RHEUMATIC HEART DISEASE

1. RHEUMATIC HEART DISEASE
ANIKA DAHAL
LECTURER
MMIHS

2. Rheumatic heart disease
• Rheumatic Heart Disease is the permanent
heart valve damage resulting from one or
more attacks of ARF.
• It is thought that 40-60% of patients with
ARF will go on to developing RHD.
• The commonest valves affecting are the
mitral and aortic, in that order. However all
four valves can be affected

3. • Rheumatic heart disease is the most
serious complication of rheumatic fever.
• Acute rheumatic fever follows 0.3% of
cases of group A beta-hemolytic
streptococcal pharyngitis in children.

4. • As many as 39% of patients with acute
rheumatic fever may develop varying
degrees of pancarditis with associated
valve insufficiency, heart failure,
pericarditis, and even death.

5. • With chronic rheumatic heart disease,
patients develop valve stenosis with
varying degrees of regurgitation, atrial
dilation, arrhythmias, and ventricular
dysfunction.
• Chronic rheumatic heart disease remains
the leading cause of mitral valve stenosis
and valve replacement in adults.

6. • Acute rheumatic heart disease often
produces a pancarditis characterized by
endocarditis, myocarditis, and
pericarditis. Endocarditis is manifested
as valve insufficiency.
• The mitral valve is most commonly and
severely affected (65-70% of patients),
and the aortic valve is second in
frequency (25%). The tricuspid valve is
deformed in only 10% of patients and is
almost always associated with mitral and
aortic lesions.

7. • The pulmonary valve is rarely affected.
Severe valve insufficiency during the acute
phase may result in congestive heart
failure and even death (1% of patients).
• Whether myocardial dysfunction during
acute rheumatic fever is primarily related
to myocarditis or is secondary to
congestive heart failure from severe valve
insufficiency is not known. Pericarditis,
when present, rarely affects cardiac
function or results in constrictive
pericarditis.

8. Epidemiology
• The incident of rheumatic fever following
streptococcal throat infection in the western
countries is 0.3 percent in the general
population and 1 to 3% in crowded population.
• The commonest age group is 5 to 15 years
• The both sexes are nearly equally affected
• Mitral valve disease and chorea are more
common in female

9. • Aortic valve involvement is seen more
often seen in males.
• Rheumatic heart disease consistutes
from 16.5 to 50% of the cardiac patient
in a hospial.

10. Predisposing factors
• Poor socio-economic conditions leading
to unhygienic living conditions and over
crowded households help spread
streptococcal infections.
• Under and poor nutrition alter the
immunological response and increase
susceptibility.

11. ETIOLOGY
Rheumatic fever

12. • The etiology of Rheumatic fever is unknown. A
strong association with beta hemolytic
streptococci of group A is indicated by a number
of observations:
• A history of preceeding sore throat (50% of
patients)
• Epidemics of streptococcal infection followed by
higher incidence of RF
• Seasonal variation of RF and streptococcal
infection are identical

13. • Streptococcal infection is followed by
recurrence of RF
• Penicillin prophylaxis for streptococcal
infection prevents recurrences of RF in
those patients who had it earlier
• More than 85% of patients with RF show
elevated levels of anti-streptococcal
antibody titer

14. Streptococcus infection :Bacteremia,
Impetigo, Cellulitis, Pneumonia,
Tonsillitis, Septic arthritis, Joint pain,
Osteomyelitis

15. PATHOPHYSIOLOGY
• Rheumatic heart disease is most dangerous
complication of Rheumatic fever. Bacteria called
streptococcus pyogens lurk in body.
• Attack skin cell lining the nose, throat and
windpipe causing sore throat, headache, fever.
• The disease progresses to Rheumatic heart
disease leads to an auto immune attack in body.

16. CONTD…
• This means that body begins to attack the
narrowing structure in the heart
• As blood flows, bacteria can infect the heart
valves, leading to symptoms such as
• Irregular heartbeat, valvular stenosis, or
narrowing, infection, arthritis, movement
disorders, stomach pain, rashes, clots and low
blood count.

