Pediatric Cardiac Pediatric Cardiac EmergenciesEmergencies

Graham ThompsonGraham Thompson

Francois BelangerFrancois Belanger

Feb 26Feb 26thth 2004 2004

ObjectivesObjectives Review transitional changes in pediatric Review transitional changes in pediatric

cardiologycardiology CHD – presentations in ED, blue vs pink, ED TxCHD – presentations in ED, blue vs pink, ED Tx Post-op issues presenting to the EDPost-op issues presenting to the ED PacersPacers SVT in the PEDSVT in the PED Acquired Cardiac DiseaseAcquired Cardiac Disease

– PericarditisPericarditis– KDKD– ARFARF

MIs in the pediatric worldMIs in the pediatric world Cardiac ArrestCardiac Arrest PALS Guidelines (attached)PALS Guidelines (attached)

Neonatal CirculationNeonatal Circulation

Changes at BirthChanges at Birth Decreased PVR from expansion of lung and high Decreased PVR from expansion of lung and high

PaOPaO22

Increased SVR from stopping placental Increased SVR from stopping placental circulationcirculation

Functional closure of PFO from high LA pressuresFunctional closure of PFO from high LA pressures Reversal of flow and then closure of PDAReversal of flow and then closure of PDA Closure of ductus venosusClosure of ductus venosus Slow change from R dominant to L dominant Slow change from R dominant to L dominant

circulation circulation (Malformations often cause changes to this (Malformations often cause changes to this

pattern (can be life saving i.e. PDA in CoA!!)pattern (can be life saving i.e. PDA in CoA!!)

Normal Vitals for ChildrenNormal Vitals for Children

Case #1Case #1

4 do boy with central cyanosis4 do boy with central cyanosis Term baby of 17 yo girl, minimal Term baby of 17 yo girl, minimal

prenatal careprenatal care ROM 6 hrs, uncomplicated deliveryROM 6 hrs, uncomplicated delivery Left hospital w/in 24 hrsLeft hospital w/in 24 hrs Bottle feeding well until today, now Bottle feeding well until today, now

only 1 oz/feedonly 1 oz/feed WOB increased over the dayWOB increased over the day

Case (cont)Case (cont) Brought into resusc Brought into resusc

roomroom Hr 165 RR 75 BP Hr 165 RR 75 BP

65/28 LA 65/28 LA SpOSpO22 79% R/A, 79% R/A,

cyanosedcyanosed 100% supplied 100% supplied

– SpOSpO22 – 84% – 84% IV started, NS bolus 70 IV started, NS bolus 70

cccc

A/E good, clear, mild-A/E good, clear, mild-moderate WOBmoderate WOB

S1 S2 (? Single) IV/VI S1 S2 (? Single) IV/VI systolic murmursystolic murmur

Pulses good, liver 2cm Pulses good, liver 2cm BCMBCM

Font flat, soft, neuro Font flat, soft, neuro intactintact

Bld wk, ECG and CXR Bld wk, ECG and CXR pendingpending

Congenital Heart DiseaseCongenital Heart Disease

0.5-0.8% of live births (not including PDA 0.5-0.8% of live births (not including PDA or bicuspid aorta)or bicuspid aorta)

Increased in syndromes Increased in syndromes – T21 – endocardial cushion defects, ASD, VSDT21 – endocardial cushion defects, ASD, VSD– XO – CoA, bicuspid AVXO – CoA, bicuspid AV

Increased with maternal prenatal drugsIncreased with maternal prenatal drugs– Lithium – Ebstein anomalyLithium – Ebstein anomaly– VPA – CoA, HLHS, VSD, ASVPA – CoA, HLHS, VSD, AS

40-50% dx in 140-50% dx in 1stst wk, 50-60% by 1 wk, 50-60% by 1stst month month

Frequency of major CHDFrequency of major CHD (% of total) (% of total)

VSD VSD 25–3025–30 ASD (sec) ASD (sec) 6–86–8 PDA PDA 6–86–8 CoACoA 5–75–7 TOFTOF 5–75–7 PSPS 5–75–7 AS AS 4–74–7 d-TGAd-TGA 3–53–5 HLHS HLHS 1–31–3

HRHS HRHS 1–31–3 Truncus Truncus 1–21–2 TAPVR TAPVR 1–21–2 Tricuspid atresia Tricuspid atresia 1–21–2 Single ventricle Single ventricle 1–2 1–2 DORV DORV 1–21–2 Others Others 5–105–10

* Excluding PDA in * Excluding PDA in preterm neonates, preterm neonates, bicuspid AV, physiologic bicuspid AV, physiologic PPS, and MVPPPS, and MVP

PresentationsPresentations Most complex CHD are picked up on Most complex CHD are picked up on

routine prenatal ultrasound (up to 75%)routine prenatal ultrasound (up to 75%) Complex CHD is often picked up prior to Complex CHD is often picked up prior to

D/C from the nursery with cyanosis or D/C from the nursery with cyanosis or abnormal heart sounds abnormal heart sounds

BUT…….Timing and symptoms relate to BUT…….Timing and symptoms relate to physiologic changes of newborn and the physiologic changes of newborn and the lesion (obstruction vs shunt) so they may lesion (obstruction vs shunt) so they may show up in the ED!!!show up in the ED!!!

