Acquired Heart Conditions

2.4c
Oct. 24, 2016
Dra. Punongbayan

JONES CRITERIA (1993)

Topic Outline MAJOR MANIFESTATIONS
I. Acquired Heart Disease  Involves large joints simultaneously or in
A. Acute Rheumatic Fever succession
B. Valvular Heart Disease  Migratory from below
C. Infective Endocarditis  Responds dramatically to salicylates
D. Kawasaki Disease  Most common manifestation
Arthritis  The inclusion of polyarthritis as a major
ACQUIRED HEART DISEASES (70%) manifestation is applicable only for
ACUTE RHEUMATIC FEVER moderate- to high-risk populations and
 Immunologic lesion that occurs as a delayed sequela of group only after careful consideration and
A β-hemolytic streptococcal infection of the pharynx but not the exclusion of other causes of arthralgia
skin such as autoimmune, viral, or reactive
 Attack rate of post-streptococcal infection 0.3-3% arthropathies (2015 Jones Criteria)
 Predisposing factors:  Includes some or all of the following in
o Family history of RF increasing order of severity:
o Low socioeconomic status o Tachycardia
 Poverty  Out of proportion to the fever
 Poor hygiene  Absence makes the diagnosis of
 Medical deprivation myocarditis unlikely
o 6-15 years old  Most common manifestation of
 No incidence of RF in <5 y/o carditis
 Incidence of exudative tonsillopharyngitis is found in 5 y/o o Heart murmur of valvulitis
and above  MR or AR
Carditis
 Exudates and ulcers in younger age group mainly have o Pericarditis
(50%)
viral causes  Friction rub
 Spectrum:  Pericardial effusion
o TonsilitisRFValvular HDEndocarditis  Chest pain
 ECG changes
o Cardiomegaly
RHEUMATIC FEVER
 Seen on CXR
 Inflammatory lesion found mainly in the: o Signs of CHF
o Heart
 Gallop rhythm
o Brain
 Distant heart sounds
o Joints
 Cardiomegaly
o Skin
 Nonpruritic serpiginous or annular
 Valvular damage:
erythematous evanescent rashes
o Mitral valve > Aortic valve > Tricuspid valve > Pulmonary
 Most prominent on the:
valve
o Trunk
 Aschoff bodies in the atrial myocardium
o Inner proximal portions of the
o Inflammatory lesions with swelling
extremities
o Fragmentation of collagen fibers
 Never on the face
o Altered staining of connective tissue
 History of streptococcal pharyngitis 1-4 weeks before the  Disappear on exposure to cold and
onset of symptoms reappear after a hot shower or if covered
with a blanket
 Pallor, malaise, easy fatigability, abdominal pain
 Family history of rheumatic fever Erythema
 Attack rate: Marginatum
o 0.3% - strep tonsillitis treated with penicillin (<3%)
o 3% - strep tonsillitis untreated
o 60% - known rheumatic patients with strep infection

 Particularly seen in recurrent cases

 Hard, painless, nonpruritic, freely movable
swellings 0.2-2 cm in diameter
 Found symmetrically, singly or in clusters
on the extensor surfaces of large & small
THEORIES OF PATHOGENESIS OF RHEUMATIC FEVER joints, over the scalp or along the spine
 Cytotoxic Theory  Last for weeks
o GAS produces enzymes that are cytotoxic for mammalian Subcutaneo
cardiac cells us Nodules
o Unable to explain the latent period between GAS (<1%)
pharyngitis and the onset of acute RF
 Immunologic Theory
o Antigenecity of GAS products and constituents and the
immunologic cross reactivity between GAS components and
mammalian tissues

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 Occurs often in prepubertal girls o It is reasonable to consider performing serial

