Acquired Heart DiseaseAcquired Heart Disease in Childhood in Childhood

Dr. Maged M. El SamadyDr. Maged M. El SamadyMBBCh, MSc, MRCPCH, FRCP (UK)MBBCh, MSc, MRCPCH, FRCP (UK)

Al Yamamah HospitalAl Yamamah Hospital

بسم الله الرحمن الرحيم

ويسألونك عن الروح قل الروح من أمر ربي وما �أوتيتم من العلم إال قليًال

صدق الله العظيم

OutlineOutline

Acute Rheumatic Fever and Rheumatic Acute Rheumatic Fever and Rheumatic Heart Disease.Heart Disease.

Kawasaki Disease.Kawasaki Disease.

ACUTE RHEUMATIC ACUTE RHEUMATIC FEVERFEVER

Acute Rheumatic Fever (ARF)Acute Rheumatic Fever (ARF)DefinitionDefinition::

ARF is an inflammatory disease thatARF is an inflammatory disease that

occurs as a delayed nonsuppurativeoccurs as a delayed nonsuppurative

sequel of group A streptococcalsequel of group A streptococcal

upper respiratory infectionupper respiratory infection..

Definition (cont.)Definition (cont.)::

In its classic form ARF is acuteIn its classic form ARF is acute , ,

febrile, and largely self-limited febrile, and largely self-limited disorderdisorder..

It involves joints, heart, brain, It involves joints, heart, brain, and skin in varying and skin in varying combinationscombinations..

EtiologyEtiology::

ARF development requires an ARF development requires an antecedent infection withantecedent infection with : :

A specific organismA specific organism::

group A streptococcusgroup A streptococcus At a specific siteAt a specific site : :

the upper respiratory tractthe upper respiratory tract..

Etiology (cont.)Etiology (cont.)::

There is evidence that group A There is evidence that group A streptococci serotypes vary in their streptococci serotypes vary in their rheumatogenic potentialrheumatogenic potential..

Strains causing clusters or epidemics Strains causing clusters or epidemics usually belong to a limited number of usually belong to a limited number of serotypes (e.g. 3, 5, 18, 24, and serotypes (e.g. 3, 5, 18, 24, and others)others)..

PathogensisPathogensis::

Various theories were explaining Various theories were explaining the mechanism by which group the mechanism by which group A streptococci elicit the A streptococci elicit the connective tissue inflammatory connective tissue inflammatory response that constitutes ARF response that constitutes ARF includingincluding::

Pathogenesis (cont.)Pathogenesis (cont.)::11 . .Toxic effects of streptococcal Toxic effects of streptococcal

products initiating tissue injuryproducts initiating tissue injury..

22 . .Inflammation mediated by antigen-Inflammation mediated by antigen-antibody complexes, localized to sites antibody complexes, localized to sites of tissue injuryof tissue injury..

33 . .Autoimmune phenomena induced Autoimmune phenomena induced by similarity of certain streptococcal by similarity of certain streptococcal and human tissue antigensand human tissue antigens..

Pathogenesis (cont.)Pathogenesis (cont.)::

Several observations suggest that Several observations suggest that specific genetic constitution of specific genetic constitution of the host may predispose to the the host may predispose to the occurrence and / or recurrence occurrence and / or recurrence of ARFof ARF..

ARF EpidemiologyARF Epidemiology::

It mirrors the epidemiology of streptococcal It mirrors the epidemiology of streptococcal pharyngitispharyngitis..

The peak age incidence is 5 to 15 years, but both The peak age incidence is 5 to 15 years, but both primary and recurrent cases can occur in adults. primary and recurrent cases can occur in adults. ARF is rare before the age of 4 yearsARF is rare before the age of 4 years..

There is no clear-cut gender predisposition, although There is no clear-cut gender predisposition, although females are more likely to develop certain females are more likely to develop certain manifestations such as Sydenhammanifestations such as Sydenham’’s choreas chorea..

ARF age at diagnosis in a high incidence population ARF age at diagnosis in a high incidence population in Australia (in Australia (Arch Dis Child 2001 ;85:223-227)Arch Dis Child 2001 ;85:223-227)

ARF Epidemiology (cont.)ARF Epidemiology (cont.)::

There is no known racial predispositionThere is no known racial predisposition..

