ACQUIRED HEART DISEASES

Rheumatic Fever and Rheumatic Heart

Disease
Rheumatic Fever
• Rare in the developed world, but remains the most important cause of heart
disease in children worldwide.

• Remain endemic in some parts of Asia (China, Indonesia, India, Pakistan,

Bangladesh), Africa and South America.

• Uncommon in Malaysia

• Improvements in sanitation, reduction in overcrowded living conditions,

treatment of streptococcal pharyngitis with 10-day course of antibiotics and
changes in streptococcal virulence have led to its virtual disappearance in
developed countries.
• Acute rheumatic fever is a short-lived, multisystem
autoimmune response to a preceding infection with group A
β-haemolytic streptococcus.

• Mainly affects children aged 5–15 years.

• Progresses to chronic rheumatic heart disease in up to 80%

of cases.

• After a latent interval of 2–6 weeks following a pharyngeal or

skin infection, polyarthritis, mild fever and malaise develop.
Pathogenesis RHD and valvular damage

Group A Produce antibody

Streptococcal against M proteins
sore throat of Streptococci
Cardiac myosin and
sarcolemma has Antibody cross
structural similarity to reacts with
streptococcal M protein myosin and
CD4 sarcolemma
T-cell

Acute Inflammation of
carditis in Endocardium,
Valvular Myocardium and
Rheumatic
damage pericardium
fever
in RHD
 Erythema Marginatum

Subcutaneous Nodules
Chronic rheumatic heart disease
• The most common form of long-term damage from scarring and
fibrosis of the valve tissue of the heart is mitral stenosis.

• If there have been repeated attacks of rheumatic fever with carditis,

this may occur as early as the second decade of life, but usually
symptoms do not develop until early adult life.

• Although the mitral valve is the most frequently affected, aortic,

tricuspid and rarely, pulmonary valve disease may occur.
Pathology (Mitral Stenosis/Regurgitation)

Progressive Fusion of the Mitral Mitral Stenosis Increase left atria

with or pressure, leads to
Fibrosis, Valve Commissures
pulmonary venous
Calcification on and Shortening of without congestion and
Chordae Tendineae
Mitral Valve Regurgitation breathlessness
Clinical Features (Mitral Stenosis)
• Symptoms • Signs
• Breathlessness (Pulmonary • Atrial Fibrillation
Congestion) • Mitral Facies
• Fatigue (low CO) • Auscultation- Loud S1, Mid Diastolic
• Oedema, Ascites (RHF) Murmur
• Palpitation • Crepitation, Pulmonary Oedema
• Haemoptysis
• Cough
• Chest Pain
Pathology (Aortic Stenosis/Regurgitation)
Progressive Fibrosis Distortion and Stenosis with or
on Aortic and Increase Rigidity of without
Tricuspid Valves the Cusps Regurgitation
Clinical Features

Signs • Pulses
• Mild to Moderate • Collapsing Pulse
• Often asymptomatic • Low diastolic pressure
• Palpitation • Murmur
• Early Diastolic Murmur (aortic
regurgitation)
• Severe • Systolic Murmur (aortic stenosis)
• Dyspnoea • Others
• Angina • Displaced Apex
• Fourth Heart Sound
• Crepitation

Symptoms
Rheumatic heart disease(Ix , Mx)

Rheumatic heart disease is a chronic,

disabling and sometimes fatal
disease. It is 100% preventable
 Lab Findings
• FBC : anemia , leucocytosis
• CRP : Elevated
• ESR : Increased
• ASO titre : >200 Todd units (peak at 3 weeks , back to normal
by 6 weeks)
• Anti-DNAse B test : typically remain elevated longer than ASO
antibody titers
• Throat culture-GAB streptococci
• ECG : prolonged PR interval , 2nd/ 3rd degree
heart block , ST depression , T inversion
• Echocardiograph : valvular edema , mitral
regurge , LA , LV dilatation , pericardial
effusion , decreased contractility
 Diagnosis
• Rheumatic fever is mainly a clinical dx
• No single diagnostic sign or specific lab test available for
diagnosis
• Diagnosis based on MODIFIED JONES CRITERIA
 Differential diagnosis
• Juvenile rheumatoid arthritis
• Septic arthritis
• Sickle cell arthropathy
• Kawasaki disease
• Myocarditis
• Scarlet fever
• Leukemia
 Treatment
1. Primary prevention (eradication of streptococci)
2. Anti-inflammation (aspirin, steroid)
3. Supportive management & management of complication
(surgical/medical)
4. Secondary prevention (prevent recurrent attack)
 Primary prevention of rheumatic fever
• Treat strep. Tonsillipharyngitis