 Consist of: echocardiography/Doppler studies in any patient diagnosed
o Choreic movements or suspected ARF even if documented carditis is not
 Spontaneous purposeless present on diagnosis
movements followed by motor o Echocardiography/Doppler testing should be performed to
weakness assess whether carditis is present in the absence of
o Hypotonia auscultatory findings particularly in moderate to high-risk
o Emotional lability populations and when ARF is considered likely
Sydenham’s o Echocardiography/Doppler findings not consistent with
o Hyperactivity
Chorea carditis should exclude that diagnosis in patients with a
o Separation anxiety
(10-15%) heart murmur otherwise thought to indicate rheumatic
o Obsessions and compulsions
 May be related to dysfunction of basal carditis
ganglia and cortical neurons
 Increased titers of “antineuronal MINOR MANIFESTATIONS
antibodies” in >90%  Not considered a minor manifestation if
Arthralgia
 Patients have no control over these arthritis is present
movements; some are not even aware of  Exceeds 38.5C in low-risk, and 38C in
it moderate- and high-risk populations
Fever o In most settings, including all low-risk
CLINICAL MANEUVERS TO ELICIT CHOREA populations, fever associated with ARF
 Milkmaid’s grip (irregular contractions of hand muscles while usually exceeds 38.5C
squeezing the examiner’s fingers  CRP > 30 mg/dL
Elevated acute
 Spooning and pronation of the hands when the patient’s arms  ESR > 60 mm/hr (in low-risk)
phase
are extended reactants  ESR > 30 mm/hr (in high-risk)
 Wormian darting movements of the tongue upon protrusion  Prolonged PR interval on the ECG
 Examination of handwriting to evaluate fine motor movements  Especially in Limb 2
Prolonged PR
interval on ECG  Normal PR interval: 0.12-0.2sec
MINOR MANIFESTATIONS o Not exceeding 5 small squares
 Not considered a minor manifestation if
arthritis is present  Any one of the following can serve s evidence of preceding
Arthralgia o Arthritis: with manifestations of infection
inflammation o Increased or rising ASO titer or other streptococcal
o Arthralgia: purely painful joints antibodies (anti-DNAse B) – a rise in titer is better evidence
Fever  At least 38.8⁰C than a single titer result
Elevated  CRP and ESR o A positive throat culture for Group A Beta-hemolytic
acute  Values increase whenever there is streptococci
phase inflammation o A positive rapid Group A streptococcal carbohydrate
reactants  Very nonspecific antigen test in achild whose clinical presentation suggests
a high pretest probability of streptococcal pharyngitis
Prolonged  Especially in Limb 2
 Diagnosis of recurrent rheumatic fever
PR interval  Normal PR interval: 0.12-0.2sec
o With a reliable past histtory of ARF or established RHD,
on ECG o Not exceeding 5 small squares
and in the face of documented GAS infection
 Two major manifestations, or;
EVIDENCE OF ANTECEDENT
 One major plus two minor manifestations, or;
GROUP A STREPTOCOCCAL INFECTION
 Three minor manifestations
 History of sore throat or scarlet fever unsubstantiated by o May be sufficient for a presumptive diagnosis
laboratory data  Possible rheumatic fever
o Not adequate evidence of recent infection o Where there is genuine uncertainty, it is reasonable to
 Streptococcal antibody tests consider offering 12 months of secondary prophylaxis
o Most reliable laboratory evidence followed by a reevaluation to include a careful history and
o Onset of clinical manifestations coincide with the peak of the physical examination in addition to a repeat
streptococcal antibody response echocardiogram
o Antistreptolysin O (ASO) titer o In a patient with recurrent symptoms (particularly involving
 Detects a previous/past gas infection of the throat. Never the joints) who has been adherent to prophylaxis
forget to request for this! recommendations but lacks serological evidence of Group
 Well standardized A streptococcal infection and lacks echocardiographic
 Elevated in 80% of patients evidence of valvulitis, it is reasonable to conclude that the
 333 Todd units in children recurrent symptoms are not likely related to ARF, and
 250 Todd units in adults discontinuation of antibiotic prophylaxis may be
 A single low titer does not exclude RF appropriate
 4-fold rise in titer in 2 samples 10 days apart
 Titers usually become elevated 2 weeks after
streptococcal infection, peaks at 4-6 weeks, and
DIAGNOSIS
decreases after another 2 weeks
 Thus, up to 8 weeks only. If beyond 8 weeks, ASO is not  Highly probable when either:
useful. o 2 major plus (+) ASO or
o Antideoxyribonuclease B test o 1 major and 2 minor manifestations plus (+)ASO
 240 Todd units in children  Absence of supporting evidence of a previous group A
 120 Todd units in adults streptococcal infection makes the diagnosis doubtful
o Streptozyme test  Exception to the Jones criteria:
 Slide agglutination test but less standardized o Chorea may occur as the only manifestation of RF
o Indolent carditis may be the only manifestation in patients
who come to medical attention months after the onset of RF
JONES CRITERIA (2015) o Occasionally, patients with RF recurrences may not fulfill the
 AHA scientific statement: Revision of Jones criteria for the Jones criteria
diagnosis of ARF in the era of 2D echo:  The following tips help in applying JC:
o Valvulitis is the most consistent feature of ARF o Two major manifestations are always stronger than 1 major
o 2D echocardiography is being used increasingly to plus 2 minor
diagnose carditis o Arthralgia or a prolonged PR interval cannot be used as a
o Thus, the concept of subclinical carditis has become minor manifestation in the presence of arthritis or carditis
incorporated into other guidelines and concensus respectively
statements as a valid RF manifestation o Absence of evidence of an antecedent group A strep
o Subclinical carditis refers exclusively to the circumstance in infection is a warning that acute RF is unlikely (except when
which classic auscultatory findings of valvular dysfunction chorea is present)
either are not present or are not recognized by the clinician o Clearly distinguish between Still’s murmur and MR murmur
but 2D echo reveal mitral or aortic valvulitis o The possibility of the early suppression of full clinical
 AHA statement concludes the following: manifestation should be sought during the history taking
o Echocardiography with Doppler should be performed in all
cases of confirmed and suspected ARF