The disease peaks in cooler months of the yearThe disease peaks in cooler months of the year..

Crowding and low socioeconomic status are the main Crowding and low socioeconomic status are the main environmental factors favoring the occurrence of environmental factors favoring the occurrence of ARFARF..

ARF remains a common and important health ARF remains a common and important health problem in developing countriesproblem in developing countries..

Crowdedness is an environmental factor favoring occurrence of ARFCrowdedness is an environmental factor favoring occurrence of ARF

ARF PathologyARF Pathology::

Exudative and proliferativeExudative and proliferative

inflammatory lesions in theinflammatory lesions in the

connective tissues of the heartconnective tissues of the heart , ,

joints, and subcutaneous tissuesjoints, and subcutaneous tissues , ,

characterize ARFcharacterize ARF..

Pathology (cont.)Pathology (cont.)::

In the heartIn the heart::

Aschoff nodulesAschoff nodules are virtually pathognomonic are virtually pathognomonic..They consist of a central area of fibrinoid They consist of a central area of fibrinoid surrounded by lymphocytes, plasma cells, and surrounded by lymphocytes, plasma cells, and large basophilic cells; some of them are large basophilic cells; some of them are multinucleatedmultinucleated..

Cardiac findings may involve pericardium, Cardiac findings may involve pericardium, myocardium, or endocardiummyocardium, or endocardium..

Acute Rheumatic Fever VegetationsAcute Rheumatic Fever Vegetations

Aschoff NoduleAschoff Nodule

Pathology (cont.)Pathology (cont.)::

In the jointsIn the joints::

The arthritis of ARF is characterized byThe arthritis of ARF is characterized by

a fibrinous exudate and sterile effusiona fibrinous exudate and sterile effusion

without erosion of joint surfaces orwithout erosion of joint surfaces or

pannus formationpannus formation..

ARF is licking the jointsARF is licking the joints

but is biting the heart but is biting the heart

ARF Clinical ManifestationsARF Clinical Manifestations::

The The latent periodlatent period between the between the antecedent streptococcal antecedent streptococcal infection and the onset of ARF infection and the onset of ARF symptoms ranges between 1 and symptoms ranges between 1 and 5 weeks ( average is 19 days ) 5 weeks ( average is 19 days ) for both primary and recurrent for both primary and recurrent attacksattacks..

Cl. Manifestations (cont.)Cl. Manifestations (cont.)::

Five clinical features are so Five clinical features are so characteristic that they are recognized characteristic that they are recognized as as majormajor manifestationsmanifestations::

CarditisCarditis

PolyarthritisPolyarthritis

ChoreaChorea

Erythema marginatumErythema marginatum

Subcutaneous nodulesSubcutaneous nodules

ArthritisArthritis::

Joint involvement ranges from Joint involvement ranges from arthralgia alone to acute disabling arthralgia alone to acute disabling arthritis (swellig,warmth,erythema, arthritis (swellig,warmth,erythema, limitation of motion, and severe limitation of motion, and severe tenderness)tenderness)..

Arthritis (cont.)Arthritis (cont.)::

The larger joints of the extremities are usually The larger joints of the extremities are usually involvedinvolved::

Most frequently: Most frequently: kneesknees and and anklesankles..

Frequently: Frequently: wristswrists and and elbowselbows..

Occasionally:Occasionally: hips hips and and small joints of hands and small joints of hands and feetfeet..

Rarely: Rarely: shouldersshoulders, , lumbosacrallumbosacral, , cervicalcervical, , sternoclavicularsternoclavicular, and , and temromandibulartemromandibular joints joints..

Ankle ArthritisAnkle Arthritis

Arthritis (cont.)Arthritis (cont.)::

Characteristically, Characteristically, migratory polyarthritismigratory polyarthritis pattern is noticedpattern is noticed..

In most instances, inflammation to any joint In most instances, inflammation to any joint begins to subside spontaneously within one begins to subside spontaneously within one weekweek..

The total duration of polyarthritis is no more The total duration of polyarthritis is no more than 3 or 4 weeks leaving no residual joint than 3 or 4 weeks leaving no residual joint

damagedamage..

CarditisCarditis::

ARF may involve endocardium, ARF may involve endocardium, myocardium, and pericardium myocardium, and pericardium ( i.e. can induce pancarditis )( i.e. can induce pancarditis )..

