AQUIRED HEART DISEASE-I

Samson Nadew(MD)

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  Common acquired heart diseases Includes-
- Acute rheumatic fever with chronic rheumatic heart disease
- Infective endocarditis
- Cardiomyopathies
- Myocarditis of different etiologies- viral, bacterial ,toxic
- Pericardial diseases-TB, bacterial

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 Rheumatic fever and chronic rheumatic heart
disease
 Rheumatic Fever
 ETIOLOGY.
-evidence to support the link between GAS pharyngitis and acute
rheumatic fever and CRHD.
-As many as ⅔ of the patients have a history of URTI
- the peak age and seasonal incidence of acute rheumatic fever
closely parallel those of GAS infections.
- Patients almost always have serologic evidence of a recent GAS
infection.

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 Rheumatic fever…
-Not all of the serotypes of GAS can cause rheumatic fever.
 -certain serotypes of GAS (M types 1, 3, 5, 6, 18, 24) are more
frequently isolated than are other serotypes.
EPIDEMIOLOGY.
 In some developing countries, the annual incidence is
currently as high as 282/100,000 population.
 Worldwide, CRHD remains the most common form of
acquired heart disease in all age groups
 as much as 50% of all cardiovascular disease and as much as
50% of all cardiac admissions in many developing countries.

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 Rheumatic fever…
 In the United States, early in the 20th century, acute rheumatic fever
was a leading cause of death among children and adolescents, with
annual incidence rates of 100–200/100,000 population
 By the early 1980s, the annual incidence was as low as 0.5/100,000
population.

 Factors leading to this dramatic decrease are

- improvements in living conditions
- availability of medical care and use of antibiotics.
- Antibiotic therapy of GAS pharyngitis -in preventing initial attacks and
recurrences of the disease.

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 Rheumatic fever…
Development of acute rheumatic fever is dependent on
various host factors.
-specific characteristics of the infecting GAS
-5–15 yr of age, the age of greatest risk for GAS pharyngitis.
-Previous attack of acute rheumatic fever-recurrence rate of 30%
-? genetic predisposition to acute rheumatic fever- a higher concordance
rate in monozygotic twins.
-? an association between the presence of both specific HLA markers and
a specific B-cell alloantigen (D8/17) and susceptibility to acute
rheumatic fever.

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 Rheumatic fever…
 PATHOGENESIS.
 The pathogenic link between a GAS URTI and an attack of acute
rheumatic fever is still not clear.
 Obstacle-the inability to establish an animal model. Several
theories, two are most accepted.
1. An immune-mediated pathogenesis
-the immunologic cross reactivity -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).
-clinical similarity to other immune-mediated illnesses
-latency period b/n infection and acute rheumatic fever

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 Rheumatic fever…
2. The cytotoxicity theory
- suggests that a GAS toxin may be involved in the pathogenesis of acute
rheumatic fever.

- GAS produces several enzymes that are cytotoxic for mammalian

cardiac cells, such as streptolysin O, which has a direct cytotoxic effect
on mammalian cells in tissue culture.

- can not explain the latency period between pharyngitis and acute
rheumatic fever.

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 Rheumatic fever…
 CLINICAL MANIFESTATIONS AND DIAGNOSIS.
No clinical or laboratory finding is pathognomonic
-T. Duckett Jones in 1944 proposed guidelines to aid in diagnosis and to
limit overdiagnosis.
-The Jones criteria are intended only for the diagnosis of the initial attack
of acute rheumatic fever.
- 5 major and 4 minor criteria and an absolute requirement for evidence
(microbiologic or serologic) of recent GAS infection.
- 2 major criteria OR 1 major + 2 minor criteria and meets the
absolute requirement- for diagnosing acute rheumatic fever.

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 Rheumatic fever…
 There are 3 circumstances in which the diagnosis of acute
rheumatic fever can be made without strict adherence to
the Jones criteria.

