History

Fact Explanation
Chest pain, chest tightness [1] Rheumatic fever (RF) is an autoimmune disease. Repeated RF episodes may cause rheumatic heart disease (RHD) [7] due to progressive and permanent valvular lesions [8] Chest pain occurs due to pancarditis and valvular heart lesions [1]
Shortness of breath [2] As a symptom of heart failure in severe rheumatic carditis [1]
Swelling of the ankles [1] As a symptom of heart failure in severe rheumatic carditis [1]
Recent history of sore throat [9] Rheumatic fever can occur after infections of oropharynx by the group A beta-hemolytic Streptococcus [5]
Age between 5-15 years [1] Rheumatic fever is more common in this age group [6]
Fever [1] Minor criteria for the diagnosis of rheumatic fever [1]
Joint pain [1] Non cardiac manifestations of rheumatic fever may be present.Migrating arthritis is a major criteria and arthralgia is a minor criteria for the diagnosis of rheumatic fever [1]
Emotional lability, uncoordinated movements and muscle weakness [1] Occurs in Sydenham’s chorea,which is a major criteria for the diagnosis of rheumatic fever [1]
Subcutaneous lumps [1] Non cardiac manifestations of rheumatic fever may be present.Subcutaneous nodules are a minor criteria for the diagnosis of rheumatic fever [1]
Skin lesions [4] Due to erythema marginatum (non cardiac manifestations of rheumatic fever) which is a minor criteria for the diagnosis of rheumatic fever [4]
Past history of rheumatic fever or rheumatic heart disease [1] Minor criteria for the diagnosis of rheumatic fever [1]
Recent history of scarlet fever [3] Rheumatic fever can occur after infections of oropharynx by the group A beta-hemolytic Streptococcus [5]
overcrowding and unemployment [10] Studies have shown that there is a trend towards increased risk of rheumatic heart disease in association with overcrowding and unemployment due to poor living conditions [10]
References
  1. Rheumatic fever and rheumatic heart disease,WHO Technical Report Series, November 2001 [viewed on 19 July 2014] Available from: http://www.who.int/cardiovascular_diseases/resources/en/cvd_trs923.pdf
  2. XAVIER JR. JL, SOEIRO AD, LOPES AS, SPINA GS, SERRANO JR. CV, OLIVEIRA JR. MT. Clinically Manifested Myocarditis in Acute Rheumatic Fever Jos? Arq Bras Cardiol [online] 2014 Feb, 102(2):e17-e20 [viewed 21 June 2014] Available from: doi:10.5935/abc.20140017
  3. CUNNINGHAM MW. Streptococcus and rheumatic fever Curr Opin Rheumatol [online] 2012 Jul, 24(4):408-416 [viewed 21 June 2014] Available from: doi:10.1097/BOR.0b013e32835461d3
  4. NARULA J., CHANDRASEKHAR Y., RAHIMTOOLA S.. Diagnosis of Active Rheumatic Carditis : The Echoes of Change. Circulation [online] 1999 October, 100(14):1576-1581 [viewed 13 September 2014] Available from: doi:10.1161/01.CIR.100.14.1576
  5. MERLINI AB, STOCCO CS, SCHAFRANSKI MD, ARRUDA P, BAIL L, BORGES CL, DORNELLES CF. Prevalence of group a Beta-hemolytic streptococcus oropharyngeal colonization in children and therapeutic regimen based on antistreptolysin levels: data from a city from southern Brazil. Open Rheumatol J [online] 2014:13-7 [viewed 13 September 2014] Available from: doi:10.2174/1874312901408010013
  6. MAYOSI BM, GAMRA H, DANGOU JM, KASONDE J, 2ND ALL-AFRICA WORKSHOP ON RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE PARTICIPANTS. Rheumatic heart disease in Africa: the Mosi-o-Tunya call to action. Lancet Glob Health [online] 2014 Aug, 2(8):e438-9 [viewed 13 September 2014] Available from: doi:10.1016/S2214-109X(14)70234-7
  7. OLIVER J, PIERSE N, BAKER MG. Improving rheumatic fever surveillance in New Zealand: results of a surveillance sector review. BMC Public Health [online] 2014 May 29:528 [viewed 13 September 2014] Available from: doi:10.1186/1471-2458-14-528
  8. MARIJON ELIO, OU PHALLA, CELERMAJER DAVID, FERREIRA BEATRIZ, MOCUMBI ANA OLGA, SIDI DANIEL, JOUVEN XAVIER. Echocardiographic screening for rheumatic heart disease. Bull World Health Org [online] 2008 February, 86(2):84-84 [viewed 13 September 2014] Available from: doi:10.2471/BLT.07.046680
  9. REMéNYI BO, WILSON NIGEL, STEER ANDREW, FERREIRA BEATRIZ, KADO JOSEPH, KUMAR KRISHNA, LAWRENSON JOHN, MAGUIRE GRAEME, MARIJON ELOI, MIRABEL MARIANA, MOCUMBI ANA OLGA, MOTA CLEONICE, PAAR JOHN, SAXENA ANITA, SCHEEL JANET, STIRLING JOHN, VIALI SATUPAITEA, BALEKUNDRI VIJAYALAKSHMI I., WHEATON GAVIN, ZüHLKE LIESL, CARAPETIS JONATHAN. World Heart Federation criteria for echocardiographic diagnosis of rheumatic heart disease—an evidence-based guideline. Nat Rev Cardiol [online] December, 9(5):297-309 [viewed 13 September 2014] Available from: doi:10.1038/nrcardio.2012.7
  10. OKELLO EMMY, KAKANDE BARBARA, SEBATTA ELIAS, KAYIMA JAMES, KUTEESA MONICA, MUTATINA BONIFACE, NYAKOOJO WILSON, LWABI PETER, MONDO CHARLES K., ODOI-ADOME RICHARD, JUERGEN FREERS, DASGUPTA KABERI. Socioeconomic and Environmental Risk Factors among Rheumatic Heart Disease Patients in Uganda. PLoS ONE [online] 2012 August [viewed 13 September 2014] Available from: doi:10.1371/journal.pone.0043917

