The document discusses several common pediatric acquired heart diseases, with a focus on rheumatic fever and rheumatic heart disease. It describes the etiology, pathogenesis, clinical features, diagnosis, treatment and prevention of acute rheumatic fever. Long-term, recurrent rheumatic fever can lead to rheumatic heart disease, causing valvular lesions primarily of the mitral and aortic valves.

1. Acquired heart
diseases
1

2. Common pediatric acquired heart diseases in
this lecture
– Rheumatic fever
– Rheumatic heart disease
– Infective endocarditis
– Myocarditis
– pericarditis
2

3. Acute rheumatic fever
3

4. Acute Rheumatic Fever
• Introduction
• Etiology
• Epidemiology
• Pathogenesis
• Clinical features
• Diagnosis
• Treatment
• Future treatments
4

5. –Acute rheumatic fever: inflammatory disease with
devastating sequelae
–Link to pharyngeal infection with group A beta
hemolytic streptocci
–Continues to be a problem worldwide:
–sporadic outbreaks in developed countries
–frequent occurrences in developing countries
–Still gaining understanding of etiology
–link between genetic predisposition and clinical
manifestations
–Best prevention still correct use of antibiotics
5

6. • Etiology
– Rheumatogenic strains of GAS serotypes (M 1, 3,
5, 6, 18, 24)
– ⅔ of the patients with an acute episode of
rheumatic fever have a history of an upper
respiratory tract infection several weeks before
– the peak age and seasonal incidence of acute
rheumatic fever closely parallel those of GAS
infections
6

7. Epidemiology
– Most common form of acquired heart disease in
all age groups world wide
– Important cause of chronic heart disease and
death in developing world
– Estimated 30 million people suffer from ongoing
heart disease from ARF, 70% dying at average age
35 years old
– Accounts for
• 50% of all cardiovascular disease
• 50% of all cardiac admissions in many developing
countries
– Underdiagnosed and undertreated
7

8. • In some developing areas of the world, the annual
incidence is 282/100,000 population
• Males and females equally affected
• Factors associated with acute rheumatic fever
– Socioeconomic status
• Overcrowding,
• poverty,
• lack of access to medical care
– Virulence of strain of GAS
• serotypes of GAS (M types 1, 3, 5, 6, 18, 24) are associated with
ARF
8

9. Host factors
• Age : peak incidence in children 5 - 15 years old
• Previous history of acute rheumatic fever
• genetic predisposition
geography
• In tropics/subtropics: year-round incidence with peak in
colder months
9

10. Pathogenesis
• Still not clearly defined
• Group A strep pharyngeal infection precedes clinical manifestations of ARF
by 2 - 6 weeks
• Two seriously considered theories:
– the cytotoxicity theory
– the immunologic theory.
• The cytotoxicity theory
– suggests that cytotoxic effect of GAS streptolysin O toxin may be responsible
for the pathogenesis.
– Limitation : inability to explain the latent period between GAS pharyngitis
and the onset of acute rheumatic fever.
• The immunologic theory (theory of molecular mimicry)
– Antibodies made against group A strep cross-react with human tissue (e.g.,
heart, brain, joint).
– Common antigenic determinants are shared between certain components of
GAS (M protein, protoplast membrane, cell wall group A carbohydrate,
capsular hyaluronate) and specific mammalian tissues (e.g., heart, brain, joint)
10

11. • Most important antigenic proteins in external
layer of cell wall
M, T, R proteins
11

12. 12

13. 13

14. Clinical Features
Following upper airway infection with GAS
Silent period of 2 - 6 weeks
Sudden onset of fever, pallor, malaise, fatigue
14

15. Clinical Features (continued)
Characterized by:
• Arthritis
• Carditis
• Sydenham’s chorea
• Erythema marginatum
• Subcutaneous nodules
Called “major manifestations” of Jones criteria either
because of frequency or specificity
15

16. 16

17. • Minor manifestations
Clinical features
• Fever
• Arthralgia
Laboratory features
• Elevated c-reactive protein or
• Erythrocyte sedimentation rate
• Prolonged PR interval on EKG
17

18. Rheumatic Carditis
– Most serious manifestation
– occurs in about 50–60% of all cases of acute rheumatic
fever
– May lead to death in acute phase or at later stage
– Any cardiac tissue may be affected
– Endocarditis (valvulitis), is a universal finding
– Most common valvular lesion : mitral and aortic
– Serious and long-term illness is related entirely to valvular
heart disease
– Valvular insufficiency is characteristic of both acute and
convalescent stages of acute rheumatic fever,
18

19. – whereas valvular stenosis usually appears several
years or even decades after the acute illness
– mitral stenosis and aortic stenosis appear earlier
in developing countries
– Recurrent attacks of acute rheumatic fever in
patients who had carditis with the initial attack
are associated with high rates of carditis
– The major consequence of acute rheumatic
carditis is chronic, progressive valvular disease,
particularly valvular stenosis
19

20. Carditis (continued)
Clinical signs:
• tachycardia
• Murmurs
• Cardiomegaly
• Rhythm disturbances (prolonged PR interval)
• Pericardial friction rubs
• Cardiac failure
• peripheral and pulmonary edema
20

21. Carditis (continued)
Mitral and aortic regurgitation most common
• Apical systolic and basal diastolic murmurs
Pericarditis usually asymptomatic
• Occasionally causes chest pain, friction rubs or distant
heart sounds
21

22. Diagnosis
• Jones criteria
– Criteria developed to prevent overdiagnosis
– Some criticism regarding validity
– Still important as guidelines
• Probability of ARF high with
– Evidence of previous infection with streptococcal
upper airway infection and
– 2 major criteria
or
– 1 major criteria and 2 minor criteria
22

