SUBJECT: Pediatrics 2

TOPIC: Acquired Heart Disease

LECTURER: Dra. Punongbayan
SHIFTING/DATE: September 1, 2008
TRANS GROUP: Where art thou Doc Ryan?

ACQUIRED HEART DISEASE 3. Erythema marginatum (<10%) – nonpruritic

serpiginous or annular erythematous evanescent
Rheumatic Heart Disease rashes most prominent on the trunk and inner
proximal portions of the extremities; never on the
Acute Rheumatic Fever face (disappear on exposure to cold and reappear
• Immunologic lesion that occurs as a delayed sequela after a hot shower or if covered with a blanket);
of grp A streptococcal infection of the pharynx but blanches on pressure
not the skin
• Attack rate of post-streptococcal infection 0.3-3% 4. Subcutaneous nodules (2-10%) – particularly
• Predisposing factors: family history of RF, low seen in recurrent cases; hard, painless, nonpruritic,
socioeconomic status (poverty, poor hygiene, freely movable swellings 0.2-2 cm in diameter;
medical deprivation), 6-15 years old found symmetrically, singly or in clusters on the
extensor surfaces of large & small joints, over the
Rheumatic Fever scalp or along the spine; last for weeks
• Diffuse inflammatory lesion of connective tissues 5. Sydenham’s chorea (15%) – occurs more often
found mainly in the heart, brain, joints & skin in prepubertal girls; consist of choreic movements
• Valvular damage: mitral valve > aortic valve > (spontaneous purposeless movements followed by
tricuspid > pulmonary valve motor weakness),hypotonia, emotional lability,
hyperactivity, separation anxiety, obsessions &
• Aschoff bodies in the atrial myocardium compulsions; may be related to dysfunction of BG
(inflammatory lesions with swelling, fragmentation of & cortical neurons; increased titers of
collagen fibers, altered staining characteristics of “antineuronal antibodies” in >90%
connective tissue)
• History of streptococcal pharyngitis 1-5 weeks before Minor manifestations:
the onset of symptoms 1. Arthralgia – not considered a minor
• Pallor, malaise, easy fatigability, abdominal pain manifestation if arthritis is present
• Family history of rheumatic fever 2. Fever – at least 38.8 C
• Autoimmune or “cross-reactivity” injury between 3. Elevated acute phase reactants (CRP and ESR)
streptococcal antigens & heart protein has been 4. Prolonged PR interval on the ECG
established
• Level of auto-antibodies are too low and show little Evidence of Antecedent Group A Streptococcal
correlation with RF Infection
1. History of sore throat/scarlet fever
Jones Criteria (1993) unsubstantiated by lab. data is not adequate
Major manifestations: evidence of recent infection.
1. Arthritis (70%) – involves large joints 2. A negative rapid strep antigen detection test
simultaneously or in succession; responds should be confirmed by a conventional throat
dramatically to salicylates culture.
2. Carditis (50%) – includes some or all of the 3. Streptococcal antibody tests are the most
following in increasing order of severity: reliable lab.evidence. The onset of the clinical
a. Tachycardia (out of proportion to the fever) – manifestations coincide with the peak of the
its absence makes the diagnosis of myocarditis streptococcal antibody response.
unlikely a. Antistreptolysin 0 (ASO) titer is well
b. Heart murmur of valvulitis – MR or AR standardized; elevated in 80% of patients; 333
c. Pericarditis – friction rub, pericardial effusion, Todd units in children and 250 Todd units in
chest pain, ECG changes adults; a single low titer does not exclude RF; a
d. Cardiomegaly – seen on chest X-ray 4-fold rise in titer in 2 samples taken 10 days
e. Signs of CHF – gallop rhythm, distant heart apart; peaks at 4-6 wks & decreases after
sounds, cardiomegaly another 2 weeks
Rheumatic Carditis b. Antideoxyribonuclease B test – 240 Todd
• Valvulitis – murmurs units in children & 120 Todd units in adults
• Myocarditis – unexplained cardiomegaly or CHF
or gallop
c. Streptozyme test – slide agglutination test but
• Pericarditis – friction rub or pericardial effusion less standardized
• Miscellaneous findings – conduction
disturbances in the ECG; 2D echo findings Diagnosis

