2. OBJECTIVES
ļ¶Define rheumatic fever and rheumatic heart disease.
ļ¶Describe the aetiology, risk factors, clinical manifestations,
diagnostic criteria and management of RF
ļ¶Explain the pathophysiology and symptoms in RHD.
ļ¶Explain the management of RHD.
ļ¶Describe about preventive methods.
3. INTRODUCTION
ā¢ Rheumatic heart disease (RHD) is the most common
acquired heart disease in children, especially in
developing countries.
ā¢ RHD is a chronic heart condition caused by rheumatic
fever that can be prevented and controlled.
5. RHEUMATIC FEVER
ā¢ Rheumatic fever is an immunologically mediated
inflammatory disorder, which occurs as a sequel to
group A streptococcal pharyngeal infection.
ā¢ Multisystem disease affecting connective tissue
particularly of the heart, joints, brain, cutaneous and
subcutaneous tissues
ā¢ RF ā not a communicable disease but results from a
communicable disease (streptococcal pharyngitis).
6. ā¢ RF ļ ļ ļ RHD (rheumatic heart disease);
a crippling disease.
ā¢ Epidemiological point of view these cannot be
separated.
[WHO CHRONICLE 1969]
ā¢ RF and RHD ļ diseases of the poor most prevalent
in underdeveloped and developing countries.
ā¢ The clinical course of rheumatic fever involves a
childhood infection with complications in adulthood
(cardiac defect).
7. EPIDEMIOLOGY
ā¢ RF and RHD is the most common cause of heart
disease in 5-30 age groups throughout the world.
ā¢ It accounts for 12-65% of hospital admissions related
to CVD in developing countries.
8. IN INDIA
ā¢ RHD is prevalent in range of 5-7/1000 in 5-15 age
groups.
ā¢ About 1 million cases of RHD
ā¢ RHD constitutes 20-30% hospital admissions due to
CVD.
ā¢ Streptococcal infections common in children living in
under āprivileged conditions and RF accounts for 1-
3% of the cases.
9. ā¢ Age: 6-15 years
ā¢ Untreated streptococcal infection
ā¢ Familial predisposition
ā¢ Over crowding
ā¢ Poverty ,Poor hygiene
ā¢ Lack of access to medical care
RISK FACTOR
10. CAUSES
ā¢ Everyday oral activities. Activities such as brushing your
teeth or chewing food can allow bacteria to enter your
bloodstream
ā¢ An infection or other medical condition. From an
infected area, such as a skin sore. Gum disease, a
sexually transmitted infection.
ā¢ Weakened immune system
ā¢ Certain dental procedures. Some dental procedures
that can cut your gums may allow bacteria to enter your
bloodstream.
11. PATHOGEN
ā¢ S. Pyogenes also known as Group A Streptococcus
(GAS) is the causative agent in Group AStreptococcal
infections including Streptococcal pharyngitis, acute
rheuamtic fever , scatlet fever, and acute
glomerulonephritis.
13. ā¢ Based on currently based evidence, RF is caused
by group A streptococcal (GAS) pharyngeal
infection.
ā¢ Postulated that series of preceding streptococcal
infection is needed to prime the immune system
prior to final infection that directly causes the
disease.
ā¢ Group A strep pharyngeal infection
precedes clinical manifestations of
ARF by 2 - 6 weeks.
PATHOPHYSIOLOGY
14. ā¢ Body produce antibodies against streptococci .
ā¢ These antibodies cross react with human tissues
because of the antigenic similarity between
streptococcal components and human connective
tissues (molecular mimicry) [there is certain
amino acid sequence that is similar btw GAS and human tissue]
ā¢ Immunologically mediated inflammation & damage
(autoimmune) to human tissues which have antigenic
similarity with streptococcal components- like heart,
joint, brain connective tissues
PATHOPHYSIOLOGY
16. STREPTOCOCCUS SORE THROAT
ā¢ Tender lymph nodes
ā¢ Strawberry tounge
ā¢ Excoriated nares( crusted lesions) in infants
ā¢ Tonsillar exudates in older children
ā¢ Abdominal pain
ā¢ More common in winter/ rainy season.
