acquired heart disease in pediatrics.pptx

8/18/2019 Acquired Heart Disease in Pediatrics.pptx

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LOGO

Acquired heart disease in

pediatrics

Onanong manacharoen MD.

Bhuddasothorn hospital

January 2015


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Contents

Acute rheumatic e!er 1

"a#asa$i disease2

%necti!e endocarditis&

Myocarditis'


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LOGO

Acute rheumatic e!er


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Contents


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(tiology

Acute rheumatic fever is a systemicdisease of childhood,often recurrent that

follows group A beta hemolytic

streptococcal infection )&*+It is a delayed non-suppurative sequelae

to URI with GAB streptococci!

It is a diffuse inflammatory disease ofconnective tissue,primarily involving

heart,blood vessels,"oints, subcutaneous

tissue and #$%


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(pidemiology

Ages &-'& yrs are most susceptibleRare () yrs

Girls*boys

#ommon in )rd world countries

+nvironmental factors-- over crowding,

poor sanitation, poverty


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Pathophysiology

,roup A streptococcus

)-heumatogenic strains+

M 1&5/1'112'22

3haryngitiss$in inection

4usceptile 6ost

)67A D- '21&+8

%mmune -eaction

)6%-9CM%-+

-heumatic :e!er

Antigenic mimicry

1;& #$s.


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3athophysiology

ibrinoid degeneration of connective

tissue,inflammatory edema, inflammatory cell

infiltration proliferation of specific cells

resulting in formation of Ashco nodules

resulting in--Pancarditis in the heart

- Arthritis in the joints

- Ashcoff nodules in the subcutaneous

tissue

-Basal ganglion lesions resulting in

chorea


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Jone


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Jone


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Clinical maniestation

litting fleeting migratory polyarthritis, involvingma"or "oints

#ommonly involved "oints-2nee,an2le,elbow wrist

6ccur in 789,involved "oints are e:quisitely tender

In children below 5 yrs arthritis usually mild but

carditis more prominent

Arthritis do not progress to chronic disease

Resolve in 1 wk!ramatic response to A"A in #$-$% hrs

1.Arthritis)5*+


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;anifest! as pancarditis


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6ccur in &-'89 of cases

;ainly in girls of '-'& yrs age

;ay appear'-> mo after the attac2 of rheumatic fever

#linically manifest! as involuntary movementclumsiness, deterioration of handwriting,

emotional lability or grimacing of face

&.4ydenham


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#linical signs- pronator sign,mil2ing sign of

hands


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6ccur in (&9!

Unique,transient lesions of '-5 inches in sie

@ale center with red irregular margin;ore on trun2s limbs non-itchy not on face

orsens with application of heat

0'ten associated with

chronic carditis

'.(rythema Marginatum


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6ccur in '89

@ainless,pea-sied,palpable nodules sie 8!&-5 cm!

;ainly over e:tensor surfaces of

"oints,spine,scapulae scalp

Associated with strong seropositivity

Always associated withsevere carditis

5.4ucutaneous nodules


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Other eatures )Minor criterias+ever up to ).Cc

Arthralgia

igh +%R, +levated #-reactive protien+#G- prolonged @R interval,8!58 sec=

5nd or )rd degree bloc2s,

%0 depression, 0 inversion


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%n!estigation

+vidence of GAB streptococcal infection

A%6 titre *5&8 0odd units or rising in 5-/ wee2s

wee2s= 789

Anti-1$Ase B .&9

0hroat culture-GAB streptococci 5&-/89


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(?ceptions to Jones Criteria

&horea alone, i' other causes have been ecluded

Insidious or late-onset carditis with no othereplanation

2atients with documented R.! or prior rheumatic

'ever, one major criterion,or o' 'ever,arthralgia or

high &R2 suggests recurrence


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Dierential Diagnosis

3uvenile rheumatiod arthritis?

