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WHO Technical Report Series923

RHEUMATIC FEVER ANDRHEUMATIC HEART DISEASE

Report of a WHO Expert ConsultationGeneva, 29 October1 November 2001

World Health OrganizationGeneva 2004

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WHO Library Cataloguing-in-Publication Data

WHO Expert Consultation on Rheumatic Fever and Rheumatic Heart Disease(2001 : Geneva, Switzerland)Rheumatic fever and rheumatic heart disease : report of a WHO Expert Consultation,Geneva, 29 October 1 November 2001.

(WHO technical report series ; 923)

1.Rheumatic fever 2.Rheumatic heart disease 3.Endocarditis 4.Cost of illness I.TitleII.Series

ISBN 92 4 120923 2 (NLM classification: WC 220)ISSN 0512-3054

World Health Organization 2004

All rights reserved. Publications of the World Health Organization can be obtained from Marketing andDissemination, World Health Organization, 20 Avenue Appia, 1211 Geneva 27, Switzerland (tel: +41 22791 2476; fax: +41 22 791 4857; email: bookorders@who.int). Requests for permission to reproduce ortranslate WHO publications whether for sale or for noncommercial distribution should be addressedto Publications, at the above address (fax: +41 22 791 4806; email: permissions@who.int).

The designations employed and the presentation of the material in this publication do not imply theexpression of any opinion whatsoever on the part of the World Health Organization concerning the legalstatus of any country, territory, city or area or of its authorities, or concerning the delimitation of its frontiersor boundaries. Dotted lines on maps represent approximate border lines for which there may not yet befull agreement.

The mention of specific companies or of certain manufacturers products does not imply that they areendorsed or recommended by the World Health Organization in preference to others of a similar naturethat are not mentioned. Errors and omissions excepted, the names of proprietary products aredistinguished by initial capital letters.

The World Health Organization does not warrant that the information contained in this publication iscomplete and correct and shall not be liable for any damages incurred as a result of its use.

This publication contains the collective views of an international group of experts and does not necessarilyrepresent the decisions or the stated policy of the World Health Organization.

Typeset in Hong KongPrinted in Singapore

2003/15621

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Contents

1. Introduction 1References 1

2. Epidemiology of group A streptococci, rheumatic fever and rheumaticheart disease 3Group A streptococcal infections 3Rheumatic fever and rheumatic heart disease 5Determinants of the disease burden of rheumatic fever and rheumatic

heart disease 7References 8

3. Pathogenesis of rheumatic fever 13Introduction 13Streptococcal M-protein 14Streptococcal superantigens 14The role of the human host in the development of rheumatic fever and

rheumatic heart disease 15Host-pathogen interaction 16The role of environmental factors in RF and RHD 16Conclusions 16References 18

4. Diagnosis of rheumatic fever 20Jones criteria for the diagnosis of rheumatic fever 2020022003 WHO criteria for the diagnosis of rheumatic fever and

rheumatic heart disease (based on the revised Jones criteria) 22Diagnosis of rheumatic carditis 24

Valvulitis/endocarditis 24Myocarditis 25Pericarditis 26

Diagnosis of extracarditic manifestations of RF 26Major manifestations 26

Arthritis 26Sydenhams chorea 31Subcutaneous nodules 34Erythema marginatum 35

Minor manifestations 36New diagnostic techniques for rheumatic carditis 36

Echocardiography 36Endomyocardial biopsy 36Radionuclide imaging 37

References 37

5. Diagnosis of rheumatic fever and assessment of valvular diseaseusing echocardiography 41The advent of echocardiography 41Echocardiography and physiological valvular regurgitation 41

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The role of echocardiography in the diagnosis of acute rheumaticcarditis and in assessing valvular regurgitation 42

Clinical rheumatic carditis 42Classification of the severity of valvular regurgitation using

echocardiography 42Diagnosis of rheumatic carditis of insidious onset 43The use of echocardiography to assess chronic valvular heart

disease 43Diagnosis of recurrent rheumatic carditis 43Diagnosis of subclinical rheumatic carditis 44Conclusions: the advantages and disadvantages of Doppler

echocardiography 45References 46

6. The role of the microbiology laboratory in the diagnosis of streptococcalinfections and rheumatic fever 50Diagnosis of streptococcal infection 50Laboratory tests that support a diagnosis of RF 51The role of the microbiology laboratory in RF prevention programmes 53References 54Appendix. WHO collaborating centres for reference and research on

streptococci 55

7. Chronic rheumatic heart disease 56Mitral stenosis 56Mitral regurgitation 60Mixed mitral stenosis/regurgitation 61Aortic stenosis 61Aortic regurgitation 62Mixed aortic stenosis/regurgitation 64Multivalvular heart disease 64References 65Pregnancy in patients with rheumatic heart disease 67References 68