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CLINICAL COURSE VALVULAR HEART DISEASES

 Only carditis can cause permanent cardiac damage.  Congenital or acquired
o Signs of mild carditis disappear rapidly in weeks but those  Almost all acquired VHDs are rheumatic in origin
of severe carditis may last for 2-6 months.  Mitral valve 3/4; aortic valve 1/4 of cases
 Arthritis subsides within a few days to several weeks  Tricuspid valve involvement is rare
o Even without treatment and does not cause permanent  Pulmonary valve involvement almost never occurs
damage.  Under Valvular Heart Diseases:
 Chorea gradually subsides in 6 months or longer o Mitral Stenosis
o End usually does not cause permanent neurologic sequelae. o Mitral Regurgitation
o Aortic Regurgitation
DIAGNOSTIC TESTS
 CBC MITRAL STENOSIS
 ESR/CRP  Most common valvular involvement in adults
 Throat culture  Thickening of leaflets and fusion of the commissures 
 ASO titer (most important) calcification with immobility of the valve  won’t be able to
 Chest X-ray prevent backflow of the blood
 ECG  LA and right-sided (ventricle) heart chambers become
 2D echo – valvular involvement dilated and hypertrophied
 If with pulmonary venous hypertension:
MANAGEMENT o Pulmonary congestion and edema
 Antibiotic Therapy o Fibrosis of alveolar walls
o Once the diagnosis of Acute RF has been made and o Hypertrophy of pulmonary arterioles
regardless of the throat culture results, the patient should o Loss of lung compliance
receive 10 days of oral Penicillin or Erythromycin or a single
IM inection of benzathine Penicillin to eradicate GAS from
the upper respiratory tract
 After this initial course of antibiotics, patient should be started
on long term antibiotic prophylaxis