Carditis is the most important Carditis is the most important manifestation of ARF as it is the only manifestation of ARF as it is the only one that can cause significant one that can cause significant permanent organ damage and deathpermanent organ damage and death..

Carditis (cont.)Carditis (cont.)::

The diagnosis of carditis requires the presence The diagnosis of carditis requires the presence of one of the followingof one of the following::

11 . .New organic cardiac murmur (e.g. apical New organic cardiac murmur (e.g. apical systolic murmur of MR, apical mid-systolic murmur of MR, apical mid-diastolic (carey-coombs) murmur of MS, diastolic (carey-coombs) murmur of MS, or basal diastolic murmur of AR)or basal diastolic murmur of AR)..

22 . .CardiomegalyCardiomegaly..

33 . .Pericardial friction rub (i.e. pericarditis) or Pericardial friction rub (i.e. pericarditis) or muffled heart sounds due to PEmuffled heart sounds due to PE..

44 . .Congestive heart failureCongestive heart failure..

Carditis (cont.)Carditis (cont.)::

Different rhythm disturbances may Different rhythm disturbances may occur during the course of ARF, the occur during the course of ARF, the commonest of which is first degree commonest of which is first degree atrioventricular block (prolonged P-R atrioventricular block (prolonged P-R interval)interval)..

SydenhamSydenham’’s Choreas Chorea::

It is characterized byIt is characterized by::

11 . .Rapid purposeless involuntary Rapid purposeless involuntary movements in the extremities and face movements in the extremities and face and may sometimes be unilateraland may sometimes be unilateral..

22 . .HypotoniaHypotonia..

33 . .Inability to maintain a tetanic muscle Inability to maintain a tetanic muscle contractioncontraction..

44 . .Emotional labilityEmotional lability..

SydenhamSydenham’’s Chorea (cont.)s Chorea (cont.)::

Chorea usually occurs after a latent Chorea usually occurs after a latent period that is longer than that period that is longer than that associated with other manifestations associated with other manifestations of ARFof ARF..

Chorea frequently occurs in Chorea frequently occurs in ““purepure”” formform..

Subcutaneous NodulesSubcutaneous Nodules::

These are firm painless subcutaneous These are firm painless subcutaneous lesionslesions..

Varying in size from few millimeters toVarying in size from few millimeters to 22 cmcm..

They occur in crops over bony They occur in crops over bony prominences and tendonsprominences and tendons..

Subcutaneous Nodules (cont.)Subcutaneous Nodules (cont.)::

They do not appear until at leastThey do not appear until at least

33 weeks after onset of ARF and last weeks after onset of ARF and last for 1 to 2 weeksfor 1 to 2 weeks..

Nodules almost always appear in Nodules almost always appear in association with severe carditisassociation with severe carditis..

Subcutaneous NoduleSubcutaneous Nodule

Erythema MarginatumErythema Marginatum::

The rash begins as an erythematous The rash begins as an erythematous macule or papule, which extends macule or papule, which extends outward while the skin in the center outward while the skin in the center returns to normalreturns to normal..

The lesions may be raised or flat, are The lesions may be raised or flat, are neither pruritic nor induratedneither pruritic nor indurated..

They blanch on pressureThey blanch on pressure..

Erythema Marginatum (cont.)Erythema Marginatum (cont.)::

The lesions vary in sizeThe lesions vary in size..

They appear in the trunk and proximal They appear in the trunk and proximal extremities, sparing the faceextremities, sparing the face..

The lesions migrate from place to The lesions migrate from place to place leaving no residual scarringplace leaving no residual scarring..

Erythema MarginatumErythema Marginatum

Erythema MarginatumErythema Marginatum

Erythema Marginatum (magnified)Erythema Marginatum (magnified)

InvestigationsInvestigations::No specific laboratory test is diagnostic of No specific laboratory test is diagnostic of ARFARF..

Usually there is polymorph nuclear Usually there is polymorph nuclear leucocytosisleucocytosis and mild to moderate and mild to moderate normocytic normochromic normocytic normochromic anemiaanemia..

Evidence of acute inflammation is Evidence of acute inflammation is prominent including raisedprominent including raised CRP CRP and and ESRESR..

