AQUIRED HEART DISEASES

Outline
I. Acute rheumatic fever
II.Infective endocarditis
III.Viral myocarditis
IV.Congestive heart disease
 I. Acute rheumatic fever
 Etiology:
– GAS (causes pharyngitis, impetigo & pyoderma and
others)
– Common age: 5-15yrs (sometimes as young as 3yrs)
– Complications from spread to adjacent structures
• Suppurative: Cervical lymphadenitis, peritonsillar
abscess, retropharyngeal abscess, otitis media,
mastoiditis, and sinusitis
• Non suppurative: Acute rheumatic fever and acute
poststreptococcal glomerulonephritis
 Pathogenesis
- Cytotoxicity theory
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
- Immunologic theory
Immunologic cross reactivity between GAS components
(M protein, protoplast membrane, cell wall group A
carbohydrate, capsular hyaluronate) and mammalian
tissues (heart, brain, joint).
 Clinical manifestations and diagnosis
- Jone’s criteria
- For initial attack of acute rheumatic fever
• 2 major with supportive evidence
• 1 major and 2 minor with supportive evidence
• Chorea
• Indolent carditis
- For recurrence
• 1 major and 1 minor with supportive evidence
 Jone’s criteria

MAJOR MINOR SUPPORTING

MANIFESTATIONS MANIFESTATIONS EVIDENCE OF
ANTECEDENT GAS
INFECTION
1. Carditis Clinical features: - Positive throat
2. Polyarthritis - Arthralgia culture
3. Erythema - Fever - Rapid streptococcal
Marginatum Laboratory antigen test
4. Subcutaneous features: - Elevated or
Nodules - Elevated acute increasing strept
5. Chorea phase reactants: antibody titer
ESR, CRP
- Prolonged PR
interval
 Migratory polyarthritis
• occurs in about 75% of patients with acute rheumatic fever
• typically involves larger joints, particularly the knees, ankles, wrists,
and elbows
• Rheumatic joints are generally hot, red, swollen, and exquisitely
tender; even the friction of bedclothes is uncomfortable
• Characteristically migratory
• dramatic response to even small doses of salicylates
• Synovial fluid in acute rheumatic fever usually has 10,000-100,000
white blood cells/mm3 with a predominance of neutrophils, a protein
level of about 4 g/dL, a normal glucose level, and forms a good mucin
clot.
 Carditis
• occurs in about 50-60% of all cases of acute rheumatic fever
• pancarditis, with active inflammation of myocardium, pericardium,
and endocardium
• Most cases consist of either isolated mitral valvular disease or
combined aortic and mitral valvular disease
• 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.
• Acute rheumatic carditis usually presents as tachycardia and cardiac
murmurs, with or without evidence of myocardial or pericardial
involvement.
• 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.
• Patterns of valvular disease
– Mitral insufficiency
is the result of structural changes that usually include some loss
of valvular substance and shortening and thickening of the
chordae tendineae.
With mild disease, signs of heart failure are not present, the
precordium is quiet, and auscultation reveals a high-pitched
holosystolic murmur at the apex that radiates to the axilla.
DDx: Viral myocarditis, Viral pericarditis, IE, Kawasaki
disease, CHD, MVP, Innocent murmurs
Complications: cardiac failure that may be precipitated by
progression of the rheumatic process, the onset of atrial
fibrillation, or infective endocarditis
– Mitral stenosis
results from fibrosis of the mitral ring, commissural adhesions,
and contracture of the valve leaflets, chordae, and papillary
muscles over time (10yr or more but earlier in our setup)
Rt sided heart failure
Hemoptysis caused by rupture of bronchial or pleurohilar veins
and, occasionally, by pulmonary infarction may occur.
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 at the apex
– Aortic insufficiency
In chronic rheumatic aortic insufficiency, sclerosis of the aortic
valve results in distortion and retraction of the cusps.
The pulse pressure is wide with bounding peripheral pulses.
Systolic blood pressure is elevated, and diastolic pressure is
lowered.
In severe aortic insufficiency, the heart is enlarged, with a left
ventricular apical heave.
Diastolic murmur is heard over the upper and midleft sternal
border with radiation to the apex and upper right sternal border.
 Chorea
• occurs in about 10-15% of patients with acute rheumatic fever
• usually presents as an isolated, frequently subtle, neurologic behavior
disorder
• Emotional lability, incoordination, poor school performance,
uncontrollable movements, and facial grimacing, exacerbated by
stress and disappearing with sleep, are characteristic.
• Occurs months from GAS infection
• Clinical maneuvers to elicit features of chorea include
(1) milkmaid's grip
(2) pronator sign
(3) wormian darting movements of the tongue upon protrusion
(4) examination of handwriting to evaluate fine motor movements.
 Erythema Marginatum

