INTRODUCTION:

Rheumatic heart disease, a neglected disease, continues to be a burden in India and other developing countries. It is a result of an autoimmune sequalae in response to group A beta haemolytic streptococcus (GAS) infection of the pharynx. Acute rheumatic fever (RF), a multisystem inflammatory disease, is followed by rheumatic heart disease (RHD) and has manifestations of joints, skin and central nervous system involvement. Although the burden has come down in developed countries, RHD continues to be a prominent cause of morbidity and mortality in developing countries of the world.¹

BURDEN OF DISEASE:

According to the Global Burden of Disease (GBD) estimates, worldwide RHD caused 33,194,900 cases and 319,400 deaths in 2015. About 25.5% of left heart failures and 5.3% of right heart failures are considered to be due to RHD. Globally, RHD accounts for the loss of 10,513,200 disability-adjusted life years every year. In addition, RHD is also a significant cause of maternal mortality. Primarily, RHD burden is located in low- and middle-income countries (LMICs) of Asia and Africa. These countries contribute about 80% of the RHD global burden. Besides, the premature loss of life, RHD also imposes huge economic burden.²

In India, RHD is an endemic disease; there were about 13.17million cases of RHD in the year 2015, and about 1 in 100 RHD deaths occur every year. Despite the high morbidity and mortality load, RHD has not attracted enough attention of policymakers and program planners in India, although evidence-based effective prevention and control strategies are available which can be implemented even in weak health systems at low cost. Ideally, improvement in living standards (primordial prevention) and better access to medical care for treatment of GAS sore throat (primary prevention) can prevent large number of RF cases. The prompt diagnosis of RF and RHD cases and administration of long-acting penicillin injections every month (secondary prevention) can prevent recurrence of RF to avoid further damage to heart valves. However, currently, most of the RHD cases are recognized at late stage when expensive tertiary level care remains the only option for the prevention of premature mortality (tertiary prevention).³

DIAGNOSIS OF RHEUMATIC FEVER:

The criteria for the diagnosis developed by Dr T. Duckett Jones have been modified, revised and updated by the American Heart Association (AHA)⁴.

Major manifestations Minor manifestations

· Carditis Fever

· Arthritis Arthralgia

· Subcutaneous nodules Elevated Sedimentation rate

· Erythema marginatum CPR Increased

· Chorea. Prolonged PR interval

Rheumatic carditis resulting in a more or less permanent damage to the heart is the main virulent manifestation of RF.Presence of two major or one major and two minor manifestations with an evidence for recent GAS infection (essential criterion) indicate acute RF. Evidence for recent GAS infection can be in the form of a positive throat culture, elevated anti-streptococcal, antibodies or presence of features for recent scarlet fever, rare in our country.

IDENTIFICATION OF RHEUMATIC FEVER/ RHEUMATIC HEART DISEASE:

The detection of RF/RHD in the vast population is challenging. RHD can occur only after a patient has had RF. Evaluation of data from various studies indicates that about 65 per cent patients get clinically recognizable RHD following RF. The RF and RHD are detected based on symptoms, audible murmurs, and echocardiography evidence of structural and functional abnormalities of the affected valves. The ability to detect murmurs and differentiating functional from pathological murmurs depends upon clinical skills of the physician, settings of auscultation, and so on. Thus, auscultation-based methods of screening RF/RHD have their limited sensitivity and speciﬁcity. The morphological and Doppler-based detection of RHD in echocardiography study has high sensitivity and speciﬁcity in detection being more objective and subject to validation. The echocardiography detects RHD in patients without being clinically evident called subclinical RHD. The prevalence of subclinical RHD is about seven to ten timeshigher than clinically evident RHD.⁵

DIAGNOSTIC TEST:

There is no single specific test to diagnose RF. Diagnosis of RF is based upon a medical assessment along with various tests such as echocardiography to check the valves, an ECG which shows the rhythm of the heart, blood tests and throat swabs (to look for recent group A streptococcal infection). RHD is also diagnosed by a medical assessment and echocardiography.

The diagnostic tests can be considered as those meant for (i) Diagnosis of RF, (ii) Presence of active vs. inactive RF in recurrences, and (iii) Identification of carditis and valve damage in RHD.

