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Heart disease resources

Primary prevention of pulmonary vented or diagnosed, and adequately treated, disease it must be recognized as a specific form of both and chronic heart disease. Such PULMONARY HEART DISEASE STUDY GROUP: recognition can be facilitated by the accep- Chairman: ROY H. BEHNKE, M.D. tance of a single definition of the disease Members: S. GILBERT BLOUNT, M.D.; entity. Pulmonary heart disease, cor pulmo- J. DAVID BRISTOW, M.D.; VIRGINIA CARRIERI, R.N.; JOHN A. PIERCE, M.D.; nale, is here defined as: Alteration in structure ARTHUR SASAHARA, M.D.; or function of the right resulting ALFRED SOFFER, M.D. from disease affecting the structure or func- Consultant: RICHARD GREENSPAN, M.D. tion of the or its vasculature, except when Introduction this alteration results from disease of the left Pulmonary heart disease, cor pulmonale, is side of the heart or congenital heart disease. a major form of heart disease yet there is reasonable evidence that it can be prevented. Incidence and prevalence Support for this contention rests in the fact Effective programs of prevention are facili- that the causative or precedent pulmonary tated by an accurate assessment of the scope diseases are in large part preventable. Primary and magnitude of the health problem. Chronic prevention of the related pulmonary diseases obstructive lung disease (bronchitis and/or is thus the initial and basic step in the pre- emphysema) is by far the most important of vention of cor pulmonale. Such an under- the respiratory diseases associated with pul- taking is a major task. It can only be achieved monary heart disease. Definitive data on the by the global application of existing knowl- prevalence of the respiratory diseases and cor edge to the environment and the population at pulmonale are not available, largely because large. The force which is most apt to activate uniform diagnostic criteria and reporting are broad programs of primary prevention of not employed by all . This is true pulmonary diseases is an enlightened public not only in certifying the cause of death but demand that these diseases in fact be much more importantly in reporting patient eliminated. morbidity in office visits and hospital admis- In individuals who develop pulmonary sions. disease, the prevention of cor pulmonale The increasing toll of death and disability becomes the joint responsibility of the patient caused by chronic obstructive lung disease has and his . Early diagnosis and treat- become a matter of grave concern to federal ment of the pulmonary disorder is essential if officials, the public, and to voluntary and the anatomic and physiologic alterations private health organizations. According to the which produce cor pulmonale are to be National Center for Chronic Disease Control avoided. Thus, secondary prevention and and the Social Security Administration, em- specific treatment of pulmonary disease is physema is now second only to coronary primary prevention of pulmonary heart dis- disease in disabling the nations workers. Each ease. year some 15,000 middle aged and older American workers become unable to work Definition because of emphysema and/or chronic bron- If pulmonary heart disease is to be pre- chitis. The Social Security Administration,