17. • Rheumatic fever results from humoral and
cellular-mediated immune responses
occurring 1-3 weeks after the onset of
streptococcal pharyngitis.
• Streptococcal proteins display molecular
mimicry recognized by the immune system,
especially bacterial M-proteins and human
cardiac antigens such as myosin and
valvular endothelium. Antimyosin antibody
recognizes laminin, an extracellular matrix
alpha-helix coiled protein, which is part of
the valve basement membrane structure.

18. Clinical Features
• Clinical presentations of rheumatic
heart disease is made after confirming
antecedent rheumatic fever. The
modified Jones criteria (revised in
1992) provide guidelines for the
diagnosis of rheumatic fever.

19. CLINICAL FEATURES
• Jones’ criteria for Rheumatic Fever
• Diagnostic : 1 Required Criteria and 2
Major Criteria and 0 Minor Criteria
• Diagnostic : 1 Required Criteria and 1
Major Criteria and 2 Minor Criteria

20. Required Criteria Evidence of
antecedent Strep infection:
• ASO / Strep antibodies / Strep group A
throat culture / Recent scarlet fever /
anti-deoxyribonuclease B / anti-
hyaluronidase

21. Major Diagnostic Criteria
• Carditis
• Polyarthritis
• Chorea
• Erythema marginatum
• Subcutaneous Nodules

22. 1. PolyArthritis: Flitting and
fleeting (transient) migratory
polyarthritis, involving major
joints.
• Commonly involved joints- knee,
ankle, elbow, and wrist.
• Occurs in 80%, involved joints are
exquisitely tender.
• In children below 5 years. It is
mild but carditis is more
prominent.
• Arthritis do not progress to
chronic disease.

23. CONTD….
2. Carditis : Manifest as pancarditis (
endocarditis, myocarditis, pericarditis).
• Carditis is the only manifestation of
rheumatic fever that leaves a sequelae and
permanent damage to the organ.
• Valvulitis occur in acute phase.
• Chronic phase fibrosis, calcification and
stenosis of heart valves.

24. 3. Sydenham Chorea :
• Late neurological manifestation that typically
appears at least 3 months after the episode
of ARF when all other signs may have
disappeared.
• Occurs up to one third of cases and is more
common in female.
• Emotional lability may be the first feature and
is typically followed by purposeless involuntary
choreiform movement of the hands, feet or
face. speech may be explosive and halting

25. • ( Reason for sydenham chorea: A major
manifestation of acute rheumatic fever,
Sydenham's chorea is a result of an autoimmune
response that occurs following infection by group A
β-hemolytic streptococci that destroys cells in the
corpus striatum of the basal ganglia.
• Molecular mimicry to streptococcal antigens leading
to an autoantibody production against the basal
ganglia has long been thought to be the main
mechanism by which chorea occurs in this
condition)

26. 4. Erythema Marginatum:
• Occurs in 5%.
• Unique, transient lesions of 1-2 inches
in size.
• Pale center with red irregular margin.
• More on trunk and limbs and non itchy.
• Worsens with application of heat.

27. • (Reason for erythema marginatum :Cell-
mediated immunity appears to be responsible
for the destruction of epithelial cells. Early in
the disease process, the epidermis becomes
infiltrated with CD8 T lymphocytes and
macrophages, whereas the dermis displays a
slight influx of CD4 lymphocytes.
• These immunologically active cells are not
present in sufficient numbers to be directly
responsible for epithelial cell death. Instead,
they release diffusable cytokines, which
mediate the inflammatory reaction and
resultant apoptosis of epithelial cells. )

28. CONTD…
5. Subcutaneous nodules:
• occurs in 10%.
• Painless pea shaped, palpable nodules.
• Size from pinhead to an almond
• Mainly over joints, spine, scapula, and scalp.
• Associated with strong seropositivity.