 Penicillin VK
o 200-500mg QID x 10 days
Antibiotic
 Benzathine PCN
to eradicate
o 0.6-1.2 MU IM
streptococcus
 Erythromycin
o 250mg TID x 10 days
o If allergic to penicillin
 For 6 to 8 weeks
o ASA
 100mg/kg/day 4-6 doses
 For arthritis and mild carditis for 3-5
Anti-
days then
inflammatory
 75 mg/kg/day q 6hrs for 4 wks
agents
o Prednisone
 2mg/kg/day
 For severe carditis, cardiomegaly or Fish-mouth/button hole deformity
CHF q 6 hours for 2-3 weeks
Complete bed CLINICAL MANIFESTATIONS
rest and  If mild:
modified o Asymptomatic
activity  Dyspnea with or without exertion
 To prevent valvular heart disease o Most common manifestation
 Primary o With or without exertion
o Prevents first episode of RF  In more severe cases:
o Treats strep throat infection o Orthopnea
Prophylaxis
 Secondary o Nocturnal dyspnea
o Penicillin VK 250mg BID PO o Palpitation
o Benzathine penicillin G  Most children are asymptomatic but become symptomatic with
0.6-1.2 MU IM q21 days exertion
 Increased RV impulse along LSB
DURATION OF PROPHYLAXIS FOR PATIENTS WHO HAVE  Weak peripheral pulses with narrow PP
HAD ACUTE RF (2009 AHA RECOMMENDATIONS)  Loud S1 at the apex
 Narrowly split S2 with loud P2
CATEGORY DURATION  Opening snap is followed by a low-frequency mitral diastolic
Rheumatic fever without carditi 5 years until 21 years of age, rumble at the apex
whichever is longer
Rheumatic fever with carditis 10 years or until 21 years of PATHOPHYSIOLOGY
but without valvular disease age, whichever is longer Pulmonary HTN
Rheumatic fever with carditis 10 years or 40 years of age, Right Heart Failure
Pulmonary Congestion
and persistent valvular whichever is longer, Hepatic Congestion
LA Enlargement
IVD
heartdisease sometimes lifetime Atrial Fibrilation
Tricuspid Regurgitation
LA Thrombi
RA Enlargement
↑ LA Pressure
Pressure RV Overload
TREATMENT RVH LV Filling
Complete bed rest RV Failure
and oxygen DIAGNOSTIC FINDINGS
 0.2mg/kg at 4-hour interval  LA and RV enlarged
CXR
Morphine  For severe CHF with respiratory  Prominent MPA
distress
Restriction of
sodium and fluid
intake
Prednisone  For severe carditis of recent onset
Digoxin
Furosemide  1mg/kg every 6-12 hours

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MANAGEMENT
 MS eventually supervenes
 Preventive measures against SBE
 Afterload-reducing agents to maintain forward cardiac output
 Diuretics & digoxin for CHF
 MV repair or replacement
 Check valve function post-surgery

AORTIC REGURGITATION
 Most patients with AR have associated MV disease
 Semilunar cusps are deformed & shortened
 Dilated valve ring so that the cusps fail to appose tightly

 Lung fields show pulmonary venous congestion &

interstitial edema

CLINICAL MANIFESTATIONS
 Asymptomatic if mild
(Kerley’s B lines: Horizontal lines in the lower  Reduced exercise tolerance in severe AR or if with CHF
posterior lung fields; perpendicular to the lateral aspect  PE Findings
of the lung) o Hyperdynamic precordium
 (+) Diastolic thrill at the 3rd LICS
 Subacute bacterial endocarditis
o Wide pulse pressure and a bounding water-hammer
 Hemoptysis → rupture of small vessels in the pulse in severe AR
bronchi as a result of long-standing pulmonary o S1 is decreased in intensity
venous hypertension o S2 may be normal or single
o Hallmark: High-pitched diastolic murmur heard best at
MANAGEMENT the 3rd-4th LICS
 Good dental hygiene & antibiotic prophylaxis against SBE  More easily audible when sitting & leaning forward
 Closed mitral commissurotomy  Patients deteriorate rapidly if symptoms begin
o Without calcification  Anginal pain, CHF, multiple PVCs (Premature ventricular
 Valve replacement contractions)
o Valves are calcified
 Regular checkups for possible dysfunction of the DIAGNOSTIC FINDINGS
replaced/repaired valve ECG  LVH/LAH
 LVE, dilated ascending aorta & prominent aortic
MITRAL REGURGITATION knob
 Most common valvular involvement in children with RHD
 Shortened leaflets due to fibrosis
 Dilated LA & LV with dilated MV ring
 Asymptomatic during childhood
 Hyperdynamic apical impulse is palpable in severe MR

CXR

MANAGEMENT
 Good oral hygiene & antibiotic prophylaxis
 ACE inhibitor to reduce the dilatation of LV
 Digoxin, diuretics, afterload-reducing agents
 AV replacement before irreversible dilatation of LV develops
CLINICAL MANIFESTATIONS  Follow-up of valve function post-surgery
 PE findings
o S1 is normal or diminished INFECTIVE ENDOCARDITIS
o S2 may widely split CASE: An 18 yo male presents with DOB, chills and chest
o S3 loud pain for the past 24 hrs. He was previously diagnosed to have
 Hallmark: Systolic regurgitant murmur grade 2-4/6 at the a valvular heart disease but was lost to follow up.
apex with transmission to the left axilla
 Difference from MR is that VSD has the same type of murmur  PE: Ill-looking, T=40°C, BP 90/50, HR 110/min. He has a
but does not radiate to the axilla grade 3/6 systolic regurgitant murmur that radiates to the left
anterior axillary line.
 Short, low-frequency diastolic rumble at the apex
 What is the most likely diagnosis?
Subacute infective endocarditis
o Diagnosed in previously abnormal or damaged valves