Any newborn looking comfortably cyanotic Any newborn looking comfortably cyanotic is probably cardiac!! – mixing lesionis probably cardiac!! – mixing lesion

0-2 wks0-2 wks

Duct dependant lesionsDuct dependant lesions Takes 2-14 days for duct closureTakes 2-14 days for duct closure May present to the ED more often, because May present to the ED more often, because

of early D/C (duct hasn’t closed yet!)of early D/C (duct hasn’t closed yet!) Most common are Left outflow tract Most common are Left outflow tract

obstructions (CoA, interrupted arch, AS, obstructions (CoA, interrupted arch, AS, HLHS), TGA, TOFHLHS), TGA, TOF

In the words of Dr. PattonIn the words of Dr. Patton– ““Beware of the “septic”-like 2 wk old infant with Beware of the “septic”-like 2 wk old infant with

poor pulses: is it possibly CoA or critical AS?”poor pulses: is it possibly CoA or critical AS?”

In 1In 1stst year year CHD that presents in failure (tachypnea, sweating CHD that presents in failure (tachypnea, sweating

with feeds, feeds >30 min, FTT, pallor)with feeds, feeds >30 min, FTT, pallor) Most commonly related to L-to-R shunt (ie VSD)Most commonly related to L-to-R shunt (ie VSD) At birth, R pressures are high, shunt is minimal so At birth, R pressures are high, shunt is minimal so

defect may not present itselfdefect may not present itself Drop in R side pressures and increased L pressures Drop in R side pressures and increased L pressures

occurs over 6-8 wks causing shunt and symptomsoccurs over 6-8 wks causing shunt and symptoms Murmurs are not heard in nursery because lack of Murmurs are not heard in nursery because lack of

shuntshunt Late TOF presentations that are relatively protected Late TOF presentations that are relatively protected

from failure by RVOT obstruction. May present with from failure by RVOT obstruction. May present with tet spells.tet spells.

ChildhoodChildhood

Restrictive L-to-R shuntsRestrictive L-to-R shunts– ASD, Small VSDASD, Small VSD

Mild CoA, mild AS – may present with Mild CoA, mild AS – may present with fatigue or leg pain/wkness or HTNfatigue or leg pain/wkness or HTN

The very rare TOFThe very rare TOF

Presentation of CHDPresentation of CHD

Approach to CHD DxApproach to CHD Dx

CyanoticCyanotic– Increased Pulmonary flow Increased Pulmonary flow – Decreased pulmonary flow (RV outflow Decreased pulmonary flow (RV outflow

obstruction)obstruction) AcyanoticAcyanotic

– Increased volume load (ASD, VDS, PDA)Increased volume load (ASD, VDS, PDA)– Increased pressure load (LV outflow Increased pressure load (LV outflow

obstruction)obstruction)

Approach to CHDApproach to CHD

Hx in CHDHx in CHD

Cyanotic episodesCyanotic episodes– Central vs peripheralCentral vs peripheral

Feeding patternFeeding pattern– diaphoresisdiaphoresis– Stopping frequentlyStopping frequently– Prolonged (>30 min)Prolonged (>30 min)– FTTFTT

Gestational ageGestational age Maternal Hx of Maternal Hx of

medications, medications, infectionsinfections

Prenatal U/SPrenatal U/S Genetic D/OGenetic D/O Previous child with Previous child with

CHDCHD Exercise Exercise

intoleranceintolerance SyncopeSyncope R/O sepsis stuff!!!R/O sepsis stuff!!!

Px in CHDPx in CHD VSVS

– TachypneaTachypnea– ArrhythmiasArrhythmias– 4 limb BPs (SBP in 4 limb BPs (SBP in

legs N > by 10, if not legs N > by 10, if not look for LVOT look for LVOT obstruction)obstruction)

– 4 limb SpO24 limb SpO2– TT00C – hypermetabolicC – hypermetabolic

ColourColour DiaphoresisDiaphoresis Wt gainWt gain

Heart sounds and Heart sounds and murmurs (don’t murmurs (don’t forget to listen to forget to listen to the back – CoA)the back – CoA)

Distal pulses, R/F Distal pulses, R/F delaydelay

PrecordiumPrecordium Abdomen – HSMAbdomen – HSM ClubbingClubbing

Cyanotic Heart DiseaseCyanotic Heart Disease

Central vs PeripheralCentral vs Peripheral CentralCentral

– 50 g/l of non-oxygenated Hgb50 g/l of non-oxygenated Hgb– Level of hypoxia varies according to hct, Level of hypoxia varies according to hct,

so polycythemic kids can look cyanotic so polycythemic kids can look cyanotic at higher SpO2sat higher SpO2s

PeripheralPeripheral– Perfusion issuesPerfusion issues

Hyperoxic testHyperoxic test

TechnicallyTechnically– Do gasDo gas– Give 100% O2Give 100% O2– After 10 min, redo gasAfter 10 min, redo gas– Mixing cardiac lesion won’t be able to Mixing cardiac lesion won’t be able to

get PaO2 > 100-150get PaO2 > 100-150 PracticallyPractically

– SpO2 won’t get into 90% in mixing SpO2 won’t get into 90% in mixing cardiac lesioncardiac lesion