1139/88 currently paying out $90 million annually to or emphysema had risen to 25,000. Studies in support people totally disabled by chronic two hospitals, Colorado General Hospital and lung disease, has reported that respiratory Denver Veterans Administration Hospital, disease was the primary diagnosis in 38,263 show that the death certificate understates workers who were retired prematurely be- emphysema and/or chronic bronchitis as a cause of disability in 1965. This number cause of death in men over 40 by almost 30% represents 14.3% of all workers who first and the presence of disabling emphysema received benefits under the disability program and/or chronic bronchitis at the time of death that year. A considerably higher portion of by 50% or more. Another study indicates that men than women had a respiratory disability, the prevalence at death of chronic respiratory particularly emphysema. This disease was the disease is found to be nine times greater than leading cause of respiratory disability with its underlying cause death rate. 16,879 workers receiving benefits for pulmo- Chronic obstructive lung disease is often nary disease. complicated by the development of cor It is estimated that more than three million pulmonale and congestive . The Americans over the age of 45 have demonstra- exact morbidity and mortality due to cor ble emphysema or significant chronic bronchi- pulmonale and right heart failure is very tis and the number of United States citizens difficult to determine. In some instances death partially or totally disabled by emphysema is attributed to heart disease with congestive and/or chronic bronchitis is estimated at from failure while in others the lung disease is listed five to ten million. Furthermore, the number as the primary cause. Stuart-Harris found 30 of persons with increases to 40% of patients with congestive heart fail- sharply with advancing age-89% of those ure in Sheffield, England had cor pulmonale to granted disability benefits because of emphy- some degree. In the United States it is sema were 50 years of age or over. estimated that 10 to 30% of congestive heart A report by The Task Force on Chronic failure admissions to medical centers have cor Bronchitis and Emphysema sponsored by the pulmonale as a predisposing condition. More National Tuberculosis and Respiratory Dis- definitive data on the frequency of cor ease Association and the Service pulmonale and its relationship to other forms reveals that the number of deaths attributed of cardiopulmonary disease are needed. It is to emphysema and bronchitis is doubling estimated that if pneumonia and conges- every five years. While emphysema has in all tive heart failure deaths in which emphysema probability always existed, there is no ques- and/or chronic bronchitis played the major or tion that its reported incidence is growing a substantial contributory role were included, sharply. This is probably due to an actual the true number of deaths from chronic increase in incidence combined with a height- obstructive lung disease would approximate ened interest and diagnostic acuity on the part 75,000 per year. of the medical profession. Thus, in 1955 As previously stated, vital statistics on emphysema was reported as a cause of death these diseases lack precision. Nevertheless, it in 3,639 persons in the United States while in is clear that incidence is increasing. Limited 1959 the number was up to 7,728. By 1967 surveys suggest that as high as 25% of the reported deaths from chronic bronchitis and/ population over 40 years of age have abnor-

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mal lung function. This leads to an estimate of monary disease than their counterparts who up to 14 million persons in the United States do not smoke. with some degree of chronic obstructive Studies of pulmonary function supply data pulmonary disease. There is no accurate base on alterations in respiratory physiology which upon which to estimate what per cent of these correlate well with clinical symptomatology will develop cor pulmonale and right heart and physical findings. There is a diminution in failure, but there is every reason from present many parameters of function: timed vital clinical experience to believe that the number capacity, peak expiratory flow, and diffusing will be large. capacity in individuals who smoke. These same individuals have increased airway resis- Prevention of the causative pulmonary diseases tance and a larger than normal debt There is need for more information on with exercise. Like the clinical symptoms of pathogenic mechanisms leading to chronic respiratory disease these functional abnormali- respiratory disease. Nevertheless much is ties improve with the cessation of the cigarette known about prevention. The basic risk habit. Pathological studies on and the factors are cigarette smoking, infection, and tracheobronchial tree of smokers vs. nonsmok- air pollution. ers demonstrate the deleterious effects of smoking. A grading of emphysema (panlobu- Smoking lar, centrilobular, or mixed type) with lung It is well established that smoking is macrosections is similarly discriminating, re- injurious to health in many ways. Cigarette vealing a greater incidence and degree of em- smoking is the major cause of pulmonary physema in the lungs of smokers. The relation- heart disease in that it is the most important ship between smoking, bronchitis-emphysema cause of the chronic non-neoplastic broncho- and pulmonary heart disease is such that there pulmonary diseases in the United States. is little doubt that a radical reduction or Epidemiological and laboratory evidence elimination of the cigarette habit would result strongly supports the view that cigarette in a greatly lowered incidence of the chronic smoking can contribute to the development of respiratory diseases and cor pulmonale. pulmonary emphysema in man. If these advantages are to be realized there Prospective studies indicate that morbidity must be increased education of the public, and mortality from bronchitis and emphysema with particular emphasis upon programs are much higher among smokers than among which discourage the young from smoking. non-smokers and are directly related to the The adult population which is habituated to number of cigarettes smoked. It now appears cigarettes must be convinced of the health clear that women who smoke also have in- advantages of stopping. The present tempo of creased risk from these same diseases. Clinical education will have to be increased if early studies of respiratory disease symptoms—per- gains are to be significantly multiplied. sistent cough, sputum production and short- However, along with these broad programs ness of breath—are found to be two to three including severe restrictions on cigarette ad- times more prevalent in smokers compared vertising, there must come an increased with non-smokers. Smokers more frequently awareness by the physician and all other require hospitalization and treatment for pul- health personnel of their individual responsi-