23. • Diagnosis: Jones Criteria
– Major criteria
• polyarthritis
• Carditis
• Sydenham’s chorea
• Erythema marginatum
• Subcutaneous nodues
23

24. Diagnosis: Jones Criteria (continued)
– Minor manifestations
• Fever
• Arthralgia
• Elevated c-reactive protein or
erythrocyte sedimentation rate
• Prolonged PR interval on EKG
24

25. Diagnosis: Evidence of Previous Infection
– Positive throat culture or rapid streptococcal
antigen test
– Antisteptolysin antibody
Elevated or increasing streptococcal antibody titer
– Antibodies to other strep antigens
• Anti-DNAase B, anti-hyaluronidase, anti-
streptokinase,anti-nicotinamide
25

26. • There are 3 circumstances in which the
diagnosis of acute rheumatic fever can be
made without strict adherence to the Jones
criteria.
– Chorea .
– indolent carditis.
– recurrences of acute rheumatic fever .
26

27. Diagnosis: Laboratory Studies
– None capable of diagnosing ARF: clinical diagnosis
– Can help eliminate other diseases
– Aids in diagnosis
– Monitor inflammatory process
– Evaluate extent of cardiac involvement
27

28. Diagnosis: Laboratory Studies (continued)
– CBC: not very helpful
– CRP, ESR: non-specific indicators of inflammation
but helpful for monitoring treatment
– Tests for anti-streptococcal antibody
– CXR
– EKG: prolonged PR interval in 1/3 patients
• not specific to ARF
• not associated with later cardiac sequelae
– Echocardiographic findings include pericardial
effusion, decreased ventricular contractility, and
aortic and/or mitral regurgitation
28

29. 29

30. Treatment
Supportive
– bed rest .
– For carditis with heart failure
• digoxin,
• fluid and salt restriction,
• diuretics,
• Oxygen
Antibiotic therapy
– 10 days of orally administered penicillin or
erythromycin, or
– single intramuscular injection of benzathine
penicillin
30

31. Anti-Inflammatory Therapy
• Agents such as acetaminophen can be used to control
pain and fever while the patient is being observed for
more definite signs of acute rheumatic fever or for
evidence of another disease.
31

32. • ASA in patients with typical migratory polyarthritis
and those with carditis
• The usual dose of aspirin is 100 mg/kg/day in 4
divided doses PO for 3–5 days, followed by 75
mg/kg/day in 4 divided doses PO for 4 wk.
32

33. • In patients with carditis and cardiomegaly or
congestive heart failure , corticosteroids.
» The usual dose of prednisone is 2 mg/kg/day in 4
divided doses for 2–3 wk followed by a tapering of the
dose that reduces the dose by 5 mg/24 hr every 2–3
days.
» At the beginning of the tapering of the prednisone dose,
aspirin should be started at 75 mg/kg/day in 4 divided
doses for 6 wk.
33

34. COMPLICATIONS.
infective endocarditis
34

35. PROGNOSIS
– The prognosis for patients with acute rheumatic fever depends on
• the clinical manifestations present at the time of the initial
episode,
• the severity of the initial episode
• the presence of recurrences.
– Approximately 70% of the patients with carditis during the initial
episode of acute rheumatic fever recover with no residual heart
disease;
– the more severe the initial cardiac involvement, the greater the risk
for residual heart disease.
– Patients without carditis during the initial episode are unlikely to
have carditis with recurrences.
– patients with carditis during the initial episode are likely to have
carditis with recurrences, and the risk for permanent heart damage
increases with each recurrence.
35

36. – Patients who have had acute rheumatic fever are susceptible to
recurrent attacks following reinfection of the upper respiratory tract
with GAS.
– Therefore, these patients require long-term continuous
chemoprophylaxis
– Approximately 20% of patients who present with “pure” chorea who
are not given secondary prophylaxis develop rheumatic heart disease
within 20 yr.
– Therefore, patients with chorea, even in the absence of other
manifestations of rheumatic fever, require long-term antibiotic
prophylaxis.
36

37. PREVENTION
Primary Prevention
– Appropriate antibiotic therapy instituted before the 9th day
of symptoms of acute GAS pharyngitis
37

38. Secondary Prevention
 Penicillin G benzathine , every 4 wk Intramuscular
 < 27 kg: 600,000 units
 >27 kg: 1,200,000 units
OR
 Penicillin V 250 mg, twice a day Oral
OR
 Sulfadiazine or sulfisoxazole
FOR PEOPLE WHO ARE ALLERGIC TO PENICILLIN AND SULFONAMIDE
DRUGS
 Erythromycin 250 mg, twice a day Oral
 Duration of prophylaxis
 patients with carditis with their initial episode of acute rheumatic
fever should receive antibiotic prophylaxis well into adulthood and
perhaps for life
 Patients who did not have carditis : untill they reach their early 20s
and after at least 5 yr have elapsed since their last episode of acute
rheumatic fever.
 The decision to discontinue prophylactic antibiotics depends on
epidemiologic factors such as the risk for exposure to GAS infections.
38

39. -
Rheumatic Heart Disease
39

40. • Rheumatic involvement of the valves and endocardium is
the most important manifestation of rheumatic fever .
• The valvular lesions
• Begin as small verrucae composed of fibrin and blood cells
along the borders of one or more of the heart valves
• As the inflammation subsides, the verrucae tend to
disappear and leave scar tissue.
• With repeated attacks of rheumatic fever, new verrucae
form near the previous ones, and the mural endocardium
and chordae tendineae become involved
• Valves involved
• The mitral ,
• followed by the aortic valve;
• right-sided heart manifestations are rare
40