17. Clinical features of RF
ā¢ Streptococcal sore throat with
fever
ā¢ Recurrence of fever with
manifestation of acute rheumatic
fever
ā¢ Shortness of breath
18. FEATURES
Following upper airway infection with GAS
Silent period of 2 - 6 weeks
Sudden onset of fever, pallor, malaise, fatigue.
Commonly GAS streptococcal infection is
subclinical; such cases confirmed using streptococcal
antibody testing .
19. ā¢ There is no definitive test.
ā¢ Diagnosis of ARF relies on presence of combination of
typical clinical features together with evidence of the
precipitating GAS infection .
ā¢ This uncertainty led Dr.T.Duckett Jones in 1944 to
develop a set of criteriaļ Jones Criteria to aid
diagnosis.
ā¢ Now Diagnosis based on MODIFIED JONES
CRITERIA .
20. Major criteria: oint
ā¢ Migratory polyarthritis of large joint
ā¢ Usually >5 joints affected and mainly large joints
Knees, ankles, wrists, elbows, shoulders
ā¢ Redness, warmth, swelling, pain , movement limitation
ā¢ Quick to appear, lasts 3- 7 days, subside and appear
in other joint.
ā¢ Respond to salicylates and NSAIDS
ā¢ Commoner in older patients.
ā¢ Small joints and cervical spine less commonly
involved.
21. Major criteria: Carditis
ā¢ Early manifestation 90% (within 2 weeks of onset)
ā¢ Pancarditis
ā« Pericarditis : precordial pain, friction rub, ST and T
changes
ā« Endocarditis: pansystolic murmur of MR w/wo AR
murmur
ā« Others : cardiac enlargement, soft S1, S3 gallop,
congestive cardiac failure,
Carditis is the only manifestation of rheumatic fever
that leaves a sequelae & permanent damage to the
organ
22. Major criteria: odules
ā¢ Late manifestation 3- 20% ( 6 weeks after onset of
RF)
ā¢ Non- tender subcutaneous nodules on bony
prominence
ā¢ Small, painless, mobile hard lumps beneath skin.
ā¢ Most common along -
extensor surfaces of joint-Knees, elbows, wrists
ā¢ Also: on bony prominences, tendons, dorsi of feet,
oicnciputor cervical spine.
ā¢ Pinhead to almond size
24. Major criteria:
rythema marginatum
ā¢ Early manifestation <3%
ā¢ Erythematous, serpiginous, non-pruritic, macular
lesions with pale centre.
ā¢ Pink macules - Clear centrally ,serpiginous spreading
edge. (has slightly elevated red margins)
ā¢ More on trunks & limbs, Almost never on face.
ā¢ Worsens with application of heat.
ā¢ Often associated with chronic carditis.
25.
26. Major criteria :
ydenhamās Chorea
ā¢ Neurological manifestation of rheumatic fever
ā¢ Late manifestation 10-15% ( 3 months after onset)
ā¢ Semi- purposeful , jerky movements ļ deranged
speech, muscular incoordination, facial grimacing
ā« Exacerbated by stress and disappears with sleep
ā¢ More common in females
ā¢ Clinical maneuvers to elicit chorea:
ā« Demostrate milkmaidās grip
ā« Handwritingexamination
ā¢ Self- limiting ( 2-6 weeks)
27. ā¢ FEVER
ā¢ Present at onset of acute illness
ā¢ High grade fever >39ĀŗC
ā¢ Lasts for about 12 weeks, tends to
recur.
ā¢ Alternates with normal temperature.