29. • ( reason for subcutaneous nodules: is a
densely packed layer of macrophages
and fibroblasts which tend to be
arranged radially)

30. Minor Diagnostic Criteria Fever
• Arthralgia
• Previous rheumatic fever or rheumatic
heart disease
• Acute phase reactions: ESR / CRP /
Leukocytosis increased
• Prolonged PR interval

31. • After a diagnosis of rheumatic fever is
made, symptoms consistent with heart
failure, such as difficulty breathing,
exercise intolerance, and a rapid heart
rate out of proportion to fever, may be
indications of carditis and rheumatic
heart disease.

32. • Physical findings in a patient with rheumatic heart
disease include cardiac and noncardiac
manifestations of acute rheumatic fever. Some
patients develop cardiac manifestations of chronic
rheumatic heart disease.
Cardiac manifestations of acute rheumatic fever
• Pancarditis is the most serious and second most
common complication of rheumatic fever (50%). In
advanced cases, patients may complain of dyspnea,
mild-to-moderate chest discomfort, pleuritic chest
pain, edema, cough, or orthopnea

33. • The murmurs of acute rheumatic fever are typically
due to valve insufficiency. Heart failure may
develop secondary to severe valve insufficiency or
myocarditis.
• The physical findings associated with heart failure
include tachypnea, orthopnea, jugular venous
distention, rales, edema, and swelling of the
peripheral extremities.
• A pericardial friction rub indicates that
pericarditis is present.
• Increased cardiac dullness to percussion and
muffled heart sounds are consistent with
pericardial effusion.

34. Cardiac manifestations of chronic
rheumatic heart disease
• Valve deformities, thromboembolism,
cardiac hemolytic anemia, and atrial
arrhythmias are the most common
cardiac manifestations of chronic
rheumatic heart disease.

35. • Chronic valvular heart disease develops in at
least half of those affected by rheumatic
fever with carditis.
• Mitral valve is affected in more than 90% of
cases: the aortic valve is the next most
frequently affected, followed by the
tricuspid and then the pulmonary valve.

36. – Mitral Regurgitation
– Mitral Stenosis
– Aortic Regurgitation
– Aortic Stenosis
– Tricuspid Regurgitation
– Tricuspid stenosis

37. Mitral regurgitation
• Commonest manifestation
• Rheumatic disease is the principal cause of
MR
Mitral valve prolapse
Also known as “floppy” mitral valve. Progressive
elongation of the chordae tendinae may lead
to increasing MR and if chordae rupture
occurs, regurgitation may suddenly become
severe

38. Symptoms
• Dyspnea
• Fatigue
• Palpitation
• Oedema

39. Signs
• Atrial fibrillation/flutter
• Cardiomegaly
• Apical pansystolic murmur + thrill
• Crepitations, pulmonary oedema
• Signs of RHF and pulmonary
hypertension

40. Rheumatic mitral stenosis
• Almost rheumatic in origin
• Although in the elderly it can be caused by heavy
calcification of the mitral valve apparatus
• Mitral valve orifice is slowly diminished by
progressive fibrosis, calcification of the valve
leaflets and fusion of cusps and subvalvular
apparatus

41. Pathophysiology
Rheumatic endocarditis
Scarring of the valve leaflets & the chordae
tendinae
Contractures & adhesions develop between the
commissure (the junctional areas) giving “fish
mouth” appearance

43. Pathophysiology cont…
Obstruction of blood flow & creation of a
pressure difference between the left atrium & the
left ventricle during asystole.
Left atrial pressure volume elevations cause
increased pulmonary vasculature pressure &
subsequent hypertrophy vessels.
In chronic mitral stenosis, pressure overload
occurs in the left atrium, the pulmonary bed, &
the right ventricle.

