PEDIATRIC CARDIOLOGY

Ruby Ann L. Punongbayan, MD

ACQUIRED HEART DISEASE
• Rheumatic heart disease
• Infective endocarditis
 ACUTE RHEUMATIC FEVER
• Immunologic lesion that occurs as a delayed
sequela of grp A streptococcal infection of the
pharynx but not the skin
• Attack rate of post-streptococcal infection
0.3-3%
• Predisposing factors: family history of RF, low
socioeconomic status (poverty, poor hygiene,
medical deprivation), 6-15 years old
 RHEUMATIC FEVER
• Diffuse inflammatory lesion of connective
tissues found mainly in the heart, brain, joints
& skin
• Valvular damage: mitral valve > aortic valve >
tricuspid > pulmonary valve
• Aschoff bodies in the atrial myocardium
(inflammatory lesions with swelling,
fragmentation of collagen fibers, altered
staining characteristics of connective tissue)
 RHEUMATIC FEVER
• History of streptococcal pharyngitis 1-5 weeks
before the onset of symptoms
• Pallor, malaise, easy fatigability, abdominal pain
• Family history of rheumatic fever
• Autoimmune or “cross-reactivity” injury between
streptococcal antigens & heart protein has been
established
• Level of auto-antibodies are too low and show
little correlation with RF
 Jones Criteria (1993)
• Major manifestations:
1. Arthritis (70%) – involves large joints
simultaneously or in succession;
responds dramatically to salicylates
2. Carditis (50%) – includes some or all
of the following in increasing order of
severity:
 Jones Criteria (1993)
a. Tachycardia (out of proportion to the fever)
– its absence makes the diagnosis of
myocarditis unlikely
b. Heart murmur of valvulitis – MR or AR
c. Pericarditis – friction rub, pericardial
effusion, chest pain, ECG changes
d. Cardiomegaly – seen on chest X-ray
e. Signs of CHF – gallop rhythm, distant heart
sounds, cardiomegaly
 Rheumatic Carditis
• Valvulitis – murmurs
• Myocarditis – unexplained
cardiomegaly or CHF or gallop
• Pericarditis – friction rub or pericardial
effusion
• Miscellaneous findings – conduction
disturbances in the ECG; 2D echo
findings
 Jones Criteria (1993)
3. Erythema marginatum (<10%) –
nonpruritic serpiginous or annular
erythematous evanescent rashes most
prominent on the trunk and inner
proximal portions of the extremities;
never on the face (disappear on
exposure to cold and reappear after a
hot shower or if covered with a blanket);
blanches on pressure
 Jones Criteria (1993)
4. Subcutaneous nodules (2-10%) –
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 joints, over the scalp or
along the spine; last for weeks
 Jones Criteria (1993)
5. Sydenham’s chorea (15%) – occurs more
often in prepubertal girls; consist of choreic
movements (spontaneous purposeless
movements followed by motor
weakness),hypotonia, emotional lability,
hyperactivity, separation anxiety, obsessions
& compulsions; may be related to dysfunction
of BG & cortical neurons; increased titers of
“antineuronal antibodies” in >90%
 Jones Criteria (1993)
• Minor manifestations:
1. Arthralgia – not considered a minor
manifestation if arthritis is present
2. Fever – at least 38.8 C
3. Elevated acute phase reactants (CRP
and ESR)
4. Prolonged PR interval on the ECG
EVIDENCE OF ANTECEDENT GROUP A
STREPTOCOCCAL INFECTION
1. History of sore throat/scarlet fever
unsubstantiated by lab. data is not adequate
evidence of recent infection.
2. A negative rapid strep antigen detection test
should be confirmed by a conventional throat
culture.
3. Streptococcal antibody tests are the most
reliable lab.evidence. The onset of the clinical
manifestations coincide with the peak of the
streptococcal antibody response.
EVIDENCE OF ANTECEDENT GROUP A
STREPTOCOCCAL INFECTION
a. Antistreptolysin 0 (ASO) titer is well standardized;
elevated in 80% of patients; 333 Todd units in
children and 250 Todd units in adults; a single low
titer does not exclude RF; a 4-fold rise in titer in 2
samples taken 10 days apart; peaks at 4-6 wks &
decreases after another 2 weeks
b. Antideoxyribonuclease B test – 240 Todd units
in children & 120 Todd units in adults
c. Streptozyme test – slide agglutination test but less
standardized
 Diagnosis
• 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
 Diagnosis
• 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.
 Diagnosis
• The following tips help in applying JC:
 Two major manifestations are always
stronger than 1 major plus 2 minor.
 Arthralgia or a a prolonged PR interval cannot
be used as a minor manifestation in the
presence of arthritis or carditis respectively.
 Absence of evidence of an antecedent grp A
strep,infection is a warning that acute RF is
unlikely (except when chorea is present).
 Diagnosis
 Clearly distinguish between Still’s
murmur and MR murmur.
 The possibility of the early suppression
of full clinical manifestation should be
sought during the history taking.
Subtherapeutic doses of aspirin may
suppress full manifestations.
 Clinical Course
1. Only carditis can cause permanent cardiac
damage. Signs of mild carditis disappear
rapidly in weeks but those of severe carditis
may last for 2-6 months.
2. Arthritis subsides within a few days to
several weeks even without treatment and
does not cause permanent damage.
3. Chorea gradually subsides in 6-7 months or
longer and usually does not cause
permanent neurologic sequelae.
 Management
• CBC, ESR, CRP, throat culture, ASO titer,
chest X-ray, ECG, 2-D echo
• Benzathine Penicillin G 0.6-1.2 M units IM to
eradicate streptococci; Pen VK 200-500 mg
QID for 10 days; Erythromycin 40 mg/kg/day
TID for 10 days if allergic to Penicillin
• Salicylates and steroids are started if definite
diagnosis is made for 6-8 wks.
 Management
• Prednisone 2mg/kg/day in 4 divided doses for
2-6 weeks indicated only in cases of severe
carditis
• Mild to moderate carditis – aspirin alone 90-
100 mg/kg/day in 4-6 doses (adequate blood
level is 20-25 mg/100 mL); continued for 4-8
weeks and 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:
• Joint symptoms
• New organic murmurs
• Changing heart size
• CHF (in the absence of long-standing valvular disease)
• Subcutaneous nodules
• Sleeping pulse rate > 100/minute
• Erythema marginatum
• Chorea
• A positive test for CRP
• Fever for at least 3 consecutive days
 RF Recurrences
• A recurrent attack refers to a new episode of RF
in a patient with previous history, and not a
relapse of the first episode
• Occur during repeated bouts of GABHS
infection, both clinical and subclinical
• Importance of secondary prophylaxis
• May be more severe
• Residual valvular disease a risk factor
 Management
• Treatment of CHF includes some or all
of the ff: complete bed rest and oxygen;
morphine 0.2 mg/kg at 4-hr interval for
severe CHF with respiratory distress;
restriction of sodium and fluid intake;
prednisone for severe carditis of recent
onset; digoxin; furosemide 1 mg/kg
every 6-12 hrs
 Management
• Sydenham’s chorea: reduce physical &
emotional stress; Benzathine Penicillin
G 1.2 M units and every 28 days for
prevention of recurrence; aspirin not
needed in patient with isolated chorea
• Presence or absence of permanent
cardiac damage determines the
prognosis
Recommended Anti-inflammatory Agents