Cyanotic Heart DiseaseCyanotic Heart Disease

The 5 T’sThe 5 T’s– Tetralogy Of Fallot (TOF)Tetralogy Of Fallot (TOF)– Truncus ArteriosusTruncus Arteriosus– Tricuspid Lesions (atresia, Ebstein’s)Tricuspid Lesions (atresia, Ebstein’s)– Transposition of the Great Arteries (TGA)Transposition of the Great Arteries (TGA)– Total Anomalous Pulmonary Venous Total Anomalous Pulmonary Venous

Return (TAPVR)Return (TAPVR) + PA/severe PS+ PA/severe PS

TOFTOF

Tetralogy Tetralogy – RV outflow RV outflow

obstruction obstruction – Overriding aortaOverriding aorta– VSD (usually non VSD (usually non

restrictive)restrictive)– RVHRVH

TOF clinicalTOF clinical

5 - 10% of CHD5 - 10% of CHD Most common cyanotic CHD after infancyMost common cyanotic CHD after infancy Cyanotic or acyanotic depending on degree Cyanotic or acyanotic depending on degree

of RVOT obstructionof RVOT obstruction– Blue if ++ obstruction = R-L shunt (present Blue if ++ obstruction = R-L shunt (present

earlier)earlier)– Pink if minimal obstruction = L-R shuntPink if minimal obstruction = L-R shunt

RVH (felt under xyphoid)RVH (felt under xyphoid) Loud, probably single S2Loud, probably single S2 III-V/VI SEM at LSBIII-V/VI SEM at LSB

TOF - DxTOF - Dx

Boot shaped heartBoot shaped heart Decreased pulm. Decreased pulm.

Vasc.Vasc. 20% R sided arch20% R sided arch RVHRVH

““Tet” SpellsTet” Spells Most frequent in 1Most frequent in 1stst 2 yrs (peak 2 yrs (peak

at 2-4 mo)at 2-4 mo) After startle, crying or upon After startle, crying or upon

wakingwaking Hyperpnea, restless, increasing Hyperpnea, restless, increasing

cyanosis, syncopecyanosis, syncope Sudden decrease in pulmonary Sudden decrease in pulmonary

flow, increased R-L shuntflow, increased R-L shunt Prolonged spells can lead to Prolonged spells can lead to

LOC, acidosis, szsLOC, acidosis, szs Tx Tx

– Calm childCalm child– Knee to chest or Squat positionKnee to chest or Squat position– O2O2– Morphine 0.1-0.2mg/kg SCMorphine 0.1-0.2mg/kg SC– HCO3 if prolongedHCO3 if prolonged– ketamineketamine– Phenylephrine to increase Phenylephrine to increase

systemic pressuressystemic pressures

TGATGA

d-TGA is 2 systems d-TGA is 2 systems in parallelin parallel

l-TGA is l-TGA is physiologically physiologically corrected (lung-LA-corrected (lung-LA-RV-aorta-body-RA-RV-aorta-body-RA-LV-lung)LV-lung)

TAPVRTAPVR

A – supracardiacA – supracardiac B – cardiacB – cardiac C – cardiacC – cardiac D - infracardiacD - infracardiac

TruncusTruncus

Tricuspid atresiaTricuspid atresia

No RA outflowNo RA outflow Requires ASD (natural or balloon) to Requires ASD (natural or balloon) to

have R-L shunt. have R-L shunt. Requires PDA or VSD to allow L-R Requires PDA or VSD to allow L-R

shuntshunt RV usually really small, so Fontan RV usually really small, so Fontan

works wellworks well Usually Dx at birth, but may be laterUsually Dx at birth, but may be later

Ebstein’s AnomalyEbstein’s Anomaly

Downward Downward displacement of an displacement of an abnormal TVabnormal TV

Atrialization of RVAtrialization of RV

Evaluation of Cyanotic Evaluation of Cyanotic InfantInfant

ABCABC IV accessIV access Physical examPhysical exam Labs, hyperoxic testLabs, hyperoxic test CXRCXR Consider medsConsider meds Call cardioCall cardio

PGE1PGE1 Relaxation of ductal smooth muscleRelaxation of ductal smooth muscle Infusion of 0.05-0.2 ug/kg/minInfusion of 0.05-0.2 ug/kg/min

– 80 cc NS in 500ug vial run at BW = 80 cc NS in 500ug vial run at BW = 0.1ug/kg/min (or 36cc in 225ug vial)0.1ug/kg/min (or 36cc in 225ug vial)

Side effectsSide effects– JitterinessJitteriness– FeverFever– HypotensionHypotension– ApneaApnea

Relative contraindicationRelative contraindication– TAPVR – may worsen systemic perfusionTAPVR – may worsen systemic perfusion

CXR in CHDCXR in CHD

Egg on a string – TGAEgg on a string – TGA Snowman – TAPVRSnowman – TAPVR Boot – TOFBoot – TOF Super huge heart – Ebstein’sSuper huge heart – Ebstein’s Backward E – CoA – notched ribs by Backward E – CoA – notched ribs by

collateralscollaterals

Acyanotic Heart DiseaseAcyanotic Heart Disease

RVOT obstruction presents with RVOT obstruction presents with shockshock

CoACoA VSDVSD ASDASD

Surgical ProceduresSurgical Procedures

Post-op issues in the EDPost-op issues in the ED

ShuntsShunts– Can obstruct, especially passive flow (glenn)Can obstruct, especially passive flow (glenn)