41. Patterns of valvular disease
– MITRAL INSUFFICIENCY
– MITRAL STENOSIS
– AORTIC INSUFFICIENCY
– TRICUSPID VALVE DISEASE
– PULMONARY VALVE DISEASE
41

42. MITRAL INSUFFICIENCY
Pathophysiology
– Mitral insufficiency is the result of some loss of valvular
substance and shortening and thickening of the
chordae tendineae.
– heart failure is caused by a combination of mitral
insufficiency coupled with inflammatory disease of the
pericardium, myocardium, endocardium, and
epicardium.
– Because of the high volume load and inflammatory
process, the left ventricle becomes enlarged.
– The left atrium dilates as blood regurgitates into this
chamber.
42

43. – Increased left atrial pressure results in
pulmonary congestion and symptoms of left-
sided heart failure.
– Spontaneous improvement usually occurs with
time, even in patients in whom mitral
insufficiency is severe at the onset.
– The resultant chronic lesion is most often mild
or moderate in severity, and the patient is
asymptomatic.
– More than half of patients with acute mitral
insufficiency no longer have the mitral murmur
1 yr later.
– In patients with severe chronic mitral
insufficiency, pulmonary arterial pressure
becomes elevated, the right ventricle and
atrium become enlarged, and right-sided heart
failure subsequently develops
43

44. Clinical Manifestations
 The physical signs of mitral insufficiency depend on its severity.
 With mild disease,
 No signs of heart failure ,
 the prericordium is quiet,
 a high-pitched holosystolic murmur at the apex that radiates to the
axilla.
 With severe mitral insufficiency,
 signs of chronic heart failure .
 The heart is enlarged,
 heaving apical left ventricular impulse
 an apical systolic thrill.
 accentuated 2nd heart sound if pulmonary hypertension is present.
 A prominent 3rd heart sound .
 A holosystolic murmur at the apex with radiation to the axilla.
 short mid-diastolic Rumbling Murmur .
44

45. Investigation
Mild disease
 Normal electrocardiogram and roentgenograms
With more severe insufficiency,
 ECG
 prominent bifid P waves,
 signs of left ventricular hypertrophy,
 right ventricular hypertrophy if pulmonary hypertension is
present.
 CXR,
 prominence of the left atrium and ventricle
 Congestion of perihilar vessels,.
 Calcification of the mitral valve
 Echocardiography
 enlargement of the left atrium and ventricle, and
 Doppler studies demonstrate the severity of the mitral
regurgitation.
 Heart catheterization
 left ventriculography 45

46. Complications.
• cardiac failure
– precipitated by
» progression of the rheumatic process,
» the onset of atrial fibrillation,
» infective endocarditis.
• The effects of chronic mitral insufficiency may become
manifest after many years
» right ventricular failure
» atrial and ventricular arrhythmias
46

47. Treatment.
 Medical treatment
 In patients with mild mitral insufficiency,
 prophylaxis against rheumatic recurrences .
 Treatment of complicating
 heart failure ,
 arrhythmia
 infective endocarditis .
 Afterload-reducing agents (ACE inhibitors) may
 reduce the regurgitate volume
 preserve left ventricular function.
47

48.  Surgical treatment
 annuloplasty
 valve replacement
 Prophylaxis against bacterial endocarditis is
warranted in these patients for dental or other
surgical procedures.
 The routine antibiotics taken by these patients for
rheumatic fever prophylaxis are insufficient to
prevent endocarditis
48

49. MITRAL STENOSIS
Pathophysiology.
 Mitral stenosis of rheumatic origin results from
 fibrosis of the mitral ring,
 commissural adhesions, and
 contracture of
 the valve leaflets,
 chordae, and
 papillary muscles over time.
 It takes 10 yr or more for the lesion to become fully
established, although the process may occasionally be
accelerated.
 Rheumatic mitral stenosis is seldom encountered before
adolescence and is not usually recognized until adult life.
 Significant mitral stenosis results in increased pressure and
enlargement and hypertrophy of the left atrium, pulmonary
venous hypertension, increased pulmonary vascular resistance,
and pulmonary hypertension.
 Right ventricular and atrial dilatation and hypertrophy ensue
and are followed by right-sided heart failure
49

50. Clinical Manifestations.
 The correlation between symptoms and the severity
of obstruction is good.
 Patients with mild lesions are asymptomatic.
 More severe degrees of obstruction are associated
with exercise intolerance and dyspnea.
 Critical lesions can result in
 orthopnea,
 paroxysmal nocturnal dyspnea,
 pulmonary edema,
 atrial arrhythmias.
 pulmonary hypertension manifested by,
 functional tricuspid insufficiency,
 hepatomegaly,
 ascites,
 edema.
 Hemoptysis caused by
 rupture of bronchial and pleurohilar veins
 pulmonary infarction
50

51. clinical manifestations (continued)
• increased Jugular venous pressure in severe disease
– with heart failure,
– tricuspid valve disease,
– severe pulmonary hypertension.
• In mild disease, heart size is normal .
• moderate cardiomegaly is usual with severe mitral
stenosis.
• Cardiac enlargement can be massive when atrial
fibrillation and heart failure supervene.
• A parasternal right ventricular lift is palpable when
pulmonary pressure is high.
51

52. Clinical manifestations (Continued)
• The principal auscultatory findings are
– a loud 1st heart sound,
– an opening snap of the mitral valve, and
– a long, low-pitched, rumbling mitral diastolic murmur
with presystolic accentuation at the apex.
– The mitral diastolic murmur may be virtually absent in
patients who are in heart failure.
– A holosystolic murmur secondary to tricuspid
insufficiency may be audible.
– In the presence of pulmonary hypertension, the
pulmonic component of the 2nd heart sound is
accentuated.
– An early diastolic murmur may be caused by
associated aortic insufficiency or secondary pulmonary
valvular insufficiency
52