• <3% of patients with acute rheumatic

fever
• consists of erythematous, serpiginous,
macular lesions with pale centers that
are not pruritic
• occurs primarily on the trunk and extre
mities, but not on the face, and it can be
accentuated by warming the skin.
 Subcutaneous Nodules
• ≤1% of patients with acute rheumatic fever
• consist of firm nodules approximately 1 cm in diameter along the
extensor surfaces of tendons near bony prominences
 Principles of treatment
• Bed rest
• Antibiotics
• Antiinflammatory
• Sedatives for chorea
• Prevention: 1ry prophylaxis & 2ry prophylaxis
 II.Infective endocarditis
 Infective endocarditis is an infection of the heart’s
endocardial surface
 Typically involves the valves
– May involve all structures of the heart
• Chordae tendinae
• Sites of shunting
• Mural lesions
– Infection of vascular shunts, by strict definition, is
endarteritis, but lesion is the same
 Etiology
• Viridans-type streptococci (common after dental
procedures) and Staphylococcus aureus (in patients with
no underlying heart disease) are the most common
• group D enterococci: more often after lower bowel or
genitourinary manipulation
• Pseudomonas aeruginosa or Serratia marcescens:
intravenous drug users
• fungal organisms: after open heart surgery.
• Coagulase-negative staphylococci: in the presence of an
indwelling central venous catheter.
• Gram negative organisms
- P. aeruginosa most common
- HACEK - slow growing, fastidious organisms that
may need 3 weeks to grow out of culture
Haemophilus sp.
Actinobacillus
Cardiobacterium
Eikenella
Kingella
• No organisms are detected in 6% of the cases
 Pathophysiology
• Turbulent blood flow disrupts valve surface
(endocardium) to produce suitable (sticky) site for
bacterial attachment
• Platelet deposition + fibrin may lead to non-bacterial
thrombus or vegetation
• Bacteraemia – delivers organisms to the damaged
(sticky) endocardial surface resulting in adherence &
colonisation
• Eventual invasion of valve leaflets results in infected
vegetation (sheath of fibrin & platelets, ideal conditions
for further bacterial multiplications, protection from
polymorphs)
• Acute
– Toxic presentation
– Progressive valve destruction & metastatic infection
developing in days to weeks
– Most commonly caused by S. aureus
• Subacute
– Mild toxicity
– Presentation over weeks to months
– Rarely leads to metastatic infection
– Most commonly S. viridans or enterococcus
 Clinical manifestations
• Fever
• Heart murmur
• Nonspecific signs – petechiae, subungal or “splinter” hemorrhages,
clubbing, splenomegaly, neurologic changes
• More specific signs - Osler’s Nodes, Janeway lesions, and Roth
Spots
• Blood culture*
• CBC
• ESR and CRP
• RFT
• Urinalysis
 Petechiae
1. Nonspecific
2. Often located on extremities
or mucous membranes
 Splinter Hemorrhages

1. Nonspecific
2. Nonblanching
3. Linear reddish-brown lesions found under the nail bed
4. Usually do NOT extend the entire length of the nail
 Osler’s Nodes

1. More specific
2. Painful and erythematous nodules
3. Located on pulp of fingers and toes
4. More common in subacute IE
 Janeway Lesions

1. More specific
2. Erythematous, blanching macules
3. Nonpainful
4. Located on palms and soles
 Duke criteria

Major criteria Minor criteria

1. Blood culture positive for 1. Predisposition – heart
typical IE-causing condition or i.v. drug abuse
microorganism 2. Fever – temp. >38 °C
3. Vascular phenomena –
2. Evidence of endocardial arterial emboli etc.
involvement 4. Immunologic phenomena –
glomerulonephritis, Osler’s
nodes, Roth’s spots
Diagnosis
5. Microbiological evidence –
• 2 major criteria positive blood cultures but do
• 1 major and 3 minor not meet major criteria
• 5 minor criteria
• Echocardiographic findings in IE
– Vegetation
– Abscess
– Pseudoaneurysm
– Perforation
– Fistula
– Valve aneurysm
– Dishence of prosthetic valve
 Complications
• heart failure
• Myocardial abscess
• Arrhythmia
• Septic emboli: to CNS, pulmonary emboli
• mycotic aneurysms
• meningitis, osteomyelitis, arthritis, renal abscess,
purulent pericarditis, and immune complex-mediated
glomerulonephritis.
 Principles of treatment
Antibiotics
Antifungal
Surgery
 Prevention
• antimicrobial prophylaxis before dental and other surgical
procedures
- Prosthetic cardiac valve or prosthetic material used for cardiac
valve repair
- Previous infective endocarditis
- Unrepaired cyanotic CHD
- Completely repaired CHD with prosthetic material or device,
during the first 6 mo after the procedure
- Repaired CHD with residual defects at the site or adjacent to
the site of a prosthetic patch
- Cardiac transplantation recipients who develop cardiac
valulopathy
• Patients undergoing respiratory tract procedures
• patients undergoing cardiac surgery with placement of
prosthetic material
 III.Viral myocarditis
 characterized by inflammatory cell infiltrates, myocyte
necrosis, or myocyte degeneration
 Causes:
• Viruses:
- Enteroviruses
- Coxsackievirus
- Influenza A and B
- Adenovirus
- Herpes
- HIV
• Bacteria:
– Beta-hemolytic Streptococcus
– Corynebacterium diphtheria
– Borrelia burgdorferi
– Enterococcus spp
– Chlamydia psittaci
– Neisseria meningitidis
– Mycoplasma pneumonia
– Staphylococcus aureus
• Protozoa:
– Trypanosoma cruzii
– Toxoplasma gondi
• Helminths
– Trichinella spiralis
– Echinococcus
• Autoimmunity
– Infection associated
– Auto-immune disease associated
• Hypersensitivity
– Penicillins, Methyldopa, Sulfamethoxazole
• Toxicity
– Catecholamines, Cocaine, Ethanol
 Pathogenesis