Think of this child presents with URTI or gastro Think of this child presents with URTI or gastro causing dehydrationcausing dehydration

BT shunts are BT shunts are activeactive flow, and when obstructing flow, and when obstructing have increased cyanosis and changed shunt have increased cyanosis and changed shunt murmurmurmur

Glenn shunts are Glenn shunts are passivepassive flow and present with flow and present with SVC syndrome and decreased murmurSVC syndrome and decreased murmur

– Can flood lungsCan flood lungs If R-L shunt is too high, then may present similar If R-L shunt is too high, then may present similar

to failure – tachypnea, crackles, wet chestto failure – tachypnea, crackles, wet chest

Post-op issues in the EDPost-op issues in the ED

MI – especially in atrial or arterial MI – especially in atrial or arterial switches for TGAswitches for TGA

ArrhythmiasArrhythmias– AV block for TGA, VSDs, ASDsAV block for TGA, VSDs, ASDs– WPW in Ebstein’s anomalyWPW in Ebstein’s anomaly– Atrial flutterAtrial flutter

Post-op issues in the EDPost-op issues in the ED Post pericardotomy syndromePost pericardotomy syndrome

– Sustained febrile period 1 wk – 2 months Sustained febrile period 1 wk – 2 months (mean 3-4 wks) after sx(mean 3-4 wks) after sx

– Thought to be related to autoimmune responseThought to be related to autoimmune response– Pericarditis symptoms, CXR and ECGPericarditis symptoms, CXR and ECG– Tamponade may occurTamponade may occur– Usually self limiting in 2-3 wksUsually self limiting in 2-3 wks– TxTx

Bed restBed rest NSAIDsNSAIDs SteroidsSteroids Pericardiocentesis if needed.Pericardiocentesis if needed.

CHD presentations to EDCHD presentations to ED 5 yr retrospective study at UCLA ED5 yr retrospective study at UCLA ED Only 8 new CHD presentationsOnly 8 new CHD presentations

– Age ranged from 1 wk to 5 monthsAge ranged from 1 wk to 5 months– ASD – 3ASD – 3– VSD – 1VSD – 1– AS – 1AS – 1– ALCAPA – 1ALCAPA – 1– CoA – 1CoA – 1– TAPVR – 1TAPVR – 1

– Savitsky E Savitsky E J.Emerg MedJ.Emerg Med 2003, 24(3):239 2003, 24(3):239

CHD in the EDCHD in the ED

Really great references on CHDReally great references on CHD– Woods WA et al Woods WA et al Emerg Med ReportsEmerg Med Reports

2003 24(6) – CHD in the ED2003 24(6) – CHD in the ED– Woolridge DP et al Woolridge DP et al Ped Emerg Med Ped Emerg Med

ReportsReports 2002 7(7) – CHD in the PED Pt 1 2002 7(7) – CHD in the PED Pt 1– Woolridge DP et al Woolridge DP et al Ped Emerg Med Ped Emerg Med

ReportsReports 2002 7(8) – CHD in the PED pt 2 2002 7(8) – CHD in the PED pt 2

CaseCase

3 mo boy to ED with grey spells3 mo boy to ED with grey spells Has happened 3 times in past 2 daysHas happened 3 times in past 2 days Hasn’t taken more than 1 oz for past 3 Hasn’t taken more than 1 oz for past 3

feedsfeeds Intermittent WOB, no URTI sympt.Intermittent WOB, no URTI sympt. Had been seen last month for Had been seen last month for

decreased feeding, difficulty breathing, decreased feeding, difficulty breathing, stuffy nose and pallor– dx – stuffy nose and pallor– dx – bronchiolitisbronchiolitis

Case (cont)Case (cont)

Looked dusky at triageLooked dusky at triage Taken directly to resusc. roomTaken directly to resusc. room OO22 and monitors, IV started and monitors, IV started

immediatelyimmediately Pinked up with OPinked up with O22, SpO, SpO22 = 99% = 99%

ECGECG

SVTSVT

Post AdenosinePost Adenosine

Child had echo showing Ebstein’s Anomaly

Non-pharmacologic Tx for Non-pharmacologic Tx for SVTSVT

Interrupts about 25% of SVTInterrupts about 25% of SVT Cooperative childrenCooperative children

– Blow against strawBlow against straw– Crouch downCrouch down

Uncooperative or Small Children and Uncooperative or Small Children and InfantsInfants– Push legs into chestPush legs into chest– Carotid sinus massageCarotid sinus massage– Ocular pressureOcular pressure– Diving reflexDiving reflex

Carotid massage for SVTCarotid massage for SVT

Randomized X-over trial of carotid Randomized X-over trial of carotid massage vs valsalva maneuvermassage vs valsalva maneuver

148 episodes148 episodes No difference in efficacyNo difference in efficacy Total of 25% responded to one of the Total of 25% responded to one of the

therapiestherapies

Carotid massage is not recommended Carotid massage is not recommended in young patients in young patients (but no reason (but no reason given!)given!)