Investigations (cont.)Investigations (cont.)::

Lab. Evidence of recent group A streptococcal Lab. Evidence of recent group A streptococcal infection are usually there includinginfection are usually there including::

Positive throat swab culturesPositive throat swab cultures..

Positive rapid streptococcal antigen testPositive rapid streptococcal antigen test..

Elevated or rising antistreptococcal antibody Elevated or rising antistreptococcal antibody titers e.gtiters e.g:.:.

Antistreptolysin O (ASO)Antistreptolysin O (ASO),,

Antideoxyribonuclease B (anti-DNase B)Antideoxyribonuclease B (anti-DNase B),,

or Antihyaluronidase titersor Antihyaluronidase titers . .

Investigations (cont.)Investigations (cont.)::

ECG may reveal evidence of rhythm ECG may reveal evidence of rhythm disturbance (e.g. prolonged P-R interval)disturbance (e.g. prolonged P-R interval)..

CXR may show findings suggesting congestive CXR may show findings suggesting congestive heart failure or pericarditisheart failure or pericarditis..

Echocardiography may document myocardial Echocardiography may document myocardial and valvular dysfunctions and pericardial and valvular dysfunctions and pericardial effusioneffusion..

Rheumatic Mitral Valve RegurgeRheumatic Mitral Valve Regurge

DiagnosisDiagnosis::

Modified Jones CriteriaModified Jones Criteria::

Two major criteria or one major and Two major criteria or one major and two minor criteria indicate a high two minor criteria indicate a high probability of ARF, provided that probability of ARF, provided that there is supporting evidence of there is supporting evidence of recent streptococcal infectionrecent streptococcal infection..

?? ??ExceptionsExceptions?? ??

Modified Jones CriteriaModified Jones CriteriaMajor criteriaMajor criteriaMinor criteriaMinor criteriaEvidence of recent Evidence of recent

strept. infectionstrept. infection

CarditisCarditisFeverFeverPositive throat Positive throat culturescultures

PolyarthritisPolyarthritisArthralgiaArthralgiaPositive rapid Positive rapid streptococcal antigen streptococcal antigen testtest

ChoreaChoreaProlonged P-R Prolonged P-R intervalinterval

Elevated Elevated antistreptococcal antistreptococcal antibodies e.g. ASO , antibodies e.g. ASO , anti DNase B, or anti DNase B, or antihyaluronidase antihyaluronidase titerstiters

Erythema Erythema marginatummarginatum

Elevated acute Elevated acute phase reactants e.g. phase reactants e.g. ESR and CRPESR and CRP Subcutaneous Subcutaneous

nodulesnodules

Differential DiagnosisDifferential Diagnosis

Juvenile rheumatiod arthritisJuvenile rheumatiod arthritis

Septic arthritisSeptic arthritis

Sickle-cell arthropathySickle-cell arthropathy

Kawasaki diseaseKawasaki disease

MyocarditisMyocarditis

Scarlet feverScarlet fever

LeukemiaLeukemia

ARF TreatmentARF Treatment::

11 . .Bed rest for inflamed joints and / or Bed rest for inflamed joints and / or congestive heart failure (CHF)congestive heart failure (CHF)..

22 . .Specific therapy for CHF e.g. diureticsSpecific therapy for CHF e.g. diuretics..

33 . .Oral penicillin V 40 mg /kg /day for 10 Oral penicillin V 40 mg /kg /day for 10 days to eradicate throat streptococcidays to eradicate throat streptococci..

Oral cephalosporin or erythromycin may be Oral cephalosporin or erythromycin may be an alternative in case of penicillin an alternative in case of penicillin allergyallergy..

Treatment (cont.)Treatment (cont.)::

44 . .Anti-inflammatory agents: Anti-inflammatory agents: AspirinAspirin in a dose of 90-100 mg / kg / day in in a dose of 90-100 mg / kg / day in 4 divided doses after food is very 4 divided doses after food is very effective decreasing fever, toxicity, effective decreasing fever, toxicity, and joint inflammation. After 2 and joint inflammation. After 2 weeks aspirin dose can be reduced weeks aspirin dose can be reduced to 60-70 mg / kg / day for an to 60-70 mg / kg / day for an additional 6 weeksadditional 6 weeks..