53. Investigation findings
 ECG :
 In mild lesions : normal
 in severe disease
 prominent and notched P waves
 right ventricular hypertrophy .
 Atrial fibrillation .
 CXR :
 In mild lesions : normal
 in moderate or severe lesions
 left atrial enlargement and prominence of the pulmonary artery
 Enlargement of right-sided heart chambers;
 calcifications of the mitral valve
 perfusion in the apices of the lung (the reverse of normal).
 Echocardiography
 distinct narrowing of the mitral orifice during diastole
 left atrial enlargement,
 Doppler can estimate the transmitral pressure gradient.
 Cardiac catheterization 53

54. Treatment.
• Surgical valvotomy
• balloon catheter mitral valvuloplasty .
– indicated for symptomatic, stenotic, pliable, noncalcified valves
of patients without atrial arrhythmias or thrombi
54

55. AORTIC INSUFFICIENCY
• In chronic rheumatic aortic insufficiency, sclerosis
of the aortic valve results in distortion and
retraction of the cusps.
• Regurgitation of blood leads to volume overload
with dilatation and hypertrophy of the left
ventricle.
• Combined mitral and aortic insufficiency is more
common than aortic involvement alone
55

56. Clinical Manifestations.
– palpitations
– Excessive sweating
– heat intolerance
– Dyspnea on exertion
– orthopnea
– pulmonary edema;
– angina precipitated by heavy exercise.
– Nocturnal attacks with
• sweating,
• tachycardia,
• chest pain, and
– hypertension may occur.
56

57. Clinical manifestations (continued)
– wide pulse pressure
– bounding peripheral pulses.
– Systolic blood pressure is elevated,
– In severe aortic insufficiency, the heart is enlarged, with a left
ventricular apical heave.
– A diastolic thrill may be present.
57

58. – The diastolic murmur heard over the upper and mid left
sternal border with radiation to the apex and the aortic
area.
– Characteristically, it has a high-pitched blowing quality and
is easily audible in full expiration.
– A systolic ejection murmur is frequent because of the
increased stroke volume.
– An apical presystolic murmur (Austin Flint murmur)
resembling that of mitral stenosis is sometimes heard and
is a result of the large regurgitant aortic flow in diastole
that prevents the mitral valve from opening fully
58

59. Investigations
– CXR
• show enlargement of the left ventricle and aorta.
– ECG
• may be normal,
• but in advanced cases it reveals signs of left ventricular
hypertrophy and strain with prominent P waves.
– The echocardiogram
• shows a large left ventricle and diastolic mitral valve flutter or
oscillation caused by regurgitant flow hitting the valve leaflets.
• Doppler studies demonstrate the degree of aortic runoff into the
left ventricle.
– Magnetic resonance angiography (MRA)
• can be useful in quantitating regurgitant volume.
– Cardiac catheterization
• is necessary only when the echocardiographic data are equivocal
59

60. Prognosis
– Mild and moderate lesions are well tolerated.
– Many adolescents with severe regurgitation are symptom free and
tolerate advanced lesions into the 3rd–4th decades.
– Unlike mitral insufficiency, aortic insufficiency does not regress.
– Patients with combined lesions during the episode of acute rheumatic
fever may have only aortic involvement 1–2 yr later.
Treatment
• medical
– afterload reducers (ACE inhibitors) and
– prophylaxis against
• recurrence of acute rheumatic fever and
• the development of infective endocarditis.
• Surgical intervention
– valve replacement
60

61. PULMONARY VALVE DISEASE
– Pulmonary insufficiency usually occurs on a
functional basis secondary to pulmonary
hypertension
– a late finding with severe mitral stenosis.
– The murmur (Graham Steell murmur) is similar
to that of aortic insufficiency, but peripheral
arterial signs (bounding pulses) are absent.
– The correct diagnosis is confirmed by two-
dimensional echocardiography and Doppler
study
61

62. TRICUSPID VALVE DISEASE
– Primary tricuspid involvement is rare after rheumatic
fever.
– Tricuspid insufficiency is more common secondary to
right ventricular dilatation resulting from unrepaired
left-sided lesions.
– The signs of tricuspid insufficiency
• prominent pulsations of the jugular veins,
• systolic pulsations of the liver,
• and a blowing holosystolic murmur at the LLSB that increases
in intensity during inspiration.
– Concomitant signs of mitral or aortic valve disease,
with or without atrial fibrillation, are frequent
Treatment.
– Treatment of left-sided lesions .
– Tricuspid valvuloplasty may be required in rare cases
62

63. Infective Endocarditis
63

64. • DEFINITION
• CLASSIFICATION
• ETIOLOGY
• EPIDEMIOLOGY
• PATHOGENESIS
• CLINICAL MANIFESTATIONS
• DIAGNOSIS
• TREATMENT
• PROGNOSIS
• COMPLICATIONS
• PREVENTION.
64

65. Definition and classification
• Infectious Endocarditis (IE): an infection of
the heart’s endocardial surface
• Classified into four groups:
– Native Valve IE
– Prosthetic Valve IE
– Intravenous drug abuse (IVDA) IE
– Nosocomial IE
65

66. Further classification
• Acute Subacute
– Affects normal heart valves • Often affects damaged
– Rapidly destructive heart valves
– Metastatic foci • Indolent nature
– Commonly Staph.
• If not treated, usually fatal
– If not treated, usually fatal
by one year
within 6 weeks
66