1. Chorea may occur as the only manifestation of acute

rheumatic fever.
2. indolent carditis may be the only manifestation in patients
who 1st come to medical attention months after the onset
of acute rheumatic fever.
3. recurrences of acute rheumatic fever

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 Rheumatic fever…
 Major Manifestations:
 1. CARDITIS.
- Carditis and resultant CRHD are the most serious
manifestations of acute rheumatic fever .
- Rheumatic carditis is characterized by pancarditis, with
active inflammation of myocardium, pericardium, and
endocardium.
- Cardiac involvement during acute rheumatic fever varies
in severity from fulminant, potentially fatal exudative
pancarditis to mild, transient cardiac involvement.
- Endocarditis (valvulitis), which manifests by 1 or more
cardiac murmurs, is a universal finding in rheumatic
carditis, whereas the presence of pericarditis or
myocarditis is variable.

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 Rheumatic fever…
 Myocarditis and/or pericarditis without evidence of endocarditis is
rarely due to rheumatic heart disease.
 Most cases consist of either isolated mitral valvular disease or
combined aortic and mitral valvular disease. Isolated aortic or right-
sided valvular involvement is uncommon.
 Serious and long-term illness is related to valvular heart disease as a
consequence of a single attack or recurrent attacks of acute rheumatic
fever. Valvular insufficiency is characteristic of both acute and
convalescent stages of acute rheumatic fever, whereas valvular stenosis
usually appears several years or even decades after the acute illness.

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 Rheumatic fever…
 In developing countries such as ours, where acute rheumatic fever
often occurs at a younger age, MS and AS may develop sooner and can
occur in young children.
Acute rheumatic carditis usually presents as
-tachycardia ,cardiac murmurs,cardiomegaly ,CHF with hepatomegaly
and peripheral and pulmonary edema.
-Echocardiographic findings include pericardial effusion, decreased
ventricular contractility, and aortic and/or mitral regurgitation.
-Echocardiographic demonstration of regurgitant lesion without
accompanying auscultatory evidence does not satisfy the Jones criteria
for carditis.

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 Rheumatic fever…
 Carditis occurs in about 50–60% of all cases of acute rheumatic fever.

 Recurrent attacks of acute rheumatic fever in patients who had carditis

with the initial attack are associated with high rates of carditis(30%)

 The major consequence of acute rheumatic carditis is chronic,

progressive valvular disease, particularly valvular stenosis, which
require valve replacement and predispose to infective endocarditis.

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 Rheumatic fever…
 MIGRATORY POLYARTHRITIS.
 Arthritis occurs in about 75% of patients.
 involves larger joints, particularly the knees, ankles, wrists, and
elbows. Involvement of the spine, small joints of the hands and feet, or
hips is uncommon.
 Rheumatic joints are generally hot, red, swollen, and exquisitely
tender.
 The pain can precede and can appear to be disproportionate to the
other findings.
 The joint involvement is characteristically migratory in nature; a
severely inflamed joint can become normal within 1–3 days without
treatment, as 1 or more other large joints become involved.

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 Rheumatic fever…
 If you suspect acute rheumatic fever with arthritis -withhold
salicylates and observe for migratory progression. A
dramatic response to even small doses of salicylates- absence of
such a response should suggest an alternative diagnosis.
 Rheumatic arthritis is typically not deforming.
 Synovial fluid in acute rheumatic fever usually has 10,000–
100,000 white blood cells/mm3 with a predominance of
neutrophils, a protein of about 4 g/dL, a normal glucose, and
forms a good mucin clot.
 arthritis is the earliest manifestation of acute rheumatic fever.
 There is inverse relationship between the severity of arthritis and
the severity of cardiac involvement.

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 Rheumatic fever…
 CHOREA.
-Sydenham chorea occurs in about 10–15% of cases and usually presents
as an isolated, frequently subtle, neurologic behavior disorder.
-Hypotonia, Emotional lability, and incoordination are the triads of
Sydenhams chorea.
-poor school performance, uncontrollable movements, and facial
grimacing, exacerbated by stress and disappearing with sleep, are
characteristic.
-Chorea occasionally is unilateral.
-The latent period from acute GAS infection to chorea is usually longer.

