PEDIATRIC

CARDIOLOGY
LECTURE V
Infective endocarditis
Rheumatic heart disease
Endomyocardial fibrosis
Infective endocarditis
Infective endocarditis
• Infection of the endocardium
• Valves
• Inner lining of the heart
• Inner lining of the great vessels
• Division
• Acute
• Sub-acute
• Often just academic!!
• Incidence up because of cardiac procedures
• Surgeries
• Catheterisation
• Prosthetic valves
IE: Risk factors
• Bacteraemia
• Dental & other surgical procedures
• IV drug users
• Localised infection
• Abscess, pyelonephritis etc
• Damaged endothelium
• CHD (except secundum ASD) => High jet velocity
• Post CHD repair
• Rheumatic valvular disease
• Prosthetic valves
• Mitral valve prolapse
IE: Pathogenesis
• Structural abnormalities of the heart or great arteries =>
• Turbulence =>
• Endothelial damage=>
• Thrombus formation =>
• Medium for bacteria to adhere =>
• Infected vegetation =>
• Platelets and fibrin are deposited over the organisms =>
• Larger vegetation.
IE: Causative organisms
• Streptococcus viridans, enterococci, and Staphylococcus
aureus (60%)
• HACEK organisms (mostly in neonates and
immunocompromised)
• Haemophilus
• Actinobacillus
• Cardiobacterium
• Eikenella
• Kingella
• Fungi
• Culture negative
IE: History
• Often hx of CHD
• Recent surgery: Open heart, dental urological etc
• Gradual onset
• Prolonged low-grade fever
• Non-specific
• Fatigue
• weakness
• Anorexia
• Pallor
• Arthralgia
• Myalgias
• Weight loss
• Diaphoresis.
IE: Physical examination
• Fever: (80-90%)
• Murmur (~100%)
• Splenomegaly (70%)
• Skin manifestation
• Oslers nodes (rare)
• Petechiae
• Janeway lesion (rare)
• Splinter haemorrhages (rare)
• Others
• Hematuria
• Embolic phenomenon: Cerebral abscess, pulmonary embolism
• Clubbing (rare)
• Signs of heart failure
 Duke’s Criteria
• Major Criteria

B : Blood culture +ve

Typical micro-organisms in 2 seperate cultures or
• Persistently +ve blood cultures drawn 12 hours apart or
• Single +ve blood culture for Coxiella burnetti

• E : Endocardial involvement
Positive echocardiogram (vegetation, abscess or valve dehiscence) or
• New valvular regurgitation

• Minor criteria
Fever > 38 oC 
• Immunologic phenomena (glomerulonephritis, Osler’s nodes, Roth’s spots, Rheumatoid factor)
• Vascular phenomena (major arterial emboli, septic pulmonary infarcts, mycotic aneurysm,
intracranial hemorrhage, conjuntival hemorrhage, Janeway lesions)
• Echocardiography findings (suggestive but not definitive)
• Predisposition (heart condition or IV drug user) 
• Microbiologic evidence (Positive blood culture but not meeting major criteria)

