Acquered heart diseases


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Acquered heart diseases

  1. 1. Acquired heart diseases 1
  2. 2. Common pediatric acquired heart diseases in this lecture – Rheumatic fever – Rheumatic heart disease – Infective endocarditis – Myocarditis – pericarditis 2
  3. 3. Acute rheumatic fever 3
  4. 4. Acute Rheumatic Fever• Introduction• Etiology• Epidemiology• Pathogenesis• Clinical features• Diagnosis• Treatment• Future treatments 4
  5. 5. –Acute rheumatic fever: inflammatory disease withdevastating sequelae–Link to pharyngeal infection with group A betahemolytic 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. 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. 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. 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. 9. Host factors • Age : peak incidence in children 5 - 15 years old • Previous history of acute rheumatic fever • genetic predispositiongeography • In tropics/subtropics: year-round incidence with peak in colder months 9
  10. 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. 11. • Most important antigenic proteins in external layer of cell wall M, T, R proteins 11
  12. 12. 12
  13. 13. 13
  14. 14. Clinical Features Following upper airway infection with GAS Silent period of 2 - 6 weeks Sudden onset of fever, pallor, malaise, fatigue 14
  15. 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. 16
  17. 17. • Minor manifestations Clinical features • Fever • Arthralgia Laboratory features • Elevated c-reactive protein or • Erythrocyte sedimentation rate • Prolonged PR interval on EKG 17
  18. 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. 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. 20. Carditis (continued) Clinical signs: • tachycardia • Murmurs • Cardiomegaly • Rhythm disturbances (prolonged PR interval) • Pericardial friction rubs • Cardiac failure • peripheral and pulmonary edema 20
  21. 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. 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. 23. • Diagnosis: Jones Criteria – Major criteria • polyarthritis • Carditis • Sydenham’s chorea • Erythema marginatum • Subcutaneous nodues 23
  24. 24. Diagnosis: Jones Criteria (continued) – Minor manifestations • Fever • Arthralgia • Elevated c-reactive protein or erythrocyte sedimentation rate • Prolonged PR interval on EKG 24
  25. 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. 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. 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. 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. 29
  30. 30. TreatmentSupportive – bed rest . – For carditis with heart failure • digoxin, • fluid and salt restriction, • diuretics, • OxygenAntibiotic therapy – 10 days of orally administered penicillin or erythromycin, or – single intramuscular injection of benzathine penicillin 30
  31. 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. 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. 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. 34. COMPLICATIONS.infective endocarditis 34
  35. 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. 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. 37. PREVENTION Primary Prevention – Appropriate antibiotic therapy instituted before the 9th day of symptoms of acute GAS pharyngitis 37
  38. 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. 39. - Rheumatic Heart Disease 39
  40. 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
  42. 42. MITRAL INSUFFICIENCYPathophysiology – 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. 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. 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. 45. InvestigationMild disease  Normal electrocardiogram and roentgenogramsWith 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. 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. 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. 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. 49. MITRAL STENOSISPathophysiology. 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. 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. 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. 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. 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. 54. Treatment.• Surgical valvotomy• balloon catheter mitral valvuloplasty . – indicated for symptomatic, stenotic, pliable, noncalcified valves of patients without atrial arrhythmias or thrombi 54
  55. 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. 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. 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. 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. 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. 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. 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. 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 frequentTreatment. – Treatment of left-sided lesions . – Tricuspid valvuloplasty may be required in rare cases 62
  63. 63. Infective Endocarditis 63
  65. 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. 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. 67. Etiologic agents in Pediatric Infective EndocarditisCOMMON: 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. 68. Etiology continuedUNCOMMON: NATIVE VALVE OR OTHER CARDIAC LESIONS – Streptococcus pneumoniae – Haemophilus influenzae – Coagulage-negative staphylococci – Coxiella burnetii (Q fever) – Neisseria gonorrhoeae – Brucella – Chlamydia psittacli 68
  69. 69. Etiology continued– Chlamydia trachomatis[*]– Chlamydia pneumoniae[*]– Legionella[*]– Bartonella[*]– HACEK group[†]– Streptobacillus moniliformis[*]– Pasteurella multocida[*]– Campylobacter fetus– Culture negative (6% of cases) 69
