Sick contacts: older brother with cold
• P/E
– Afebrile, HR 160, RR70, BP 80/50, sats 90%
– Nurses describes infant with nasal congestion, moderate
indrawing
• Sounds like bronchiolitis….but how do you know it’s
not CHF??
Presentation: what’s the diff?
• Poor feeding, failure to thrive, tachypnea,
diaphoresis, wheeze
• Two bigs: Big heart, big liver
• Two fasts: Fast RR, Fast HR
Ddx CHF
• Cardiac
– Structural
1) Overcirculation (L-R shunts): VSD, large PDA, AV canal
defect
2) Forward flow obstruction: Ao stenosis, CoA, HLH
3) Myocardial dysfunction: anomalous coronary (Eg
ALCAPA), myocarditis, cardiomyopathies
– Electrical
• Extracardiac: AVMs
Causes of CHF by age
< 2 weeks
> 2 weeks
Child
Shunts
Obstructive lesions
Coronary artery anomalies
Cardiomyopathies
Myocarditis
Arrhythmias
AVMs
Adolescent
L – R shunts
• VSD = 25% of all CHD
• Gradually increasing L → R shunt with drop in
PVR
– also present when tipped into failure by illness
(e.g. bronchiolitis)
CASE ECG
CXR
So what’s the plan?
•
•
•
•
•
Oxygen
IV access
Elevate head of bed
Admit
Supportive therapy for bronchiolitis (that’s a
whole other talk….)
• Diuretics
CASE 4
• 4 year old complaining that her heart feels funny
– Chest pain? Palpitations?
• Started this morning and ?comes and goes; URTI
symptoms x 2 days
• Feels “dizzy” sometimes, no syncope
• PmHx: known to have an ASD, followed every 1 –
2 years by Cardiology
• On exam: HR 128, BP 80/50, RR 30, Sats 95%,
afebrile
– Nurse says no acute distress
CASE 4
• Child has nasal congestion
• Normal heart sounds, no rubs, no extra
sounds, 2/6 SEM at LUSB
• Clear lung fields
• You get an ECG… what worrisome findings are
you looking for?
CASE ECG
What’s going on?
• Cardiac
– Arrhythmia
– Myocarditis/pericarditis
– Ischemia, HOCM, etc etc etc
• Non-cardiac
• Then again kid says he feels funny…. You get
another ECG
CASE ECG
Paediatric arrhythmias in the emergency department
(Clausen et al 2011)
SVT
• In infants:
– 50% = idiopathic
– 25% congenital heart disease
– 10-20% WPW syndrome
• HR >220 infants, >180 children
• Complications:
– Syncope
– Shock
– CHF
How do you treat stable SVT?
• Stable
– Vagal maneuvers: What can you try?
•
•
•
•
•
Blow through plugged straw
Ice on face (don’t cover mouth) – no more than 15 – 30 sec!
Bear down, try to puff up stomach
Stand upside down
Orbital pressure = NO!
– Adenosine
• Unstable
– SVT with poor perfusion… PALS
Approach syncope…. Similar to adults
• Benign
– Orthostatic, vasovagal, breath-holding
• Pathologic
– Neuro: Seizures
– Cardiac (<5%)
•
•
•
•
•
Myocardial: HOCM, myocarditis
Aortic disease: Aortic stenosis, aneurysm, dissection
Conduction: LQTS, heart block, WPW, ARVD
Coronary artery disease: anomalous origin, aneurysms
Other: pulm HTN, electrolyte abN
SCD in athletes <35 yo
•
•
•
•
•
•
•
•
HCM – 36%
Anomalous origin of a coronary artery – 17%
Myocarditis – 6%
Arrhythmogenic right ventricular dysplasia – 4%
MVP – 4%
Aortic stenosis – 3%
Coronary atherosclerosis – 3%
Other – 12%
Cardiac warning signs
•
•
•
•
•
History of known heart disease
Sudden fainting without prodrome
Syncope during exercise, emotional stimulus
Incontinence during syncope
Family history of sudden death (including
single person MVC, drowning)
• Family/personal history of deafness
Long QTS
• What is prolonged QT interval in kids?
– Up to 490ms in neonate  440 ms adolescents
• Find a LQT?