• Definitive Diagnosis requires 2 Major  or 3 Minor + 1 Major or  5 Minor 

IE: Investigation
• Laboratory
• Blood culture:
• At least 3 in 24 hours
• FBC: anemia, high WBC
• ESR: Elevated
• Microscopic hematuria
• Echocardiogram
• Visualise vegetations + others
• Beware of false negatives and positives
IE: Management
• Antibiotics
• 1st line: Gentamycin + flucloxacillin + Penicillin
• 2nd line: Ceftriazone
• Combination may change because of
• Current antibiotics profile
• Culture results
• Vancomycin and Methicillin may be used
• Treatment for 4-6weeks
• May require surgical treatment as well
IE: Prognosis
• Recovery rate = 80-90%
• Fungal => poor prognosis
IE: Prevention
• Very important in management
• Good oral hygiene (most important)
• When to give prophylaxis depends on:
• Surgical procedure to be undertaken
• Type of CHD
• Patient’s immune status
• Given orally 1 hour before procedure or parenteral
(30mins)
• Antibiotics choice depends on risk profile
• Amoxicillin, Ampicillin, clindamycin etc OR
• Combinations: e.g. Ampicillin + gentamycin
Rheumatic Heart Disease
Rheumatic heart disease
• Introduction
• Only permanent effect of rheumatic fever (RF)
• Peak age 5-14yrs
• Affects 15·6–19·6 million people worldwide
• 2-3% of those infected with GABHS => RF
• Almost lost in developed countries
• Most common acquired heart disease in Africa
RHD: Pathogenesis
• Group A beta-haemolytic streptococcus pharyngitis =>
• (Genetically susceptible host)
• Immune response =>
• 1-5weeks =>
• Rheumatic fever =>
• Damage of valves
• Rheumatic heart disease
• Repeated infection
• Worsening of RHD
Rheumatic fever: Diagnosis
• Jones criteria for diagnosis (Major)
• Arthritis
• Carditis
• Erythema Marginatum
• Subcutaneous Nodules
• Sydenham's Chorea
• Jones criteria for diagnosis (Minor)
• Arthralgia
• Fever
• Elevated acute phase reactants (CRP, ESR)
• Prolonged PR interval
• Evidence of GABHS infection
• Throat swab +
• Elevated ASO
• Streptococcal antibody positive
RF: Diagnosis
• 2 major criteria
OR
• 1 Major + 2 minor + evidence of prior GABHS infection
Rheumatic heart disease (RHD)
• Often RHD patients not seen at the RF stage
• Report with advanced RHD
• RF =>
• Endocardial inflammation and damage -> permanent
• Myocarditis => Heart failure
• Pericarditis => Pericardial effusion
• Endocardial inflammation=>
• Mitral valve damage
• Regurgitation
• Stenosis
• Aortic valve
• Regurgitation
• Stenosis
RHD: Pathophysiology
• Valvulopathy
• Frequency
• Mitral >aortic>tricuspid>pulmonary
• Only mitral: 76%
• Only aortic: 13%
• Either: 97%
RHD: Pathphysiology
• Acute RF stage (uncommon)
• Carditis
• Myocarditis => Poor systolic function
• Pericardial effusion => poor diastolic function
• Valvular inflammation => regurgitation
• Fever => high output failure
• Post-RF stage
• Myocarditis and pericarditis resolves
• Continued healing and fibrosis of valves =>
• Valvulopathy =>
• Pulmonary oedema =>
• Pulmonary hypertension =>
• Heart failure =>
• Further RF => further damage to heart
RHD: Presentation
• Hardly in the RF stage
• Often at the heart failure stage
• Cough
• Dyspnoea
• Peripheral oedema
• Wasting
• Orthopnea
• PND
• If fever => pneumonia +_ other infections
• Auscultation
• Murmur of MR (PSM @ apex -> axilla)
• MS (diastolic)
• AI (diastolic)
RHD: Investigation
• RF stage:
• CBC
• ESR, CRP
• Throat culture
• ASO titer
• Chest x-ray films
• ECG
• Echocardiogram
• RHD stage
• Chest x-ray (mitralisation of the left ventricle)
• ECG (LAE, LVH)
• Echocardiogram (diagnostic)
RHD: Treatment
• RF stage:
• Carditis => steroids (doubtful efficacy)
• Arthralgia/ arthritis => Aspirin or other NSAIDs
• Sydenham’s chorea => Haloperidol
• Treat pharyngitis : Penicillin
• Sportive treatment
• RHD
• Treat heart failure
• Diuretics (Lasix) + ACE inhibitor
• Eradicate GBHS from throat => penicillin
• Surgery
• Valvulopathy + drug treatment failure
• Delay as long as possible
RHD: Prevention
• Very important!!!
• Community prevention
• Improve economic + environmental status
• Primary prevention
• Screening and treatment of GBAHS carriage with penicillin
• Treatment
• ≤27kg: Phenoxymethylpenicillin 250 mg two or three times daily
• >27kg : Phenoxymethylpenicillin 250 mg two or three times daily OR
• Amoxicillin 50 mg/kg per day for 10 days OR
• ≤27 kg: IM benzathine benzylpenicillin 600 000 IU stat
• >27kg : IM benzathine benzylpenicillin 600 000 IU stat
• Vaccine still in development stage
RHD: Prevention
• Secondary prevention
• ≤27kg: Phenoxymethylpenicillin 250 mg two or three times daily
• >27kg : Phenoxymethylpenicillin 250 mg two or three times daily OR
• ≤27 kg: IM benzathine benzylpenicillin 600 000 IU stat
• >27kg : IM benzathine benzylpenicillin 600 000 IU stat
• Duration of prophylaxis variable but often life long
RF: differential diagnosis
• Other collagen vascular diseases :
• Juvenile rheumatoid arthritis
• Systemic lupus erythematosus
• Postinfectious arthritis:
• e.g. Gonococcal arthritis
• Congenital heart disease
• Viral myocarditis/ pericarditis
Endomyocardial fibrosis (EMF)