Treatment (cont.)Treatment (cont.)::Corticosteroids are reserved forCorticosteroids are reserved for::

Patients with severe carditis Patients with severe carditis manifested by CHFmanifested by CHF,,

Patients who are unable to tolerate Patients who are unable to tolerate large doses of aspirin, orlarge doses of aspirin, or

Patients whose signs and symptoms Patients whose signs and symptoms are not adequately suppressed by are not adequately suppressed by aspirinaspirin..

Treatment (cont.)Treatment (cont.)::

Prednisolone 2 mg / kg / day in divided doses Prednisolone 2 mg / kg / day in divided doses may be initiatedmay be initiated..

After 2-3 weeks it should be withdrawn slowly After 2-3 weeks it should be withdrawn slowly over an additional 3 weeks periodover an additional 3 weeks period..

Aspirin is better to be given for one month Aspirin is better to be given for one month after discontinuation of steroids to avoid after discontinuation of steroids to avoid rebounds, which frequently occur after rebounds, which frequently occur after

steroid therapysteroid therapy . .

Treatment (cont.)Treatment (cont.)::Patients with significant SydenhamPatients with significant Sydenham’’s chorea s chorea need specific treatmentneed specific treatment..

DiazepamDiazepam in acute severe cases in acute severe cases..

HaloperidolHaloperidol was frequently used was frequently used..

Sodium valproateSodium valproate was found effective and has was found effective and has less side effectsless side effects..

ARF ProphylaxisARF Prophylaxis::

11 . .Primary prophylaxis of ARFPrimary prophylaxis of ARF::

It consists of accurate diagnosisIt consists of accurate diagnosis

and appropriate treatment ofand appropriate treatment of

streptococcal throat infectionsstreptococcal throat infections..

ARF ProphylaxisARF Prophylaxis::22 . .Secondary prophylaxisSecondary prophylaxis::

It aims atIt aims at preventing the recurrencepreventing the recurrence of ARF of ARF in patients who have already suffered a in patients who have already suffered a rheumatic attackrheumatic attack..

Benzathine penicillinBenzathine penicillin intramuscular intramuscular injections every 3-4 weeks is injections every 3-4 weeks is recommendedrecommended..

The dose is 600,000 units in patients The dose is 600,000 units in patients weighing less than 27 kg. and 1,200,000 weighing less than 27 kg. and 1,200,000 units in patients weighingunits in patients weighing more than 27 kgmore than 27 kg..

ARF ProphylaxisARF Prophylaxis::This secondary prophylaxis should be This secondary prophylaxis should be maintained maintained indefinitelyindefinitely for those with for those with rheumatic heart diseaserheumatic heart disease..

Other rheumatic subjects should be protected Other rheumatic subjects should be protected until they reach age 21 and 5 years have until they reach age 21 and 5 years have elapsed since the last rheumatic attack, elapsed since the last rheumatic attack, whichever is longerwhichever is longer..

A skin sensitivity test A skin sensitivity test shouldshould be done before be done before each injectioneach injection..

ARF ProphylaxisARF Prophylaxis::33 . .Tertiary prophylaxisTertiary prophylaxis::

It means It means bacterial endocarditis prophylaxisbacterial endocarditis prophylaxis for for patients with rheumatic heart disease patients with rheumatic heart disease whenever they undergo dental or surgical whenever they undergo dental or surgical procedures likely to evoke bacteremiaprocedures likely to evoke bacteremia..

This is not necessary in rheumatic patients free This is not necessary in rheumatic patients free of residual heart diseaseof residual heart disease..

Receiving rheumatic fever prophylaxis does not Receiving rheumatic fever prophylaxis does not exempt the patient from endocarditis exempt the patient from endocarditis

prophylaxisprophylaxis . .