MARY YVETTE ALLAIN TINA RALPH SHERYL BART HEINRICH PIPOY TLE JAM CECILLE DENESSE VINCE HOOPS CES XTIAN LAINEY RIZ KIX EZRA GOLDIE BUFF MONA AM MAAN ADI KC
PENG KARLA ALPHE AARON KYTH ANNE EISA KRING CANDY ISAY MARCO JOSHUA FARS RAIN JASSIE MIKA SHAR ERIKA MACKY VIKI JOAN PREI KATE BAM AMS HANNAH MEMAY PAU
RACHE ESTHER JOEL GLENN TONI
 SUBJECT: Pediatrics 2
TOPIC: Acquired Heart Disease
• Highly probable when either 2 major or 1 major
and 2 minor manifestations plus evidence of
antecedent streptococcal infection are present
• Absence of supporting evidence of a previous
group A strep.infection makes the diagnosis
doubtful
• Exception to the Jones criteria:
 Chorea may occur as the only manifestation
of RF
 Indolent carditis may be the only
manifestation in patients who come to
medical attention months after the onset of
RF
 Occasionally, patients with RF recurrences
may not fulfill the Jones criteria.
Page 2 of 4
withdrawn gradually over 4-6 weeks while
monitoring ESR and CRP
• Arthritis – aspirin is continued for 2 weeks and
gradually tapered in 2-3 weeks
RF Prophylaxis
• Primary – prevents 1st episode of RF
 Treat strep throat infection
• Secondary – prevents recurrences
 Pen VK 250 mgQID
 Benzathine PCN 0.6 – 1.2 M units IM every 21
days
 Arthritis – at least 5 years
 Carditis – at least 10 years
 Recurrence-free & no residual heart disease