44. • Symptoms
– Breathlessness
– Fatigue
– Oedema, ascitis
– Palpitation
– Haemoptysis
– Cough
– Chest pain

45. Signs
• AF
• Mitral Facies
• Auscultation – loud first heart sound,
opening snap, mid diastolic murmur
• Crepititions, pulmonary edema, effusion

46. Aortic regurgitation
• May be due to disease of aortic valve
cusps or dilatation of the aortic root.
Symptoms
• Often asymptomatic , palpitations(Mild-
moderate AR)

47. Severe
• Breathlessness
• Angina

48. Signs
• Large volume or “collapsing pulse”
• Quincke’s sign
• Traube sign
• Musset’s sign
• Murmur (early diastolic, systolic)

49. Aortic Stenosis
• Second most frequently affected by RF
and commonly both aortic and mitral
valve are affected

50. Symptoms
• Exertional dyspnea
• Angina
• Episodes of acute pulmonary edema

51. Signs
• Ejection systolic murmur
• Narrow pulse pressure
• Crepitations

52. TRICUSPID REGURGITATION
• 20 to 50% of RHD patient
Symptom
• Pain in the right hypochondrium
• Fatigue
• Oedema
Signs
• Raised JVP
• Systolic pulsation of liver
• Pansystolic murmur

53. INVESTIGATION
• ECG
– Prolonged PR interval
– AF
– LV hypertrophy, RV hypertrophy and LA
hypertrophy

54. • Chest Xray
– Cardiomegaly
– Sign of pulmonary venous congestion
• ECHO to check the heart valves for any
damage or infection and assessing if
there is heart failure. This is the most
useful test for finding out if RHD is
present.

55. . Laboratory examination
• Throat culture: to determine presence of
streptococcal organisms.
• WBC count and ESR ,CRP – increased
during acute phase of infection.
• Elevated antistreptolycin- o (ASO) titer.

56. Management
• Medical therapy in rheumatic heart
disease includes attempts to prevent
rheumatic fever (and thus rheumatic
heart disease).

57. • In patients who develop rheumatic
heart disease, therapy is directed
toward eliminating the group A
streptococcal pharyngitis (if still
present), suppressing inflammation from
the autoimmune response, and providing
supportive treatment for congestive
heart failure.

58. • Following the resolution of the acute
episode, subsequent therapy is directed
towards preventing recurrent rheumatic
heart disease in children and monitoring
for the complications and sequelae of
chronic rheumatic heart disease in
adults.

59. • Therapy is directed towards eliminating
the GABHS pharyngitis (if still
present), suppressing inflammation from
the autoimmune response, and providing
supportive treatment of congestive
heart failure.

60. Eliminating the GABHS pharyngitis and
prophylactic
• Single dose of benzyl penicillin 1.2 million U
IM or oral phenoxymethylpenicillin 250mg
6 hourly for 10 days
• Prophylactic dose: Inj benzathine penicillin
1.2 million unit once every 4 weeks.

61. • The American Heart Association currently
recommends that patients with rheumatic
fever without carditis receive prophylactic
antibiotics for 5 years or until aged 21 years,
whichever is longer.

62. • Patients with rheumatic fever and carditis
but no valve disease should receive
prophylactic antibiotics for 10 years or
well into adulthood, whichever is longer.
• Finally, patients with rheumatic fever with
carditis and valve disease should receive
antibiotics for at least 10 years or until
age 40 years.

63. • Alternate drugs recommended by the
American Heart Association for these
patients include PO clindamycin (20
mg/kg in children, 600 mg in adults) and
PO azithromycin or clarithromycin (15
mg/kg in children, 500 mg in adults).

64. Suppressing inflammation from the
autoimmune response
• Treatment of the acute inflammatory
manifestations of acute rheumatic fever
consists of salicylates and steroids.
Aspirin in anti-inflammatory doses
effectively reduces all manifestations of
the disease except chorea, and the
response is typically dramatic.

65. • 90 to 120 mg/kg/day in 4 divided dose
• Steroid: Prednisone
»1.0 to 2.0 mg/kg per day in divided
dose

66. • Total duration of course for the
suppressive agent (aspirin/steroid) is 12
week
• Aspirin full dose for 10 weeks and
tapered off in next 2 week
• Steroid full dose for 3 weeks and taper
gradually in next 9 weeks.