• Benzathine penicillinG 600000U IM Once OR

• Penicillin V Children 250mg Oral 2-3 times daily for 10 days
OR
• Erythromycin 20-40 mg/kg/d 2-4 times daily Oral for 10 days
 Anti-inflammation treatment
• Arthritis only : Aspirin 75-100mg/kg/day , give as 4 divided
doses for 6 weeks

• Carditis : Prednisolone 2-2.5 mg/kg/day, give as 2 divided

doses for 2 weeks
 Supportive management
• Bed rest
• Treatment of congestive heart failure
• digitalis , diuretics
• Treatment of chorea
• Diazepam , haloperidol
• Rest of joints & supportive splinting
 Secondary prevention
• Prevent recurrence

• Benzathine penicillin G 1200000U every 4 weeks IM OR

• Penicillin V 250mg twice daily Oral OR
• Erythromycin 250mg twice daily Oral
 Duration of prophylaxis
Category
Rheumatic fever with carditis
and residual heart disease
(persistent valvular disease)
Rheumatic fever with carditis
but no residual heart disease
(absence of valvular disease)
Rheumatic fever without
carditis
Duration
At least 10yrs since last
episode and at least 40 yrs ,
sometimes lifelong
prophylaxis
10y or well into adulthood
whichever is longer
5y or until age of 21y
Whichever is longer
 Surgical treatment
• Severe mitral/ aortic valve dysfunction that causes persistent
heart failure requires corrective surgery

• Commussurotomy : open a stenotic valve by scalpel incision.

• Valvuloplasty : improve blood flow by passing catheter into the
valve through a hole temporarily created in the septal wall.
• Valve replacement by Arbor technique
Infective Endocarditis
Infective endocarditis
• Often a complication of congenital or rheumatic heart disease but can also
occur in children without any abnormal valves or cardiac malformations.

• Risk is highest when there is a turbulent jet of blood, as with a VSD,

coarctation of the aorta and PDA or if prosthetic material has been inserted
at surgery.

• Turbulent flow traumatizes the vascular endothelium, creating a substrate

for deposition of fibrin and platelets, leading to the formation of a
nonbacterial thrombotic embolus (NBTE) that is thought to be the initiating
lesion for infective endocarditis.

• Leading to proliferation of bacteria within the lesion.

• Includes acute and subacute bacterial endocarditis as well as
nonbacterial endocarditis caused by viruses, fungi, and other
microbiologic agents.

• It is a significant cause of morbidity and mortality in children and

adolescents despite advances in the management and prophylaxis of
the disease with antimicrobial agents.

• The presence of the classical peripheral stigmata of infective

endocarditis should not be relied upon.
ETIOLOGY
• No relationship exists between the infecting organism and the type of
congenital defect, the duration of illness, or the age of the child.
• Staphylococcal endocarditis is more common in patients with no
underlying heart disease
• Viridans group streptococcal infection is more common after dental
procedures
• Pseudomonas aeruginosa or Serratia marcescens is seen more
frequently in intravenous drug users
• Fungal organisms are encountered after open heart surgery
• Coagulase-negative staphylococci are common in the presence of an
indwelling central venous catheter.
Clinical Features
 Osler nodes

Splinter Haemorrhages

Janeway Lesions
Infective Endocarditis
Investigation and Management

Chuah Wei Hong

Roth’s Spot
Investigations
• Blood cultureS
• Echocardiography
• Identify vegetation but not exclude
• CRP/ESR
• Also in monitoring response to treatment
• Full blood count
• Urine FEME
• Chest X-ray
Principle of Management
• Use empirical antibiotics, until culture results available
• Ensure 3 blood cultures taken before antibiotic therapy
• Do not wait for echocardiography

• Rx
• IV high dose penicillin + aminoglycoside 6/52
• Surgical removal (infected prosthetic material)
IE Prophylaxis
• Practice of good dental hygiene in all children with congenital heart
disease

