Rheumatic Heart Diseases

Epidemiology and Control

 Rheumatic fever (RF) and rheumatic heart disease (RHD) are nonsuppurative complications of Group A streptococcal pharyngitis due to a delayed immune response I n economically developed countries, rheumatic fever and rheumatic heart disease have become uncommon health problems. In Third World areas rheumatic fever remains the leading cause of heart disease in children and young adults

 evidence suggests that there has been little if any decline in the occurrence of RHD over the past few decades Recent reports from the developing world have documented rheumatic fever (RE) incidence rates as high as 206/100 000 and RHD prevalence rates as high as 18.6/1000. the incidence of acute rheumatic fever in industrialized countries has dropped precipitously to below 1/100 000, whereas in Sudan it exceeds 100/100 000. (although there has been no decline in group A streptococci pharyngitis) The prevalence of chronic rheumatic heart disease has been estimated mainly from surveys of school-going children and varies from 2.7/1000 in Nairobi to 14.3/1000 in Kinshasa

 In both developing and developed countries, pharyngitis and skin infection (impetigo) are the most common infections caused by group A streptococci. Group A streptococci are the most common bacterial cause of pharyngitis, with a peak incidence in children 515 years of age Streptococcal pharyngitis is less frequent among children in the first three years of life and among adults. It has been estimated that most children develop at least one episode of pharyngitis per year, 1520% of which are caused by group A streptococci

 The incidence of pharyngeal beta-haemolytic streptococcal infections vary between countries and within the same country depending upon:
Season age group socioeconomic conditions environmental factors the quality of health care

 congestive heart failure (CHF) that required repeated hospitalisation. A large proportion of the individuals with CHF required cardiac valve surgery within 510 years The mortality rate for RHD varied from 0.5 per 100 000 population in Denmark, to 8.2 per 100 000 population in China the estimated annual number of deaths from RHD for 2000 was 332000 worldwide The mortality rate per 100 000 population varied from 1.8 in the WHO Region of the Americas, to 7.6 in WHO South-East Asia Region.

Determinants of rheumatic fever and rheumatic heart disease

socioeconomic and environmental factors (indirect role):
Shortage of resources for providing quality health care Inadequate expertise of health-care providers Low level of awareness of the disease in the community Crowding

Determinants Socioeconomic environmental factors: (poverty, under nutrition, overcrowding ,housing).

Effects Rapid spread of group A streptococcal strains. Difficulties in accessing health care.

Impact on RF and RHD burden Higher incidence of acute streptococcal-pharyngitis and suppurative complications. Higher incidence of acute RF. Higher rates of recurrent attacks. Higher incidence of acute RF and its recurrence. Patients unaware of the first RF episode More severe evolution of disease. Untimely initiation or lack of secondary prophylaxis Higher rates of recurrent attacks with more frequent and severe heart valve involvement, and higher rates of repeated hospital admissions and expensive surgical interventions.

Health-system related factors: shortage of resources for health care; inadequate expertise of health-care providers low-level awareness of the disease in the community

Inadequate diagnosis and treatment of streptococcal pharyngitis Misdiagnosis or late diagnosis of acute RF

Inadequate secondary prophylaxis and/or noncompliance with secondary prophylaxis

Primary prevention of rheumatic fever

the adequate antibiotic therapy of group A streptococcal upper respiratory tract (URT) infections to prevent an initial attack of acute RF Primary prevention is administered only when there is group A streptococcal URT infection The therapy is therefore intermittent

Primary prevention of rheumatic fever

Although a cost-effective vaccine for group A streptococci would be the ideal solution, scientific problems have prevented the development of such a vaccine There have been no controlled studies showing that tonsillectomy is effective in reducing the incidence of RF

Secondary prevention of rheumatic fever

the continuous administration of specific antibiotics to patients with a previous attack of RF, or a welldocumented rheumatic heart disease (RHD). The purpose is to prevent colonization or infection of the upper respiratory tract (URT) with group A betahemolytic streptococci and the development of recurrent attacks of RF. Secondary prophylaxis is mandatory for all patients who have had an attack of RF, whether or not they have residual rheumatic valvular heart disease

 Health education activities Health education activities should address both primary and secondary prevention. The activities may be organized by trained doctors, nurses or teachers and should be directed at the public, teachers and parents of school-age children. Health education activities should focus on the importance of recognizing and reporting sore throats early; on methods that minimize and avoid the spread of infection; on the benefits of treating sore throats properly; and on the importance of complying with prescribed treatment regimes. Training health-care providers

 Epidemiological surveillance Surveillance of acute RF and RHD Community and school involvement

Risk factors
pharyngitis due to group A streptococci in the pathogenesis of this disease overcrowding, close person-to-person contact, and poor healthcare facilities, all of which have been shown to be the most consistent predisposing epidemiological factors for rheumatic fever.