Lim SH et al Ann Emerg Med 1998 31(1):30

Paed Drugs 2000 2(3):171

SVT and the Diving ReflexSVT and the Diving Reflex increased parasympathetic tone to the heart increased parasympathetic tone to the heart

causing bradycardia in association with systemic causing bradycardia in association with systemic vasoconstrictionvasoconstriction– ice cube application to the lip and bottom noseice cube application to the lip and bottom nose– immersion of the head into a basin of ice and water for 4-6 immersion of the head into a basin of ice and water for 4-6

ss– application of a cold, wet facial cloth to the faceapplication of a cold, wet facial cloth to the face– application of an ice bag to the faceapplication of an ice bag to the face

““The diving reflex is much more effective than the The diving reflex is much more effective than the ‘usual’ vagal maneuvers such carotid sinus ‘usual’ vagal maneuvers such carotid sinus massage, gag reflex or rectal stimulationmassage, gag reflex or rectal stimulation” ”

avoid inducing apnea and aspiration; and avoid avoid inducing apnea and aspiration; and avoid prolonged application of ‘cold’ to the face.prolonged application of ‘cold’ to the face.

Moak JP Prog Ped Card 2000 11(1):25

The Diving Reflex and SVTThe Diving Reflex and SVT The use of the diving reflex to terminate a The use of the diving reflex to terminate a

case of paroxysmal supraventricular case of paroxysmal supraventricular tachycardia (PST) is described in a 2-week-tachycardia (PST) is described in a 2-week-old infant who presented in severe old infant who presented in severe congestive heart failure with congestive heart failure with supraventricular tachycardia at a rate of supraventricular tachycardia at a rate of 300. The infant's face was placed in a basin 300. The infant's face was placed in a basin of ice water at 5 degrees C. for 5 seconds of ice water at 5 degrees C. for 5 seconds with manual occlusion of the infant's with manual occlusion of the infant's nostrils to prevent aspirationnostrils to prevent aspiration. The PST . The PST converted to a sinus rhythm of 120 within 3 converted to a sinus rhythm of 120 within 3 seconds of facial immersion. seconds of facial immersion.

Hamilton J Am Heart J 1979 97(3):371

Ice for SVTIce for SVT Has been reported to abort up to 90% of SVT in infantsHas been reported to abort up to 90% of SVT in infants Supposedly initiates the “diving reflex”Supposedly initiates the “diving reflex” 11stst report 1980 report 1980

– Filled plastic bag with equal volume of water and crushed iceFilled plastic bag with equal volume of water and crushed ice– Covered entire face “to preauricular area”Covered entire face “to preauricular area”– Conversion occurred within 15 seconds (n=10)Conversion occurred within 15 seconds (n=10)– ““We can only speculate on the emotional trauma of the ice We can only speculate on the emotional trauma of the ice

bag technique”bag technique” Do you have to cover the whole face? Do you have to Do you have to cover the whole face? Do you have to

cause apnea? – still controversial, no good datacause apnea? – still controversial, no good data Probably shouldn’t cover mouth and noseProbably shouldn’t cover mouth and nose

Complications?Complications?

Craig JE et al J Pediatr 1998 133:727

Medications for SVTMedications for SVT AdenosineAdenosine

– Transient Av node blockadeTransient Av node blockade– 0.1 mg/kg to max of 6 mg0.1 mg/kg to max of 6 mg– Repeat at 0.2 mg/kg to max of 12 mgRepeat at 0.2 mg/kg to max of 12 mg– Don’t get your knickers in a knot when the monitor Don’t get your knickers in a knot when the monitor

shows a flat lineshows a flat line– Needs proximal IV with rapid NaCl bolus ‘cause ½ life is Needs proximal IV with rapid NaCl bolus ‘cause ½ life is

a mere 15 secondsa mere 15 seconds VerapamilVerapamil

– CONTRAINDICATED in children < 1yo because of several CONTRAINDICATED in children < 1yo because of several studies demonstrated increased risk of hypotension, studies demonstrated increased risk of hypotension, malignant arrhythmias and electomechanical malignant arrhythmias and electomechanical dissociationdissociation

– 0.1 mg/kg max dose 5mg over 15 minutes0.1 mg/kg max dose 5mg over 15 minutes

Atkins, DL Clin Ped Emerg Med 20012(2):107

I I

Chamber(Chamber(s) Paceds) Paced

IIII

Chamber(sChamber(s) Sensed) Sensed

IIIIII

Response Response to Sensingto Sensing

IV IV ProgrammabilitProgrammabilit

y, Rate y, Rate ModulationModulation

V V AntiarrhythAntiarrhyth

mia Functionmia Function

O, NoneO, None O, NoneO, None O, NoneO, None O, NoneO, None O, NoneO, None

A, AtriumA, Atrium A, AtriumA, Atrium T, TriggeredT, Triggered P, Simple P, Simple programmableprogrammable

P, Pacing P, Pacing (antitachyarrh(antitachyarrhythmia)ythmia)

V, VentricleV, Ventricle V, VentricleV, Ventricle I, InhibitedI, Inhibited M, Multi-M, Multi-programmableprogrammable