Antibiotic prophylaxis
• Required in
• Oral, dental procedures
• Surgery (likely to be associated with bacteraemia)
Antibiotic Prophylaxis
Antibiotic Prophylaxis
Antibiotic Prophylaxis
HEART FAILURE
Lim Kok Hong
Definition
• Inability of the heart to provide adequate cardiac output to meet the
metabolic demand of the body.
• primary determinants of stroke volume are the afterload (pressure
work), preload (volume work), and contractility (intrinsic myocardial
function).
• molecular/cellular level, such as upregulation or downregulation of
various metabolic pathway components leading to changes in
efficiency of oxygen and other substrate utilization.
• neurohormones such as the renin–angiotensin system and the
sympathoadrenal axis.
 Heart
Failure

Low High
output output
Due to cardiac problem no basic abnormality in myocardial function, CO is
normal or increased, due to decreased in systemic
O2 content or increase O2 demand
Clinical features
• Varies according to age
• Infancy :
Symptoms : poor feeding, recurrent chest infection, failure to thrive,
excessive perspiration, weak cry, irritability
Signs : tachypnea, wheezing, subcostal and intercostal recession, nasal
flaring, tachycardia, weak pulses, hyperactive precordium, praecordial
bulge, hepatomegaly, edema generalized / usually involves eyelids,
sacrum
• Children:
Similar to adults
Fatigue, effort intolerance, anorexia, dyspnea, cough, abdominal
symptoms ( pain, nausea) , increase JVP, hepatomegaly,
edema/anarsaca, gallop rhythm, systolic murmur due to valve
regurgitation caused by advanced ventricular dilation
Diagnosis & Investigation
• X-ray : cardiac enlargement, increase pulmonary vascularity ( left to
right shunt) , perihilar pulmonary marking ( venous congestion, acute
pul. Edema)
• ECG : chamber hypertrophy
• Echocardiography : assessment of ventricular function
• Doppler : estimate Cardiac output, flow of blood,
• MRI : quantifying left & right ventricular function, volume, mass and
coronary artery anatomy, regurgitant fraction
• ABG : arterial oxygen level, respiratory/ metabolic acidosis
• Serum B-type natriuretic peptide : increased in cardiomyopathy,
volume overload HF
Treatment
General Measures
• Oxygen supplement
• Propped up position
• Keep warm
• Fluid restriction
• Optimize calories intake
• Correct anemia, electrolyte imbalance, chest infection
Pharmacological
• Diuretics
• Afterload reducer ( ACE inhibitors, Angiotensin II Receptor Blockers)
• Digitalis glycosides ( digoxin)
• α- and β-Adrenergic Agonists ( dopamine)
• Phosphodiesterase Inhibitors (Milrinone)
Diuretics
• Frusemide (loop diuretic)
• Dose: 1 mg/kg/dose OD to QID, oral or IV
• Continuous IV infusion at 0.1 – 0.5 mg/kg/hour if severe fluid overload
• Use with potassium supplements (1 - 2 mmol/kg/day) or add potassium
sparing diuretics.

• Spironolactone (potassium sparing diuretic, modest diuretic effect)

• Dose: 1 mg/kg/dose BD
Captopril
• Angiotensin converting enzyme inhibitor, afterload reduction agent
• Dose: 0.1 mg/kg/dose TDS, gradual increase up to 1 mg/kg/dose TDS
• Monitor potassium level (risk of hyperkalaemia)

Digoxin
• Role controversial
• Useful in heart failure with excessive tachycardia, supraventricular
tachyarrhythmias.
IV inotropic agents - i.e. Dopamine, Dobutamine, Adrenaline, Milrinone
• Use in acute heart failure, cardiogenic shock, post-op low output
syndrome.
Specific management

• Establishment of definitive aetiology is of crucial importance

• Specific treatment targeted to underlying aetiology. Examples:
• Surgical/transcatheter treatment of congenital heart lesion.
• Pacemaker implantation for heart block.
• Control of blood pressure in post-infectious glomerulonephritis.
• High dose aspirin ± steroid in acute rheumatic carditis.
References
• Nelson Paedriatic 20th edi
• Paediatric protocol 3rd edi
• Sunflower 4th edi