small and large "oint,symmetrical,not response%eptic arthritis

%ic2le-cell arthropathy

Dawasa2i disease;yocarditis

%carlet fever

4eu2emia


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@reatment and 3rophyla?is

@ t t


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@reatment

4tep % - primary prevention


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24

4@(3 % 3rimary 3re!ention o -heumatic :e!er

)@reatment o 4treptococcal @onsillopharyngitis+

Agent Dose Mode

Duration

Benathine penicillin , /00 000 or patients %ntramuscular Once2 $g )/0 l+

1 200 000 or patients E2 $g

or3enicillin Children 250 mg 2;& times daily Oral 10 d

)pheno?ymethyl penicillin+ Adolescents and adults 500mg 2;& times daily

:or indi!iduals allergic to penicillin

(rythromycin 20;'0 mgF$gFd 2;' times daily Oral 10 d (stolate

)ma?imum 1 gFd+

or(thylsuccinate '0 mgF$gFd 2;' times daily Oral 10 d

)ma?imum 1 gFd+

Recommendations of American Heart Association


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4tep %% Anti inlammatory treatment

#linical condition 1rugs

Arthritis only Aspirin E&-'88mgF2gFday q > hrs!:> w2s!


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Bed rest

0reatment of congestive cardiac failure?

digitalis,diuretics

0reatment of chorea?

haloperidol 8!8'-8!8)mgF2gFday


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Carditis status -ecommended duration

$o carditis Bed rest 5 w2s!

Gradual ambulation 5 w2s!#arditisno cardiac enlargement

Bed rest / w2s!Gradual ambulation / w2s!

#arditis

with cardiac enlargement

Bed rest > w2s!

Gradual ambulation > w2s!#arditisith active #

%trict bed rest for as long as Gradual ambulation ) mo!


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4@(3 % 4econdary 3re!ention o -heumatic :e!er

)3re!ention o -ecurrent Attac$s+

Agent Dose Mode

Benathine penicillin , 1 200 000 e!ery ' #ee$sG %ntramuscular

or

3enicillin 250 mg t#ice daily Oral

or

4uladiaine 0.5 g once daily or patients 2 $g )/0 l Oral

1.0 g once daily or patients E2 $g )/0 l+

:or indi!iduals allergic to penicillin and suladiaine(rythromycin 250 mg t#ice daily Oral

G%n high;ris$ situations administration e!ery & #ee$s is =ustiied and recommended



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Duration o 4econdary -heumatic :e!er

3rophyla?is Category Duration

-heumatic e!er #ith carditis and At least 10 y since last

residual heart disease episode and at least until

)persistent !al!ar diseaseG+ age '0 y sometimes lielong

prophyla?isAter !al!e surgery lielong

-heumatic e!er #ith carditis 10 y or #ell into adulthood ut

no residual heart disease #hiche!er is longer )no !al!ar

diseaseG+

-heumatic e!er #ithout carditis 5 y or until age 21 y

#hiche!er is longer

*Clinical or echocardiographic evidence.


3 i


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3rognosis

Rheumatic fever can recur whenever the individual

e:perience new GAB streptococcal infection,if noton prophylactic medicines

Good prognosis for older age group if no carditis

during the initial attac2

Bad prognosis for younger children those withcarditis with valvular lesions

accine


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LOGO

"a#asa$i disease

6i t " $i Di


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6istory o "a#asa$i Disease

0omisa2u Dawasa2i '.>' & yrs! boy Hdiagnosis un2nown

'.>5-Ecases,$on scarlet fever syndrome with desquamation

'.>/-55cases,;ucocutaneous ocular syndrome


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(tiology

Un2nown;ucocutaneous lymph node syndrome

Infectious agent most li2ely? virus

Acute vasculitis disease,self limited

Associate with genetics,environment

( id i l


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(pidemiology

;edian age of affected children J 5!) years

789 of cases in children ( / yrs, &9 of cases inchildren * '8 yrs

;ales?females J '!&-'!E?'

Recurs in )-&9

@ositive family history in '9 but ')9 ris2 ofoccurrence in twins

( id i l


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(pidemiology

&-'8/!>?'88,888children under & years of ageIncomplete form '89

#ardiac involvement occurs in 58-5&9 of

patients who are not treated

the mortality rate is 8!'-59

3athophysiology


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3athophysiology

Un2nown

$o diagnostic test#linical diagnosis

3athophysiology


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3athophysiology

Di ti C it i


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Diagnostic Criteria

ever for at least & days

At least / of the following & features?

