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Rheumatic heart disease in rural Pakistan
  1. E L Kaplan
  1. Correspondence to:
    Professor Edward L Kaplan
    Department of Pediatrics, University of Minnesota Medical School, 420 Delaware Street SE, Minneapolis, MN 55455, USA;


In the developing countries of the world, rheumatic fever and rheumatic heart disease remain significant medical and public health problems

  • rheumatic heart disease
  • rheumatic fever
  • Pakistan

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In this issue of Heart, Rizvi and colleagues describe the current situation in Pakistan regarding rheumatic fever and rheumatic heart disease.1 These noteworthy data likely are representative of many countries even at the beginning of the 21st century. While one might question how quantitatively these data represent the other parts of Pakistan, and while one might wish to delve further into the epidemiology of this important cardiovascular disease in Pakistan, nevertheless the point which cannot be overlooked is that this theoretically preventable cardiovascular disease is unacceptably too prevalent. In the developing countries of the world, countries which constitute approximately two thirds of the world’s population, rheumatic fever and rheumatic heart disease (and by inference group A streptococcal upper respiratory tract infections) remain significant medical and public health problems. Uncounted numbers of children and young adults are in need of valve surgery, a procedure which few individuals or ministries of health in those countries can afford.

While there have been recent reports addressing the incidence of acute rheumatic fever and the prevalence of rheumatic heart disease in many countries where the magnitude of the problem is similar (for example, India2), the sentinel reports authored by Strasser and colleagues from the World Health Organization three or four decades ago adequately documented the cost effectiveness of control programmes, and how the lack of attention even to secondary prophylaxis resulted in higher morbidity (including hospitalisations) and mortality.3,4 Yet, little seems to have changed during these past four decades.5 Furthermore, recent published data by Veasy and colleagues from middle class US populations in Utah clearly document that the potential for medical and epidemiologic problems caused by streptococcal infections, rheumatic fever, and rheumatic heart disease is not confined to socially and economically disadvantaged populations in the still industrialising countries. Since 1985, there have been more than 600 proven cases of acute rheumatic fever in Utah.6–,8


The obvious question is why has this disease remained essentially uncontrolled around the world? The irrefutable studies by Wannamaker and colleagues in the early 1950s showed that prompt diagnosis and adequate penicillin treatment of group A streptococcal throat/tonsil infections prevent rheumatic fever (primary prophylaxis).9 During the past 10 or 15 years, although significant new microbiologic and immunologic information has been generated from studies using sophisticated molecular techniques in many basic science laboratories, the pathogenetic mechanism(s) by which the group A streptococcus incites the development of rheumatic fever and rheumatic heart disease remain, at best, incompletely defined. Furthermore, there has been little if any translation of laboratory discoveries (either relating to the organism or to its human host) to the clinic or to the bedside.

Effective control of streptococcal infections continues to be further complicated by the extraordinary and intense competition for health related funds within ministries of health in all countries. Recent commitments by international organisations such as WHO toward the control of coronary heart disease, hypertension, HIV/AIDS, tuberculosis, malaria, and others appear to have made it even more difficult for adequate attention to be given and for subsequent allocation of adequate resources for cost effective and inexpensive control of an essentially preventable cardiovascular disease, rheumatic heart disease. The effort expended and the resources required for rheumatic fever/rheumatic heart disease control are relatively minimal when compared with most other cardiovascular diseases, and the effort can be rewarded in a relatively short period of time as has been noted.


What should be attempted about a current situation such as that described by Rizvi and colleagues in Pakistan? As has been the case with so many other consequences of infectious diseases, the development of vaccines and implementation of wide immunisation programmes have at least the theoretical potential for controlling group A streptococcal infections just as they have impacted smallpox, poliomyelitis, measles, diphtheria (which also has cardiovascular sequelae), tetanus, and many others. However, although there are currently candidate vaccines in very early clinical trials, it is probably accurate to speculate that a group A streptococcal vaccine ready for widespread use will not be available for a number of years, perhaps even a decade. Whether a vaccine—either one leading to effective type-specific immunity or one based upon conserved antigens of the group A streptococcus—will prove sufficiently cost effective to allow it to be distributed among the most at-risk populations in the developing countries has not been sufficiently discussed by global health authorities. This issue should be addressed. Research into the still elusive pathogenetic mechanism(s) should be supported.

Thus, physicians and other clinicians are left with having to continue the current clearly unsatisfactory control methods and procedures as has been the case in the past, and having to make efforts to implement new laboratory developments into the every day practical clinical diagnosis and management of streptococcal infections. The importance of the roles of primary care physicians and of public health authorities around the world in implementing a relatively simple and cost effective approach surely requires additional consideration and resources.

It is the responsibility of the medical and public health communities to raise these important questions in a meaningful way in the hope that until more effective techniques (perhaps a cost effective vaccine) are available, control of situations such as that described by Rizvi and colleagues can be more effectively attempted.