Arthritis Mild Mod. Severe

Alone Carditis Carditis Carditis
Pred- 0 0 0 2-6
nisone
wks
Aspirin 1-2 3-4 6-8 2-4
wks wks wks mos
 VALVULAR 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
 Mitral Stenosis
• Most common valvular involvement in adults
• Thickening of leaflets and fusion of the
commissures-calcification 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.
 Mitral Stenosis
• 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
 Mitral Stenosis
• 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)
 Mitral Stenosis
• 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
 Mitral Stenosis
• 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
 Mitral Regurgitation
• 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
 Mitral Regurgitation
• 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
 Aortic Regurgitation
• 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
 Aortic Regurgitation
• 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
 IE
• 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
• At high risk: VSD, AS, TOF, PDA, MVP, children who
underwent valve replacement
• ~65% of cases: surgical or dental procedure
 Clinical Manifestations
• 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
 Clinical Manifestations
• 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
 Duke Criteria
• 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
 Duke Criteria
7. Presence of newly diagnosed clubbing
8. Splenomegaly
9. Splinter hemorrhages
10. Petechiae
11. High ESR
12. High CRP
13. Presence of central nonfeeding lines
14. Peripheral lines
15. Microscopic hematuria
 How to use the Duke criteria
• Definite endocarditis
1. 2 major criteria
2. One 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  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
 Treatment
• 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