KAWASAKI DISEASEKAWASAKI DISEASE

KAWASAKI DISEASEKAWASAKI DISEASE

First described in Japan in l967 by Dr. First described in Japan in l967 by Dr. Tomisaku KawasakiTomisaku Kawasaki

Acute and self-limited vasculitisAcute and self-limited vasculitis Infantile polyarteritis nodosaInfantile polyarteritis nodosa Mucocutaneous lymph node syndromeMucocutaneous lymph node syndrome Surpassed acute rheumatic fever as the leading Surpassed acute rheumatic fever as the leading

cause of acquired heart disease in children in cause of acquired heart disease in children in the USthe US

Kawasaki T. Acute febrile mucocutaneous syndrome with lymphoid involvement with specific desquamation of the fingers and toes in children [in Japanese]. Arerugi. 1967;16:178

11stst Saudi Report Saudi Report

EPIDEMIOLOGYEPIDEMIOLOGY

Positive family history in about 1% Positive family history in about 1%

The rate in a sibling is 2.1% within 1 year after onset The rate in a sibling is 2.1% within 1 year after onset of the first case in a familyof the first case in a family

The risk of occurrence in twins is 13%The risk of occurrence in twins is 13%

Incidence increased in children of parents who Incidence increased in children of parents who themselves had the illness in childhoodthemselves had the illness in childhood

ETIOLOGYETIOLOGY

UnknownUnknown Infectious, Genetic, Immune, Environmental?Infectious, Genetic, Immune, Environmental? More common during the winter and early More common during the winter and early

spring month in the USspring month in the US Occurs in epidemics with geographic wave Occurs in epidemics with geographic wave

like spreadlike spread 76% of children are <5 years old with most 76% of children are <5 years old with most

cases in less than 2 years oldcases in less than 2 years old

PATHOLOGYPATHOLOGY

Generalized systemic vasculitis involving Generalized systemic vasculitis involving blood vessels throughout the bodyblood vessels throughout the body

Aneurysms may occur in other mid-size Aneurysms may occur in other mid-size extraparenchymal muscular arteries such as extraparenchymal muscular arteries such as the celiac, mesenteric, femoral, iliac, renal, the celiac, mesenteric, femoral, iliac, renal, axillary, and brachial arteriesaxillary, and brachial arteries

Naoe S, Takahashi K, Masuda H, Tanaka N. Kawasaki disease. With particular emphasis on arterial lesions. Acta Pathol Jpn. 1991;41:785–797

DIAGNOSTIC CRITERIADIAGNOSTIC CRITERIA

DIAGNOSIS CRITERIADIAGNOSIS CRITERIA

≥≥5 days of fever and ≥4 of the 5 principal clinical 5 days of fever and ≥4 of the 5 principal clinical featuresfeatures

Typically, all of the clinical features are not present at Typically, all of the clinical features are not present at a single point in timea single point in time

In the presence of 4 of 5 classic criteria, US and In the presence of 4 of 5 classic criteria, US and Japanese experts agree that only 4 days of fever are Japanese experts agree that only 4 days of fever are necessary before initiating treatmentnecessary before initiating treatment

Lancet 2004; 364: 533–44

Current Paediatrics (2005) 15, 62–68

LABORATORY FINDINGSLABORATORY FINDINGS

Thrombocytosis appears in the second week Thrombocytosis appears in the second week the illness, peaks in the third week with a the illness, peaks in the third week with a gradual return to normal by 4 to 8 wksgradual return to normal by 4 to 8 wks

Range from 500 000 to >1 million/mm3 Range from 500 000 to >1 million/mm3

Thrombocytopenia is seen rarely in the acute Thrombocytopenia is seen rarely in the acute stage and may be a sign of disseminated stage and may be a sign of disseminated intravascular coagulationintravascular coagulation

LABORATORY FINDINGSLABORATORY FINDINGS

Elevation of acute phase reactants (ESR and CRP) is Elevation of acute phase reactants (ESR and CRP) is universal universal

Return to normal by 6 to 10 weeksReturn to normal by 6 to 10 weeks

The degree of elevation of ESR and CRP may show a The degree of elevation of ESR and CRP may show a discrepancy so both should be measured discrepancy so both should be measured

Elevation of ESR (but not of CRP) can be caused by Elevation of ESR (but not of CRP) can be caused by IVIG therapyIVIG therapy

CORONARIESCORONARIES 20% of untreated patients develop coronary aneurysm 20% of untreated patients develop coronary aneurysm

or ectasia or ectasia

20% of aneurysms go on to develop stenosis20% of aneurysms go on to develop stenosis

Ectasia or aneurysms regress in most patients within 1-Ectasia or aneurysms regress in most patients within 1-2 years (AHA)2 years (AHA)

Coronaries are ABNORMAL after aneurysms Coronaries are ABNORMAL after aneurysms regression and even when aneurysms were never regression and even when aneurysms were never detecteddetected

The long-term clinical implication is unknownThe long-term clinical implication is unknown

ECHOCARDIOGRAPHYECHOCARDIOGRAPHY

Aneurysms are classified according toAneurysms are classified according to AHA statement as: AHA statement as:

small (<5-mm internal diameter), small (<5-mm internal diameter),

medium (5 to 8 mm internal diameter), medium (5 to 8 mm internal diameter),

or giant (>8-mm internal diameter).or giant (>8-mm internal diameter).