ā¢ Weakness ,malaise , weight loss ,
anorexia
MINOR CRITERIAS
29. LAB DIAGNOSIS
ā¢ High ESR
ā¢ Anemia, leucocytosis
ā¢ Elevated C-reactive protien
ā¢ Elevated ASO or other streptococcal antibody titer
ā¢ Anti-DNase B test
ā¢ Throat culture-GABH streptococci
ā¢ ECG: prolonged PR interval
30. DIAGNOSIS- YOU SHOULD HAVE
ā¢ 2 Major + Essential criteria
OR
ā¢ 1 Major+2 Minor + Essential criteria
Along with evidence of streptococcal infection
31. OTHER FEATURES
ā¢ Aschoff bodies are nodules found in the hearts of
individuals with rheumatic fever. They result from
inflammation in the heart muscle .
ā¢
Aschoff cells
32. Antibiotic therapy:-
ļ§ Oral penicillin 500 mg BD x 10 days OR
ļ§ A single dose of Benzathine penicillin 1.2
million units I/M
ļ§ Tab. Erythromycin 250 mg BD x 10 days(in
case of penicillin allergy)
(the patient should be started on long-term antibiotic
prophylaxis)
33. Contdā¦
ā¢ Arthritis , arthralgia : Salicylates or NSAIDS (eg: aspirin)
80-100 mg/kg/day in 4-5 divided doses x 3-5wks
ā¢ Severe carditis :- Corticosteroids ( prednisolone 1-2 mg
/kg/day ;max 60 mg x 4-6 wks, then taper20-25 mg/wk)
ā¢ Sydenhamās Chorea :-
ā« Haloperidol -0.5mg/kg/day
ā« Carbamazepine or sodium valproate -15-20
mg/kg/day x1-2 wks
34. ā¢ Rheumatic fever can recur whenever the individual
experience new GABH streptococcal infection, If not
on prophylactic medicines.
ā¢ Good prognosis for older age group & if no carditis
during the initial attack
ā¢ Bad prognosis for younger children & those with
carditis with valvar lesions
PROGNOSIS
35. RHEUMATIC HEART
DISEASE
ā¢ Rheumatic heart disease is an immunologic disease
characterized by valvular damage or dysfunction followed
by one or more episodes of rheumatic fever caused by
pharyngeal infection with GAB hemolytic streptococci.
ā¢ Rheumatic Heart Disease is the permanent heart valve
damage resulting from one or more attacks of ARF.
ā¢ It is thought that 40-60% of patients with ARF will go on to
developing RHD.
ā¢ Sadly, RHD can go undetected with the result that
patients present with debilitating heart failure.
36. RHEUMATIC HEART DISEASE
ā¢ RHD is the result of damage to the heart valves which
occur after repeated episodes of ARF
ā¢ The order of frequency of involvement depends on the
hemodynamic stress placed on the various chambers
ā¢ Thus the order is mitral> aortic > tricuspid> pulmonic
ā¢ Valve incompetence is more common than stenosis
65-70% 20-25% 10% Rarely
37. The extent of the damage
depends on the heart area that
the disease strikes
38. PATHOPHYSIOLOGY
Causative agent
Group A Beta-hemolytic streptococci
Untreated strep throat
Rheumatic fever
All layers of the heart and the mitral valve become inflammed
Vegetation forms
Valvular Regurgitation
and stenosis
Heart Failure
42. VALVE CHANGES
ā¢ Leaflet thickeninig
ā¢ Commissural fusion
ā¢ Shortening and thickening of chordae
ā¢ Orifice is narrowed
43. VALVE INVOLVEMENT IN RHD
ā¢ MITRAL Valve is affected in 60 ā 70% of cases
ļ Mitral regurgitation most commonly found in
children and adolescent.
ļ Mitral stenosis represent longer term chronic
disease, commonly in adults.
ļ Most common complication in mitral stenosis is
atrial fibrillation.
45. VALVE INVOLVEMENT IN RHD
ā¢ AORTIC Valve next most commonly affected
ļ Generally associated with diseases of the mitral
valve.
