Rheumatic heart disease

Presented by:

Asmaa nouh

Supervised by:

Prof. Dr. Ashraf Mansour

Rheumatic fever

Rheumatic fever is an inflammatory disease that may affect many connective tissues of the body, especially those of the heart, joints, brain or skin. It usually starts out as a streptococcal throat infection.

Anyone can get acute rheumatic fever, but it usually occurs in children between the ages of 5 and 15 years.

About 60% of people with rheumatic fever develop some degree of subsequent heart disease.

Rheumatic fever (cont.)

Rheumatic fever can cause the heart to inflame and leave permanent damage to the heart, specifically the heart valves.

A heart valve acts like a one-way door. It makes sure that blood pumped by the heart flows in one direction.

When the heart fails, the heart valves are unable to function adequately.

Rheumatic fever (cont.)

Every part of the heart, including the the

pericardium, the endocardium and the valves may

be damaged by inflammation caused by acute

rheumatic fever. However, the most common form of

rheumatic heart disease affects the heart valves,

particularly the mitral valve.

It may take several years after an episode of

rheumatic fever for valve damage to develop or

symptoms to appear.

Antibiotics can prevent streptococcal infection from

developing into rheumatic fever.

In conclusion RHD is a chronic heart condition that

Definition:

Rheumatic heart disease describes a group of short-term (acute) and long-term (chronic) heart disorders that can occur as a result of rheumatic fever.

One common result of rheumatic fever is heart valve damage leading to valve disorder. This can lead to heart failure and sometimes the need for cardiac surgery.

Rheumatic heart disease is the most common form of heart disease among children in the world.

Magnitude of the problem:

Rheumatic heart disease (RHD) is the most common

acquired heart disease in children in many countries

of the world, especially in developing countries.

The global burden of disease caused by rheumatic

fever currently falls disproportionately on children

living in the developing world, especially where

poverty is widespread and is responsible for about

233,000 deaths annually.

At least 15.6 million people are estimated to be currently affected by RHD with a significant number of them requiring repeated hospitalization and, often unaffordable, heart surgery in the next 5 to 20 years.

The worst affected areas are sub-Saharan Africa, south-central Asia, the Pacific and indigenous populations of Australia and New Zealand.

Up to 1% of all schoolchildren in Africa, Asia, the Eastern Mediterranean region, and Latin America show signs of the disease.

Magnitude of the problem in Egypt:

Egypt is known to be endemic for rheumatic heart

disease.

A cross sectional screening of a randomly selected 3062

school children between the ages of 5 and 15 years in

Aswan was done in 2012.

The children underwent detailed physical and trans-

thoracic echocardiographic examination. A set of rigid

criteria including structural and functional changes in

left sided valves, were used to determine the presence,

distribution and severity of rheumatic affection.

Definite and probable rheumatic valve affection was

present in 23/1000 for mitral valve and 13.4/1000 for

aortic valve affection.

Mitral valve affection (mainly regurgitation) was present

in 96%, aortic regurgitation in 2.9% and combination of

both in 1.4%.

Left ventricular function was normal in all patients.

Auscultation revealed a murmur in 7.1% of children with echocardiographic evidence of rheumatic heart disease showing a 14 fold increased detection rate by echocardiography.

These findings are important for instituting early secondary prevention and stimulating future development of specific biomarkers and vaccine development.

Factors related to occurrence of RHD in Egypt were studied using a case control design. Low socioeconomic status, chronic tonsillitis, positive family history of acute rheumatic fever/RHD and paternal consanguinity were significantly associated with the occurrence of RHD.

Ecology:

a- Agent

The causative organism is Group A beta streptococci

affecting the upper respiratory tract. It is gram +ve

cocci. There are 3 theories about the pathogenesis of RF:

1- Direct infection of the Heart and valves by group A

streptococci.

2- Extracellular toxin produced by the organism damages

the target organs (Heart, synovium and brain).

3- Abnormal immune response by the human host.

b- Host

Age: any age and especially school age (5-15 years).

Sex: both sexes are equally affected.

Social standard: URT infection with low socioeconomic state at home and school because of crowding and poor hygiene.

School children are specially easily infected at school because of:

1- The crowding and poor ventilation in classes.

2- Unsound health habits among them as contact of secretions with fingers or rubbing nose.

3- School children may have some sort of nutritional deficiencies especially vit A susceptibility to RTI.

4- Immuno compromised children with chronic debilitating diseases like diabetes or renal diseases or on steroids or immunotoxic drugs e.g those of nephrotic syndrome.

c- Environment factors

Temperate climate increases the infection with streptococci as in fall and early spring but infection during winter is more serious.