RF is considered Active:
• The following tips help in applying JC: • Joint symptoms
 Two major manifestations are always • New organic murmurs
stronger than 1 major plus 2 minor. • Changing heart size
 Arthralgia or a a prolonged PR interval • CHF (in the absence of long-standing valvular
cannot be used as a minor manifestation in disease)
the presence of arthritis or carditis • Subcutaneous nodules
respectively. • Sleeping pulse rate > 100/minute
 Absence of evidence of an antecedent grp A • Erythema marginatum
strep,infection is a warning that acute RF is • Chorea
unlikely (except when chorea is present). • A positive test for CRP
 Clearly distinguish between Still’s murmur • Fever for at least 3 consecutive days
and MR murmur.
 The possibility of the early suppression of full RF Recurrences
clinical manifestation should be sought • A recurrent attack refers to a new episode of RF
during the history taking. Subtherapeutic in a patient with previous history, and not a
doses of aspirin may suppress full relapse of the first episode
manifestations. • Occur during repeated bouts of GABHS infection,
both clinical and subclinical
Clinical Course • Importance of secondary prophylaxis
1. Only carditis can cause permanent cardiac • May be more severe
damage. Signs of mild carditis disappear rapidly • Residual valvular disease a risk factor
in weeks but those of severe carditis may last for
2-6 months. Management
2. Arthritis subsides within a few days to several • Treatment of CHF includes some or all of the ff:
weeks even without treatment and does not complete bed rest and oxygen; morphine 0.2
cause permanent damage. mg/kg at 4-hr interval for severe CHF with
3. Chorea gradually subsides in 6-7 months or respiratory distress; restriction of sodium and
longer and usually does not cause permanent fluid intake; prednisone for severe carditis of
neurologic sequelae. recent onset; digoxin; furosemide 1 mg/kg every
6-12 hrs
Management • Sydenham’s chorea: reduce physical &
• CBC, ESR, CRP, throat culture, ASO titer, chest X- emotional stress; Benzathine Penicillin G 1.2 M
ray, ECG, 2-D echo units and every 28 days for prevention of
• Benzathine Penicillin G 0.6-1.2 M units IM to recurrence; aspirin not needed in patient with
eradicate streptococci; Pen VK 200-500 mg QID isolated chorea
for 10 days; Erythromycin 40 mg/kg/day TID for • Presence or absence of permanent cardiac
10 days if allergic to Penicillin damage determines the prognosis
• Salicylates and steroids are started if definite Recommended Anti-Inflammatory Agents
diagnosis is made for 6-8 wks.
Arthriti Mild Modera Severe
• Prednisone 2mg/kg/day in 4 divided doses for 2-
s Alone Carditis te Carditis
6 weeks indicated only in cases of severe carditis
Carditis
• Mild to moderate carditis – aspirin alone 90-100
Predniso 0 0 0 2-6 wks
mg/kg/day in 4-6 doses (adequate blood level is
ne
20-25 mg/100 mL); continued for 4-8 weeks and
Aspirin 1-2 wks 3-4 wks 6-8 wks 2-4 mos
 SUBJECT: Pediatrics 2
TOPIC: Acquired Heart Disease
• Congenital or acquired; almost all acquired VHDs
are rheumatic in origin
• Mitral valve ¾; aortic valve ¼ of cases
• Tricuspid valve involvement is rare
• Pulmonary valve involvement almost never
occurs
VALVULAR HEART DISEASE
Mitral Stenosis
• Most common valvular involvement in adults
• Thickening of leaflets and fusion of the
commissures-àcalcification with immobility of
the valve results
• LA and right-sided heart chambers become
dilated and hypertrophied
• If with pulmonary venous hypertension,
pulmonary congestion & edema, fibrosis of
alveolar walls, hypertrophy of pulmonary
arterioles & loss of lung compliance occurs.
• Asymptomatic if mild
• Dyspnea with or without exertion
• Orthopnea, nocturnal dyspnea, palpitation in
more severe cases
• Increased RV impulse along LSB
• Weak peripheral pulses with narrow pulse
pressure
• Loud S1 at the apex & a narrowly split S2 with
loud P2
• Opening snap is followed by a low-frequency
mitral diastolic rumble at the apex
• CXR: LA & RV enlarged; prominent MPA
• Lung fields show pulmonary venous congestion
& interstitial edema (Kerley’s B lines)
• Most children are asymptomatic but become
symptomatic with exertion
• Subacute bacterial endocarditis
• Hemoptysis can develop from the rupture of
small vessels in the bronchi as a result of long-
standing pulmonary venous hypertension
• Good dental hygiene & antibiotic prophylaxis
against SBE
• Closed mitral commissurotomy for those without
calcification
• Valve replacement if valves are calcified
• Regular checkups for possible dysfunction of the
replaced/repaired valve
Mitral Regurgitation
• 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
• S1 is normal or diminished
• S2 may widely split; loud S3
• Hallmark: systolic regurgitant murmur gr 2-4/6 at
the apex with transmission to the left axilla
• Short, low-frequency diastolic rumble at the apex
Page 3 of 4
• 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
• Asymptomatic if mild
• Reduced exercise tolerance in severe AR or if
with CHF
• Hyperdynamic precordium; (+)diastolic thrill at
the 3rd LICS
• Wide pulse pressure and a bounding water-
hammer pulse in severe AR
• S1 is decreased in intensity; S2 may be normal
or single
• High-pitched diastolic murmur heard best at the
3rd-4th LICS – hallmark and more easily audible
when sitting & leaning forward
• LVH/LAH in the ECG
• CXR: LVE, dilated ascending aorta & prominent
aortic knob
• Patients deteriorate rapidly if symptoms begin
• Anginal pain, CHF, multiple PVCs
• 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
• Follow-up of valve function post-surgery
Infective Endocarditis
• Etiology: viridans-type streptococci (alpha-
hemolytic strep) and Staphylococcus aureus
• Other causes: group D strep (enterococcus) (S.
bovis, S. faecalis), Strep pneumoniae, H.
influenzae, coagulase (-) staph, Staph
epidermidis, Pseudomonas aeruginosa, etc.
• ~6% of cases: blood cultures are (-)
• Often a complication of congenital or rheumatic
heart disease
• Can also occur in children without any abnormal
valves or cardiac malformations
• Developed countries: congenital heart disease is
the major predisposing factor (blood is ejected at
high velocity through a hole or stenotic orifice)
• Rare in infancy but may occur following open
heart surgery or associated with a central
venous line
• Vegetations form at the site of the endocardial or
intimal erosion that results from the turbulent
flow
 SUBJECT: Pediatrics 2
TOPIC: Acquired Heart Disease
Page 4 of 4
• At high risk: VSD, AS, TOF, PDA, MVP, children 13. Presence of central nonfeeding lines
who underwent valve replacement 14. Peripheral lines
• ~65% of cases: surgical or dental procedure 15. Microscopic hematuria

