Q:1 What is Rheumatic Heart Disease?     A:1 Rheumatic (roo-MAT-ik) heart disease was formerly one of the most serious forms of heart disease of childhood and adolescence. Rheumatic heart disease involves damage to the entire heart and its membranes. Rheumatic heart disease is a complication of rheumatic fever and usually occurs after attacks of rheumatic fever. The incidence of rheumatic heart disease has been greatly reduced by widespread use of antibiotics effective against the streptococcal bacterium that causes rheumatic fever.

Q:2 What causes Rheumatic Heart Disease?     A:2 Rheumatic fever causes rheumatic heart disease. Rheumatic fever results from an untreated strep throat. Rheumatic fever can damage the heart valves. If the heart valves are damaged, they will fail to open and close properly. When this damage is permanent, the condition is called rheumatic heart disease.

Q:3 Define rheumatic     A:3 Rheumatic fever is uncommon in the United States. However, rheumatic fever can occur in children who have had strep infections that were untreated or inadequately treated.

Q:4 What are the symptoms of Rheumatic Heart Disease?     A:4 Some of the most common symptoms of rheumatic heart disease are: breathlessness, fatigue, palpitations, chest pain, and fainting attacks.

Q:5 Treatment options for Rheumatic Heart Disease?     A:5 Treatment of rheumatic heart disease may include medication and surgery. Medication will aim to avoid overexertion. Surgery may be needed to replace the damaged valve(s).

Q:6 Can Rheumatic Heart Disease be Prevented?     A:6 The best way to prevent rheumatic heart disease is to seek immediate medical attention to a strep throat and not let it progress to rheumatic fever.

Q:7 What are the reasons why RF is considered post streptococcal in origin ?     A:7 1) Epidemiological evidence: Epidemiological evidence over a period of 20 Years have shown a clear sequential relationship between outbreaks of streptococcal phaiyngitis and ARF. 2) Immunologic evidence: Initial or recurrent RF does not occur without a streptococcal antibody response. Streptococcal immune response is an important cri­teria for diagnosis of ARF. 3) Prophylactic evidence: Pencillin prophylaxis prevents recurrence of ARF. Majoirity of cases of recurrence occur on discontinuation of penicillin profylaxis. 4) Clinical evidence: As clinical evidence of streptococcal throat infection is not seen in every case. It is difficult to establish Group A streptococci etiological agent.

Q:8 Discuss Jones criteria and the recent WHO recommendations in the diagnosis of ARF?     A:8 The Jones Criteria for guidance in the diagnosis of ARF was initially proposed by Dr T.Ducket Jones in 1944. More specific criteria were called major and those less specific were called minor criteria. In 1956, AHA (American Heart association) modified the criteria and made arthalgia a minor criteria and evidence of proceeding streptococcal infection as another minor criteria. In 1965 Jones Criteria was revised when evidence of antecedent streptococcal infection was made mandatory except in patients with chorea, and those with insidious onset carditis. Later revision was made in 1982 by AHA when it was pointed out that other causes of chorea should be ruled out before accepting chorea as the sole criterion for diagnosis of ARE Also it was recognised that elevated ASO titre result from Group A streptococcal infection as well as streptococcal skin infection. WHO study group in 1987 recommended that 1982 revised Jones Criteria be accepted for general use. The presciice ol’2 niajor or I major plus 2 minor criteria plus evidence of proceeding streptococcal infection indicated a higher probability of ARF Major criteria: I . Carditis 2. Polyarthritis 3. Chorea 4. Erythernamarginaturn. 5. Subcutaneous nodules Minor criteria: I. Fever 2. Arthralgia 3. Previous Rheumatic fever or RHD 4. Acute phase reactants elevated ESR, CRP, Leucocytosis 5. Prolonged PR interval Supporting Evidence of streptococcal infection: 1. Increased titre of antistreptococcal antibodies ASO and others. 2. Positive throat culture for group A streptococcus 3. Recent scarlet fever.