School environment has a major role in spread of U.R.T infections as:

1- Site of school if it is near of a source of air pollution like factory with smoke or dust infection or in rural areas due to combustion of biofuels such as wood, crop residues and animal dung.

2- Over crowded or ill ventilated classrooms.

3- Unsanitary water circuits without soaps to wash hands or without proper amount of water.

4- Overcrowding in school buses.

5- Canteen personnel if they have not sound habits and not routinely washing their hands after coughing or sneezing.

6- Home environment with smoking parents and low socioeconomic students with humid overcrowded houses.

Symptoms:

symptoms may include:

Chest pain.

Heart palpitations.

Breathlessness on exertion.

Breathing problems when lying down.

Waking from sleep with the need to sit or stand up.

Swelling of lower limbs.

Fainting.

Pathology:

1- The connective tissues of the heart:

a- Acute inflammatory stage (2-3 weeks):

The myocardium: cellular infiltrate and damage to muscle cells.

The pericardium: It is covered with fibrinous exudates.

The endocardium and valve leaflets: Edematous and infiltrated by lymphocytes.

b- Proliferative stage:

The myocardium: Ashcoff bodies (the pathognomonic lesion of rheumatic fever) consist of large multinucleated cells arranged around a vascular core of fibrinoid material fibrosis.

The valve leaflets: vegetations made up of masses of eosinophilicmaterial along the edge of the valves fibrosis scarred thickened valve cusps.

Pathology(cont.):

2- The connective tissue of synovium and skin:

edematous cellular infiltrate and also serous effusion into the joint space. There is never erosion of joint surfaces.

3- CNS:

cellular degeneration and hyalinization of small blood vessels scattered throughout the cortex, cerebellum, and basal ganglia. No Ashcoff bodies have been found in the CNS.

4- Subcutaneous nodules:

central area of fibrinoid necrotic material surrounded by fibroblasts and occasional lymphocytes.

Impact of RHD on the child

Physical impact: the child is deprived from doing

exertion, decreased activity compared with other

colleagues as during PE classes.

Psychological impact: feeling of fear about the

future, anxiety, depression and sense of being

hopeless.

Economic impact: high cost of treatment, surgery

and rehabilitation.

Impact of RHD on the family:

1- Shock: the patient and parents have inappropriate social responses.

2- Denial: as they do not accept the fact. It is a defense and grief stage.

3- Anger: the targets for rage are doctors and hospital personnel.

4- Depression: due to sense of hopelessness about the future and realization of the decreased activity.

5- Seeking information: the patients looking after information about the condition and for its treatment to minimize its effects.

6- An economic burden on the family, for the cost of treatment, diagnosis, hospitalization and surgeries and rehabilitation equipments.

7- Complications of Rheumatic fever: Vulvular affection congestive heart failure and/or pulmonary hypertension some sort of handicapping.

Impact of RHDs on the society:

Decreased efficiency for work by patients, the cost

of treatment, diagnosis, hospitalization and

rehabilitation equipments are burdens especially in

developing countries.

Prevention:

Primary prevention

1- eradication of streptococcal infection

Early diagnosis (using clinical and lab testing) and treatment of streptococcal infection is mandatory for prevention of rheumatic heart disease.

It may be difficult to establish a diagnosis of streptococcal pharyngitis in the individual patient on clinical grounds alone.Tests for group A streptococci in the upper respiratory tract are thus important in ensuring appropriate clinical management of the patient.

The streptococcal infection can usually be eradicated by a

single intramuscular injection of benzathine

benzylpenicillin or by ten days' treatment with oral

penicillin, although treatment failures have been reported.

Other alternatives to penicillin: e.g. Erythromycin,

clindamycin, ampicillin, amoxicillin & cephalexin.

2- Immunoprophylaxis:

It is one possible alternative approach in the future. It

includes immunization against the more common

serotypes of group A streptococci, in particular, those

associated with rheumatic fever.

3- Health education:

Since the attacks of acute rheumatic fever occur

primarily in children, education of parents regarding the

importance of recognizing and tracing streptococcal

sore throat to prevent rheumatic heart disease is

warranted.

Health education can be accomplished through the

primary health care system. also through media e.g; in

areas where all levels of the population can be reached

by television.

Since there are more teachers than health professionals, and more schools than health centres, the school can be the ideal area both for health education and for identifying children with pharyngitis and the early manifestations of rheumatic fever. Health fairs in schools can be effective in increasing the awareness of schoolchildren and parents.