Clinical Manifestations How to use the Duke criteria

• Prolonged fever
• Fatigue, myalgia, arthralgia, chills, headache,
nausea, vomiting
• New of changing heart murmur
• Splenomegaly, petechiae
• Embolic stroke, cerebral abscess, mycotic
aneurysm, hemorrhage
• Meningismus, increased ICP, altered sensorium,
focal neurologic signs
• Osler nodes – tender, pea-sized intradermal
nodules in the pads of fingers & toes
• Janeway lesions – painless small erythematous
or hemorrhagic lesions on the palms & soles
• Splinter hemorrhages – linear lesions beneath
the nails
• Identification of IE is most often based on a high
index of suspicion during evaluation of an
infection in a child with an underlying
contributory factor
Diagnosis
• Blood culture (3-5 separate blood collections;
causative agent recovered in 90% of cases)
• Antimicrobial pretreatment reduces the yield of
blood culture to 50-60%
• TEE cardiography, 2D echo, Doppler (>1 cm
lesions and fungating masses are at greatest risk
for embolization)
• Absence of vegetations does not exclude
endocarditis
Duke Criteria
Major criteria
 (+) blood culture (2)
 Evidence of endocarditis on echocardiography:
1. Intracardiac mass on a valve or other site
2. Regurgitant flow near a prosthesis
3. Abscess
4. Partial dehiscence of prosthetic valves
5. New valve regurgitant flow
Definite endocarditis
1. 2 major criteria
2. 1 major and 3 minor
3. 5 minor criteria
Prognosis & Complications
• Despite the use of antibiotic agents, mortality
remains at 20-25%
• Serious morbidity occurs in 50-60% of children
with documented IE (heart failure due to aortic
or mitral valve vegetations)
• Myocardial abscesses
• Systemic or pulmonary emboli
• Mycotic aneurysms
• Heart block
Treatment
• Several weeks are required for a vegetation to
organize completely à therapy must be
continued through this period to avoid
recrudescence
• Total of 4-6 weeks is recommended
• Nonstaphylococcal disease: fever resolves in 5-6
days with antibiotics
• Surgery for severe aortic or mitral valve
involvement with intractable heart failure,
myocardial abscess, recurrent emboli, new heart
block, increasing size of vegetations while
receiving therapy
• Staphylococcus: Oxacillin with optional addition
of Gentamicin; if resistant – Vancomycin
• Streptococcus: Penicillin G Na or Ceftriaxone plus
Gentamicin
• Prosthetic valve endocarditis due to Staph:
Oxacillin + Rifampicin + Gentamicin

Minor criteria
1. Predisposing conditions
2. Fever
3. Embolic-vascular signs
4. Immune complex phenomena (GN, arthritis,
rheumatoid factor, Osler nodes, Roth spots)
5. A single (+) blood culture or serologic evidence
of infection
6. Echocardiographic signs not meeting the major
criteria
7. Presence of newly diagnosed clubbing
8. Splenomegaly
9. Splinter hemorrhages
10. Petechiae
11. High ESR
12. High CRP