Examination

Fact Explanation
Tachycardia / Increased rate of sleeping pulse [1] Monitor the sleeping pulse.Tachycardia may occur as a result of cardiac arrhythmia or as a result of acute decompensation of heart in cardiac failure [1]
Audible friction rub [1] Due to pericarditis.Can be supported by echocardiographic evidence of pericardial effusion [1]
Shifted apex beat [1] Due to cardiomegaly (myocarditis) [2]
Heart murmurs [4] Presence of apical holosystolic murmur of mitral regurgitation (with or without apical mid-diastolic murmur, Carey Coombs), or basal early diastolic murmur in patients who do not have a history of rheumatic heart disease.On the other hand, in an individual with previous rheumatic heart disease, a definite change in the character of any of these murmurs or the appearance of a new significant murmur indicates the presence of carditis [1]
Red,swollen,warm joints (Arthritis) [1] Arthritis is the most frequent major manifestation of RF, occurring in up to 75% of patients in the first attack of RF.Typically present as migratory polyarthritis, more often in larger joints (ankles and knees commonly) [1]
Sydenham’s chorea [1] Characterized by emotional lability, uncoordinated movements and muscle weakness [1]
Subcutaneous nodules [1] The subcutaneous nodules are round, firm, freely movable, painless lesions varying in size from 0.5–2.0cm [1]
Erythema marginatum [1] appear first as a bright pink macule or papule that spreads outward in a circular or seripiginous pattern. The lesions are multiple, appearing on the trunk or proximal extremities, rarely on the distal extremities, and never on the face [1]
Ankle edema, ascites [1] Occur due to heart failure in severe rheumatic heart disease [1]
Inflamed throat [3] Recent history of streptococcal infection [3]
References
  1. Rheumatic fever and rheumatic heart disease,WHO Technical Report Series, November 2001 [viewed on 19 July 2014] Available from: http://www.who.int/cardiovascular_diseases/resources/en/cvd_trs923.pdf
  2. XAVIER JR. JL, SOEIRO AD, LOPES AS, SPINA GS, SERRANO JR. CV, OLIVEIRA JR. MT. Clinically Manifested Myocarditis in Acute Rheumatic Fever Jos? Arq Bras Cardiol [online] 2014 Feb, 102(2):e17-e20 [viewed 21 June 2014] Available from: doi:10.5935/abc.20140017
  3. CUNNINGHAM MW. Streptococcus and rheumatic fever Curr Opin Rheumatol [online] 2012 Jul, 24(4):408-416 [viewed 21 June 2014] Available from: doi:10.1097/BOR.0b013e32835461d3
  4. NARULA J., CHANDRASEKHAR Y., RAHIMTOOLA S.. Diagnosis of Active Rheumatic Carditis : The Echoes of Change. Circulation [online] 1999 October, 100(14):1576-1581 [viewed 13 September 2014] Available from: doi:10.1161/01.CIR.100.14.1576