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Subacute
 Diagnosed in previously abnormal 2007 AHA STATEMENT: PROPHYLACTIC ANTIBIOTIC
Infective
or damaged valves REGIMENS FOR A DENTAL PROCEDURE
Endocarditis
SITUATION AGENT ADULT CHILDREN
Acute  Develops in previously normal Oral Amoxicillin 2g 50 mg/kg
Endocarditis valves Ampicillin OR 2g IM or IV 50 mg/kg IM
Unable to
 Infective endocarditis in SLE take oral
or IV
Libman-Sacks  “Sterile endocarditis” Cefazotin or
medication
Endocarditis  Result from autoimmune damage to Ceftriaxone 1g IM or IV
cardiac valves Cefalexin OR 2g 50 mg/kg

ETIOLOGY Allergic to Clindamycin 600mg 20 mg/kg

penicillin or OR
 Viridans-type streptococci and Staphylococcus aureus  most
ampicillin;
common causes
oral Azithromycin 500mg 15 mg/kg
 Other causes: or
o Group D strep (Enterococcus) (S. bovis, S. faecalis) Clarithromycin
o S. pneumoniae
Allergic to Cefazotin or 1g IM or IV 50 mg/kg IM
o H. influenza
penicillin or Ceftriaxone or IV
o Coagulase (-) Staphylococcus epidermidis
ampicillin & 600 mg IM
o Pseudomonas aeruginosa
unable to OR or IV 20 mg’kg IM
 In 6% of cases take oral Clindamycin or IV
o Blood cultures are negative medication
 Often a complication of congenital or rheumatic heart disease
 Note: Amoxicillin is the first line. Only give IM/IV ampicillin if
 Can also occur in children without any abnormal valves or child cannot take oral meds. Cefalexin if allergic to ampicillin
cardiac malformations and Cefatozin if allergic and cannot take oral.
 In developed countries  Amoxicillin Oral Oral Azithromycin (If allergic to penicillin) 
o Congenital heart disease is the major predisposing factor Clindamycin(If patient cannot take oral)
o Blood is ejected at high velocity through a hole or stenotic
orifice HISTORY OF IE
 Rare in infancy but may occur following open heart surgery or
 Prior or congenital heart disease
associated with a central venous line
 Preceding dental, urinary tract or intestinal procedure
 Vegetations form at the site of the endocardial or intimal
 IV drug user
erosion that results from the turbulent flow  Turbulent flow 
damage to vascular endothelium  formation of Non-bacterial  Central venous catheter
thrombotic embolus  Prosthetic heart valve

WHO ARE AT RISK? CLINICAL MANIFESTATIONS

 Patients with Congenital heart diseases because turbulent
blood flow increases the susceptibility to IE
o VSD, AS, MVP,TOF, PDA children who underwent valve
replacement
 Turbulent flow traumatizes the vascular endothelium →
creation of substrate for deposition of fibrin and platelets
→ formation of nonbacterial thrombotic embolus (NBTE)
 Development of transient bacteremia colonizes the NBTE →
proliferation of bacteria within the lesion
 65% of cases: Surgical or dental procedure  hence need for
prophylaxis
 When to consider IE  Hx of Congenital Heart Disease (VSD,
AS, MVP), Rheumatic Heart Disease, or Surgical and Dental
Procedures
ENDOCARDITIS PROHYLAXIS
 Patients with cardiac conditions associated with the highest
risk for adverse outcomes should have prophylaxis before
surgery
 Patients with previous endocarditis
 Unrepaired congenital cyanotic heart conditions
 Completely repaired congenital heart defects with prosthetic
material or device
 Repaired CHD with residual defects at the site or adjacent to
the site of prosthetic patch or device
 Unique clinical manifestations include osler nodes,
janeway lesions, splinter hemorrhages
 Always look for an underlying contributory factor
 Multisystemic
 Prolonged fever
 Fatigue, myalgia, arthralgia, chills, headache, nausea, vomiting
 New murmur or a changing heart murmur
 Splenomegaly, petechiae
 Embolic stroke, cerebral abscess, mycotic aneurysm,
hemorrhage
 Meningismus, increased ICP, altered sensorium, focal
neurologic signs
 OSLER NODES
o Tender, pea-sized intradermal nodules in the pads of fingers
& toes
 JANEWAY LESIONS
o Painless small erythematous or hemorrhagic lesions on the
palms & soles
 SPLINTER HEMORRHAGES
o Linear lesions beneath the nails
 Identification of Infective Endocarditis is most often based on a
high index of suspicion during evaluation of an infection in
a child with an underlying contributory factor