'! #hanges in the e:tremities +dema, erythema, desquamation

5! @olymorphous e:anthem, usually truncal

)! #on"unctival in"ection

/! +rythemaFor fissuring of lips and oral cavity

&! #ervical lymphadenopathy


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Diagnostic criteria

:e!er E 5

d

Bilateral painless non e?udati!e

con=uncti!itis

7ip crac$ing and issuring stra#errytounge inlammation o oral mucosanoulcer

Cer!ical lymphadenopathy unilateral )E1.5cm+

3olymorphous e?antheminduration oBC, scar

-edness and s#elling o hands and eet

#ith desquamation


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www.themegallery.com


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>oncardiac maniestation


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>oncardiac maniestation

• omitting diarrhea gallladder hydrops

ele!ated transaminases,%• 4terile pyuria proteinuria-enal

• Cough rhinorrhea iniltrate on chestradiograph)pneumonia+-esp.

• Arthalgia and arthritisJoint

• (le!ated (4- C-3 leu$ocytosishypoaluminemiaBlood

• Mononuclear pleocytosis o C4:)aseptic

meningitis+ irritaility and acial palsy>eurologic


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3hases o Disease


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3hases o Disease

Acute


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Beau s line

Atypical or %ncomplete "a#asa$i Disease


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Atypical or %ncomplete "a#asa$i Disease

@resent with ( / of & diagnostic criteria#ompatible laboratory findings

%till develop coronary artery aneurysms

$o other e:planation for the illness

;ore common in children ( ' year of age;ore cardiac complication

%n!estigation


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%n!estigation

7a 4tudies Imaging

C di l M i t ti " $i


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Cardio!ascular Maniestations o "a#asa$i

$one

asculitis of coronary artery

%uggestive of myocarditis and endocarditis


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Coronary Arterial Changes

Coronary artery anormalities

'&9 to 5& 9 of untreated patients develop

coronary artery changes

1ays '8-)8

)-E9 if treated in first '8 days of fever with IIG ;ost commonly pro:imal, can be distal

Giant aneurysm


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Coronary Aneurysms

L 3atients most li$ely to de!elop aneurysms

Mounger than > months, older than 7 years ;ales

evers persist for greater than '/ days

@ersistently elevated +%R,#R@ Anemia

igh B#

0hrombocytosis

4ow albumin @ts who manifest sFs of cardiac involvement

Cardio!ascular 4equelae


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Cardio!ascular 4equelae

8!)-59 mortality rate due to cardiac disease '89 from early myocarditis

Aneurysms may thrombose, cause ;IFdeath

;I is principal cause of death in D1 )59 mortality

;ost often in the first year

;a"ority while at restFsleeping

About 'F) asymptomatic

@reatment and management


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@reatment and management

@reatment


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@reatment

IIG? 5gF2g as one-time dose

Beneficial effect 'st reported by

3apanese

;echanism of action is unclear

%ignificant reduction in #AA in pts

treated with IIG plus aspirin vs! aspirin

alone


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Acute "a#asa$i Disease @reatment

IIG

E8-.89 defervesce show symptom

resolution within 5-) days of treatment

Retreat those with failure of response to

'st dose or recurrent symptoms Up to

5F) respond to a second course

*5doses failedNpulse methyl

prednisolone,immunosuppressive agent



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Aspirin igh dose


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Aspirin 1ecrease to low dose -7

wee2s or until platelet levels normalieFaneurysmsolvedNOcoronary thrombosis

$o evidence effect on #AA when used alone 1ue to potential ris2 of Reye syndrome instructparents about symptoms of influena or varicella

1ipyridamole )-& mgF2gFdayif A%A contraindicated

eparin,warfarin if giant aneurysm



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Aggressive support with diuretics

inotropes for some patients with

myocarditis

Antibiotics while e:cluding bacterial

infection

:ollo#ing echocardiography


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g g p y

1 2 & '

At irst time

o diagnosis

or

Hithin 2

#$s

/ #$s

later

/ mo.