Q:9 How will you diagnose carditis in a patient with ARF and assess severity of carditis?     A:9 The 4 major criteria for diagnosis of carditis are: 1. Organic murmur (MR, AR, Carey Coombs murmur) not previously present 2. Cardiornegaly 3. Congestive Cardiac failure 4. Pericardial friction rub or effusion. If any one of this is unequivocal carditis is present. Assessment of severity of carditis is by the degree of cardiomegaly, heart failure, loudness of apical systolic murmur and pericarditis. In mild carditis, there is only minimal cardiac enlargement and an apical systolic murmur of grade I II/VI. Moderate to severe carditis is associated with definite cardiornegaly of heart failure and grade III IV/VI apical systolic murmur. The more severe the CHF and cardiac enlargement, the more severe is the carditis. The loudness of the murmur does not always correlate with severity.

Q:10 What are the common murmurs associated with AR?     A:10 The murmur of rheumatic carditis occur in the first week in 76% and within 3 months in 93%. Mitral valve involvement is three times more common than aortic valve involvement. The valvular involvement is due to inflammation of the valve leaf­lets and chordae tendinae. The three important murmurs are: Apical systolic murmur due to mitral regurgitation is heard early in the course of disease and is due to mitral valvulitis, edema, thickening and verrucae with damaged swollen musculotendinous structures. Dilatation of mitral annulus due to myocarditis cardiac enlargement can also cause MR. Apical mid diastolic murmur Carey Coomb’s murmur This is due tautness & oedema of valve and chordae due to mitral valvulitis. It is localized soft and easily missed and occurs after S3. S I is not loud, OS is not present and there is no presystolic accentuation. Aortic early diastolic murmur from aortic valvulitis Soft high pitched or cooing (Seagul) type of murmur heard in 20% patients. Signifies carditis Functional ejection murmurs may be heart at the base. These may disappear with tachycardia, congestive cardiac failure or if associated pericarditis or effusion.

Q:11 What is the significance of arthralgia in contrast to arthritis in the Indian context?     A:11 We see many cases of rheumatic fever especially in children with out the ­classical polvarthritis. Hence polyarthralgia in children in our set up has to be given significance. However this alone is not enough to diagnose ARF. It should be supported by other clinical signs or unequivocal laboratory evidence of rheumatic fever (not an elevated ASO alone). However in a patient with preexisting rheumatic heart disease, not on secondary prophylaxis, polyarthralgia alone with markedly elevated ESR and elevated ASO titre can be considered as rheumatic reactivation.

Q:12 What is the incidence of various clinical manifestations of what is the sequence of these manifestations?     A:12 Highest incidence is for arthritis and carditis, least for erythema marginatum with chorea and subcutaneous nodules in between. Arthritis is seen in about 76% cases, carditis in 50% chorea in 7 20% and subcutaneous nodules in 5 20%. Erythema marginatum is very rare. In young children carditis dominates. As age advancesioint symptoms become more striking than carditis. Among the minor manifestations fever is present in 90% cases. Arthralgia and arthritis together constitute 90% of all patients in Indian context.

Q:13 What is the natural history of RF in the first episode with special ref­erence to carditis?     A:13 Usual duration of ARF is less than 3 months. Only 5% go beyond 6 months. Rheumatic arthritis has excellent prognosis with no residual arthritis or deformity. Rheumatic choreamay rarely recur. Insidious development of mitral valve disease is seen in 23% of patients with corea over a 20 year period. Tile prognosis in those without carditis is excellent. If no organic murmurs are noted in the first episode, no chronic rheumatic heart disease occurs in five year follow up. If no carditis is diag­nosed in the first episode, 96% patients are free of heart disease at 5 years and 94% at 10 years. With mild carditis and mitral regurgitation (mild), a normal heart is seen in 82% in 5 years and 70% in 10 years. If there is severe cardiac failure in the first episode, only 30% of patients will be normal by 5 years and by 10 years it is 40%. Seventy percent of mitral regurgitation disappear by 5 years while Aortic regurgita­tion if present in the first episode seldom disappears. Carey Coomb’s murmur never persists. Progression to mitral stenosis is of same frequency in males and females in case of severe ARF, but in mild cases, progression to NIS is seen more in females.