67. Providing supportive treatment of
congestive heart failure.
• Include digoxin and diuretics,
supplemental oxygen, bed rest, and
sodium and fluid restriction as
additional treatment for patients with
acute rheumatic fever and heart failure

68. Mitral Valve Regurgitation:
Medical management:
• Vasodilators, e.g. ACE inhibitors.
• Diuretics.
• Digoxin if atrial fibrillation is present.
• Anticoagulants if atrial fibrillation is present.
• Antibiotic prophylaxis against infective
endocarditis

69. Surgical management:
• Open surgical vulvuloplasty is primarily used.
• Further repair or reconstruction of the valve
may be necessary & can be achieved by
annuloplasty.
• Annuloplasty entails reconstruction of the
annulus, with or without the aid of prosthetic
rings.

70. Mitral Valve Stenosis:
Medical management:
 Anticoagulant to decrease the risk for
developing arterial thrombus
 Endocarditis prophylaxis is essential for any
patient with MI.
 Symptomatic patients with MS may initially be
managed with beta blocker, diuretics are used to
reduced pulmonary congestion

71. Surgical management:
 Vulvuloplasty or mitral valve replacement.
 Valvotomy may be performed percutaneously by
balloon dilatation or by surgical commissurotomy.
Balloon vulvuloplasty is the procedure of the
choice in young patients with pliable valve,
minimal valvular calcification
 Patient heavy calcified valves and or associated
significant mitral regurgitation require valve
replacement

72. Percutaneous balloon valvuloplasty

73. Aortic Valve Regurgitation:
Valve repair-
• Aortic valve repair is surgery to preserve
the valve and to improve its function.
• Patients don't need long-term medications
to prevent blood clots (anticoagulation
therapy) after a valvuloplasty.

74.  Valve replacement-
• In many cases, the aortic valve has to be
replaced to correct aortic valve regurgitation.
• Surgeon removes the aortic valve and replaces it
with a mechanical valve or a tissue valve.
• Mechanical valves, made from metal, are durable,
but they carry the risk of blood clots forming on
or near the valve

75. Management of VHD cont…
Prevention:
 Treating sore throat in time.
 Taking good care of your teeth and gums.
Practicing good oral hygiene helps prevent
bloodstream infections that can damage your
heart valves due to endocarditis.
 Keeping heart healthy

76. Prosthetic valves
• Valvular replacement may be required for mitral,
aortic, tricuspid & occasionally pulmonic disease.
• The surgical treatment of choice for combined
aortic stenosis & aortic regurgitation is valvular
replacement.
• Prosthetic valves are categorized as mechanical
or biological.
• Mechanical valves are manufactured from man
made materials & consist of combinations of
metals, carbons etc.

77. Prosthetic valves
• Biologic valves are constructed from bovine,
porcine, & human cardiac tissue & usually
contain some made materials.
• Mechanical valves are more durable & last
longer than biologic valves however they
have increased risk of thromboembolism,
necessitating long-term anticoagulant
therapy.
• The main complication of mechanical valves
is hemorrhage from the use of
anticoagulants.

78. Prosthetic valves
• Biological valves do not require
anticoagulant therapy due to their low
thrombogenicity however they are less
durable due to tendency for early
calcification, tissue degeneration &
stiffening of the leaflets.
• Problems with either type of prosthetic
valves include paravalvular leaks &
endocarditis.

79. Prosthetic valves
• The choice of valves depends on many
factors. For e.g.
– If a patient cannot take an anticoagulant
(e.g. women of childbearing age), a biologic
valve may be considered.
– A mechanical valve may be best for a
younger patient because it is more durable.
– For patient over 65, durability is less
important than the risks of hemorrhage
from anticoagulants.

80. Prognosis
• Rheumatic heart disease is the major
cause of morbidity from rheumatic fever
and the major cause of mitral insufficiency
and stenosis in the world. Variables that
correlate with severity of valve disease
include the number of previous attacks of
rheumatic fever, the length of time
between the onset of disease and start of
therapy, and sex.
• Insufficiency from acute rheumatic valve
disease resolves in 60-80% of patients who
adhere to antibiotic prophylaxis.

81. Complications
• Potential complications include heart
failure from valve insufficiency (acute
rheumatic carditis) or stenosis (chronic
rheumatic carditis). Associated cardiac
complications include atrial arrhythmias,
pulmonary edema, recurrent pulmonary
emboli, infective endocarditis,
intracardiac thrombus formation, and
systemic emboli.