REASONS FOR 200 REVISED RECOMMENDATIONS FOR

ANTIMICROBIAL PROPHYLAXIS
1. IE is much more likely to result from exposure to the more
frequent random bacteremia associated with daily activities
than from a dental or surgical problem
2. Routine prophylaxis may prevent “an exceedingly small”
number of cases
3. Risk of antibiotic-related adverse events exceeds the
benefits of prophylaxis

2007 AHA: CARDIAC CONDITIONS ASSOCIATED WITH

THE HIGHEST RISK OF AN ADVERSE OUTCOME FROM IE
FOR WHICH DENTAL PROPHYLAXIS IS REASONABLE
1. Prosthetic cardiac valve or prosthetic material used for
cardiac valve repair A-C: Osler Nodes; D: Janeway lesion
2. Previous infective endocarditis Note: They somehow appear similar but Osler is painful while
3. Cardiac transplantation recipients who develop cardiac Janeway is painless
valvulopathy
4. Congenital Heart Disease
a. Unrepaired cyanotic CHD (palliative shunts)
b. Completely repaired CHD with prosthetic material or
device, whether placed by surgery or catheter
intervention
c. Repaired CHD with residual defects at the site
d. Prosthetic device (inhibits endothelization)
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Non-staphylococcal  Fever resolves in 5-6 days with

