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241 Pages·1980·9.78 MB·English
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Rheumatic Valvular Disease in Children Edited by Joseph B. Borman and Mervyn S. Gotsman With Contributions by J.B. Borman, A. Carpentier, S. Cotev, J.T. Davidson, A.M. Davies, V.J. Ferrans, M.S. Gotsman, S.T. Halfon, B.S. Lewis, C.M. Oakley, A.T.S. Paul, W.C. Roberts, N.M.A. Rogers, A. Simcha, T.L. Spray With 105 Figures and 43 Tables Springer-Verlag Berlin Heidelberg New York 1980 Editors: Joseph B. Borman, MB., B. Ch. (Wits), FR.C.S. (Eng), FA.C.S., FA.C.C.,Professor of Surgery, Head, Department of Cardiothoracic Surgery, Hadassah University Hospi tal and Hebrew University.JIadassah Medical School, Jerusalem, Israel Mervyn S. Gotsman, M.D., F.R.C.P., FRC.P. (G), FA.C.C., Professor of Medicine, Head, Department of Cardiology, Hadassah University Hospital and Hebrew University Hadassah Medical School, Jerusalem, Israel ISBN-13: 978-3-540-10079-9 e-ISBN-13: 978-3-642-95371-2 DOl: 10.1007/978-3-642-95371-2 Library of Congress Cataloging in Publication Data. Main entry under title: Rheumatic valvular disease in children. Bibliography: p. Includes index. 1. Rheumatic heart disease in children. 2. Rheumatic heart disease in children - Surgery. 3. Heart - Valves - Surgery. I. Borman, Joseph B. II. Gotsman, Mervyn S., 1935- [DNLM: 1. Heart valve disease - in infancy and childhood. 2. Rheumatic heart disease - In infancy and childhood. WG240 R472] RJ426. R54R5 618.92'127 80-17094 This work is subject to copyright. All rights are reserved, whether the whole or part of the material is concerned, specifically those of translation, reprinting, re-use of illustrations, broadcasting, re production by photocopying machine or similar means and storage in data banks. Under § 54 of the German Copyright Law where copies are made for other than private use, a fee is payable to the publisher, the amount of the fee to be determined by agreement with the publisher. ©Springer-Verlag Berlin Heidelberg 1980. Softcover reprint of the hardcover I st edition 1980 The use of registered names, trademarks etc. in this publication does not imply, even in the ab sence of a specific statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use. Typesetting, SatzStudio Pfeifer, Germering 2121/3321 543210 Contents List of Contributors. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. VI Acknowledgement. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . VIII Preface. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. IX S.T Halfon and AM. Davies: Epidemiology and Prevention of Rheumatic Heart Disease .. . . . . . . . . . . . . . 1 C. M.Oakley: Acute Rheumatic Carditis. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 15 V J. Ferrans and W.C. Roberts: Pathology of Rheumatic Heart Disease . . . . ...................... " 28 B.S. Lewis and M.S. Gotsman: Natural History of Rheumatic Heart Disease in Childhood. . . . . . . . . . . . . . .. 59 M.S. Gotsman and B.S. Lewis: Preoperative Assessment of the Child with Chronic Rheumatic Heart Disease . .. 72 J. T. Davidson and S. Cotev: Anesthesia and Respiratory Support . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 112 A.T .S. Paul: Closed Mitral Commissurotomy in Children. . . . . . . . . . . . . . . . . . . . . . . .. 126 A. Carpentier: Reconstructive Surgery of Rheumatic Valvular Disease in Children Under 12 Years of Age ......................................... " 149 J.B. Bonnan and A. Simcha: Mitral Valve Replacement in Children. . . . . . . . . . . . . . . . . . . . . . . . . . . .. 160 NM.A. Rogers: Aortic Valve Replacement in Children with Rheumatic Heart Disease. . . . . . .. 172 J.B. Bonnan and A. Simcha: Surgery for Multivalvular Disease in Children. . . . . . . . . . . . . . . . . . . . . . .. 180 W. C. Roberts and T. L. Spray: Prosthetic Cardiac Valves - A Comparison of the Four Basic Designs. . . . . . .. 193 M.S. Gotsman and B.S. Lewis: Long-Tenn Management of the Child After Surgery for Rheumatic Heart Disease. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 206 Subject Index. . . . . . .. .................................... 223 V List of Contributors Joseph B. Bonnan, M.B., B. Ch. (Wits), F .R.C.S. (Eng), F .A.C.S., F.A.C.C., Professor of Surgery, Head Department of Cardiothoracic Surgery, Hadassah University Hospital and Hebrew University-Hadassah Medical School, Jerusalem. Israel. Alain Carpentier, MD., PhD., Professor of Surgery, Hopital Broussais, Paris, France. S. Cotev, MD., Associate Professor of Anesthesiology and Director of Respiratory In tensive Care Unit, Department of Anesthesiology and Respiratory Intensive Care Unit, Hadassah University Hospital, Jerusalem, Israel. J.T. Davidson, M.D., F.F.A.R.C.S., Professor and Head, Department of Anesthesio logy and Respiratory Intensive Care Unit, Hadassah University Hopsital, Jerusa lem, Israel. AM. Davies, M D., Professor and Head, Department of Medical Ecology, Hebrew Uni versity-Hadassah Medical School, Jerusalem, Israel. Victor i. Ferrans, M.D., Ph. D., Pathology Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland, United