ļ Aortic stenosis is one of the most common and
most serious valve disease problems in elderly
population.
ā¢ Tricuspid and pulmonary valves re much less
commonly affected
ļ Usually affected in very severs RHD when all
valves are affected.
50. ā¢ Elongated leaflets
ā« Leafletplication
ā« Leafletresection
ā¢ Holes in the leaflets
ā« Pericardial patchrepair
ā¢ Short leaflets
ā« Most often repaired bychordoplasty
51. ā¢ Repair of the chordae tendinae
ā¢ Mostly used for mitral valve
ā¢ Gore-Tex can be used to create chordae tendinae.
54. ā¢ Performed when valvuloplasty is not suitable
ā¢ Approached through a median sternotomy or mitral
valve (at times) ā right thoracotomy incision
55. ā¢ Two types of prosthetic valves :-
ā« Mechanicalvalves
ā« Tissue(biologic) valves
58. NEXT GENERATION OF
MECHANICAL VALVE: TRILEAFLET
VALVE ļµ More
physiological
ļµ Better
hemodynamic
s ācentral
blood flowā
ļµ Reduced
thrombosis
risk
59. ā¢ These are animal tissue valves: pigs(porcine),
cows(bovine).
ā¢ Viability is 7-10 yrs.
ā¢ Do not generate thrombi. So no need for long term
anticoagulation.
Indications :
ā¢ Women of child bearing age
ā¢ Others who cannot tolerate long term
anticoagulation.
- patients older than 70yrs
- patients with H/O peptic ulcer disease
60. ā¢ Obtained from cadaver tissue donations
ā¢ Used for aortic and pulmonic valve replacement
ā¢ Aortic valve and a portion of the aorta / pulmonic valve
and a portion of the pulmonary artery are harvested from
the cadaver and stored cryogenically
ā¢ Non thrombogenic
ā¢ Viability ā 10 to 15 years
61. ā¢ Patientās own pulmonic valve and a portion of the
pulmonary artery excised for use as the aortic valve
(aortic valve autograft) āRoss procedure
ā¢ Anticoagulation not required as non-thrombogenic
ā¢ Viability ā more than 20 years
ā¢ Most aortic valve auto grafts are double valve replacement
procedures
ā« Where pulmonic valve is replaced with a homograft
62. ā¢ More durable
ā¢ Can be used if the patient
has hypercalcemia,
endocarditis or sepsis.
ā¢ Do not deteriorate or
become infected as easily
as the tissue valves.
ā¢Life long anticoagulation wit
warfarin required.
ā¢Increased risk of thrombo
embolism.
ā¢Not suitable for women of
child bearing age.
63. SELECTION OF AN ARTIFICIAL
VALVE
ā¢ RISK BENEFIT RATIO
ā¢ MULTIFACTORIAL
ā« Age
ā« Site ofinvolvement
ā« Special situation(Pregnancy, Associated cardiac
abnormalities)
ā« Patientās preference(Anticoagulation, regular
follow up)
64. FACTORS
ā¢ Age
ļ¼>65 years : bioprosthesis
ļ¼<65 years : mechanical
ā¢ Anticoagulation
ļ¼Ready : mechanical
ļ¼No / contraindication : bioprosthesis
ā¢ Prosthesis
ļ¼mechanical at other site : mechanical
ļ¼Reoperation / Infective endocarditis : bioprosthesis
67. PREVENTION
PRIMARY-10 days
course of penicillin
therapy;
SECONDARY-
Secondary prevention
is directed at
preventing acute
GABHS pharyngitis in
patients at substantial
risk of recurrent
acute rheumatic fever
68. PRIMARY PREVENTION
ļ Detection and Mx. of streptococcal throat infection.
ļ Antibiotic prophylaxis in highly prevalent areas with
Benzathine penicillin.