disease antibiotics
Severe aortic or mitral
valve involvement
with intractable heart
failure, myocardial  Surgery while receiving therapy
abscess, recurrent
emboli, new heart
block, increasing size
of vegetations
Janeway Lesions Splinter Hemorrhages  Vancomycin plus Gentamicin
o Patients without prosthetic
DIAGNOSIS valve
o High risk of S. aureus,
 2 separate blood collection
enterococcus and Strep.
 Causative agent recovered in 90%
viridans
of cases Empirical therapy
Blood culture  suspect Staphylococcus
 Higher number of blood samples,
o If patient is severe looking,
higher chance of getting the
toxic, highly febrile, negative
etiology
sensorium
 Treat for Streptococcus
 Reduces the yield of blood culture otherwise
Antimicrobial
to 50-60%
pretreatment  Aqueous Pen G Na
o 200,000 U/kg/day IV q 4/6
 >1 cm lesions and fungating Native valve hours
TEE cardiography,
masses are at greatest risk for endocarditis due to  Ceftriaxone
2D echo, Doppler
embolization Strep. viridans and o 100 mg/kg/day IV od
Strep. bovis  Ceftriaxone plus Gentamicin
 Absence of vegetations on the valves does not exclude  3 mg/kg/day IV od or q 8
endocarditis hours
 Nafcillin or Oxacillin(200
DUKE CRITERIA Endocarditis due to
mg/kg/day IV q 4 or 6 hours)
MAJOR CRITERIA Staph with prosthetic
plus Gentamicin (3 mg/kg/day
1. (+) blood culture on (2) sites material
IV q 8 hours)
2. Evidence of endocarditis on echocardiography: Penicillin allergic
*Mnemonics: RADIN (Regurgitant, Abscess, patients with  Cefazolin(100 mg/kg/day IV q 8
Dehiscence, Intracardiac, New) endocarditis due to hours) plus optional
 Intracardiac mass on a valve or other site Staph without Gentamicin
 Regurgitant flow near a prosthesis prosthetic materials
 Abscess Oxacillin resistant  Vancomycin(40 mg/kg/day IV q
 Partial dehiscence of prosthetic valves strains due to Staph 8 or 12 hrs
 New valve regurgitant flow  Note: Accdg to dra., take note of empirical therapy. First,
MINOR CRITERIA vancomycin plus gentamicin. Usually mas severe ang
*Mnemonics:PHECSFI- P4H3E2C2S2FI presentation kapag Staph. aureus ang agent. If not, it is
 Predisposing conditions Strep., so treat with Aqueous Pen G Na, Ceftriaxone and
 Fever Gentamicin.
 Embolic-vascular signs
 Immune complex phenomena (GN, arthritis, rheumatoid KAWASAKI DISEASE
factor, Osler nodes, Roth spots)  Named after Tomisaku Kawasaki, reported the first 50 cases in
 A single (+) blood Culture or serologic evidence of 1967 (first case seen in 1961)
infection  Common in infants less than 5 years old (80%)
 Echocardiographic signs not meeting the major criteria  Mostly affects Asians although it occurs worldwide
 Presence of newly diagnosed Clubbing  Most common in boys (1.5:1)
 Splenomegaly  Etiology is unknown but epidemiology and clinical features
 Splinter hemorrhages support an infectious process (and autoimmune)
 Petechiae
 High ESR PATHOGENESIS
 High CRP  Acute non-specific systemic vasculitis of the blood
 Presence of central nonfeeding lines vessels of the heart (specially the left coronary artery) and
 Peripheral lines medium sized vessels  Early Myocardial Infarction
 Microscopic Hematuria  Arteritis on medium sized arteries
Definite Endocarditis is defined as: o Most common (coronary and iliac arteries)
2 major criteria  Aneurysm of coronary arteries is the most characteristic
OR finding of the disease
One major and 3 minor criteria  Interstitial myocarditis, pericarditis, inflammation of the SA
OR nodes and AV conduction system, valvulitis and endocarditis
5 minor criteria may also be present
 Inflammatory infiltrates of left coronary artery
o Usually involved macrophages, CD8Tcells and PMNs, IgA
PROGNOSIS AND COMPLICATIONS plasma cells
 Despite the use of antibiotic agents, mortality remains at 20-  Vessel loose structural integrity and weakens dilation,
25% saccular or fusiform aneurysm formation thrombi formation
 Serious morbidity occurs in 50-60% of children with  blood flow obstruction
documented infective endocarditis  There is no diagnostic test that could diagnose Kawasaki
o Heart failure due to aortic or mitral valve vegetations disease, clinicians must rely on the clinical manifestations and
 Myocardial abscesses a high degree of suspicion
 Systemic or pulmonary emboli
 Mycotic aneurysms
 Heart block DIAGNOSTIC CRITERIA
Fever plus 4 out of 5 criteria or
TREATMENT Presence of coronary lesions
o Proven by 2D echo or angiography
 Several weeks are required for a vegetation to organize
o Even when fewer than four minor features are present
completely → therapy must be continued through this period to
avoid recrudescence
 Total of 4-6 weeks is recommended  High Grade Fever
O
o Spiking up to 40 C and persisting more than 5 days,
remittent and unresponsive to antibiotics

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PEDIA2: Acyanotic Congenital Heart Conditions, Obstructive Lesions 2.4b

 Plus presence of at least 4 of the following features: PHASES

1. Bilateral bulbar conjunctival injections without  Lasts for 1 -2 weeks
exudates (Mapula na mata)
 Fever and other acute signs of illness
2. Erythema of the oral and pharyngeal mucosa with
 Perineal desquamation
strawberry tongue (papilla are raised) and dry, Acute
cracked lips without ulceration  Periungual desquamation of the finger and
Febrile
3. Edema and erythema of the hands and feet only toes beginning 1-3 weeks
Phase
4. Polymorphous exanthema (Non-vesicular)  Extreme irritability
o (Non-vesicular with accentuation on the groin  Tachycardia
area; common on the trunk)  Pericarditis with small effusion
5. Unilateral Non-suppurative cervical lymphadenopathy  3rd - 4th week
(> 1.5 cm in diameter)
 Begins when fever and other acute signs have
 Note: Accdg to dra., para mas madali maalala, puro abated
namumula. Pula ang mata, kamay, paa, groin, dila plus Subacute
Phase  Desquamation, thrombocytosis, and the
cracked lips.
development of coronary aneurysms (highest
risk of sudden death in those who have
developed aneurysms)
 6 to 8 weeks after onset of illness
Convale-  Begins when all clinical signs have
scent disappeared
 Continues until ESR and CRP return to normal