S, shockS, shock

D, Dual D, Dual

(A + V)(A + V)D, Dual D, Dual

(A + V)(A + V)D, Dual D, Dual

(T + I)(T + I)C, C, CommunicatingCommunicating

R, Rate R, Rate ModulatingModulating

D, Dual (P + S)D, Dual (P + S)

North American Society of Pacing and North American Society of Pacing and Electrophysiology Generic Pacemaker Electrophysiology Generic Pacemaker

CodeCode

Different Types of PacingDifferent Types of Pacing

DDDR PacingDDDR Pacing

Pacemaker ComplicationsPacemaker Complications

Most common include Most common include – infection at siteinfection at site– Electromagnetic interferenceElectromagnetic interference

Cell phones – unlikelyCell phones – unlikely Antitheft devices – unlikelyAntitheft devices – unlikely MRI, cautery etc.MRI, cautery etc.

CaseCase

14 yo girl gradual onset of chest pain 14 yo girl gradual onset of chest pain x 2dx 2d

Now acutely SOB, increased painNow acutely SOB, increased pain Worse when lying downWorse when lying down Recent viral URTI symptomsRecent viral URTI symptoms Previously wellPreviously well

PericarditisPericarditis

PericarditisPericarditis

Normal pericardial fluid volume = 10-15ccNormal pericardial fluid volume = 10-15cc Inflammatory response causes increased Inflammatory response causes increased

fluid accumulation (can be > 1L)fluid accumulation (can be > 1L) Symptoms – precordial pain improved with Symptoms – precordial pain improved with

sitting, cough, dyspnea, vomiting, feversitting, cough, dyspnea, vomiting, fever Px – friction rub (variable), muffled heart Px – friction rub (variable), muffled heart

sounds, tachy, venous distention, pulsus sounds, tachy, venous distention, pulsus paradoxusparadoxus

Etiology of PericarditisEtiology of Pericarditis CONGENITAL ANOMALIES CONGENITAL ANOMALIES

– Absence (partial, complete)Absence (partial, complete)– CystsCysts– Mulibrey nanism (Mulibrey nanism (mumuscle, scle, liliver, ver,

brbrain, ain, eyeye) with congenital e) with congenital pericardial thickening and pericardial thickening and constriction constriction

INFECTIOUS INFECTIOUS – ViralViral (coxsackievirus B, EBV, (coxsackievirus B, EBV,

influenza, adenovirus)influenza, adenovirus)– Bacterial (Bacterial (Streptococcus, Streptococcus,

Pneumococcus, Staphylococcus, Pneumococcus, Staphylococcus, Meningococcus, MycoplasmaMeningococcus, Mycoplasma, , tularemia)tularemia)

– Immune complex (Immune complex (Meningococcus, Meningococcus, Haemophilus influenzaeHaemophilus influenzae))

– TuberculosisTuberculosis– Fungal (histoplasmosis, Fungal (histoplasmosis,

actinomycosis)actinomycosis)– Parasitic (toxoplasmosis,Parasitic (toxoplasmosis,

echinococcosis)echinococcosis)

CONNECTIVE TISSUE DISEASESCONNECTIVE TISSUE DISEASES

– Rheumatoid arthritisRheumatoid arthritis– Rheumatic feverRheumatic fever– Systemic lupus erythematosusSystemic lupus erythematosus– Systemic sclerosisSystemic sclerosis– SarcoidosisSarcoidosis

METABOLIC-ENDOCRINE METABOLIC-ENDOCRINE – UremiaUremia– HypothyroidismHypothyroidism– ChylopericardiumChylopericardium

HEMATOLOGY-ONCOLOGY HEMATOLOGY-ONCOLOGY – Bleeding diathesisBleeding diathesis– Malignancy (primary, metastatic)Malignancy (primary, metastatic)– Radiotherapy-inducedRadiotherapy-induced

OTHER OTHER – Trauma Trauma – Iatrogenic (catheter related)Iatrogenic (catheter related)– PostpericardiotomyPostpericardiotomy– Aortic dissectionAortic dissection– IdiopathicIdiopathic– Familial Mediterranean feverFamilial Mediterranean fever

Pericarditis - TxPericarditis - Tx

To tap or not to tap? To tap or not to tap? – No good dataNo good data– In typical viral pericarditis, probably don’t have toIn typical viral pericarditis, probably don’t have to– If any doubt of bacterial causes, just do itIf any doubt of bacterial causes, just do it– Other reasons to tapOther reasons to tap

Significant symptomsSignificant symptoms Possible malignancyPossible malignancy Abnormal diastolic fnctn on echoAbnormal diastolic fnctn on echo

NSAIDs, steroids. Again, no good evidence.NSAIDs, steroids. Again, no good evidence.