Endomyocardial fibrosis
• Thickening of the endocardium of ventricles
• Extends to inner 3rd of myocardium
• Destroys valves
• Diastolic filling defect
• Severe regurgitation: AV valve
• Dilated atria
• Cardiomegaly
• Sidedness
• Right ventricle
• Left ventricle
• Both ventricles
EMF: Epidemiology
• Not well understood
• Prevalence changes for location
• Age: Onset in children and adolescents
• Associations
• Infective agents: Malaria, mycoplasma pneumonia, schistosomiasis
• Autoimmunity
• Hypereosinophilia
• Genetics
• Herbal medications
EMF: Pathophysiology
• Trigger (???) =>
• Inflammation =>
• Necrosis and/or thrombosis
• Endomyocardial scar
• Mitral and tricuspid regurgitation
• Dilated LA + RA
• Clinical course
• Subclinical
• Clinical progressive disease
• Burnt out (no residual abnormality)
EMF: Clinical presentation
• Right ventricle
• Hepatomegaly
• Raised JVP
• Ascites (massive)
• Minimal peripheral oedema
• Often no murmur
• Perioral hyperpigmentation
• Left
• MR murmur
• Signs of left heart failure
• Bilateral (most common)
• Signs of both right and left EMF
EMF: Investigations
• No specific lab investigation
• Eosinophilia (common)
• Hypoproteinemia (advanced stage)
• ECG
• LAE
• RAE
• Others (non-specific)
• Atrial fibrillation (30% of advanced cases)
• Chest x-ray
• Cardiomegaly
• Echocardiogram
• RAE
• LAE
• Fibrosed and small RV +- LV
EMF: differential diagnosis
• Rheumatic heart disease
• Dilated cardiomyopathy
• Constrictive pericarditis
• Tuberculous pericarditis
• Endocardial fibroelastosis
• Löeffler endocarditis
• Ebstein's anomaly
• Hypertrophic cardiomyopathy
EMF: Management
• Medical management
• Acute disease
• Steroids
• Heart failure
• Diuretics
• ACE inhibitors
• β-blockers
• Oral anticoagulants
• Surgical
• Resecting the fibrous endocardium and
• Correcting the atrio-ventricular valve abnormalities
EMF: Prognosis
• Poor
• Most may die within 2 years (maybe 5yrs)
• Recurs after surgical therapy
• Cause of death
• Heart failure
• Thrombo-embolic phenomenon => stroke, pulmonary embolism
Pericarditis
Pericarditis
• Inflammation of the pericardium
• Infectious
• RHD
• Bacterial
• Tuberculous
• Viral
• Non-infectious
• Malignancy
• Uremia
• Autoimmune diseases
• Idiopathic (30%)
Pericarditis: Pathophysiology
• Initial insult =>
• Inflamed pericardium =>
• Exudate + WBCs =>
• Decrease diastolic function
• Cardiac compromise =>
• Fluid build-up =>
• Cardiac tamponade =>
Pericarditis: Viral
• Most common
• Present with 10-14days prodrome (fever, malaise, etc)
• Often associated with myocarditis
• Causative agents
• Coxsackievirus
• Echovirus
• Adenovirus
• Epstein-Barr virus
• Influenza
• HIV
• Presentation
• Fever
• Chest pain
• Friction rub
Pericarditis: Bacterial
• Less common
• Higher mortality
• Presentation
• Toxic
• High fevers
• Irritability
• Chest pain.
• Source
• Local spread: Pneumonia
• Distant spread
• Primary: Post surgery
• Organisms (most common)
• Staphylococcus aureus
• Haemophilus influenzae
Pericarditis: Tuberculous
• TB endemic area with significant pericardial effusion =>
TB pericarditis except proven otherwise
• Predisposition
• Immunocompromised
• Endemic area (e.g. Ghana)
• Spread by:
• Direct extension from LN
• Blood borne from distant site
• Often no extra-pulmonary TB present
• Gradual effusion build-up => massive effusion!!
• Other signs of TB present (wasting, night sweat etc)
Pericarditis: Presentation
• Generally:
• Chest pain:
• Substernal
• Sharp
• Worse on inspiration
• Relieved by sitting upright & leaning forward
• Examination
• Pericardial friction rub
• Loudest when upright and leaning forward
• Distant heart sound
• Others due to specific cause
Pericarditis: Investigation
• CBC
• Acute phase reactants: CRP, ESR
• Chest x-ray
• Troponin: -> myocardial involvement
• Tuberculin skin test
• Pericardiocentesis
• Culture: Bacterial, viral, Tuberculous
• LDH
• Adenosine deaminase
• WBCs
• etc
Pericarditis: Investigation
• ECG
• Stage I: ST segment elevation
• Stage II: Flat to inverted T-waves
• Stage III: T wave inversions
• Stage IV: ECG returns to pre-pericarditis state.
• Echocardiogram:
• Diagnostic
• Quantify
• Type:
• Debris or not!!!
Pericarditis: Treatment
• Viral / Idiopathic pericarditis
• NSAIDs
• Colchicine
• Corticosteroids
• Bacterial pericarditis
• Antibiotics: Penicillin, cepahalosporins,
• Sensitivity of organism to determine
• Analgesia
• Antipyretics
• Tuberculous
• Corticosteroids
• Anti-tuberculous treatment
Pericarditis: Treatment
• Pericardiocentesis: indication
• Cardiac tamponade
• Hemodynamic compromise
• Purulent pericarditis
• Suspected neoplastic pericarditis
• Pericardial window (resistant cases)
 Pericarditis:
• Complications
• Cardiac tamponade
• Constrictive pericarditis
• Prognosis
• Viral: Very good
• Bacterial:
• Fatal if untreated.
• Aggressive treatment => good prognosis (?40% mortality)
• Tuberculous:
• Fatal if untreated,
• Good when treated (?%)
Conclusion
• We have learnt about
• Infective endocarditis
• Rheumatic heart disease
• Endo myocardial fibrosis
• Pericarditis
Thank you!!!