Q:14 What is the value of various laboratory investigations in ARF especially with reference to antistreptococcal antibodies and their temporal relationship?     A:14 The laboratory finding are useful in documenting the presence of inflammatory reaction and in establishing preceding streptococcal infection. Acute phase reactants include elevated ESR, CRP, serum hexosamine. ESR and CRP, most commonly used, are invariably elevated. Mild normocytic hypochromic anemia may be seen in chronic rheumatic process. Prolonged PR interval in ECG is due to increased vagal activity and does not signify carditis and is considered a minor criteria. The diagnosis of recent streptococcal infection can be made only tentatively by throat culture as many people are carriers of this bacteria. Anti streptococcal antibodies are more reliable in establishing a diagnosis of recent streptococcal infection. These antibodies are: Antistreptolysin (ASO), Anti DNase B (ADNB), Anti. streptokinase (ASK), Anti NADase. Commonly used ones are ASO and ADNB. Single ASO titre is con­sidered elevated if it is more than 250 Todd units in adults and 333 units in children. Above the age of 5years. ASO should be repeated after 2 weeks if initial titre is nondiagnostic. Rise in titre of 2 or more dilution is diagnostic of recent streptococcal infection. Sensitivity of ASO test is 80% with serial evaluation. Peak ASO Valves are obtained 4 6 weeks following streptococcal infection and remain elevated for 4-6 weeks. Then the ASO titre falls rapidly in the next few weeks. In some it may persist for longer periods. If the latent period of ARF is short (less than 10 days Or very long as in chorea) ASO titre is usually not elevated; whereas if the latent period is around 4 weeks, ASO is elevated in 80%. Anti DNaseB tends to remain longer than other antistreptococcal antibodies and are likely to be positive in chorea and late onset carditis. Anti streptozyme tests is a hernagglutination reaction to con­centration of extra cellular streptococcal antigen absorbed to red cells. It is almost 100% sensitive but specificity is low. Radiological evaluation, ECG, and Echo Doppler evaluation are useful in assessment of presence and severity of cardiac involve­ment.

Q:15 What are the goals of treatment of ARF? When will you prefer sali­cylates and when will you use steroids? What is the usual duration of treat­ment?     A:15 Goals of treatment of ARF: a. Relief of inflanunatory symptoms like joint pain and fever. b. Control of toKic maidfestations Re carditis and CBF Anti inflammatory drugs reduce the burden on the heart and may tilt the balance in favour of a critically ill patient. c. Eradication of streptococci from respiratory tract by penicillin therapy Steroids and salicylates are not curativie and do not shorten the duration of ARR Development of chronic valvular heart disease is not influenced by anti inflammatory therapy. Salicylates are preferred in patients with moderate or severe arthritis with no carditis. With carditis and no cardiornegaly or failure, salicylates or steroids may be used. Steroids are preferred in patients with carditis and heart failure. Duration of therapy must be estimated according to the expected course of the attack. For arthritis usual duration is 6 weeks. In severe carditis, longer duration of therapy is needed.

Q:16 What is rebound phenomenon? How can you prevent it? What is chronic rheumatic fever?     A:16 Reappearance of clinical and or laboratory evidence of rheumatic ac­tivity when anti inflationary therapy, especially steroids, is discontinued is called reboundphenomenon. This usually occurs within 2 weeks of stopping treatment. It can be prevented by gradually tapering steroids and supplementing xspirin during the period. Usually RF subsides by 4 8 weeks even without treatinent. Rarely it may persist upto 6 months this is called chronic rheumaticfever

Q:17 What is the risk of recurrence for untreated RF ?     A:17 The chance for recurrence RF depends upon various factors. They are number of previous episodes, short time interval from previous episode, severity of previous carditis, younger age, low socio economic status and non favourable occupation (eg. teachers, nurses). Temperate climate and seasonal factors are also important. In the first 5 years, people not taking prophylaxis have a recurrence rate of 19%, 11 % in the next 5 years and 6% in next 5 years. With antibiotic prophylaxis the corresponding figures are 2.67%, 1.3% and 0.4%. Maximum recurrence is likely in the first year after the first episode and there after the incidence decrease.