Wise words from Dr. Patton

ARFARF

Major CriteriaMajor Criteria– CarditisCarditis– PolyarthritisPolyarthritis– ChoreaChorea– Erythema Erythema

MarginatumMarginatum– Subcutaneous Subcutaneous

NodulesNodules

Minor CriteriaMinor Criteria– ClinicalClinical

FeverFever ArthralgiaArthralgia

– InvestigationsInvestigations Increased ESR or Increased ESR or

CRPCRP Prolonged PR Prolonged PR

intervalinterval

Plus Evidence of GAS infection (swab, ASOT, antiDNase B)Plus Evidence of GAS infection (swab, ASOT, antiDNase B)

Dx = 2 major criteria, 1 major + 2 minorDx = 2 major criteria, 1 major + 2 minor

CaseCase

4 mo boy transferred to pediatrics for 4 mo boy transferred to pediatrics for significant HSM and fever for 7 dayssignificant HSM and fever for 7 days

Very fussy, not tolerating feedsVery fussy, not tolerating feeds Erythematous blanching rash on day 3 Erythematous blanching rash on day 3

now gonenow gone WBC - 50’s consistently, no blasts seen WBC - 50’s consistently, no blasts seen

hgb 99 -105 plts low 400’shgb 99 -105 plts low 400’s Cultures negativeCultures negative Mild resp distress, likely b/c HSM (CXR Mild resp distress, likely b/c HSM (CXR

N)N)

Case (cont)Case (cont)

Repeat SWU negativeRepeat SWU negative U/S abdo – significant HSMU/S abdo – significant HSM hem/onc investigations startedhem/onc investigations started

At 11 days – plt – 1340, rpt at 1360At 11 days – plt – 1340, rpt at 1360

Echo – 3 giant coronary aneurismsEcho – 3 giant coronary aneurisms

Dx of KDDx of KD

Supporting Evidence for KDSupporting Evidence for KD

Sterile pyuria – 70%Sterile pyuria – 70% Arthritis – 40%Arthritis – 40% GI symptoms (hydrops of GB and diarrhea) GI symptoms (hydrops of GB and diarrhea)

– 25%– 25% Aseptic meningitisAseptic meningitis Anterior uveitisAnterior uveitis IrritabilityIrritability Elevated ESRElevated ESR Thrombocytosis at 1 wk from start of Thrombocytosis at 1 wk from start of

symptomssymptoms

Incomplete KDIncomplete KD

At least 10% of children will CAAs At least 10% of children will CAAs never meet diagnostic criterianever meet diagnostic criteria

Review of 127 pts Tx for KD Review of 127 pts Tx for KD – 36% did not meet criteria, but this grp 36% did not meet criteria, but this grp

had higher incidence of CAAs than those had higher incidence of CAAs than those who did meet criteriawho did meet criteria

Infants < 1 y have up to 45% Infants < 1 y have up to 45% incomplete KDincomplete KD

Up to 30% of atypical KD < 1yo had Up to 30% of atypical KD < 1yo had CAAsCAAs

Incomplete KD in InfantsIncomplete KD in Infants

Review of KD Dx in Review of KD Dx in children < 6 mochildren < 6 mo

Of 33 cases Of 33 cases reported, 28% had reported, 28% had atypical disease atypical disease compared to 7-compared to 7-10% in > 1yo.10% in > 1yo.

%%

feverfever 9797

rashrash 8282

conjunctivitconjunctivitisis

6161

ExtremitiesExtremities 6161

MMMM 5757

nodesnodes 3030Genizi et al Clin Pediatr 2003 42:263

KD-Initial TherapyKD-Initial Therapy

Lang et al Best Pract Res Clin Rheum 2002 16(3):427

IVIG in KDIVIG in KD

Dosing schemesDosing schemes– 400 mg/kg x 4 days400 mg/kg x 4 days– 1 g/kg x 1 1 g/kg x 1 – 2 g/kg x12 g/kg x1

Highest reduction of CAAs and Highest reduction of CAAs and quickest relief of fever in 2g/kg dosequickest relief of fever in 2g/kg dose

May be repeated fever not May be repeated fever not responsive in 24-48 hrsresponsive in 24-48 hrs

Tx and Timing of Tx and Timing of PresentationPresentation

Children Tx with IVIG within 10 days Children Tx with IVIG within 10 days of symptoms have reduced rates of of symptoms have reduced rates of giant CAAs (<2%) compared with giant CAAs (<2%) compared with those Tx at 15 days (6.1%)those Tx at 15 days (6.1%)

Benefit after 10 days has not been Benefit after 10 days has not been adequately studied. adequately studied.

Current suggestion include IVIG if still Current suggestion include IVIG if still evidence of active inflammation (ie evidence of active inflammation (ie fever)fever)

CaseCase

Risk factors for MI in KidsRisk factors for MI in Kids

MI Etiology in NeonatesMI Etiology in Neonates Congenital heart disease    Congenital heart disease    

– Anomalies of the origin of the coronary arteries   Anomalies of the origin of the coronary arteries   – Anomalies producing left ventricular hypertrophy    Anomalies producing left ventricular hypertrophy    – Pulmonary atresia with intact ventricular septum   Pulmonary atresia with intact ventricular septum   – Transposition of the great arteries  Transposition of the great arteries  – Truncus arteriosus   Truncus arteriosus   

Endocardial fibroelastosis    Endocardial fibroelastosis     Mediocalcinosis of the coronary arteriesMediocalcinosis of the coronary arteries Poor coronary perfusion Poor coronary perfusion Hypoxia    Hypoxia     Sepsis Sepsis Disseminated intravascular coagulation     Disseminated intravascular coagulation      EmbolismEmbolism

MI Etiology in ChildrenMI Etiology in Children Kawasaki diseaseKawasaki disease Idiopathic or inherited Idiopathic or inherited

cardiomyopathy  cardiomyopathy   Myocarditis  (Viral, Myocarditis  (Viral,

Rheumatic)Rheumatic) Collagen vascular diseaseCollagen vascular disease Substance abuseSubstance abuse

– CocaineCocaine– Glue sniffing Glue sniffing

Chest trauma Chest trauma Iatrogenic    Iatrogenic     Post-cardiac transplant    Post-cardiac transplant     Post bypass hypoperfusion Post bypass hypoperfusion Other complications of Other complications of

congenital heart disease congenital heart disease surgery, especially damage surgery, especially damage to coronary arteries.  to coronary arteries.  