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 Rheumatic fever…
 Onset can be insidious, with symptoms being present for several
months before recognition.
Clinical maneuvers to elicit features of chorea
1. demonstration of milkmaid's grip (irregular contractions of the
muscles of the hands while squeezing the examiner's fingers)
2. spooning and pronation of the hands when the patient's arms are
extended
3. wormian darting movements of the tongue upon protrusion
4. examination of handwriting to evaluate fine motor movements.
chorea rarely leads to permanent neurologic sequelae

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 Rheumatic fever…
 ERYTHEMA MARGINATUM.
 Erythema marginatum is a rare (<3% of patients with acute rheumatic
fever) but characteristic rash of acute rheumatic fever.
 It consists of erythematous, serpiginous, macular lesions with pale
centers that are not pruritic.
 It occurs primarily on the trunk and extremities, but not on the face,
and it can be accentuated by warming the skin.
 SUBCUTANEOUS NODULES.
 Subcutaneous nodules are a rare (≤1% of patients with acute
rheumatic fever) and consist of firm nodules approximately 1 cm in
diameter along the extensor surfaces of tendons near bony
prominences.
 There is a correlation between the presence of these nodules and
significant rheumatic heart disease.

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 Rheumatic fever…
 Minor Manifestations.
=The 2 clinical minor manifestations are
-arthralgia (in the absence of polyarthritis as a major criterion) and
-fever (typically temperature ≥102°F and occurring early in the course
of illness).

=The 2 laboratory minor manifestations are

-elevated acute-phase reactants (e.g., CRP, ESR)
-prolonged PR interval on ECG.
*However, a prolonged PR interval alone does not constitute evidence of
carditis or predict long-term cardiac sequelae.

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 Rheumatic fever…
 Recent Group A Streptococcus Infection- an absolute
requirement for the diagnosis of acute rheumatic fever is supporting
evidence of a recent GAS infection.
- develops 2–4 wk after an acute episode of GAS pharyngitis at a time
Cl fxs of pharyngitis dissapear and only 10–20% of the throat culture
or rapid streptococcal antigen test results are positive.
- One third of patients have no history of an antecedent pharyngitis.
- Therefore, evidence of an antecedent GAS infection is usually based on
elevated or increasing serum antistreptococcal antibody titers.

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 Rheumatic fever…
-A slide agglutination (Streptozyme) test for detection of GAS antigens
- purported to detect antibodies against 5 different GAS antigens.
- If only a single antibody is measured (usually ASO), only 80–85% of
patients with acute rheumatic fever have an elevated titer
- however, 95–100% have an elevation if 3 different antibodies
(antistreptolysin O, anti-DNase B, antihyaluronidase) are measured.
- Except for patients with chorea, clinical findings of acute rheumatic
fever generally coincide with peak antistreptococcal antibody
responses.

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 Rheumatic fever…
 TREATMENT.
1. Supportive
-bed rest and monitored closely for evidence of carditis.
-They can be allowed to ambulate as soon as the signs of acute
inflammation have subsided.
-patients with carditis require longer periods of bed rest.
2. Antibiotic Therapy.
-should receive 10 days of orally administered penicillin or
erythromycin, or a single intramuscular injection of benzathine
penicillin to eradicate GAS from the upper respiratory tract. Regadless
of throat swab result
-After this initial course of antibiotic therapy, the patient should be
started on long-term antibiotic prophylaxis.

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 Rheumatic fever…
3. 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.
a. oral salicylates- Patients with typical migratory polyarthritis and
those with carditis without cardiomegaly or congestive heart failure
- 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.
b. corticosteroids -Patients with carditis and cardiomegaly or
congestive heart failure.