Efforts to ensure primary prevention of rheumatic fever at the community level should be focused on education of the general public on the importance of proper treatment of sore throat, and on the importance of early diagnosis and effective treatment of streptococcal pharyngitis, especially in children.

Secondary prevention

Secondary prevention refers to the early detection of

disease and implementation of measures to prevent the

development , recurrence or worsening of the disease.

Diagnosis:

According to revised Jones criteria, the diagnosis of

rheumatic fever can be made when two of the major

criteria, or one major criterion plus two minor criteria, are

present along with evidence of streptococcal infection

(elevated or rising antistreptolysin O titre or DNAase).

Major criteria:

1- Carditis (40%-80% of cases)

2- Polyarthritis (about 75% of cases)

3- Chorea (10-15% of cases)

4- Erythema marginatum

5- Subcutaneous nodules

Minor criteria:

1- Fever: Not higher than 38.8°C.

2- Arthralgia: Joint pain in absence of redness, warmth and limitation of movement.

3-Raised erythrocyte sedimentation rate or C reactive protein.

4-Leukocytosis.

5-ECG showing features of heart block, such as a prolonged PR interval (Cannot be included if carditis is present as a major symptom).

6-Previous episode of rheumatic fever or inactive heart disease.

Evidence of group A streptococcal infection:

Culture may be negative in cases of early antibiotic

therapy, in Rheumatic chorea, or in late onset

carditis.

Recent scarlet fever.

Elevated or rising titres of streptococcal antibodies.

Treatment:

WHO in 2001 stated the long-term management of individuals who have been diagnosed with ARF or RHD, excluding management of heart failure. It also discussed issues relating to population-based ARF/RHD control strategies. In the case of ARF/RHD, this has become synonymous with secondary prophylaxis.

Continuous administration of specific antibiotics to patients with a previous attack of rheumatic fever, or well-documented rheumatic heart disease to prevent colonization of infection of the upper respiratory tract with group A beta-hemolytic streptococci and the development of recurrent attacks of rheumatic fever.

Two main methods of prophylaxis are available:

IM Benzathin penicillin 1.200.000u every/ 3wks.

Or

Oral penicillin V 250mg bid (twice/day)

Sulfadiazine 500mg od (once/day)

Erythromycin 250mg bid (twice/day) (Nelson 1992)

The regular I.M. injection of penicillin is the most effective

method available, even for individuals shown to be compliant

with oral antibiotics, the rheumatic fever recurrence rate is

higher than in comparable patients receiving IM Benzathin

penicillin.

The effectiveness of secondary prophylaxis is impaired

by factors affecting adherence to antibiotic regimens

and by the broad determinants that drive the ongoing

incidence of ARF.

These factors relate to overcrowded housing, poor

access to health services, limited educational

opportunities and poor environmental conditions, all of

which are strongly related to poverty.

From the available evidence, secondary prevention should include:

Strategies aimed at improving the delivery of secondary prophylaxis and patient care.

The provision of patient (and family) education.

Support for patients to improve self-management.

Coordination of available health services.

Structured and sustained routine care and follow up.

The establishment of local, regional and/or national control programs.

A commitment to advocacy for necessary and appropriate resources for people at risk of or with ARF/RHD.

Duration of secondary prophylaxis:

Patients without carditis in a previous attack should have

prophylaxis for a minimum of 5 years after the last attack,

and at least until the age of 18 years and sometimes later.

Patients with cardiac involvement in the initial attack should

continue prophylaxis at least until the age of 25 years, and

later if environmental conditions or other risk factors

warrant it.

Penicillin prophylaxis for rheumatic fever should be

continued through pregnancy. However, sulfadiazine

represents a risk to the fetus and alternative antibiotics

should be substituted.

For patients with chronic valvular rheumatic heart

disease, secondary prophylaxis for prolonged periods,

even for life, has sometimes been recommended.

Patients who have had cardiovascular surgery for

rheumatic heart disease are still at risk for recurrence of

rheumatic fever and require secondary prophylaxis.

Secondary prevention programs are the most realistic

approach to the prevention of rheumatic fever and

rheumatic heart disease at this time.

Tertiary Prevention:

Tertiary prevention refers to the treatment options

for patients diagnosed with rheumatic heart

disease.

The goal of tertiary prevention measures is to

minimize further damage, as once the heart

valves have been damaged, the prevention

measures have palliative rather than curative

effect, and to reduce the disabilities caused by

symptoms and complications.

Depending on the type and severity of a

patient’s rheumatic heart disease,

tertiary prevention may include:1. Surgical valve repair.

2. Balloon mitral valvotomy (BMV).

3. Valve replacement or drug therapy.