Genetic/metabolic disorders    Genetic/metabolic disorders    – Homocysteinuria  Homocysteinuria  – Familial hypercholesterolemiaFamilial hypercholesterolemia– Progeria Progeria – Pseudoxanthoma elasticum  Pseudoxanthoma elasticum  – Mucopolysaccharidoses, Fabry's, Mucopolysaccharidoses, Fabry's,

  Alkaptonuria, Hurler's   Alkaptonuria, Hurler's syndrome, Pompe's Disease     syndrome, Pompe's Disease     

– Hyperbetalipoproteinemia, Hyperbetalipoproteinemia, familial combined familial combined hyperlipidemia, and hyperlipidemia, and hypoalphalipoprotenemia   hypoalphalipoprotenemia   

Other systemic disorders   Other systemic disorders   – Nephrotic syndrome     Nephrotic syndrome     – Sepsis     Sepsis     – Occult malignancy Occult malignancy – Idiopathic or premature coronary Idiopathic or premature coronary

artery disease without artery disease without predisposing genetic diseasepredisposing genetic disease

Symptoms of MIs in KidsSymptoms of MIs in Kids

– NauseaNausea– VomitingVomiting– IrritabilityIrritability– Pallor Pallor – DiaphoresisDiaphoresis

– Poor feedsPoor feeds– SyncopeSyncope– WeaknessWeakness– WOB/SOBWOB/SOB– AnxietyAnxiety– PainPain

Pediatric MIs on ECGPediatric MIs on ECG Wide Q waves (>35 milliseconds)   Wide Q waves (>35 milliseconds)   

– Particularly I, aVL, V5, V6, but any lead other Particularly I, aVL, V5, V6, but any lead other than aVR than aVR

ST segment changes of >2 mmST segment changes of >2 mm– Elevation in any lead, especially in the Elevation in any lead, especially in the

presence of reciprocal changes.presence of reciprocal changes.– ST depression in V1 -V3 .ST depression in V1 -V3 .

Ventricular arrhythmias   Ventricular arrhythmias   – Calculated QTc of >0.48.Calculated QTc of >0.48.

Based on autopsy proven MI Based on autopsy proven MI (Towbin JA (Towbin JA Am J CardAm J Card 1992 1992 70:1545)70:1545)

? Prospective use?? Prospective use?Reich JD et al AJ Emerg Med 1998 16(3):296

Troponin Levels in KidsTroponin Levels in Kids

< 7 days< 7 days <0.35<0.35 8-30 days8-30 days <0.2<0.2 31-120 days31-120 days <0.1<0.1 > 120 days> 120 days <0.03<0.03

Tx for MI in ChildrenTx for MI in Children

Very small literature bank, most on KD kids Very small literature bank, most on KD kids Only case reports of streptokinase or urokinaseOnly case reports of streptokinase or urokinase Theoretical risk of using streptokinase as it is Theoretical risk of using streptokinase as it is

contraindicated in pts with recent GAS infection contraindicated in pts with recent GAS infection (and what kid doesn’t meet that criteria?!)(and what kid doesn’t meet that criteria?!)

tPA has been usedtPA has been used– Case study from 2000 in 7yo boy post-KDCase study from 2000 in 7yo boy post-KD– Resolution of symptoms, improved LV function and no Resolution of symptoms, improved LV function and no

thrombus seen on cath at 1 month (echo + @ thrombus seen on cath at 1 month (echo + @ presentation)presentation)

Krendel et al Ann Emerg Med 2000 35(5): 502

Tx for MI in ChildrenTx for MI in Children

Angioplasty pretty difficult in children Angioplasty pretty difficult in children <5<5

IIa/IIIb inhibitors are not approved in IIa/IIIb inhibitors are not approved in children (and may have a higher risk children (and may have a higher risk of bleeding?)of bleeding?)

Cardiac Arrests in KidsCardiac Arrests in Kids

20 yrs of cardiac arrest attended by 20 yrs of cardiac arrest attended by EMSEMS

Of 5505 arrests, 2% were in children Of 5505 arrests, 2% were in children (0-17 y.o.)(0-17 y.o.)

Overall survival = 5% Overall survival = 5%

Engdahl J et al Resuscitation 2003 58:131

Cardiac Arrests in KidsCardiac Arrests in Kids

Majority of Majority of CA occur in CA occur in 0-1 yo0-1 yo

Mostly Mostly related to related to SIDS and SIDS and respiratory respiratory causescauses

Cardiac Arrests in KidsCardiac Arrests in Kids