67. Etiologic agents in Pediatric Infective Endocarditis
COMMON: NATIVE VALVE OR OTHER CARDIAC LESIONS
– Viridans group streptococci (S. mutans, S. sanguis, S. mitis)
– Staphylococcus aureus
– Group D streptococcus (enterococcus) (S. bovis, S. faecalis)
67

68. Etiology continued
UNCOMMON: NATIVE VALVE OR OTHER CARDIAC
LESIONS
– Streptococcus pneumoniae
– Haemophilus influenzae
– Coagulage-negative staphylococci
– Coxiella burnetii (Q fever)
– Neisseria gonorrhoeae
– Brucella
– Chlamydia psittacli
68

69. Etiology continued
– Chlamydia trachomatis[*]
– Chlamydia pneumoniae[*]
– Legionella[*]
– Bartonella[*]
– HACEK group[†]
– Streptobacillus moniliformis[*]
– Pasteurella multocida[*]
– Campylobacter fetus
– Culture negative (6% of cases)
69

70. Etiology continued
PROSTHETIC VALVE
– Staphylococcus epidermidis
– Staphylococcus aureus
– Viridans group streptococcus
– Pseudomonas aeruginosa
– Serratia marcescens
– Diphtheroids
– Legionella species[*]
– HACEK group[†]
– Fungi[‡]
70

71. Epidemiology
• often a complication of congenital or
rheumatic heart disease
• can also occur in children without any
abnormal valves or cardiac malformations.
• rare in infancy; in this age group, it usually
follows open heart surgery or is associated
with a central venous line
71

72. Epidemiology
• Patients with congenital heart lesions in
which blood is ejected at high velocity
through a hole or stenotic orifice are most
susceptible to endocarditis.
• Vegetations usually form at the site of the
endocardial or intimal erosion that results
from the turbulent flow
72

73. Epidemiology
• In ≈30% of patients with infective endocarditis, a
predisposing factor is recognized.
• A surgical or dental procedure can be implicated in ≈65% of
cases in which the potential source of bacteremia is
identified.
• Poor dental hygiene in children with cyanotic heart disease
results in a greater risk for endocarditis.
• Primary bacteremia with Staphylococcus aureus is another
risk for endocarditis (10% risk).
• The occurrence of endocarditis directly after heart surgery is
relatively low, but it is frequently an antecedent event
73

74. Mitral Valve Prolapse
and Infective Endocarditis
20 Male
18 Female
16
14
12
10
8
6
4
2
0
<19 20-29 30-39 40-49 50-59 >60
Rev Infect Dis 1986;8:117-137
74

75. Pathophysiology
1. Turbulent blood flow disrupts the
endocardium making it “sticky”
2. Bacteremia delivers the organisms to
the endocardial surface
3. Adherence of the organisms to the
endocardial surface
4. Eventual invasion of the valvular
leaflets
75

76. Clinical manifestations
HISTORY
– Prior congenital or rheumatic heart disease
– Preceding dental, urinary tract, or intestinal
procedure
– Intravenous drug use
– Central venous catheter
– Prosthetic heart valve
76

77. Clinical manifestations
• SYMPTOMS
– Fever
– Chills
– Chest and abdominal pain
– Arthralgia
– myalgia
– Dyspnea
– Malaise
– Night sweats
– Weight loss
– CNS manifestations (stroke, seizures, headache
77

78. Clinical manifestations
SIGNS
– Elevated temperature
– Tachycardia
– Vascular- Embolic phenomena (Roth spots,
petechiae, splinter nail bed hemorrhages, CNS
or ocular lesions)
– Immune complex phenomena
(glomerulonephritis, arthritis,Osler nodes, Roth
spot, )
– Janeway lesions
– New or changing murmur
– Splenomegaly 78

79. CLINICAL MANIFESTATIONS
– Arthritis
– Heart failure
– Arrhythmias
– Metastatic infection (arthritis, meningitis,
mycotic arterial aneurysm, pericarditis,
abscesses, septic pulmonary emboli)
– Clubbing
79

80. Petechiae
1. Nonspecific
2. Often located on extremities
or mucous membranes
dermatology.about.com/.../
blpetechiaephoto.htm
Harden Library for the Health Sciences
Photo credit, Josh Fierer, M.D. www.lib.uiowa.edu/ hardin/ 80
medicine.ucsd.edu/clinicalimg/ Eye-Petechiae.html md/cdc/3184.html

81. Splinter Hemorrhages
1. Nonspecific
2. Nonblanching
3. Linear reddish-brown lesions found under the nail bed
4. Usually do NOT extend the entire length of the nail
81

82. Osler’s Nodes
American College of Rheumatology
webrheum.bham.ac.uk/.../ default/pages/3b5.htm www.meddean.luc.edu/.../
Hand10/Hand10dx.html
1. More specific
2. Painful and erythematous nodules
3. Located on pulp of fingers and toes
4. More common in subacute IE
82

83. Janeway Lesions
1. More specific
2. Erythematous, blanching macules
3. Nonpainful
4. Located on palms and soles
83

84. LABORATORY
– Positive blood culture
– Elevated erythrocyte sedimentation rate; may be
low with heart or renal failure
– Elevated C-reactive protein
– Anemia
– Leukocytosis
– Immune complexes
– Hypergammaglobulinemia
– Hypocomplementemia
84

85. – Cryoglobulinemia
– Rheumatoid factor
– Hematuria
– Renal failure: azotemia, high creatinine
(glomerulonephritis)
– Chest radiograph: bilateral infiltrates, nodules,
pleural effusions
– Echocardiographic evidence of valve
vegetations, prosthetic valve dysfunction or
leak, myocardial abscess, new-onset valve
insufficiency
85