Differential Diagnoses

Fact Explanation
Innocent murmur [1] Mitral and aortic valve may be present even without rheumatic fever,but other diagnosing criteria are absent [1]
Myocarditis — viral or idiopathic [1] May give the same signs and symptoms of myocarditis in rheumatic carditis, but other diagnosing criteria are absent [1]
Pericarditis — viral or idiopathic [1] May give the same signs and symptoms of pericarditis in rheumatic carditis, but other diagnosing criteria are absent [1]
Hypertrophic cardiomyopathy [1] Left ventricular hypertrophy is present but fever or other diagnosing criteria of rheumatic carditis are absent [1]
Lyme carditis [1] Clinical picture has similarities to acute rheumatic fever; but in Lyme disease, complete heart block may be commoner, myopericardial involvement tends to be milder, and valves seem not to be affected [1]
Congenital heart defects [1] May have murmurs and cardiomegaly, but fever or other diagnosing criteria of rheumatic carditis are absent [1]
Mitral valve prolapse [2] In mitral valve prolapse, a mid-systolic click, followed by a late systolic murmur heard best at the apex is common. Also there are seven special proteins found to be significantly different in abundance between the patients with rheumatic heart disease and patients with mitral valve prolapse [2]
References
  1. Rheumatic fever and rheumatic heart disease,WHO Technical Report Series, November 2001 [viewed on 19 July 2014] Available from: http://www.who.int/cardiovascular_diseases/resources/en/cvd_trs923.pdf
  2. ZHENG D, XU L, SUN L, FENG Q, WANG Z, SHAO G, NI Y. Comparison of the Ventricle Muscle Proteome between Patients with Rheumatic Heart Disease and Controls with Mitral Valve Prolapse: HSP 60 May Be a Specific Protein in RHD Biomed Res Int [online] 2014:151726 [viewed 20 June 2014] Available from: doi:10.1155/2014/151726

Investigations - for Diagnosis

Fact Explanation
Echocardiography [1] Provides information about the size of atria and ventricles, valvular thickening,leaflet prolapse and ventricular dysfunction.The severity of valvular regurgitation has been classified based on the echocardiographic findings [1]
Endomyocardial biopsy [1] Since myocarditis is an obligatory component of cardiac involvement in RF, the value of endomyocardial biopsy has been investigated for diagnosing rheumatic carditis [1]
Radionuclide imaging [1] Radiolabelled anti myosin antibodies and radiolabeled leukocytes are used to image myocardial inflammation [1]
Chest x ray [1] Detection of cardiomegaly, effusions [1]
Electrocardiogram [1] To detect arrhythmias and prolonged PR interval (minor criteria for the diagnosis of rheumatic fever) [1]
Antistreptolysin-O test [2] Diagnosis of rheumatic fever requires prior streptococcal infection. Although a single elevated titre is useful, it is recommended to perform another test 3-4 weeks after the onset of rheumatic fever [1]
Throat swab culture [1] Positive throat culture for Group A beta-hemolytic streptococci gives an evidence of prior streptococcal infection. [1]
Positive rapid direct Group A strep carbohydrate antigen test [1] Gives an evidence of prior streptococcal infection. [1]
Erythrocyte sedimentation rate/ C reactive proteins [1] Acute phase reactants: Leukocytosis, elevated erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) is a minor criteria for the diagnosis of rheumatic fever [1]
References
  1. Rheumatic fever and rheumatic heart disease,WHO Technical Report Series, November 2001 [viewed on 19 July 2014] Available from: http://www.who.int/cardiovascular_diseases/resources/en/cvd_trs923.pdf
  2. MERLINI AB, STOCCO CS, SCHAFRANSKI MD, ARRUDA P, BAIL L, BORGES CL, DORNELLES CF. Prevalence of group a Beta-hemolytic streptococcus oropharyngeal colonization in children and therapeutic regimen based on antistreptolysin levels: data from a city from southern Brazil. Open Rheumatol J [online] 2014:13-7 [viewed 13 September 2014] Available from: doi:10.2174/1874312901408010013