1141/90 bility and capability as effective educators. All conditions are important determinants of the patients should be queried about their smok- continuing endemism of pulmonary disease ing habits, and they should be adequately and their correction is mandatory for any informed of the health risks and the benefits to successful program of primary prevention. be derived from stopping smoking. Physicians Bacteria and the viruses are also implicated, have a responsibility to strongly admonish all although much less specifically, as a cause of patients against smoking. There must be pulmonary heart disease due to their associa- adequate medical recognition of the physio- tion with both acute and chronic bronchitis logic and psychologic effects attendant upon and emphysema. Their exact role in these stopping and a willingness to treat the diseases is somewhat unclear. There is insuffi- withdrawal state with the seriousness it cient data to implicate them as the primary requires if the effort is to be successful. The cause of injury although viral agents are under health professions must take a position of increasing suspicion and study in this regard. leadership in the development and expansion There is, however, evidence that chemical of community assistance resources (e.g., stop irritants, such as are found in cigarette smoke smoking clinics) to which an individual may or air pollutants, may create favorable circum- be referred for help in breaking the cigarette stances in the lower respiratory tract, which is habit. normally quite sterile, for the growth of infectious agents. Frequently there are or- Infection ganisms indigenous to the upper respiratory A wide variety of infectious agents are able tract. In other instances they are totally to damage the lung and derange its functions foreign to it. Whatever the sequence of sufficiently to produce pulmonary heart dis- pathogenesis it is probable that these or- ease. This cause and effect relationship is ganisms do damage tissue once they are particularly evident with infections caused by established within the lower tract. Investiga- specific bacteria. For example, tuberculosis tion of the sequence of events and the specific organisms can destroy lungs or produce role of infectious agents is needed. This is fibrosis which will eventuate in , pul- essential if prevention is to be effective in the monary hypertension and cor pulmonale, [of] early stages of the disease prior to irreversible which the basic etiology is infection, the basic tissue damage. inflammation and tissue damage, and the clinical disability in great part, Air Pollution cardiac. The risk of acquiring such a lung The relationship of air pollution to pulmo- infection with serious cardiac consequences is nary disease and consequently to pulmonary less now than ever before due to the wider heart disease is twofold. First there is the application of public health measures and the highly important exposure of individuals to ever increasing spectrum of specific toxic substances because of their occupation. agents. These health advantages are still Secondly there is the much greater problem of denied to large segments of the population the general pollution of the atmopshere with living in over-crowded conditions with pover- noxious substances to which large segments of ty, substandard housing and admittedly inad- the population are exposed. equate delivery of basic health care. These The significance of a special occupational

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pollutant is related both to the size of the gathering techniques and methodology were industrial population exposed and the extent inadequately standardized, making meaningful of pulmonary damage which it is capable of comparison impossible. Much of the experi- effecting. The awareness of the specific mental work has involved exposing animals to capability of particulate silica, for example, to levels of toxic agents far greater than those produce pulmonary fibrosis with progressive existing in the human environment. severe pulmonary and cardiac disability has During specific episodes of extremely high led to a heightened and intensified application contamination such as have occurred in of occupational safety techniques in appropri- London, New York and Los Angeles there ate industries. Older views of simple particu- have been significant increases in morbidity late industrial exposure have been supple- and mortality within the exposed population. mented by newer concepts of occupational The evidence suggests that these excessive hypersensitivity diseases as, for example, rates occur primarily in those patients with berylliosis. In an industrial nation where new pre-existing respiratory and/or cardiovascular manufacturing processes are constantly being disease. Most studies have attempted to developed, exposure to a wide range of correlate levels of pollution with the more irritants of unknown potential for both acute easily diagnosable entity bronchitis rather and chronic pulmonary disease is always than with pulmonary emphysema. There is no possible. If serious and widespread inadver- convincing evidence which clearly relates tent exposures are to be prevented there must pollution and emphysema. This is at least in be increasing emphasis on accurate and part due to the great difficulty in establishing careful industrial occupational histories of an accurate early diagnosis of this disease. exposed employees correlated with physical, Some studies have positively correlated pul- laboratory, and radiologic findings. Industry monary disease, especially bronchitis, with must be willing to utilize what may eventual- levels of smoke and sulfur dioxide. It has been ly prove to be unnecessary industrial health suggested that the effect of pollution may be controls during the initiation and development widespread throughout the tracheobronchial stages of new processes until the actual hazard tree thus setting the stage whereby minimal to employees is evaluated. There must be chemical damage could facilitate bacterial greater interest in the development and the colonization. This may be a primary mecha- vigorous application of appropriate laws and nism in the development of acute and chronic regulations where hazards are found to exist. recurrent bronchitis. The significance of general air pollution in Despite measurable differences in the level the production of pulmonary disease is of air pollution there is little evidence that complex and difficult to determine. Existing morbidity and mortality from respiratory retrospective epidemiologic studies provide an diseases is significantly different in rural as inadequate base upon which to determine the compared with urban areas. This is particular- roles played by multiple variables. In many of ly true when the important variables of age, these studies the number of observations were smoking habits, and occupation on the two small, there were wide variations in meteoro- resident groups are taken into consideration. logic and climatic conditions as well as in A great deal of investigation is necessary to type and quantity of pollutants, and data evaluate critically the relationship between