DIAGNOSIS OF RF:

1. The diagnosis of RF is dependent on some laboratory tests included as minor criteria and consist of the following:

(i) Acute phase reactants (leukocytosis, elevated sedimentation rate and presence of C reactive protein CRP).

(ii) Prolonged PR interval in the electrocardiogram.

2. The diagnosis requires presence of essential criteria in the form of evidence for recent GAS infection and consists of:

(i) Elevated antistreptococcal antibodies,

(ii) Positive throat culture for GAS, and

(iii) Evidence for recent scarlet fever- rare in India.

PRESENCE OF ACTIVE VS. INACTIVE RF IN RECURRENCES:

Two investigations have been tried to assess the presence or absence of active RF in patients with recurrences besides ESR, CRP and evidence for recent GAS infection.

(i) Induced subcutaneous nodules (SCN)

(ii) Myocardial biopsy

RHEUMATIC CARDITIS AND VALVE DAMAGE:

The severity of RF is related to its capacity to cause cardiac damage. Clinically carditis has been reported by several investigators in the initial attack in India. Rheumatic carditis has been considered to be a pancarditis causing pericardial, myocardial and endocardial disease which can be identify by an echocardiogram. In an evaluation of patients with RF the final diagnosis regarding the presence or absence of carditis is determined by clinical findings related to the mitral and aortic valve disease. The commonest clinical finding is the presence of mitral regurgitation with or without aortic regurgitation.

PATHOGENESIS OF RHD:

It is well known that RF causes permanent damage only to the cardiac valves. Clinically the mitral aortic, tricuspid and pulmonary valves are involved in order of frequency. Mitral valve involvement is the commonest and the pulmonary valve involvement is rare.

The valves are composed of a central core of connective tissue covered on both sides by endothelium. The central core of connective tissue is derived from the ventricular myocardium-muscle and inter-myocardial connective tissue. Histopathology indicates absence of myocardial and connective tissue damage in carditis due to acute RF. Immuno-histopathology excludes myocardial damage in RF. Hence, the site of damage in the valves derived from the ventricular myocardium has to be the valve endothelium. Endothelium consists of two components – the endothelial cells and the basement membrane to which the cells are attached. It is suggested that the valve damage is related to the valve endothelium- the endothelial cells, the basement membrane and the substance binding these together.⁶

MANAGEMENT:

There has been no significant change in the management of acute RF in the last 50 years. Patients need penicillin to eradicate GAS present in throat. Anti-inflammatory agents - aspirin or steroids are used to control rheumatic activity but do not cure RF. These suppress the inflammatory response which lasts for about 12 weeks in more than 80 per cent patients. The standard dose of aspirin (90-120mg/kg/day) is given for ten weeks and tapered in the next two weeks. The dose of prednisone 60 mg/day above 20 kg and 40 mg /day below 20 kg in weight is given for three weeks and tapered in the next nine weeks. The standard 12week course can be reduced to four to eight weeks depending on the patient's response. Patients without carditis can have weekly follow up of ESR and CRP. If they normalize, the course can be reduced to a shorter period. Aspirin is preferred over steroids as long as the carditis is mild and the patient is not in congestive failure. However, with severe carditis and congestive failure steroid is the drug of choice because of the more potent suppressive effect.

Immunosuppressive agents like azathioprine and cyclosporine A have also been considered for acute rheumatic fever. Despite of the concerns of side effects, toxicity and late onset of lymphomas with the use of these immunosuppressive it is possible to argue that a short course of 6 to 8 weeks may result in a greater benefit than harm.⁷

SURGICAL MANAGEMNET:

It is now well accepted that rheumatic endocarditis involving heart valves is the main cause of morbidity and mortality in RF. Surgical management consisting of mitral and /or aortic valve replacement in patients whose congestive failure cannot be controlled by aggressive medical treatment during acute RF.