Investigations - Fitness for Management

Fact Explanation
Erythrocyte sedimentation rate/ C reactive proteins [1] High counts indicate a severe disease [1]
Full blood count [1] Detection of high white blood cell counts due to streptococcal infection [1]
References
  1. Rheumatic fever and rheumatic heart disease,WHO Technical Report Series, November 2001 [viewed on 19 July 2014] Available from: http://www.who.int/cardiovascular_diseases/resources/en/cvd_trs923.pdf

Investigations - Followup

Fact Explanation
Echocardiography [1] Regular assessments with echocardiography are mandatory to confirm and monitor cardiac involvement [2] It is important to detect progression to severe chronic mitral or aortic lesions. [3] Also important to detect infective endocarditis which may occur secondary to rheumatic heart disease [1]
Acute phase reactants [1] Check for the reduction of acute phase reactants [1]
References
  1. Rheumatic fever and rheumatic heart disease,WHO Technical Report Series, November 2001 [viewed on 19 July 2014] Available from: http://www.who.int/cardiovascular_diseases/resources/en/cvd_trs923.pdf
  2. DE SILVA K S H, PERERA S N, SAMARASINGHE M D. Echocardiographic assessment of rheumatic carditis: a long term follow up study. J of Cey Coll of Phy [online] 2014 April [viewed 19 June 2014] Available from: doi:10.4038/jccp.v43i1-2.6805
  3. MEIRA ZM, GOULART EM, COLOSIMO EA, MOTA CC. Long term follow up of rheumatic fever and predictors of severe rheumatic valvar disease in Brazilian children and adolescents Heart [online] 2005 Aug, 91(8):1019-1022 [viewed 19 June 2014] Available from: doi:10.1136/hrt.2004.042762

Investigations - Screening/Staging

Fact Explanation
Antistreptolysin-O test [1] To detect prior streptococcal infection [2] Although a single elevated titre is useful, it is recommended to perform another test 3-4 weeks after the onset of rheumatic fever [1]
Throat swab culture [1] Positive throat culture for Group A beta-hemolytic streptococci gives an evidence of prior streptococcal infection. [1]
Positive rapid direct Group A strep carbohydrate antigen test [1] Gives an evidence of prior streptococcal infection. [1]
Echocardiography [1] Done to screen for the detection of rheumatic heart disease.The severity of valvular regurgitation has been classified based on the echocardiographic findings [1]
Electrocardiogram [1] To detect arrythmias and prolong PR interval [1]
References
  1. Rheumatic fever and rheumatic heart disease,WHO Technical Report Series, November 2001 [viewed on 19 July 2014] Available from: http://www.who.int/cardiovascular_diseases/resources/en/cvd_trs923.pdf
  2. MERLINI AB, STOCCO CS, SCHAFRANSKI MD, ARRUDA P, BAIL L, BORGES CL, DORNELLES CF. Prevalence of group a Beta-hemolytic streptococcus oropharyngeal colonization in children and therapeutic regimen based on antistreptolysin levels: data from a city from southern Brazil. Open Rheumatol J [online] 2014:13-7 [viewed 13 September 2014] Available from: doi:10.2174/1874312901408010013