later

7ong term

(!aluation

in

3ersistent

Coronaryaneurysm


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LOGO

%necti!e endocarditis

%necti!e (ndocarditis


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ebrile illness

@ersistent bacteremia#haracteristic lesion of microbial infection

of the endothelial surface of the heart

(tiology


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gy

Agent

ostcongenital heart disease?06,%1,AR

normal heart?$B,I1U,#entral line,immunocompromise

+nvironment

bacteremia,age(5yrs!,procedure

&0*predisposing actor

(tiology


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gy

Gram positive organisms


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(pidemiology

:requency o %( increase

1120;'500

Pediatric patients

with preexist HDincreased risk of IE

3athophysiology


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p y gy

$onbacterial 0hrombotic +ndocarditis»

+ndothelial in"ury» ypercoagulable state

4esions seen at coaptation points of valves• Atrial surface mitralFtricuspid

• entricular surface aorticFpulmonic

;odes of endothelial in"ury• igh velocity "et

• low from high pressure to low pressure chamber

• low across narrow orifice of high velocity

Bacteria deposited on edges of low pressure sin2 orsite of "et impaction bacterial endocarditis

enturi (ect

3latelet;irinthromi

enturi (ect


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3athophysiology


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(ndothelial damage

3latelet;irin thromi

Microorganism adherence

Bacterial endocarditis)!egetation+

7ocal tissue

damage

4ystemic

inlammation%mmune

reactionemolism

3athophysiology


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+mbiliation )8->89cardiac,spleen,2idney,brain

Blood vessel emboliNfungusDidneyN focal nephritis,diffuse glomerulonephritis

cardiacNabscess,;,Ao,0,@

%pleenNinfarct,rupture

BrainNto:ic encephalitis,myelitis

6slerKs nodeNto:ic,allergic vasculitis,emboli

%necti!e (ndocarditis


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Acute


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$ative valve endocarditis


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4hould e suspected in any child or adult #ith


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Classical 3eripheral Maniestations


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4ess common today

$ot seen in tricuspid endocarditis@etechiae most common

ascular phenomena?

ma"or arterial emboli,septic pulmonary

infarcts,mycotic aneurysm,intracranial

hemorrhage,con"unctival hemorrhage,

"aneway lesions,clubbing finger

Immunologic phenomena?

glomerulonephritis,osler nodes,

rothKs spots,rheumatoid factor

4ucon=uncti!al 6emorrhages


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Jane#ay 7esions


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0;10*

4plinter 6emorrhage


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0;10*

Oslerodes


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;*

-oth


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0;/*

-e!ised Du$e Criteria


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Ma=or Minor

,oals o @herapy


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+radicate

infection

@herapy

Deiniti!ely treat

4equelae o

destructi!e

intra;cardiac

and e?tra;

cardiaclesions

Antiiotic @herapy


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0reatment tailored to etiologic agent

Important to note ;I#F;B# relationship for eachcausative organism and the antibiotic used

igh serum concentration necessary to penetrate

avascular vegetation

Antiiotic @herapy


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4treptococcal iridans endocarditis

@enicillin sensitivehrs!

Gentamicin)mgF2gFday in ' or ) divided doses

@enicillin resistance


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4urgical @reatment o %ntra;Cardiac Complications


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Relative indications

@erivalvular e:tension of infection

@oorly responsive %! aureus $+

Relapse of $+

#ulture negative $+F@+ with persistent fever


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@rophylactic regimen targeted against li2ely

organism

%trep! viridans P oral, respiratory, eosphogeal

+nterococcus P genitourinary, gastrointestinal

%! aureus P infected s2in, mucosal surfaces

3re!ention I the underlying lesion


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igh ris2 lesions @rosthetic valves

@rior I+

#yanotic congenital heart

disease

@1A

AR, A%, ;R,;% with ;R %1

#oarctation

%urgical systemic-

pulmonary shunts

Intermediate ris2 ;@ with murmur

@ure ;%

0ricuspid disease

@ulmonary stenosis

Bicuspid Ao valve with no

hemodynamic significance

3re!ention I the underlying lesion


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4owFno ris2 ;@ without murmur

0rivial valvular regurg!