Management of chorea: It has a self-limiting course, hence parents need reassurance. The children could be treated with sedatives like phenobarbitone 30 mg thrice daily. chlorpromazine, Valium, diphenhydramine or promethazine can be used as sedatives. Haloperidol 5 to 10 mg twice daily has been used effectively.⁷

RHEUMATIC HEART DISEASE:

Surgical management of valve disease was the standard approach till balloon mitral valvotomy was introduced in 1985. Mitral stenosis could be corrected surgically either by closed valvotomy, open commissurotomy or by valve replacement if the valve was calcified. Balloon valvotomy provides results as good as surgical valvotomy and has become the treatment of choice.

For mitral regurgitation the choice of treatment would be valve repair especially in younger patients to avoid long-term anti-coagulant therapy. Most patients with mitral or aortic valve regurgitation end up with valve replacement. Hence, although surgical help is very useful, it is expensive and requires prolonged care with anticoagulant therapy with the associated complications of valve thrombosis and systemic embolic.⁷

PREVENTION:

Prevalence of RF/RHD has been attributed to overcrowding and unhygienic living related to low socio-economic status. Unhygienic living results in persistent GAS in the environment. Since GAS spreads by droplet dissemination, overcrowding causes cross infection from person to person. Low socio-economic status may undermine nutrition and seriously limit access to medical treatment. Poor nutritional status is believed to contribute to a decreased immune response. The result is not only endemic RF but also a more severe or virulent disease.⁸

It is possible at the same time that the initial attack of RF is mild and results in mild carditis, which remains subclinical, undiagnosed, and as such the patient does not get prophylaxis to prevent recurrences. In low socio-economic settings, recurrences causing further cardiac damage result in symptomatic RHD with multivalve involvement and congestive failure identified as the first attack of severe RF.⁸

The strategies for prevention consist of primordial prevention, primary prevention and secondary prevention.

Primordial prevention:

It requires, preventing the development of ‘risk factors’ in the community to prevent the disease in the population and thus protect individuals. Requirements for primordial prevention in relation with RF and RHD consist of

(i) Improvement in socio-economic status

(ii) Prevention of overcrowding

(iii) Improving nutritional status

(iv) Availability of prompt medical care

(v) Public education regarding the risk of RF from sore throat specially below the age of 15 yr.

Improvement in nutrition improves immune response and the capacity of individuals to resist and fight infection. Public education is the most important component for primordial prevention. Unless parents know that a sore throat can cause RF and RHD, it is most unlikely to be seen by a physician and treated. Improvement in socio-economic status and preventing overcrowding cannot be relied upon to reduce the burden of RHD.

Primary prevention:

Primary prevention is theoretically feasible but practically extremely difficult to achieve. Primary prevention requires identification of (GAS) sore throat and use of penicillin to eradicate the streptococci. Primary prevention is possible if an anti-streptococcal vaccine becomes available. The requirement for primary prevention consists of

(i) Public awareness regarding danger of RF from sore throat.

(ii) Identification of sore throat as being due to GAS infection.

(iii) Use of injectable penicillin to cure the infection.

Secondary prevention:

Secondary prevention requires identification of those with RF or RHD and maintenance of a registry. Once identified, the patient needs injections of benzathine penicillin, given once in two to three weeks, depending on age, body size and muscle mass. Benzathine penicillin is painful, may result in fever and very rarely in anaphylactic reactions. Most physicians are very reluctant to give penicillin injections. The necessity of penicillin prophylaxis is due to the fact that RF has a tendency for recurrences in those who have had RF in the past. Each new attack causes further damage to the valve tissue making the disease worse than before. Secondary prevention can reduce the damage of recurrences but cannot prevent the initial damage. Further, secondary prevention cannot reduce the burden of RHD in the community. Secondary prevention has been found to help in disappearance of clinical findings of RHD. However, disappearance of murmurs does not indicate that the heart disease has disappeared. Recurrence of RF results not only in appearance of murmurs but also the valve damage is worse than before.⁸

NURSING MANAGEMENT:

Nurses together with physicians and health officers are involved in the primary prevention of rheumatic fever. The first approach to preventing initial attack of rheumatic fever is to look for and treat streptococcal infections adequately. Secondary prevention is the specific preventive care of the patient with rheumatic heart disease or documented previous rheumatic fever. The medical and nursing intervention for people with rheumatic fever is to control and alleviate infecting streptococci if they are still present with the purpose of protecting the heart against the damaging effect of carditis. Nursing interventions may include.