ļ Promote health :-improve living conditions, hygiene,
avoid over crowding, access to medical facilities,
education
69. PRIMARY PREVENTION
ā¢ AIM ; Prevent the first attack of RF, by identifying all patients
with streptococcal throat infection and treating them with
pencillin.
ā¢ Theoretically simple , in practise its difficult, not feasible.
ā¢ Many infections are in apparent or if apparent are not brough
to attention of health services.
ā¢ VIABLE APPROACH; concentrate on high risk groups ie
school age children.
70. Secondary Prevention of Rheumatic Fever
(Prevention of Recurrent Attacks)
Agent Dose Mode
Benzathine penicillin G 1 200 000 U every 3 weeks* Intramuscular
Penicillin V
or
250 mg twice daily Oral
For individuals allergic to penicillin and sulfadiazine
Erythromycin 250 mg twice daily Oral
Recommendations of American HeartAssociation
71. Duration of Secondary Rheumatic Fever
Prophylaxis
Recommendations of Am erican Heart Association
Category Duration
Rheumatic fever without carditis At least 5 y or until age 18 y,(whichever
is longer)
Rheumatic fever with carditis and
heart disease (persistent valvular
disease*)
At least 10 y since last residual
episode and at least until age 40 y
sometimes lifelong prophylaxis
RF with carditis disease but no residual
heart disease (no valvular disease*)
Rheumatic fever 10 y or well into
adulthood (whichever is longer )
More severe valvular disease Post-
valve surgery cases
*Clinical or echocardiographic
evidence.
Lifelong
Recommendations of American Heart Association
72. Supportive management &
management of complications
ā¢ Bed rest
ā¢ Treatment of congestive cardiac failure:
-digitalis, diuretics
ā¢ Treatment of chorea:-diazepam or haloperidol
ā¢ Rest to joints & supportive splinting
73. PENICILLIN PROPHYLAXIS IS
NOT EASY!!!!
ā¢ Give the test dose of penicillin injection every time
the patient comes for the injection. Give the
injection deep I/M.
ā¢ The most serious adverse effect of penicillin is
anaphylaxis.
ā¢ The most common side effects are diarrhea,
maculopapular rash, urticarial rash, fever,
bronchospasm, vasculitis, , and exfoliative
dermatitis.
74. PATIENT MONITORING
ā¢ Keep epinephrine and emergency equipment at hand
in case of anaphylaxis.
ā¢ Watch closely for anaphylaxis and serum sickness.
ā¢ In long-term therapy, monitor electrolyte levels and
CBC.
ā¢ Assess neurologic status, especially for seizures and
decreasing level of consciousness.
ā¢ Watch for evidence of super infection
75. PATIENT TEACHING
ā¢ Teach patient to recognize anaphylaxis
symptoms and to contact emergency
medical services immediately.
ā¢ Tell patient drug may cause diarrhoea.
ā¢ Tell female patient that drug may make
hormonal contraceptives ineffective.
Advise her to use barrier birth control if
she wishes to avoid pregnancy.
76. NON- MEDICATED MEASURES
ā¢ Improvement of living standards.
ā¢ Breaking the poverty ādisease āpoverty cycle.
ā¢ Improvements in socio-economic conditions.
77. Ensuring that patients
understand their disease, are
informed regarding their
future and receive secondary
prophylaxis
EDUCATION
ļ¼Health education is critical at all levels
ļ¼Lack of parental awareness of the causes and
consequences of ARF/RHD is a key contributor
to poor adherence amongst children on long-term
prophylaxis
79. REFERENCES
ā¢ Suzanne C , Brenda G. Textbook of medical surgical
nursing,2003;9
ā¢ Black J M, Hawks J H . Medical surgical
nursing,2005;7
ā¢ Woods S L .Cardiac nursing,1995;3;847-850
ā¢ www.medicalcriteria.com
ā¢ www.wikipedia.com
ā¢ www.emedicine.medscape.com