Isolated A%1

Implanted device


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1ental procedures

2nown to producebleeding

0onsillectomy

%urgery involving GI,

respiratory mucosa

+sophageal dilation

+R#@ for obstruction

Gallbladder surgery

#ystoscopy, urethraldilation

Urethral catheter if

infection present

Urinary tract surgery,

including prostate

I1 of infected tissue


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3rophylactic regimens:or dentaloralrespiratory tract

procedures

Situation Agent Regimen

Standard generalprophylaxis

Amoxicillin C:50mg/kg PO1hr e!ore procedure

A:" g PO 1hr e!oreprocedure

#nale to take oralmedications

Ampicillin C:50mg/kg $% or $& 'ithin(0 min e!ore procedure

A:" g $% or $& 'ithin (0min

e!ore procedure


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@his #allet card is to e gi!en to patients y their physician. 6ealthcare

proessionals please see ac$ o card or reerence to the complete statement.

$ame? SSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSneeds protection from

BAC@(-%A7 (>DOCA-D%@%4 because of an e:isting6(A-@ CO>D%@%O>

1iagnosis? SSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSS@rescribed by? SSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSS 1ate? SSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSSS


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LOGO

Myocarditis

Myocarditis


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Inflammatory process of myocardium

#ellular damage,degeneration,necrosis$ot ischemic heart disease

ulminant disease acute heart failure and

arrhythmias

%ome patients are asymptomaticNunderdiagnosed

owever, asymptomatic myocarditis may be a

cause of une:plained deaths in '9 of cases

(tiology


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iruses? +nteroviruses


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3rotooa? 0rypanosoma cruii

0o:oplasma gondi6elminths? 0richinella spiralis

+chinococcus

Autoimmunity? Infection associated

Auto-immune disease associated @rimary autoimmunity

6ypersensiti!ity? @enicillins

;ethyldopa

%ulfametho:aole

@o?icity #atecholamines

#ocaine

+thanol

(pidemiology


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@revalence of clinical myocarditis '-'8?'88,888

&-'>9 of myocardial disease in childrenIntrauterine infection


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0hree phases?

iral Replication

Autoimmune in"ury

1ilated cardiomyopathy


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%n!estigation


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#TR? cardiomegaly,pulmonary edema

+DG? sinus tachycardia,R% low voltage, wave,%0-0 change,0,%0,A,A bloc2

+cho? 4 enlargement,poor contraction

+ndomyocardial biopsy? lymphocyte infiltrate

gold standard

sens!)->)9

%n!estigation


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0he 1allas criteria ?

Biopsy?acute myocarditis,borderline myocarditis, no myocarditis

ollow up ?ongoing myocarditis,revolving

myocarditis,Resolved myocarditis

iral studies ?culture myocardiumNstandard

?culture blood,stool,urineNunreliable

Antibody / fold rising


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#ardiac biomar2ers i!e! creatine 2inase and

troponin 0 and I are routinely measured#D;B is not useful due to low predictive value!

0rop 0 * 8!'ngFm4 had a sensitivity of &)9 and aspecificity of ./9

+%R found to have low sensitivity and specificity!+cho changes i!e! 4 dysfunction .9=, and

segmental wall motion abnormalities /9=, donot differentiate myocarditis from other

cardiomyopathies!

@reatment


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0reatment is dictated by clinical signs and

symptoms!;any proposed therapies, most have only atheoretical basis! %ome have been tested inanimal models

#onventional heart failure therapy is currentlythe only accepted therapy for myocarditisincluding A#+ inhibitors, angiotensin receptorbloc2ing agents, diuretics, V-bloc2ers or

amiodarone,digitalis


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Immunosuppression

prednisolone,aathioprine,cyclosporineNnoteffective

IIG

Interferon-V

+:tracorporeal membrane o:ygenation


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$ewborn,Infant esp!co:sac2ie BNmortalityE&9

#hildN mortality'8-5&9

N recover &89

N sequelae 5&9

Berieri


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eavy alcohol inh! ability to absorb! thiamine