Viral Replication

↓
Autoimmune injury

↓
Dilated cardiomyopathy
• Viral replication
– Cardiotropic RNA viruses are taken into myocytes by
receptor-mediated endocytosis.
– Directly translated intracellularly to produce viral
protein.
– Virus infection directly contributes to cardiac tissue
destruction by cleaving the cytoskeleton protein
dystrophin, leading to a disruption of the dystrophin-
glycoprotein complex.
• Autoimmunity
– Viral replication concludes with activation of the host
system.
– Ideally, the immune system should down-regulate to a
resting state once viral proliferation is controlled.
– If host immune activation continues unabated 
autoimmune disease.
– T cells target the host’s own tissue through molecular
mimicry.
• Dilated Cardiomyopathy
– Re-modelling mechanisms lead to dilated
cardiomyopathy (DCM).
– The persistent myocyte viral gene expression 
progressive DCM.
– Cytokines: activate matrix metalloproteinases
(gelatinase, collagenase, elastases).
• Clinical presentation
– May be asymptomatic
– May include a viral prodrome of fevers, myalgias,
respiratory symptoms or gastroenteritis.
– May present with rapidly deteriorating LV function or
arrhythmias and heart block
• Diagnosis
– Gold Standard is endomyocardial biopsy.
– Cardiac biomarkers i.e. creatine kinase and troponin T
and I are routinely measured
– Trop T > 0.1ng/mL had a sensitivity of 53% and a
specificity of 94%
– ESR found to have low sensitivity and specificity.
– Echo changes i.e. LV dysfunction (in 69%), and
segmental wall motion abnormalities (64%), do not
differentiate myocarditis from other cardiomyopathies
• Principle of treatment: supportive
 IV.Congestive heart failure
 Pathophysiology of congestive heart failure
• Essential functions of the heart
- To cover metabolic needs of body tissue (oxygen,
substrate) by adequate blood supply
- To receive all blood coming back from tissue to the
heart
• Systemic oxygen transport = CO * systemic oxygen
content
• Cardiac output (CO) = heart rate (HR) * stroke vol (SV)
• Control of HR: ANS and hormonal control
• Control of SV: preload, contractility, afterload
• Adaptive mechanisms of the heart to increased load
1. The Frank-Starling mechanism
- Stretching of the myocardial fibers during diastole by
increasing end-diastolic volume → ↑force of contraction
during systole
2. Ventricular hypertrophy
- Increased mass of contractile elements → ↑strength of
contraction
3. Increased sympathetic adrenergic activity
- Increased heart rate (β adrenergic receptors), increased
contractility (by increasing Ca concentration) &
vasoconstriction (β adrenergic receptors)
4. Increased activity of the renin-angiotensin-aldosterone
system
• These adaptive mechanisms finally fail and even become
deleterious over the long run resulting in hypermetabolism,
increased afterload, arryhythmogenesis and increased myocardial
oxygen requirements. And also down regulation of β adrenergic
receptors and direct myocardial damage by the cathecolamines.
• Heart failure is a pathophysiological process in which
the heart as a pump is unable to meet the metabolic
requirements of the tissue for oxygen and substrates.
 Approach to the child with congestive heart failure

i. Clinical manifestations
– Dyspnea (most sensitive symptom)
– Edema
– Hepatic congestion
– Ascites
– Orthopnea, Paroxysmal Nocturnal Dyspnea (PND)
- Fatigue (especially with exertion), intruption of
feeding, poor growth
- S3 gallop
ii. Lab investigations
– CXR
» cardiomegally, cephalization of the pulmonary vessels,
plueral effusion
– ECG, ECHO
– CBC
» Since anemia can exacerbate heart failure
– Serum electrolytes and creatinine
» before starting high dose diuretics
– Fasting Blood glucose
» To evaluate for possible diabetes mellitus
– Thyroid function tests
» Since thyrotoxicosis can result in A. Fib,
and hypothyroidism can results in HF.
– Viral studies
» If viral mycocarditis suspected
iii.Principles of treatment
– Positioning
– Oxygen
– Drugs: treatment is directed at restoring balance to these
neuroendocrine systems.
• Duiretics
• Digoxin
• β-blockers
• ACE inhibitors
• Angiotensin receptor blockers
• Aldosterone blockers
– Surgery
– Treatment of the precipitating factors