82. Nursing management
Assessment
Subjective data:
Important Health information:
Past health history: Rheumatic fever,
infective endocarditis, congenital defects,
myocardial infarction, chest trauma,
cardiomyopathy, syphilis.

83. Nursing management
Assessment
Subjective data:
Functional Health Patterns:
Health perception – health management: IV
drug abuse, fatigue.
Activity – exercise – Palpitations, generalized
weakness, activity intolerance, dizziness,
fainting, dyspnea on exertion, cough,
hemoptysis, orthopnea.

84. Nursing management
Subjective data:
Sleep – rest – Paroxysmal nocturnal dyspnea.
Cognitive – Perceptual – Angina or atypical
chest pain.
Objective data:
General: Fever
Integumentary: Diaphoresis, flushing, cyanosis,
clubbing, peripheral edema,

85. Objective data:
Respiratory: Crackles, wheezes, hoarseness
Cardiovascular: Abnormal heart sounds,
including clicks, systolic & diastolic murmurs,
S3 & S4 dysrythmias, including atrial
fibrillation, premature ventricular contractions,
tachycardia, increase or decrease pulse
pressure, hypotension, water hammer or
thready peripheral pulses.
Gastrointestinal: Ascitis, hepatomegaly.

86. Nursing management
Nursing Diagnosis:
• Activity intolerance related to insufficient oxygenation
secondary to decreased cardiac output & pulmonary
congestion as evidenced by weakness, fatigue,
shortness of breath, increase or decrease in pulse rate.
• Excess fluid volume related to heart failure secondary
to incompetent valve as evidenced by peripheral edema,
weight gain, adventitious breath sounds, neck vein
distension.

87. Nursing management
Nursing Diagnosis:
• Decrease cardiac output related to valvular
incompetence as evidenced by murmurs, dyspnea,
dysrythmias, peripheral edema.
• Deficient knowledge related to lack of experience &
exposure to information about disease & treatment
process as evidenced by verbalization of
misconceptions about measures to prevent
complications & requests for information.

88. Nursing Interventions:
• Activity intolerance:
 Monitoring cardio-respiratory response to activity (e.g.
vital signs) to plan appropriate interventions.
 Encourage alternate rest & activity periods to conserve
energy & decrease cardiac demands.
 Encourage patient to choose activities that gradually
build endurance to prevent cardiac tolerance.
 Assist the patient / significant other to establish realistic
activity goals to promote feelings of accomplishment.

89. Nursing Interventions:
• Excess fluid volume:
 Monitoring changes in peripheral edema to detect
hypervolemia.
 Monitor respiratory pattern for symptoms of difficulty (e.g.
dyspnea, tachypnea) to assess for fluid congestion in the
lungs.
 Monitor vital signs & intake & output to assess
hemodynamic response to & effectiveness of interventions.
 Weigh patient daily
 Administer prescribed diuretics.

90. Nursing Interventions:
• Decreased cardiac output:
 Monitoring vital signs, cardiovascular status &
respiratory status to assess for manifestations of
decreased cardiac output (e.g. fatigue, malaise,
shortness of breath, dyspnea on exertion, palpitations.)
 Monitor for cardiac dysrythmias, including
disturbances of both rhythm & conduction, to detect
changes from baseline.
 Administer inotropic medication as ordered to increase
myocardial contractility.

91. Nursing Interventions:
• Decreased cardiac output:
 Elevate head of bed to reduce venous return, reduce
oxygen demand.
 Promote bed rest/activity limitation to decrease cardiac
workload & oxygen demand.

92. Nursing Interventions:
• Deficient knowledge:
 Explain pathophysiology of disease process to ensure
knowledge base.
 Describe disease process.
 Discuss lifestyle changes to prevent complications & / or
control the disease (e.g. smoking cessation) to prevent an
increased cardiac workload.
 Instruct patient on purpose & action of each medication.
 Provide patient with written information about action,
purpose & side effects of each medication.