8. Medical management of rheumatic fever 69General measures 69Antimicrobial therapy 69Suppression of the inflammatory process 69Management of heart failure 70Management of chorea 71References 71

9. Surgery for rheumatic heart disease 73Indications for surgery in chronic valve disease 73

Mitral stenosis (MS) 74Mitral regurgitation (MR) 74Aortic stenosis (AS) 74Aortic regurgitation (AR) 74

Contra-indications to surgery 75

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Treatment options 76Balloon valvotomy (commissurotomy) 76Surgical treatment 76

Long-term complications 77Long-term postoperative management 77The role of surgery in active rheumatic carditis 78References 80

10. Primary prevention of rheumatic fever 82Epidemiology of group A streptococcal upper respiratory tract infection 82Diagnosis of group A streptococcal pharyngitis 82Laboratory diagnosis 83Antibiotic therapy of group A streptococcal pharyngitis 85Special situations 87Other primary prevention approaches 87References 87

11. Secondary prevention of rheumatic fever 91Definition of secondary prevention 91Antibiotics used for secondary prophylaxis: general principles 91

Benzathine benzylpenicillin 91Oral penicillin 92Oral sulfadiazine or sulfasoxazole 93

Duration of secondary prophylaxis 93Special situations 93Penicillin allergy and penicillin skin testing 94References 95

12. Infective endocarditis 97Introduction 97Pathogenesis of infective endocarditis 97Microbial agents causing infective endocarditis1 98Clinical and laboratory diagnosis of infective endocarditis 98Medical and surgical management of infective endocarditis 100Prophylaxis for the prevention of infective endocarditis in patients with

rheumatic valvular heart disease 101Summary 105References 105

13. Prospects for a streptococcal vaccine 106Early attempts at human immunization 106M-protein vaccines in the era of molecular biology 106Immunization approaches not based on streptococcal M-protein 107Epidemiological considerations 107Conclusion 108References 108

14. The socioeconomic burden of rheumatic fever 111The socioeconomic burden of rheumatic fever 111Cost-effectiveness of control programmes 112References 113

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15. Planning and implementation of national programmes for theprevention and control of rheumatic fever and rheumatic heart disease 115Secondary prevention activities 116Primary prevention activities 116Health education activities 116Training health-care providers 117Epidemiological surveillance 117Community and school involvement 117References 118

16. Conclusions and recommendations 120

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WHO Expert Consultation on Rheumatic Fever andRheumatic Heart Disease

Geneva, 29 October1 November 2001

MembersAlan Bisno, Department of Medicine, Veterans Administration Medical Center,

Miami, Florida, USA.

Eric G Butchart, Director, Cardiothoracic Surgery, University Hospital, Cardiff,Wales, UK.

NK Ganguly, Director-General, Indian Council of Medical Research, New Delhi,India.

Tesfamicael Ghebrehiwet, Consultant, Nursing & Health Policy, International Coun-cil of Nurses, Geneva, Switzerland.

Hung-Chi Lue, Professor of Pediatrics, National Taiwan University Hospital, Taipei,Taiwan.

Edward L Kaplan, Department of Pediatrics, University of Minnesota MedicalSchool, Minneapolis, MN, USA. (Co-Chair).

Nawal Kordofani, Programme Coordinator, RF/RHD Prevention Programme, ShaabTeaching Hospital, Khartoum, Sudan.

Diana Martin, Principal Scientist, Institute of Environmental Science & Research,Kenepuro Science Centre, Porirua, New Zealand.

Doreen Millard, Consultant Paediatrician, Paediatrics & Paediatric Cardiology,Kingston, Jamaica.

Jagat Narula, Hahnemann University School of Medicine, Philadelphia, USA. (Co-Rapporteur).

Diego Vanuzzo, Servizio di Prevenzione Cardiovascolari, Centro per la Lotta alleMalattie Cardiovascolari, P. le Santa Maria Misericordia, Udine, Italy.

Salah RA Zaher, Assistant Professor of Pediatrics, University of Alexandria, Alexan-dria, Egypt. (Co-Rapporteur).

WHO SecretariatDerek Yach, Executive Director, Noncommunicable and Mental Health Cluster

(NMH).

Rafael Bengoa, Director, Management of Noncommunicable Diseases (MNC).

Shanthi Mendis, Coordinator, Cardiovascular Disease (CVD). (Co-Chair).

Porfirio Nordet, Cardiovascular Disease (CVD).

Dele Abegunde, Cardiovascular Disease (CVD).

Francesca Celletti, Cardiovascular Disease (CVD).

Claus Heuck, Blood Safety and