1. Early detection of sign and symptoms:

Nurse must assess the temperature frequently, pulse to detect widening pulse pressure, hemodynamic changes and heart murmurs and report to physician. She also takes measures to reduce the fever and prevent infection by using aseptic techniques.

2. Provide comfort and reduce pain:

Position the child to reduce joint pain; warm baths and gentle range-of-motion exercises help to alleviate some of the joint discomforts; use pain indicator scales with children so they are able to express the level of their pain.

3. Provide diversional activities and sensory stimulation:

For those who do not feel very ill, bed rest can cause distress or resentment; be creative in finding diversional activities that allow bed rest but prevent restlessness and boredom, such as a good book; quiet games can provide some entertainment, and plan all activities with the child’s developmental stage in mind.

4. Promote energy conservation:

Provide rest periods between activities to help pace the child’s energies and provide for maximum comfort; if the child has chorea, inform visitors that the child cannot control these movements, which are as upsetting to the child as they are to others.

5. Prevent injury:

Protect the child from injury by keeping the side rails up and padding them; do not leave a child with chorea unattended in a wheelchair, and use all appropriate safety measures.

6. Maintaining cardiac output:

Assess for sign and symptoms of acute rheumatic carditis. Nurse should be alert to client’s complaints of chest pain, palpitations and precordial tightness. And monitor patients for tachycardia or bradycardia. She should auscultate heart sound every 4 hours to find out any abnormality and document murmur or pericardial friction rub. Monitor the clients for development of chronic rheumatic endocarditis which may include valvular disease or heart failure and report to physician immediately.

REHABILITATION:

Medical rehabilitation, including physical and occupational therapy, can help to do daily activities while managing a rheumatic condition. A rehabilitation program helps to improve in strength and flexibility and the movement of joints and muscles - all with less pain and stiffness. Such a program will not cure a rheumatic condition, but it can help to become better able to function and more independent.

Nurses may make important contributions towards the rehabilitation of the child with rheumatic fever or rheumatic heart disease. They can advice both child and family regarding prevention of recurrence; they can help school nurses and teachers to understand what heart diseases in childhood means. They can use school health services in finding and following cases.

REFERENCES:

1. Negi P, Sondhi S, Asotra S et al. Current Status of Rheumatic Heart Disease in India. Indian Journal2019. (Available online)

2. Watkins DA, Johnson CO, Colquhoun SM, Karthikeyan G, Beaton A, Bukhman G, et al. Global, regional, and national burden of rheumatic heart disease, 1990-2015. The New England Journal of Medicine2017; 377:713-22.

3. Basu UP. Preliminary observations on acquired diseases of the heart and aorta as met with in Bengal. Indian Med Gaz. 60:307–10.

4. Guidelines for the diagnosis of rheumatic fever. Jone criteria. 1992 update. Special Writing Group of the Committee on Rheumatic fever, Endocarditis and Kawasaki disease of the Council on Cardiovascular Disease in the young of the American Heart Association. JAMA. 1992; 268:2069–73.

5. Kumar R. Rheumatic Heart disease: a neglected public health priority. Indian Journal of Public health. 2019: 63(1); 1-3.

6. Tandon R. Rheumatic fever pathogenesis: Approach in research needs change. Annals of Pediatric Cardiology. 2012; 5:169–78.

7. Kumar R K, Tondon R. Rheumatic disease and Rheumatic heart disease: the last 50 years. IJMR. 2013 Apr; 137(4): 643–658.

8. Dajani A, Taubert K, Ferrieri P, Peter U, Shulman S. Treatment of acute streptococcal pharyngitis and prevention of rheumative fever: a statement for health professionals. Committee on Rheumatic fever, Endocarditis and Kawasaki Disease of the Council on Cardiovascular disease in the young, the American Heart Association. Pediatrics. 1995; 96:758–64.

Received on 31.03.2020 Modified on 10.04.2020

Asian J. Nursing Education and Research. 2020; 10(3): 360-364.

DOI: 10.5958/2349-2996.2020.00076.2