Q:18 How will you treat rheumatic chorea?     A:18 Treatment of chorea is specific sedative and tranquilisers. Haloperidol is effective in controlling chorea. Start with small dose and gradually increase (5 15 mg/day in divided doses). Recently sodium valproate has also been found to be useful (30 mg/kg/day in divided doses). Therapy may be continued for 2 months or even longer.

Q:19 What is the status of secondary antibiotic prophylaxis of RF and when will you stop prophylaxis?     A:19 By secondary prophylaxis, we are preventing recurrence of RF by means of continuous chemo prophylaxis: Methods Available: 1) Benzathine penicillin. Once in 3 week I M injection. For children less than 30 kg 6 lakhs units; and adults and children weighing more than 30 kg 12 lakhs units. 2) Oral prophylaxis: a) Sulphadiazine: I gm daily for adults and children more than 30 kg and 500 gm daily children less than3Okg. b) Penicillin. (Potassium Phenoxymethyl Penicillin) 250 mg twice dailyfor adults or penicillin G 2 to 2.5 lakh units daily. c) Erythromycin: 250mg twice daily for adults who are allergic to penicillin and sulphadiazin. Recurrence rate while on benzathine penicillin secondary prophylaxis is one in 250 patients years. Recurrence rate is high on oral prophylaxis, one in 25 patient years with oral sulphadiazine and one in 20 patient years with oral penicillin. Patients without carditis in any previous attacks should have I for minimum 5 years after last attack and atleast still 18 years of age. Those with carditis in the initial attack with no evidence of cardiac lesion on letter evaluation should continue prophylaxis till 25 years and in high risk for longer time. For patients with chronic valvular heart diseases, prophylaxis should be continued for prolonged periods and in some high risk patients life long

Q:20 What is primary prophylaxis? What is the role of vaccination?     A:20 Primary prophylaxis is the treatment of upper respiratory inflection due to Group A streptococci to prevent an initial attack of rheumatic fever. is by penicillin, oral penicillin or by erythromycin in those who are penicillin. The drugs should be given for 10 days. The primary pre strongly recommended for individual patients with streptococcal ph& is difficult to impliment on a community basis. No vaccine is currently available. Type specific antibodies were produced using M protein preparation but cross reaction with host low immunogenicity has limited its role.

Q:21 What are the important differential diagnosis you would concider?     A:21 The most important differential diagnosis is rheumatoid arthritis. The jont symptoms last longer, smallerjoints are affected, temporomandibular joints and sternoclavicular joints tend to get involved and morning stiffness is common. Joint deformities occur in long standing cases. Other inflammatory arthiritis, poncents disease, gonococcal arthritis, septic arthritis an vascular disease have to be differentiated. Acute leukemia is an important condition in children which has to be considered. If patient presents with carditis, the differential diagnosis is viral myocarditis and pericarditis. The most important point in rheumatic carditis is the presence of murmurs. Other causes of chorea should be considered when chorea is the sole manifestation. Functional systolic murmurs may create confusion. Echo Doppler studies may help in diagnosis of MR, A R etc. Another important diagnosis to be considered is infeclive endocarditis. It can produce joint symptoms, murmurs, fever and acute phase recants. This is more so in people with pre exiting RHD. Initiating steroids in a patient with infective endocarditis on a mistaken diagnosis of ARF can be catastrophic. Left atrial myxoma is another condition to be ruled out. In equivocal cases it is better to complete investigations before starting anti inflammatory drugs, so that the full blown picture of the condition appears. Delay in starting anti inflammatory drugs for a few days not affect the long term prognosis,

Q:22 What is the role of prophylaxis after cardiac surgery?     A:22 Those patients who have undergone cardiac surgery should also continue to receive prophylaxis. In cases over 35 years, decision can be individualised.

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