– Extended electrolytes, drug history
– Refer to cardiology urgently
– B blockers
– ICDs
A shout out to acquired cardiac
disease
•
•
•
•
•
Myocarditis
Pericarditis
Endocarditis
Kawasaki disease
Rheumatic heart disease
Myocarditis… same as in adults?
• Spectrum of presentation
– Beware non-cardiac presentations eg. Pneumonia,
wheeze not responsive to bronchodilators
• Most common cause dilated CM in kids
• Usually infectious, presents in the winter
following viral prodrome
CASE 5
• A 4 month old infant presents with increasing cyanosis
• Had low grade fever, cough, rhinorrhea and poor
feeding for preceding 4 days
• Today developed increased WOB and progressively
worsening cyanosis
• Mom says has a history of tricuspid atresia and a “BT
shunt”
• T 38.3, HR 170, RR 70, BP 80/50, sats 75%, infant is
crying, cyanotic, grunting and has retractions
• Other than change your pants, what should you do???
What could be going on?
• 50% of patients with CHD that presented to
the ED required admission
• 10% of those admitted died
• Patients often have complex physiology and
are at risk of decompensation for a number of
reasons.
• So, yes, you should sweat ...
• A little
• Most common presentations of CHD patients
to the ED are for:
– Respiratory tract infection
– Dehydration
– CHF
– Arrhythmia
– Tet spell
– Endocarditis
Huh? A what shunt now??
• Used primarily as palliation in defects with
single ventricle pathology (ie. HLH, tricuspid
atresia)
– Try to bypass part of heart to offset workload on
single overworked/impaired ventricle (usu RV)
Systemic arterial  PA shunt: Blalock
Taussig
• Why? Increase
pulmonary blood flow
• Subcl art  Pulm art
Systemic venous  PA shunt: Glenn
• Why? Increase
pulmonary blood flow
• SVC Pulm art
• *Dependent on passive
flow to the lungs*
• Beware
intubation/ventilation
Fontan procedure
• Why? Divert blood
from RA to PA (bypass
single functioning RV)
• IVC Pulm art
• *Dependent on
passive flow to the
lungs*
Risks of shunts
• Clotting/ obstruction
• Reduced flow (from
hypovolemia/dehydration)
• Coronary steal
• Over circulation in the pulmonary system
– Beware too much oxygen
Bottom line
• If not sure, ask the parents what normal sats
should be
• You probably wont have enough time or
information to figure out exactly what’s causing
their problem- an extensive inpatient work-up is
often required
• Low threshold for admission, possibly ICU
• Low threshold for cardiology consult
• Don’t be stingy with fluids and oxygen, they often
need one or both, just monitor their response
closely
Take home points
• Shock
– Do a good cardiac exam to r/o cardiogenic shock
– If concerned about duct dependent lesion, think PGE1!
• Cyanosis
– Try oxygen first… of course
– You don’t need to figure out exact CHD – that’s what echo is for
– If concerned about duct dependent lesion, think PGE1!
• CHF:
– Symptoms may be subtle – exam is important
• JVPs don’t exist in our world – check the liver instead
– Always consider on ddx for common presentations eg dyspnea
– Different causes based on age (eg. L – R shunts)
Take home points
• Palpitations/chest pain/ syncope
– Approach similar to adults except chest pain/syncope
rarely cardiac
• Acquired heart disease
– Most common is myocarditis – keep your eye out for it
– Prevent it by looking our for Kawasaki disease
• Known CHD
– If all else fails, ask the family and consult cardiology;
low threshold for admission
– Be kind with your fluids and careful with oxygen
Thanks!
• Dr. Roger Galbraith
• Dr. Joyce Harder
• Drs. Mark Bromley and Jay Green
References
• Emergency Medicine Reports, Jan 2005: 10(1)
• Park MK. The Pediatric Cardiology Handbook
3rd Ed. (2003). Philadelphia: Mosby.
• Sharieff GQ et al. The Pediatric ECG. Emerg
Med Clin N Am. 2006; 24: 195-208.
• Durani Y et al. Myocarditis and pericarditis in
children. Pediatr Clin N Am 57. 2010; 1281–
1303.