NATURAL HISTORY
 Kawasaki disease is a self-limited disease for most of the
patients
 Cardiovascular involvement
o Most serious complication of the disease
 25% of untreated patients develop coronary artery
abnormalities including aneurysm, CA thrombosis or stenosis,
MI, Aneurysm rupture or Sudden death
 Not detected in childhood  sudden death in adolescence
(due to MI, Aneurysms)

A: Bilateral nonexudative conjunctivitis; PREDISPOSING RISK FACTOR FOR CORONARY ARTERY

B: Dry, fissured, erythematous lips and a “strawberry” tongue; ANEURYSM
C&D: Erythematous and edematous hands and feet; E: 1. Boys < 1 year of age
Erythematous truncal rash; 2. Fever for > 16 days or recurrent fever
F: Desquamating perineal rash 3. Peripheral WBC count > 30,000
4. ESR >101 mm/hr
OTHER CLINICAL AND LABORATORY FINDINGS 5. Elevated CRP or ESR for > 30 days of illness
 For infants (Atypical or Incomplete Kawasaki Disease) 6. Reappearance of elevated ESR
o Persistent Fever but 7. ECG abnormality, Abnormal Q wave in leads III, AvF
o 4 out 5 criteria may not be completed 8. Symptoms of MI
o Check for accentuation in the groin area (perineal 9. Hypoalbuminemia
desquamtation)
 Perineal desquamation is common in the acute phase; LABORATORY
Infants; red at the diaper area  NO diagnostic test
 WBC is normal to elevated
 Elevated ESR, CRP for 4-6 weeks
 Normocytic, normochromic anemia
 Platelet usually normal during the 1st week of illness, but
rapidly increase on the 2nd to 3rd week
 Sterile pyuria
 Mildly high liver enzymes

 Periungal desquamation of the fingers & toes begins 1-3
weeks (subacute phase) after onset of illness (bilog bilog na
areas na namamalat)
2D ECHO
 Should be performed at diagnosis and be repeated after 2-
3 weeks of illness
 If both are normal, a repeat study should be done 6-8 weeks
after onset of illness
 No convincing evidence of long term cardiovascular sequelae
in children who do not develop coronary artery abnormalities
within 2 months after onset of illness
 Most useful test to monitor potential development of
coronary artery abnormalities
 Recommendation: All teenagers should undergo routine PE
and 2d Echo (pero sobrang impractical accdg. To doc)

TREATMENT
 IVIG 2g/kg over 10 – 12 hours
 Aspirin 80 – 100 mg/kg/ day Q6,
oral, until 14th day of illness
ACUTE PHASE
 Saves the patient from risk of
 Extreme irritabilty in infants sudden MI
 Coronary artery aneurysm develops in the 2 -3 week of
nd rd
 Decreases prevalence of coronary
illness in untreated patients best detected by 2D disease
Echocardiography  Aspirin3-5 mg/kg/day orally OD (2
 Myocarditis manifested as tachycardia out of proportion of the CONVALESCENT months) or until 6-8 weeks after
fever occurs in at least 50% 0f the patients onset of illness
 Pericarditis with a small pericardial effusion is common in the  For those with coronary
acute phase of the illness abnormalities
 Aspirin 3-5 mg/kg/day OD +/-
LONG TERM clopidogrel 1 mg/kg/day (max 75
mg/day)
 Most experts add warfarin for those
at high risk for thrombus formation

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PEDIA2: Acyanotic Congenital Heart Conditions, Obstructive Lesions 2.4b

 Second IVIG 2 g/kg

 If poor response to 2 IVIG, IV
nd

methylprednisolone 30 mg/kg/day
REFRACTORY
for 3 days
 Abciximab, glycoprotein IIb/IIIA
inhibitor
 Mechanism of action of IVIG is unknown but results in rapid
defervescence & resolution of clinical signs in 85-90% of
patients
 IVIG reduces prevalence of CAD in 20-25% in children treated
with aspirin alone to 2-4% in those treated with IVIG and
aspirin within the first 10 days of illness
 Consideration given to children diagnosed after 10th day of
illness if fever has persisted because the anti-inflammatory
may be helpful
 Consideration given to child diagnosed after 10th day of illness
if fever has persisted because anti-inflammatory may be
helpful
 Patients with small solitary aneurysm should continue
aspirin indefinitely
 Abciximab (not available in the Philippines)
o Glycoprotein IIb/ IIIA inhibitor
o Has been used in some patients who develop giant
coronary aneurym

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