  70. 70. Etiology continuedPROSTHETIC VALVE – Staphylococcus epidermidis – Staphylococcus aureus – Viridans group streptococcus – Pseudomonas aeruginosa – Serratia marcescens – Diphtheroids – Legionella species[*] – HACEK group[†] – Fungi[‡] 70
  71. 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. 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. 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. 74. Mitral Valve Prolapse and Infective Endocarditis20 Male18 Female16141210 8 6 4 2 0 <19 20-29 30-39 40-49 50-59 >60 Rev Infect Dis 1986;8:117-137 74
  75. 75. Pathophysiology1. Turbulent blood flow disrupts the endocardium making it “sticky”2. Bacteremia delivers the organisms to the endocardial surface3. Adherence of the organisms to the endocardial surface4. Eventual invasion of the valvular leaflets 75
  76. 76. Clinical manifestationsHISTORY – Prior congenital or rheumatic heart disease – Preceding dental, urinary tract, or intestinal procedure – Intravenous drug use – Central venous catheter – Prosthetic heart valve 76
  77. 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. 78. Clinical manifestationsSIGNS – 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. 79. CLINICAL MANIFESTATIONS– Arthritis– Heart failure– Arrhythmias– Metastatic infection (arthritis, meningitis, mycotic arterial aneurysm, pericarditis, abscesses, septic pulmonary emboli)– Clubbing 79
  80. 80. Petechiae 1. Nonspecific 2. Often located on extremities or mucous membranes blpetechiaephoto.htm Harden Library for the Health SciencesPhoto credit, Josh Fierer, M.D. hardin/ Eye-Petechiae.html md/cdc/3184.html
  81. 81. Splinter Hemorrhages1. Nonspecific2. Nonblanching3. Linear reddish-brown lesions found under the nail bed4. Usually do NOT extend the entire length of the nail 81
  82. 82. Osler’s NodesAmerican College of default/pages/3b5.htm 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. 83. Janeway Lesions1. More specific2. Erythematous, blanching macules3. Nonpainful4. Located on palms and soles 83
  84. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 95. COMPLICATIONS• Four mechanisms – Embolic – Local spread of infection – Metastatic spread of infection – Formation of immune complexes – glomerulonephritis and arthritis 95
  96. 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. 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. 98. Septic Pulmonary Emboli 98
  99. 99. Septic Retinal Embolus 99
  100. 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. 101. Local spread of infection• Valve obstruction• Pericarditis• Fistulous intracardiac connections E.g. , acquired VSD 101
  102. 102. Local spread of infectionAcute S. aureus IE with perforation of the Acute S. aureus IE with mitral valve ringaortic valve and aortic valve vegetations. abscess extending into myocardium. 102
  103. 103. Metastatic spread of infection• Metastatic abscess – Kidneys, spleen, brain, soft tissues• Meningitis and/or encephalitis• Vertebral osteomyelitis• Septic arthritis 103
  104. 104. Treatment• Medical• Surgical – Intracardiac complications 104
  105. 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. 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. 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. 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. 109. Medical treatment: bacterialCommonly used antibiotic combination:Aqueous crystalline penicillin G sodium orCeftriaxone sodium plusGentamicin sulfate PlusVancomycin hydrochloride 109
  110. 110. Medical treatment: fungal• Amphotericin B• 5-fluorocytosine 110
  111. 111. Medical treatment• Treatment of complications – Heart failure • Digitalis • salt restriction • diuretic therapy 111
  112. 112. Surgical treatment• Removal of vegetations• Valve replacement 112
  113. 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. 114. Surgical treatment : Indications – recurrent emboli – new heart block – myocardial abscess – increasing size of vegetation. 114
  115. 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. 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. 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. 118. PREVENTION• Vigorous treatment of sepsis and local infections• Careful asepsis during heart surgery and catheterization . 118
  119. 119. PREVENTION: Antibiotic prophylaxisStandard general prophylaxis amoxicillinUnable to take oral meds ampicillinAllergic to penicillin clindamycin cephalexin azithromycin clarithromycinAllergic to penicillin and unable clindamycinto take oral medications cefazolin 119
  120. 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
  122. 122. • Definition• Etiology• Epidemiology• Pathophysiology• Clinical manifestation• Diagnosis• Treatment 122
  123. 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. 124. Viral myocarditisETIOLOGY 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. 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. 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. 127. CLINICAL MANIFESTATIONS• Signs and symptoms depend on – the patients 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. 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. 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. 130. TREATMENT• The role of specific treatments is controversial – Intravenous immunoglobulin (IVIG) – Corticosteroids – Specific antiviral therapy is being evaluated 130
  131. 131. Pericardial diseasesEtiology 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. 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. 133. Acute PericarditisPATHOPHYSIOLOGY• 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. 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. 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 drainageImmune 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. 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. 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. 138. THANK YOU!! 138