86. Diagnostic (Duke) Criteria
• Definitive infective endocarditis
– pathologic criteria
• microorganisms or pathologic lesions:
demonstrated by culture or histology in a
vegetation, or in a vegetation that has embolized,
or in an intracardiac abscess
– clinical criteria (see below)
• two major criteria, or one major and three minor
criteria, or five minor criteria
86

87. Diagnostic (Duke) Criteria
• Possible infective endocarditis
– findings consistent of IE that fall short of “definite”, but
not “rejected”
• Rejected
– firm alternate Dx for manifestation of IE
– resolution of manifestations of IE, with antibiotic
therapy for 4 days
– no pathologic evidence of IE at surgery or autopsy,
after antibiotic therapy for 4 days
87

88. Diagnostic (Duke) Criteria
• Major criteria
– Positive blood culture for IE
– Echocardiographic evidence of endocardial involvement
• Minor criteria
– Predisposing conditions (heart condition or IV drug use)
– Fever of 100.40F or higher
– Embolic – vascular phenomena
– Immunologic phenomena
– Microbiologic evidence not meeting major criteria
– Echocardiographic evidence not meeting major criteria
88

89. The Essential Blood Test
• Blood Cultures
– Minimum of three blood samples1
– Three separate venipuncture sites
– Obtain 10-20mL in adults and 0.5-5mL in children2
• Positive Result
– Typical organisms present in at least 2 separate samples
– Persistently positive blood culture (atypical organisms)
• Two positive blood cultures obtained at least 12 hours apart
• Three or a more positive blood cultures in which the first and last
samples were collected at least one hour apart
89

90. Duke’s Major Criteria
– Typical microorganism (strep viridans, strep bovis,
HACEK group, staph aureus or enterococci in the
absence of a primary locus)
90

91. Duke’s Major Criteria
• Evidence of endocardial involvement
(Echocardiography)
– intracardiac mass on a valve or other site
– regurgitant flow near a prosthesis
– abscess
– partial dehiscence of prosthetic valves
– new valve regurgitant flow
91

92. Risk for Endocarditis
• High risk
– prosthetic cardiac valve
– prior episodes of endocarditis
– complex cyanotic congenital cardiac defect
• transposition of great vessels,
• tetralogy of Fallot,
• single ventricle
– surgically constructed systemic -pulmonary
shunts or conduits
92

93. Risk for Endocarditis
• Moderate risk
– patent ductus arteriosus
– VSD, primum ASD
– coarctation of the aorta
– bicuspid aortic valve
– hypertrophic cardiomyopathy
– acquired valvular dysfunction
– MVP with mitral regurgitation
93

94. Risk for Endocarditis
• Low risk (negligible risk)
– isolated secundum atrial septal defect
– ASD, VSD, or PDA >6 months past repair
– “innocent” or functional murmur
– mitral valve prolapse without regurgitation
– previous coronary artery bypass surgery
94

95. COMPLICATIONS
• Four mechanisms
– Embolic
– Local spread of infection
– Metastatic spread of infection
– Formation of immune complexes –
glomerulonephritis and arthritis
95

96. Embolic complications
• Occur in up to 40% of patients with IE
• Predictors of embolization
– Size of vegetation
– Left-sided vegetations
– Fungal pathogens, S. aureus, and Strep. Bovis
• Incidence decreases significantly after
initiation of effective antibiotics
96

97. Embolic complications
• Stroke
• Myocardial Infarction
– Fragments of valvular vegetation or vegetation-
induced stenosis of coronary ostia
• Ischemic limbs
• Hypoxia from pulmonary emboli
• Abdominal pain (splenic or renal infarction)
97

98. Septic Pulmonary Emboli
http://www.emedicine.com/emerg/topic164.htm
98

99. Septic Retinal Embolus
99

100. Local spread of infection
• Heart failure
– Extensive valvular damage
– Myocardial abscesses
– Toxic myocarditis
• Paravalvular abscess (30-40%)
– Most common in aortic valve, IVDA, and S. aureus
– Higher rates of embolization and mortality
• Arrythmias
• Heart block
• Mycotic aneurysms
• Rupture of sinus of Valsalva
100

101. Local spread of infection
• Valve obstruction
• Pericarditis
• Fistulous intracardiac connections
E.g. , acquired VSD
101

102. Local spread of infection
Acute S. aureus IE with perforation of the Acute S. aureus IE with mitral valve ring
aortic valve and aortic valve vegetations. abscess extending into myocardium.
102

103. Metastatic spread of infection
• Metastatic abscess
– Kidneys, spleen, brain, soft tissues
• Meningitis and/or encephalitis
• Vertebral osteomyelitis
• Septic arthritis
103

104. Treatment
• Medical
• Surgical
– Intracardiac complications
104

105. Medical treatment
• Pre-antibiotic era - a death sentence
• Antibiotic era
– microbiologic cure in majority of patients
• Parenteral antibiotics
– High serum concentrations to penetrate
vegetations
– Prolonged treatment to kill dormant bacteria
clustered in vegetations
105

106. Medical treatment
• A total of 4–6 wk of treatment is
recommended, with serumcidal levels by tube
dilution of at least 1:8 after a dose of
antibiotic.
• Depending on the clinical and laboratory
responses, antibiotic therapy may require
modification and, in some instances, more
prolonged treatment is required.
106

107. Medical treatment
• In nonstaphylococcal disease, bacteremia usually
resolves in 24–48 hr, whereas fever resolves in
5–6 days with appropriate antibiotic therapy.
• Resolution with staphylococcal disease takes
longer
107

108. Medical treatment
• Determinants of choice of antibiotics
– Type of endocarditis
• Native valve
• Prosthetic valve
– Etiologic agent
– Sensitivity of the etiologic agent to drugs
108