#an be inherited

Increase requirementNinfection,pregnancy,Breast

feeding whose mother diet are inadequate in

thiamine

+:cessive #6 inta2e,flurosemideRequirement 8!& mgFday ,breast feeding '!/ mgFday

1ry beriberi N nervous system

et beriberiN cardiovascular system


Dietary thiamine


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ood that are rich in thiamine

hole-grain foods

;eatFfishFpoultryFeggs

;il2 and mil2 products

egetables


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1ifficulty wal2ing

4oss of muscle coordination

4oss of sensation

$eurological problems, such as memory loss,

confusion, and encephalitis

@aralysis%evere discomfort or pain

%lurred speech

0ingling or other unusual sensations in the hands orfeet

Uncontrolled eye movement


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atigue

Increased heart rate

4eg pain and swelling

@leural effusion

%hortness of breath


%nantile erieri


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5->months

1yspnea,cyanosis and right sided heart failure

hoarseness of voice

oarseness caused by nerve paralysis

eight loss, marasmic as the disease progresses

omiting,1iarrhea@ale s2in,+dema

Alterations of the cardiovascular system,

especially tachycardia

6ccasionally convulsions were observed in the

terminal stage

pseudomeningitic form in infants aged E-. months


@reatment


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0herapeutic diagnosis

thiamine 5&-&8mg


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/$i&, BP '%/6% $$Hg G(: ( thai gi)l, Goo* +o&s+ios&ess

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9(B i&estigatio&


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CBC

BC !6'3%, Plt 2"!%%%, H#!2, H+t30,P-1 52, 9y$p "5

Ele+t)olytes

1a!36, ?".0,Cl !%2,TC@2 !A, B12% ,C)%.3' 9T !2:"'

(l# 3.%, TP ".' TB 2.!, DB !." SG@T!%3, SGPT 0%

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hF /0 IMOYPK oXF @`X EYHF P9[KS .c<


125/130

B0110B= 13,B7 น, HORI .

h:K iOR F ยบบวม กนไดมาก(, -น ไมหอบ

hF /0 IMOYPK oXF @`X EYHF P9[KS . c< RNGOPYJ9:K9O ;F \CTG

hSHKS c23 .,.W N?0X c2e

1,Wf :?0HX2> B,8e 9CSG Va0X

\CTGpp K[KSK X/HRIZJ:CM9G:L\c 1e^_9:9OP NKS9COSH9OPKrJI9G:

V_;F _RGCOSH9M9IMOSM iGYJMO9:K =7?sB^i@ B7 P V/ BP0TSl17 C?0j?09:m

XOI9U 17 ? V/ IMOM MTK:W T

-a&age$e&tB0110B= 18,77 น, iOR .


126/130

B0110B= 18,77 น, iOR .

h:K iOR &ท# 6KZKS #',2##า" 0น!นาวา 1m


127/130

W0110B= 7=,37 น, iOR 3

h:K iOR F OCM9[K + )นด ไมเหน )อยยบบวมลงมาก ป$..าว!ออกด

hF /0 IMOYPK Nh.fB^ ahq8,W P0j?0TSVq17e7PL hq..f1 P

>oXF @`XL :G OCM9[KNSKCGSH9J EYHF IGZML ยบบวม `G N9MM9:? KHKO OMY MT P

hSHKS KNOS9: 17L777 asB^i@177 P V/ . P0TS

iGYJMO9:K =7?sB^i@ B7 P V/ BP0TSl17 C?0j?09:m

XOI9U B ? GSOP Y9H;22 t e TS

-a&age$e&tW0110B= 11,e7 น, iOR 3


128/130

W0110B= 11,e7 น, iOR 3

h:K iOR c0a 5d6F COSH9GK?OPRเทาเดม

;21B,= ;223W,=

V`61,3 2##า"&ท#มา 0น!นาวา *า;22.7b37 %ห&หลดTKNOS9: B7a0j?0HGIK

1B,37 ;22 .B,8 .7,77น,;223f,=pp.7,37 #$บ6 Z Z

hSHKS >KNOS9: 17L777asB^i@ 177 P V/.,B P0TS

>KNOS9: B77 a V/HS9N 9: 1 TS MTK:

17L777 asB^i@177 P V/ .,= P0TS

>KNOM9: 3 P0TS


129/130


130/130

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