States. Mervyn S. Gotsman, M.D., F.R.C.P., F.R.C.P. (G), FA.C.C., Professor of Medicine, Head Department of Cardiology, Hadassah University Hospital and Hebrew Uni versity-HadassahMedical School, Jerusalem, Israel. S.T. Halfon, M.D., M.P .R., Senior Lecturer, Department of Medical Ecology, Hebrew University-Hadassah Medical School, Jerusalem, Israel. Basil S. Lewis,M.D., MR.C.P., F .C.P. (SA), Senior Lecturer, Senior Physician Depart ment of Cardiology, Hadassah University Hospital and Hebrew University-Hadas sah Medical School, Jerusalem, Israel. Celia M. Oakley, MD., F .R.C.P., Consultant Cardiologist, Royal Postgraduate Medical School, Hammersmith Hospital, Du Cane Road, London W120HS, Great Britain. A.T.S. Paul, Senior Lecturer, Department of Surgery, University of Nairobi; Consul tant Surgeon, Kenyatta National Hospital; Late Senior Cardio-Thoracic Surgeon, Colombo General Hospital, Sri-Lanka, Hunterian Professor Royal College of Sur geons, Great Britain. William C. Roberts, MD., Pathology Branch, National Heart, Lung, and Blood Insti tute, National Institutes of Health, Bethesda, Maryland, United States. NMA. Rogers, F.R.C S., Associate Professor, Department of Thoracic Surgery, Went worth Hospital and University of Natal, Durban, South Africa. VI Arie Simcha, M.D., Senior Lecturer, Senior Physician, Departments of Pediatrics and Cardiology, Hadassah University Hospital and Hebrew University-Hadassah Medi cal School, Jerusalem, Israel. Thomas L. Spray, M.D., Pathology Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland, United States. VIl Acknowledgement The preparation of this study was supported through the Special Foreign Currency Program of the National Library of Medicine, Public Health Service, U.S. Department of Health, Education and Welfare, Bethesda, Maryland, under an agreement with the Israel Journal of Medical Sciences, Jerusalem, Israel. VIII Preface In the Middle Eastern states, in the developing countries of Southeast Asia and Africa with their vast populations, in the poverty-stricken Central and South American lands, and in other underprivileged parts of the world, rheumatic heart disease is a major problem with a high morbidity and mortality. In these areas the disease frequently attacks children, in whom the course is much more malignant than in adults. This re sults in severe pathologic changes in the cardiac valves from an early age. Stenosis of the mitral valve is one of the more common lesions, and closed mitral commissurotomy is often carried out in children. Furthermore, mitral insufficiency, aortic insufficiency, and tricuspid valve pathology may cause such life-threatening hemodynamic effects as to require valve replacement, usually electively, or even occaSionally, as an emergency. This differs from experience in the affluent sections of the population in the developed countries where severe rheumatic valvular pathology in children is uncommon, and sur gery for the advanced form of the disease is limited to adults. Recent progress in diagnostic methods, great advances in surgical skill, and the de velopment of improved valvular prostheses encourage the application of valvular sur gery - so successfully carried out in adults - to children. Such surgery is being under taken with increasing frequency, and the long-term results are encouraging. It was therefore considered important to collect the considerable but scattered information on the subject and to present it in monograph fOml. The editors, who have extensive experience with valvular surgery in children, have succeeded in gathering together an impressive list of contributors, each recognized in ternationally as an expert in the field. The text describes and contrasts in detail the severe nature and course of the disease in children as compared to adults. The monograph is original in concept. The subject matter is of special interest to epidemiologists, pathologists, pediatricians, anesthetists, cardiologists, and surgeons. It will be of great value to physicians both in the developing countries where rheumatic fever is rampant and in the affluent countries where there is less experience with valve replacement in children with rheumatic heart disease. Joseph B. Borman Mervyn S. Gotsman IX Epidemiology and Prevention of Rheumatic Heart Disease S.T. Halfon and A.M. Davies Rheumatic fever, a social disease that "licks the joints and bites the heart," is a non suppurative complication of streptococcal pharyngitis. It is ubiquitous in its dis tribution, but some families seem more susceptible than others: this susceptibility is potentiated by poverty. The epidemiologic picture depends on the interaction of three complex sets of variables, the characteristics of the host (patient), the agent (streptococcus), and the en vironment (social and physical). Small wonder, therefore, that the variations are infmite and that much is unclear, even after half a century of research. Much of the confusion devolves from variations in the clinical picture in time and place, variations in the incidence and spread of the streptococcus, and the use of different criteria for