109. Medical treatment: bacterial
Commonly used antibiotic combination:
Aqueous crystalline penicillin G sodium
or
Ceftriaxone sodium
plus
Gentamicin sulfate
Plus
Vancomycin hydrochloride
109

110. Medical treatment: fungal
• Amphotericin B
• 5-fluorocytosine
110

111. Medical treatment
• Treatment of complications
– Heart failure
• Digitalis
• salt restriction
• diuretic therapy
111

112. Surgical treatment
• Removal of vegetations
• Valve replacement
112

113. Surgical treatment : Indications
– severe aortic or mitral valve involvement with
intractable heart failure
– mycotic aneurysm
– rupture of an aortic sinus
– dehiscence of an intracardiac patch
– failure to sterilize the blood despite adequate
antibiotic level
113

114. Surgical treatment : Indications
– recurrent emboli
– new heart block
– myocardial abscess
– increasing size of vegetation.
114

115. PROGNOSIS
• In the pre-antibiotic era, infective
endocarditis was a fatal disease.
• Despite the use of antibiotic agents,
mortality remains at 20–25%.
• Serious morbidity occurs in 50–60% of
children with documented infective
endocarditis
115

116. Poor prognostic factors
• Female • Diabetes mellitus
• S. aureus • Low serum albumen
• Vegetation size • Heart failure
• Aortic valve • Paravalvular abscess
• Prosthetic valve • Embolic events
• Older age
116

117. PREVENTION.
• In patients with high or moderate risk heart
conditions, antimicrobial prophylaxis before
various procedures:
– dental and oral procedures
– surgery of the upper respiratory tract
– Surgery of the GI tract
– Urinary tract procedures
• Continuing education regarding the important
of prophylaxis
• Proper general dental care and oral hygiene
117

118. PREVENTION
• Vigorous treatment of sepsis and local
infections
• Careful asepsis during heart surgery and
catheterization .
118

119. PREVENTION:
Antibiotic prophylaxis
Standard general prophylaxis amoxicillin
Unable to take oral meds ampicillin
Allergic to penicillin clindamycin
cephalexin
azithromycin
clarithromycin
Allergic to penicillin and unable clindamycin
to take oral medications cefazolin
119

120. References
• Kliegman: Nelson Textbook of Pediatrics, 18th edition
• Prevention of bacterial endocarditis. Recommended by the American Heart
Association.Dajani AS, Taubert KA, Wilson W, et al. Circulation 1997;96:358-
366
• New Criteria for diagnosis of infective endocarditis: Utilization of specific
echocardiographic findings.
Durack DT, Lukes AS, Bright DK, et al. Am J Med 1994;96:200-209
• Antibiotic treatment of adults with infective endocarditis due to strptococci,
enterococci, staphlococci, and HACEK microorganisms. Wilson WR, Karchmer
AW, Dajani AS. JAMA 1995;274:1706-1713
120

121. MYOCARDITIS AND
PERICARDITIS
121

122. • Definition
• Etiology
• Epidemiology
• Pathophysiology
• Clinical manifestation
• Diagnosis
• Treatment
122

123. Definition:
Myocarditis refers to inflammation, necrosis, or myocytolysis of the
myocardium.
Causes of myocarditis
–Infectious
•viral infections ( most common)
•bacterial infections
•rickettsial infections
•parasitic infections
•fungal infections
–Connective tissue
–Granulomatous
–Toxic
–Idiopathic processes
123

124. Viral myocarditis
ETIOLOGY AND EPIDEMIOLOGY.
• The most common causative agents in children are
– adenovirus
– coxsackievirus B
– other enteroviruses
• The true incidence in children unknown because many mild
cases go undetected.
• typically a sporadic, but occasionally an epidemic illness.
124

125. PATHOPHYSIOLOGY
• Acute viral myocarditis is characterized by
– cellular infiltrates,
– cell degeneration and necrosis
– fibrosis.
– persistence of viral RNA or DNA in the myocardium
• Chronic viral myocarditis
– persistence of viral RNA or DNA in the myocardium
– activation of host immune response against viral-host
antigenic alterations
– Cytotoxic lymphocytes and natural killer cells, together
with persistent and possibly defective viral replication,
impair myocyte function .
– the persistent viral infection may alter the expression of
MHC antigens, with resultant exposure of neoantigens to
the immune system.
125

126. PATHOPHYSIOLOGY
– some viral proteins may share antigenic
epitopes with host cells
– Cytokines such as tumor necrosis factor-α and
interleukin 1 may be released
– The net final result of chronic viral-associated
inflammation is often dilated cardiomyopathy.
126

127. CLINICAL MANIFESTATIONS
• Signs and symptoms depend on
– the patient's age
– the acute or chronic nature of the infection.
• In early infancy,
– viral myocarditis often occurs as an acute, fulminant disease;
– A neonate may initially have fever, severe heart failure, respiratory
distress, cyanosis, distant heart sounds, weak pulses, tachycardia , mitral
insufficiency , a gallop rhythm, acidosis, and shock.
– Evidence of viral hepatitis, aseptic meningitis, and an associated rash
may be present.
– In the most fulminant form, death may occur within 1–7 days of the
onset of symptoms
• In toddlers and young children,
– occurs as an acute, but less fulminant myopericarditis;
• In older children and adolescents,
– often asymptomatic and comes to clinical attention primarily as a
precursor to idiopathic dilated cardiomyopathy
127

128. DIAGNOSIS
– The sedimentation rate,
– heart enzymes (creatine phosphokinase, lactate
dehydrogenase),
– brain natriuretic peptide (BNP)
– Serum viral titers
– PCR of ventricular biopsy
– Echocardiography
– Endomyocardial biopsy
– PCR can identify specific viral RNA or DNA
128