Management - General Measures

Fact Explanation
Aspirin, 100 mg/kg-day divided into 4–5 doses [1] Suppression of the inflammatory process.After achieving the desired initial steady-state concentration for two weeks, the dosage can be decreased to 60–70mg/kg-day for an additional 3–6 weeks [1]
Steroids [3] Suppression of the inflammatory process.Patients with pericarditis or heart failure respond favorably to corticosteroids; corticosteroids are also advisable in patients who do not respond to salicylates and who continue to worsen and develop heart failure despite anti-inflammatory therapy.Prednisone (1– 2 mg/kg-day, to a maximum of 80mg/day given once daily, or in divided doses) is usually the drug of choice [1]
Treatment of heart failure [1] Generally responds to bed rest and steroids.But in severe symptoms diuretics,angiotensin converting enzyme inhibitors and digoxin may be used [1]
Management of chorea [1] Haloperidol, diazepam are effective drugs [1]
Analgesics [1] For management of pain in arthritis.Usually paracetamol or non steroidal anti inflammatory drugs are used [1]
Control of fever [1] Paracetamol is usually used [1]
Antibiotics [1] Single-dose IM benzathine benzylpenicillin (preferable) or 10 days oral penicillin ( oral erythromycin is given if allergic to penicillin) [1]
Primordial prevention [1] Broad social, economic and environmental initiatives undertaken to prevent or limit the impact of Group A Streptococcal (GAS) infection in a population [2]
Primary prevention [1] Reducing GAS transmission, acquisition, colonisation and carriage or treating GAS infection effectively to prevent the development of acute rheumatic fever in individuals [1]
Secondary prevention [1] Administering regular prophylactic antibiotics to individuals who have had an episode of acute rheumatic fever to prevent the development of rheumatic heart disease or to individuals who have established rheumatic heart disease to prevent the progression of disease and to prevent infective endocarditis. For highly suspected acute rheumatic disease, secondary prophylaxis is usually given minimum 10 years or until age 21 (which ever is longer), or until alternative diagnosis is confirmed. Intramuscular injection of benzathine benzylpenicillin every three weeks (every four weeks in low-risk areas or low risk patients) is given.Oral penicillin is an alternative [1]
Tertiary prevention [1] Intervention in individuals with rheumatic heart disease to reduce symptoms and disability, and prevent premature death [1]
Dental review [1] To prevent infective endocarditis due to infections (patients with rheumatic heart disease,are more prone to get infective endocarditis due to infections) [1]
References
  1. Rheumatic fever and rheumatic heart disease,WHO Technical Report Series, November 2001 [viewed on 19 July 2014] Available from: http://www.who.int/cardiovascular_diseases/resources/en/cvd_trs923.pdf
  2. JOSEPH N, MADI D, KUMAR GS, NELLIYANIL M, SARALAYA V, RAI S. Clinical Spectrum of Rheumatic Fever and Rheumatic Heart Disease: A 10 Year Experience in an Urban Area of South India N Am J Med Sci [online] 2013 Nov, 5(11):647-652 [viewed 19 June 2014] Available from: doi:10.4103/1947-2714.122307
  3. KOTHARI SS. Of history, half-truths, and rheumatic fever. Ann Pediatr Cardiol [online] 2013 Jul, 6(2):117-20 [viewed 13 September 2014] Available from: doi:10.4103/0974-2069.115251

Management - Specific Treatments

Fact Explanation
Treatment of heart failure [1] Generally responds to bed rest and steroids.But in severe symptoms diuretics,angiotensin converting enzyme inhibitors and digoxin may be used [1]
Antibiotic prophylaxis [1] For prevention of infective endocarditis.Intramuscular injection of benzathine benzylpenicillin every three weeks (every four weeks in low-risk areas or low risk patients) is given.Oral penicillin is an alternative. For highly suspected acute rheumatic disease, secondary prophylaxis is usually given minimum 10 years or until age 21 (which ever is longer), or until alternative diagnosis is confirmed. [1]
Surgery for rheumatic heart disease [2] Balloon valvotomy (commissurotomy) This technique is reserved almost entirely for stenosis of the mitral valve [1]
Heart transplantation [3] Heart transplantation for endstage rheumatic heart disease can achieve an acceptable long-term survival [3]
References
  1. Rheumatic fever and rheumatic heart disease,WHO Technical Report Series, November 2001 [viewed on 19 July 2014] Available from: http://www.who.int/cardiovascular_diseases/resources/en/cvd_trs923.pdf
  2. SWAIN JD, MUCUMBISTI J, RUSINGIZA E, BOLMAN RM 3RD, BINAGWAHO A. Cardiac surgery for advanced rheumatic heart disease in Rwanda. Lancet Glob Health [online] 2014 Mar, 2(3):e141-2 [viewed 13 September 2014] Available from: doi:10.1016/S2214-109X(14)70022-1
  3. CHI NH, CHOU NK, YU YH, YU HY, WU IH, CHEN YS, HUANG SC, KO WJ, WANG SS. Heart transplantation in endstage rheumatic heart disease-experience of an endemic area. Circ J [online] 2014, 78(8):1900-7 [viewed 13 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/24965078