1143/92 general air pollution and the respiratory Systemic venous and embolism diseases. This environmental research will Idiopathic pulmonary thrombosis require large sums of money and years of Idiopathic observations made by teams (clinicians, en- Sickle cell anemia with pulmonary throm- vironmental engineers, epidemiologists and bosis statisticians) operating in widely scattered Malignant pulmonary emboli geographic areas. Because of its potential sig- Schistosomiasis ficance for populations residing in urban areas INFILTRATIVE, INFLAMMATORY OR FIBROTIC there is a compelling need for research to PULMONARY DISEASE elucidate the relationship between atmospher- and other pneumoconioses ic pollution and pulmonary disease. Tuberculosis Recognition of causative pulmonary disease Berylliosis If cor pulmonale is to be prevented, a Idiopathic diffuse interstitial fibrosis greatly increased awareness of the specific Radiation damage interrelationship between pulmonary disease Collagen vascular disorders and heart disease is essential upon the part of Malignant infiltration all physicians and allied health personnel. In too many instances this association is treated Pathogenesis of cor pulmonale casually or not recognized at all. The outline Pulmonary heart disease is produced by below is intended to provide a broad schema respiratory disease through the interaction of of associated pulmonary diseases. Specific two pathophysiologic mechanisms. The final details of recognition will be described in the common pathway in cor pulmonale and right section dealing with acute treatment. heart failure is increased pulmonary vascular resistance and pulmonary arterial hyperten- Causes of cor pulmonale sion. The mechanism most easily understood is CHRONIC OBSTRUCTIVE PULMONARY DISEASE the production of increased pulmonary resis- Chronic bronchitis with or without em- tance and pressure that results from the physema plugging of pulmonary arteries and arterioles Bronchial asthma with thrombotic material as seen in the OTHER ABNORMALITIES OF ALVEOLAR VENTI- clinical syndrome of . LATION Simple anatomic reduction of the dimension Kyphoscoliosis and other structural ab- of the arterial bed is the dominant defect. A normalities of the thorax second mechanism, classically found in chron- Pleural disease ic obstructive pulmonary disease, is more Neuromuscular disorders complex. Here the hypertension and increased Alveolar hypoventilation associated with resistance are the result of pulmonary vaso- obesity spasm triggered by alveolar hypoxia. The Residence at high altitude other consequences of respiratory failure or Airway obstruction due to hypertrophied alveolar hypoventilation—hypercapnea and tonsils and adenoids acidosis—are much less important in the PULMONARY THROMBOSIS OR EMBOLISM genesis of the deranged cardiac physiology.