diagnosis. Moreover, patients in clinical series are rarely representa tive of the totality of cases in the community and one must be wary of generalizations from personal experience. In recent years, however, the patterns have become less hazy with more precise implications for prevention and management. This chapter will present some of the evidence and practical conclusions. Incidence and Prevalence: Rheumatic Fever and Rheumatic Heart Disease Sources of Data For accurate information on incidence, i.e., the rate of appearance of new cases in a defmed community during a defined period, it would be necessary to keep the popula tion under constant medical surveillance, using standard methods of examination and diagnosis to ensure that no case is missed, however mild. In practice, we are obliged to rely on information about those cases that come to medical attention. Quite apart from the difficulties of diagnosis [1, 2] and the variability of criteria [3], rheumatic fever is not a reportable disease and its pattern may be changing [4]. Accurate, com parable information is thus rarely aVailable, and we must rely on data from admissions to hospital and death certificates. For prevalence data, i.e., the number of cases existing in a community at a given moment, we are in a better position, particularly with respect to rheumatic heart disease. Here, we have information based on surveys of schoolchildren [5-10] and military recruits [11] as well as hospital and autopsy data. The presence of mild rheu matic fever in a community can lead to a low level of reported incidence (cases are missed) but a high prevalence, which rises as cases of rheumatic heart disease are de tected in asymptomatic individuals. 1 Incidence Rheumatic fever (RF) and rheumatic heart disease (RHD) are still important public health problems in many countries, particularly for those large segments of the popula tion who live in poverty. RHD is the leading cardiovascular disease in those admitted to hospital in Algeria, Chile, Egypt, India, Iran, Morocco, Mongolia, Nigeria, and Sudan [10]. In industrialized countries with higher standards of living, however, the incidence has decreased considerably in recent years. Studies of all hospitalized cases in Baltimore for the period of 1960-1964 by Gordis [12] showed an incident rate of 15.6 per 100,000 for RF in persons aged 5-19. These comprised 13.3 per 100,000 initial attacks and 2.3 for recurrences. For all cases in the community, however, including those treated at home, the corrected in cidence was 24 per 100,000. The incidence among the black population was 2.5 times greater than that among whites for fIrst attacks and fourfold for recurrences. As part of the Jerusalem study, 8000 children initially aged 7-9 were closely followed for 3 years (1968-1970). An annual RF incidence of 8 per 10,000 was re corded with a further 4 per 10,000 suspected cases (1). This very high RF rate was achieved, in part, by sUlveillance of the population at its most susceptible age. For children aged 5-9 in Nashville, during the period 1963-1965, Quinn [13] found a total incidence of 3.6 per 10,000, less than half the Jerusalem fIgure, but based on case finding methods and a wider age span. Here too, the rate was three times higher in blacks. Comparisons between these and other surveys, however, were confounded by differences in diagnostic criteria and availability and utilization of health services by the different populations involved. Prevalence of Rheumatic Heart Disease Here too, many of the differences observed between different groups may be due to problems of diagnostic criteria and observer variation (see Sect. on "Pitfalls in Inter pretation of the Data"). On the other hand, the population is of comparable age, and limited conclusions may be drawn from the data. Table 1 summarizes the results of surveys by different investigators in a number of countries. These data serve to illustrate four characteristics of the disease: it is ubiquitous, in hot as well as in cold countries; there is considerable variation from place to place; there is an excess in poor areas; and in the case of New York, an improvement with time. Other sources of information derive from the medical examination of college students, admittedly a selected population, and military recruits. AID-year survey of 1s t-year American university students (1956- 1965) showed a history of RF, or the presence of RHD or both, in 15.8 per 1000, with a decline in reported incidence and prevalence over the period, in both whites and blacks. [14] The same picture was reported by Ru Duskey [11] in American military recruits. In 1941-1943,2.4% were rejected for RHD: in 1960-1962, the figure was 0.9%. 2

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In the Middle Eastern states, in the developing countries of Southeast Asia and Africa with their vast populations, in the poverty-stricken Central and South American lands, and in other underprivileged parts of the world, rheumatic heart disease is a major problem with a high morbidity and mortalit
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