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 Rheumatic fever…
 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, aspirin should be started at 75
mg/kg/day in 4 divided doses for 6 wk.
3. RX of heart failure. digoxin, fluid and salt restriction, diuretics, and
oxygen- for treatment of CHF in moderate to severe carditis. Be
careful about digoxin toxicity.
Termination of the anti-inflammatory therapy may be followed by
rebounds phenomena which should be left untreated unless the
clinical manifestations are severe; salicylates or steroids should be
reinstated in such cases.

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 Rheumatic fever…
Rx of Sydenham Chorea.
-anti-inflammatory agents are usually not indicated.
-Sedatives may be helpful early in the course of chorea; phenobarbital (16–32
mg every 6–8 hr PO)
-If phenobarbital is ineffective, then haloperidol (0.01–0.03 mg/kg/24 hr
divided bid PO) or chlorpromazine (0.5 mg/kg every 4–6 hr PO) should be
initiated.
COMPLICATIONS.
 The arthritis and chorea of acute rheumatic fever resolve completely without
sequelae.
 The long-term sequelae of rheumatic fever are usually limited to the heart
 Patients with VHD secondary to acute rheumatic fever are at increased risk
for developing infective endocarditis during episodes of transient bacteremia.

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 Rheumatic fever…
 PROGNOSIS.
 depends on the clinical manifestations present at the initial episode,
the severity of the initial episode, and the presence of recurrences.
 Approx 70% of the patients with carditis- 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.

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 Rheumatic fever…
 Patients who have had acute rheumatic fever are susceptible to
recurrent attacks following reinfection of the upper respiratory tract
with GAS.
 Without antibiotic prophylaxis, 75% of patients who had an
initial episode of acute rheumatic fever had 1 or more recurrences
during their lifetime-recurrences are a major source of morbidity
and mortality. The risk of recurrence is highest immediately after the
initial episode and decreases with time.
 20% of patients who present with “pure” chorea who are not given
secondary prophylaxis develop rheumatic heart disease within 20 yr.
Therefore, require long-term antibiotic prophylaxis.

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 Rheumatic fever…
 PREVENTION.
1. Primary Prevention.
 Appropriate antibiotic therapy instituted before the 9th day of
symptoms of acute GAS pharyngitis is highly effective in preventing
1st attacks of acute rheumatic fever from that episode.
2. Secondary Prevention- to prevent reccurence
 Secondary prevention is directed at preventing acute GAS pharyngitis
in patients at substantial risk of recurrent acute rheumatic fever.
Secondary prevention requires continuous antibiotic prophylaxis every
4 wks.
3. Tertiary prevention- involves prevention of further cardiac
deterioration in patients with established CRHD

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 Chronic rheumatic heart disease
 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.
 The mitral valve is affected most often, followed in frequency by the
aortic valve; right-sided heart manifestations are rare.
 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

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 Chronic rheumatic heart…
 PATTERNS OF VALVULAR DISEASE
 MITRAL INSUFFICIENCY
 Pathophysiology.
 Mitral insufficiency is the result of structural changes that
usually include some loss of valvular substance and
shortening and thickening of the chordae tendineae.
 During acute rheumatic fever with severe cardiac
involvement, 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.

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 Chronic rheumatic heart…
 The left atrium dilates as blood regurgitates. 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.

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 Chronic rheumatic heart…
 Clinical Manifestations.
 The physical signs of mitral insufficiency depend on its
severity.
With mild disease, signs of heart failure are not present
auscultation reveals a high-pitched holosystolic murmur at
the apex that radiates to the axilla.
With severe mitral insufficiency-
-Cardiomegally with a heaving apical left ventricular impulse
and often an apical systolic thrill.
-S2 is accentuated, A 3rd heart sound is prominent.
- A short mid-diastolic rumbling murmur is caused by
increased blood flow across the mitral valve as a result of
the insufficiency (not necessarily MS)

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 Chronic rheumatic heart…
 Lab
ECG -shows prominent bifid P waves
-signs of left ventricular hypertrophy
-associated right ventricular hypertrophy if pulmonary hypertension
is present.
CXR- prominence of the left atrium and ventricle(straightening of left
heart border and widening of the carinal angle >90 degree)
- Congestion of perihilar vessels ( if pulmonary hypertension)
Echocardiography -shows enlargement of the left atrium and
ventricle, and Doppler studies demonstrate the severity of the mitral
regurgitation.

