Acute rheumatic fever is a delayed autoimmune reaction to group A streptococcal infection and its long term sequelae.
Valvular involvement , particularly of the left side heart valves is the major manifestation of rheumatic hear disease.
Valvular heart disease related to endothelial damage of the valves, as these have a small core of connective tissue covered by two endothelial layers.
Involvement of the valves connective tissue along with endothelial inflammation leads to a chronic disease process.
Rheumatic fever develops after untreated Streptococcus pyogenes infection and causes rheumatic heart disease.
More than 40.5 million people worldwide estimated to be living with rheumatic heart disease, and approximately 306,000 deaths from RHD occur annually.
The mitral valve is universally involved by RHD, while aortic valve disease occurs only in about 2% of patients.
In advanced RHD the tricuspid valve and very rarely pulmonary valve can be affected.
Heterogeneity exists in relation to age of onset, mode of presentation, and specific valvular abnormality.
Rheumatic heart disease has virtually been eliminated from high income countries where rheumatic heart disease is predominantly reported in the aged population, a consequence of acute rheumatic fever several decades previous, and usually occurring during childhood.
Rheumatic heart disease is a leading non-communicable disease of the young in poor countries.
RHD causes more than 300,000 deaths annually, mainly in low and middle income countries.
Most patients with symptomatic advanced rheumatic heart disease not have a history of acute rheumatic heart disease.
In 2010 an estimated 34.2 million people worldwide had rheumatic heart disease, with 345,110 deaths and 10.1 million disability-adjusted life years lost per year.
Global burden of disease estimate 0.49% of the global population have rheumatic heart disease.
Children and adolescents between five and 15 years are the greatest risk of a primary acute rheumatic fever episode.
In a prospective observational study of 13,696 patients from low and middle income countries, nearly 15% of patients died at three years, mostly due to heart failure or sudden death: In this steady corrected valve surgery or valvoplasty was independently associated with the reduced risk of death (INVICTUS investigators).
The peak prevalence of rheumatic heart disease is between 25 and 45 years of age.
The cumulative effect of recurrent episodes of acute rheumatic fever is reflected by the delayed case detection.
Patients younger than 10 years of age predominantly have pure mitral regurgitation.
In the second decade of life RHD is characterized by mixed mitral valve disease.
By the third decade mitral stenosis dominates.
Concomitant aortic valve disease increases with age.
Secondary antibiotic prophylaxis is the corner stone of management of rheumatic fever and rheumatic heart disease.
Intramuscular penicillin G benzathine is more effective than oral antibiotic agents in preventing streptococcal pharyngitis and its use is associated with a lower incidence of recurrence of rheumatic fever.
Prophylaxis allows for regression of valvular damage and may prevent death from rheumatic heart disease.
Among children and adolescents 5 to 17 years of age with latent rheumatic heart disease, secondary antibiotic prophylaxis reduces the risk of disease progression at 2 years.