 The high frequency of RHD in the developing world necessitates aggressive prevention and control measures. The major interventions for prevention and control include: (1) reduction of exposure to group A streptococci, (2) primary prophylaxis to prevent initial episodes of RF, and (3) secondary prophylaxis to prevent recurrent episodes of RE. Because recurrent episodes of RE cause increasingly severe cardiac complications, secondary prophylaxis is the most crucial feature of an effective RHD programme. For some impoverished countries, secondary prophylaxis may be the only intervention that can realistically be implemented. In addition to this intervention, however, financial and human resources must be committed, and all of these elements must be integrated into existing primary health care systems. Because RHD continues to be a common health problem in the developing world, greater emphasis needs to be placed on the simple and cost-effective prevention and control measures that are currently available to combat this disabling disease.

 prevention of the first attack of rheumatic fever by early treatment of streptococcal pharyngitis (primary prevention) or prevention of recurrent attacks of rheumatic fever (secondary prevention) is the only way to prevent rheumatic heart disease. Secondary prevention is a more costeffective and attainable goal than primary prevention. A recent systematic review of the most effective antibiotic regimen for secondary prophylaxis45 confirms the World Health Organization recommendation3 of 3 weekly intramuscular injections of benzathine penicillin.

The decline in rheumatic fever and rheumatic heart disease in most of the Western World occurred before their origin and pathogenesis were fully understood. Many questions remain unanswered. Decreases in the frequency and, perhaps, virulence of streptococcal infections may occur after the general socioeconomic status and living conditions improve. Although this is an important goal, it is unlikely that many developing countries can substantially decrease their incidence of rheumatic fever at any time in the near future through such improvements.

 The definitive form of primary prevention of rheu matic fever would be a streptococcal vaccine that uses the surface M protein. The development of a safe and effective streptococcal vaccine has been hindered by two major obstacles. First, although a limited number of the more than 80 distinct serotypes are rheumatogenic, an effective vaccine would still have to be a highly complex mixture of M proteins from multiple serotypes. Second, M protein contains epitopes that cross-react with human tissue. Antibodies directed against these epitopes could actually produce rheumatic

 matic fever would be a streptococcal vaccine that uses the surface M protein. The development of a safe and effective streptococcal vaccine has been hindered by two major obstacles. First, although a limited number of the more than 80 distinct serotypes are rheumatogenic, an effective vaccine would still have to be a highly complex mixture of M proteins from multiple serotypes. Second, M protein contains epitopes that cross-react with human tissue. Antibodies directed against these epitopes could actually produce rheumatic

 matic fever would be a streptococcal vaccine that uses the surface M protein. The development of a safe and effective streptococcal vaccine has been hindered by two major obstacles. First, although a limited number of the more than 80 distinct serotypes are rheumatogenic, an effective vaccine would still have to be a highly complex mixture of M proteins from multiple serotypes. Second, M protein contains epitopes that cross-react with human tissue. Antibodies directed against these epitopes could actually produce rheumatic

 matic fever would be a streptococcal vaccine that uses the surface M protein. The development of a safe and effective streptococcal vaccine has been hindered by two major obstacles. First, although a limited number of the more than 80 distinct serotypes are rheumatogenic, an effective vaccine would still have to be a highly complex mixture of M proteins from multiple serotypes. Second, M protein contains epitopes that cross-react with human tissue. Antibodies directed against these epitopes could actually produce rheumatic matic fever would be a streptococcal vaccine that uses the surface M protein. The development of a safe and effective streptococcal vaccine has been hindered by two major obstacles. First, although a limited number of the more than 80 distinct serotypes are rheumatogenic, an effective vaccine would still have to be a highly complex mixture of M proteins from multiple serotypes. Second, M protein contains epitopes that cross-react with human tissue. Antibodies directed against these epitopes could actually produce rheumatic