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 Chronic rheumatic heart…
 Complications.
- cardiac failure ,atrial fibrillation, or infective endocarditis.
- The effects of chronic mitral insufficiency may become
manifest after many years and include right ventricular
failure and atrial and ventricular arrhythmias.
 Treatment.
 In patients with mild mitral insufficiency, prophylaxis
against recurrences of rheumatic fever is all that is required.
 Treatment of complicating heart failure , arrhythmias , and
infective endocarditis is described elsewhere.
 Afterload -reducing agents (ACE inhibitors) may reduce the
regurgitant volume and preserve left ventricular function.
 Surgical treatment

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 Chronic rheumatic heart…
 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.
 Significant MS 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.

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 Chronic rheumatic heart…
 Clinical Manifestations.
 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, and overt pulmonary edema, as well as
atrial arrhythmias.
 pulmonary hypertension -right ventricular dilatation may
result in functional tricuspid insufficiency, hepatomegaly,
ascites, and edema.
 Hemoptysis caused by rupture of bronchial or pleurohilar
veins and, occasionally, by pulmonary infarction may occur.
 Jugular venous pressure may be increased

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 Chronic rheumatic heart…
 parasternal right ventricular lift is palpable when
pulmonary pressure is high.
 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.
 A holosystolic murmur secondary to tricuspid insufficiency
may be audible.
 If pulmonary hypertension, P2 is accentuated.
 An early diastolic murmur may be caused by secondary
pulmonary valvular insufficiency.

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 Chronic rheumatic heart…
 AORTIC INSUFFICIENCY.
 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.
 Clinical Manifestations.
 In severe aortic insufficiency-
 Excessive sweating and heat intolerance .
 Dyspnea on exertion , orthopnea and pulmonary edema.
 wide pulse pressure ,bounding peripheral pulses, Systolic BP is
elevated, and diastolic pressure is lowered, the heart is enlarged, with
a left ventricular apical heave, A diastolic thrill may be present.

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 Chronic rheumatic heart…
 The diastolic murmur is heard over the upper and mid LSB
with radiation to the apex and the aortic area, high-pitched
blowing in quality and is easily audible in full expiration
with the patient leaning forward.
 A systolic ejection murmur due to the increased stroke
volume.
 An apical presystolic murmur (Austin Flint murmur)
 Roentgenograms show enlargement of the left ventricle and
aorta.
 ECG may reveals signs of LV hypertrophy and strain with
prominent P waves.
 The echo- a large LV. Doppler studies demonstrate the
degree of aortic runoff into the left ventricle..

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 Chronic rheumatic heart…
 Prognosis and Treatment.
 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 may have only aortic
involvement 1–2 yr later.
 Treatment consists of afterload reducers (ACE inhibitors) and
prophylaxis against recurrence of acute rheumatic fever and the
development of infective endocarditis.

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 Chronic rheumatic heart…
 TRICUSPID VALVE DISEASE.
 Primary tricuspid involvement is rare.
 Tricuspid insufficiency is more common secondary to right
ventricular dilatation resulting from unrepaired left-sided lesions.
 the signs produced by tricuspid insufficiency include
prominent pulsations of the jugular veins, systolic pulsations of the
liver, and a blowing holosystolic murmur at the lower left sternal
border that increases in intensity during inspiration.
 Concomitant signs of mitral or aortic valve disease, with or
without atrial fibrillation, are frequent.

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 Chronic rheumatic heart…
 PULMONARY VALVE DISEASE.

 Pulmonary insufficiency usually occurs on a functional

basis secondary to pulmonary hypertension and is 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 studies

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