(Circulation. 2005;111:e440.)

TREATMENTTREATMENTIVIGIVIG

The efficacy of IVIG in reducing the prevalence of The efficacy of IVIG in reducing the prevalence of coronary artery abnormalities is well establishedcoronary artery abnormalities is well established

Decreases the incidence of coronary aneurysms from Decreases the incidence of coronary aneurysms from 20% to 5% (if used before day 10)20% to 5% (if used before day 10)

Has generalized anti-inflammatory effectHas generalized anti-inflammatory effect

Patients should be treated with IVIG, 2 g/kg in a Patients should be treated with IVIG, 2 g/kg in a single infusion over 10-12 hours together with aspirin single infusion over 10-12 hours together with aspirin (AHA)(AHA)

.

Durongpisitkul K, Gururaj VJ, Park JM, Martin CF. The prevention of coronary artery aneurysm in Kawasaki disease: a meta-analysis on the efficacy of aspirin and immunoglobulin treatment. Pediatrics. 1995;96:1057–1061Terai M, Shulman ST. Prevalence of coronary artery abnormalities in Kawasaki disease is highly dependent on gamma globulin dose but independent of salicylate dose. J Pediatr. 1997;131:888–893

TREATMENTTREATMENTIVIGIVIG

Give in the first 10 days and preferably within 7 days Give in the first 10 days and preferably within 7 days of illnessof illness

Should be still administered to children presenting Should be still administered to children presenting after the 10th day of illness if they have either after the 10th day of illness if they have either persistent fever without other explanation orpersistent fever without other explanation or aneurysms and ongoing systemic inflammation, as aneurysms and ongoing systemic inflammation, as

manifested by elevated ESR or CRPmanifested by elevated ESR or CRP Live vaccines deferred for 11 monthsLive vaccines deferred for 11 months

TREATMENTTREATMENTASPIRINASPIRIN

AspirinAspirin Additive anti-inflammatory effects to IVIGAdditive anti-inflammatory effects to IVIG Does not appear to lower the frequency of the development of Does not appear to lower the frequency of the development of

coronary abnormalitiescoronary abnormalities During the acute phase of illness, aspirin is administered at 80 During the acute phase of illness, aspirin is administered at 80

to 100 mg/kg per day in 4 doses to 100 mg/kg per day in 4 doses Many centers reduce the aspirin dose after the child has been Many centers reduce the aspirin dose after the child has been

afebrile for 48 to 72 hours. Other clinicians continue high-dose afebrile for 48 to 72 hours. Other clinicians continue high-dose aspirin until day 14 of illnessaspirin until day 14 of illness

Low-dose aspirin (3Low-dose aspirin (3––5 mg/kg per day) is maintained until the 5 mg/kg per day) is maintained until the patient shows no evidence of coronary changes by 6 to 8 patient shows no evidence of coronary changes by 6 to 8 weeks after the onset of the illness weeks after the onset of the illness

For children who develop coronary abnormalities, aspirin may For children who develop coronary abnormalities, aspirin may be continued indefinitelybe continued indefinitely

PrognosisPrognosis

50% of aneurysms regress within 5 years 50% of aneurysms regress within 5 years Mild dilatation (3Mild dilatation (3––4 mm) regresses within 2 4 mm) regresses within 2

yearsyears 80% of those with moderate dilatation (480% of those with moderate dilatation (4––8 8

mm) regress within 5 years. mm) regress within 5 years. Giant aneurysms (>8 mm) are unlikely to Giant aneurysms (>8 mm) are unlikely to

resolve, and many progress to stenosis or resolve, and many progress to stenosis or complete obstruction within years of the initial complete obstruction within years of the initial diagnosis.diagnosis.