Rheumatic Valvular Disease in Children

241 Pages·1980·9.78 MB·English

by  S. T. Halfon

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