129. TREATMENT
• Supportive measures for severe congestive
heart failure or cardiogenic shock .
– Dopamine, epinephrine, and milrinone .
– All inotropic agents, including digoxin, should be
used with caution
– When used, digoxin is often started at half the
normal dosage
• Treatment of arrhythmias
• ECMO
• LVAD
129

130. TREATMENT
• The role of specific treatments is controversial
– Intravenous immunoglobulin (IVIG)
– Corticosteroids
– Specific antiviral therapy is being evaluated
130

131. Pericardial diseases
Etiology of Pericardial Diseases
 CONGENITAL ANOMALIES
 Absence (partial, complete)
 Cysts
 Mulibrey nanism (muscle, liver, brain, eye) with congenital pericardial
thickening and constriction
 INFECTIOUS
 Viral (coxsackievirus B, Epstein-Barr virus influenza, adenovirus)
 Bacterial (streptococcus, pneumococcus, staphylococcus, meningococcus,
mycoplasma, tularemia, listeria, leptospirosis)
 Immune complex (meningococcus, Haemophilus influenzae)
 Tuberculosis
 Fungal (histoplasmosis, actinomycosis)
 Parasitic (toxoplasmosis, echinococcosis)
 CONNECTIVE TISSUE DISEASES
 Rheumatoid arthritis
 Rheumatic fever
 Systemic lupus erythematosus
 systemic sclerosis
 Sarcoidosis
 Wegener granulomatosis
131

132. • METABOLIC-ENDOCRINE
– Uremia
– Hypothyroidism
– Chylopericardium
• HEMATOLOGY-ONCOLOGY
– Bleeding diathesis
– Malignancy (primary, metastatic)
– Radiotherapy-induced
• OTHER
– Trauma (penetrating or blunt injury)
– Iatrogenic (catheter related)
– Postpericardiotomy (cardiac surgery)
– Aortic dissection Idiopathic
– Familial Mediterranean fever
– Smallpox vaccination
– Pancreatitis
– Löffler syndrome
132

133. Acute Pericarditis
PATHOPHYSIOLOGY
• Pericardial inflammation results in an accumulation of fluid in
the pericardial space.
• The fluid varies according to the cause of the pericarditis and
may be serous, fibrinous, purulent, or hemorrhagic.
• Cardiac tamponade occurs when the amount of pericardial
fluid reaches a level that compromises cardiac function.
• In a healthy child, 10–15 mL of fluid is normally found in the
pericardial space,
• whereas in an adolescent with pericarditis, fluid in excess of
1,000 mL may accumulate.
• For every small increment of fluid, pericardial pressure rises
slowly; once a critical level is reached, pressure rises rapidly
and culminates in severe cardiac compression.
• Inhibition of ventricular filling during diastole, elevated
systemic and pulmonary venous pressure, and, if untreated,
eventual compromised cardiac output and shock occur
133

134. CLINICAL MANIFESTATIONS.
• The first symptom is often characterstic precordial pain .
• The major complaint is a sharp, stabbing sensation over the
precordium and often the left shoulder and back
• Cough, dyspnea, abdominal pain, vomiting, and fever
• In younger children atypical symptoms may predominate.
• symptoms or signs associated with other organ involvement
depend on the cause of the pericarditis.
• Friction rub when the effusion is small.
• Muffled heart sounds when the effusion is large
• Narrow pulses, tachycardia, neck vein distention, and
increased pulsus paradoxus
134

135. Purulent Pericarditis.
 Most often associated with bacterial infections such as pneumonia,
epiglottitis, meningitis, or osteomyelitis.
 The most common organisms implicated in purulent pericarditis are
Staphylococcus aureus, Haemophilus influenzae type b, and Neisseria
meningitidis
 Generally, signs and symptoms of the primary infection are present.
 Once the purulent process is established, if untreated, the course is
fulminant and terminated by acute cardiac tamponade and death.
 Treatment
 appropriate intravenous antibiotics
 closed pericardial aspiration
 Open pericardial drainage
Immune complex–mediated pericarditis (sterile)
• may occur 5–7 days after the initiation of therapy for severe systemic or
meningeal infection with meningococcus or H. influenzae type b.
 Therapy includes anti-inflammatory agents and pericardiocentesis if
tamponade develops.
135

136. Acute Rheumatic Fever.
– Pericarditis occurs in acute rheumatic fever as a
component of pancarditis .
– It is associated with acute valvulitis.
– Pericarditis and other manifestations of acute rheumatic
pancarditis respond to therapy with steroids.
Juvenile Rheumatoid Arthritis.
– Pericarditis is a common manifestation of juvenile
rheumatoid arthritis
– Rarely, it may be the only manifestation and precede the
onset of arthritis by months or even years.
– Differentiation of rheumatoid pericarditis from that seen
with other collagen vascular disease, particularly lupus
erythematosus, may be difficult.
– Treatment consists of steroids or salicylates, which may be
needed on a long-term basis
136

137. Uremia.
– occurs only in prolonged severe renal failure
– results from chemical irritation of the pericardium
– It may culminate in cardiac tamponade or cause recurrent
hypotension during hemodialysis.
– If adequate relief of uremic pericarditis does not occur with
hemodialysis, pericardiectomy is recommended.
Neoplastic Disease.
– Neoplastic pericardial effusion is seen in patients with
Hodgkin disease, lymphosarcoma, and leukemia
– Results from direct neoplastic invasion of the pericardium.
– Cardiac tamponade may occur late in the course of the
illness.
– Patients with malignancy may also acquire pericarditis as a
result of radiation therapy to the mediastinum.
137

138. THANK YOU!!
138