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Reconunendations Cigarette smoking Incidence and prevalence The importance of cigarette smoking as the While it is conservatively estimated that major cause of chronic obstructive pulmonary there are at least two million people with disease and consequently heart disease cannot manifestations of chronic obstructive pulmo- be overemphasized. The significance of this nary disease in the United States, there is little public health hazard has been exhaustively data to indicate the number of patients who documented in the reports of the Surgeon will develop or who already have pulmonary General of the United States Public Health heart disease. As previously noted this situa- Service and attested to by major governments tion is due in part to the vagaries of diagnosis throughout the world as well as leading as well as the inadequacies in collecting and voluntary health organizations. The lesson of reporting morbidity and mortality statistics. the moment is that an attitude of pessimism is 1. There is a need for intensified research not warranted. During the past two years it and investigation to define and identify more has become apparent that widespread efforts accurately the factors which produce cor by many individuals and groups combined pulmonale in patients with respiratory disease. with a hard hitting mass media educational The interface between pulmonary disease and campaign have been effective in reducing heart disease must be more accurately de- cigarette consumption in all age groups in the scribed and defined in cooperative efforts by United States. These trends must be acceler- scientists and laboratories whose efforts here- ated. The following recommendations are tofore have been principally confined to one included for increased emphasis. specialty area or the other. 1. Every individual working within the 2. Criteria for the diagnosis of cor pulmo- health professions should be regarded as an nale and right heart failure must be widely example to whom the public can look for disseminated, understood and utilized by all encouragement and support. To strengthen practicing physicians. Care and accuracy their position in this matter all health should be universal in the certification of the professionals should be encouraged to stop primary and secondary causes of death. The smoking. In particular, physicians, nurses and responsibility for this program of education dentists have a responsibility to counsel all lies with the medical schools and those patients on the hazards of smoking and the professional organizations with a special benefits to be derived from stopping. As a interest in cardiovascular and respiratory program of preventive these facts disease. require constant restatement and reinforce- 3. Prospective studies of morbidity and ment in the home visit, the office and the mortality from pulmonary heart disease based hospital. upon well planned protocols must be devel- 2. Communities should develop and sup- oped and financed. These studies will require port facilities and services to assist individuals cooperative data collection. The hospitals of who wish to stop smoking. Churches, veterans the Veterans Administration with their dom- organizations and voluntary health agencies as inantly male population could provide accu- well as other community groups can play an rate and significant longitudinal and terminal important role in developing these programs. data. However, the local health department work-

1145/94 ing in close cooperation with the medical Where such a risk is suspected but not yet profession should assume initiative and lead- conclusively proven, steps should be taken to ership in establishing these vitally important (a) establish the nature of the relationship services where they do not presently exist. between the agent and the respiratory system Programs similar to those for the control of and (b) minimize possible damage during the alcoholism and obesity with extensive involve- period of uncertainty. ment of laymen should be considered as models. Further research is needed to develop Infection and air pollution new and innovative ways of accomplishing A great deal more is known today about the these ends. interrelationship between infection, air pollu- 3. There is great need for expanded re- tion and the cardiopulmonary diseases than search into the physiologic and psychologic was the case less than a decade ago. Never- events attendant upon stopping smoking, theless a greatly intensified, highly inte- including the use of drugs, hypnosis and lay grated interdisciplinary research effort will be and professionally directed group . In required to resolve many of the unanswered addition, investigation of the effects of smok- questions. ing on those patients with serious pulmonary There is need for standardization of cate- and cardiac disease should be continued. gories of environmental contaminants as well Much of what is presently known in this area as the techniques for their measurement. Until has been derived from the essentially "normal" some degree of uniformity is achieved there individual. can be no meaningful comparisons. This will 4. All advertising of tobacco products require agreement by authorities in the field should be discontinued. In addition, an followed by adequate operating funds and intensified mass media educational program implementing legislation at the local, state and on the hazards of smoking and benefits of federal level. cessation should be continued indefinitely. Research to ascertain the relative impor- tance of air pollution and infection should Occupational hazards receive a high priority. Longitudinal studies of Where there is a recognized relation be- segments of the population with the minimum tween materials used in an industry and the of environmental variables supported by development of pulmonary disease, strict facilities for accurate bacteriologic and sero- regulation must be enforced to minimize risk. logic evaluation should be implemented.

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