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Da Costa's syndrome
Specialty	Psychiatry

Da Costa's Syndrome is a type of anxiety disorder named for the surgeon Jacob Mendes Da Costa who first observed it in soldiers during the American Civil War. It has also been called effort syndrome, neurocirculatory asthenia, or "soldier's heart". It causes symptoms similar to heart disease - such as fatigue upon exertion, shortness of breath, palpitations, sweating and chest pain - but upon examination, nothing is found to be physically wrong with the patient. The term is infrequently used in modern texts, and the syndrome is now usually interpreted as one of a number of imprecisely characterized "postwar syndromes".

History

Overview

The syndrome was first described among soldiers in 1869 by Arthur Bowen Richards Myers, who called it neurocirculatory asthenia or cardiovascular neurosis, but acquired its more usual name from the 1871 Da Costa study, which reported the latter's observations made during the American Civil War. Use of the term "Da Costa's syndrome" peaked in the early 20th century. Towards the mid-century, the condition was generally re-characterized as a form of neurosis. It was initially classified as "F45.30" (under somatoform disorder of the heart and cardiovascular system) in ICD-10, and is now classified under "somatoform autonomic dysfunction". A 1987 historical overview by Oglesby described it as having "a long and honourable history in the medical literature", considering it to still exist - labelled as "anxiety state" or "anxiety neurosis" - and affecting 2-4% of the population.

1861-1950

In 1871 Jacob Mendez Da Costa’s study of more than 200 soldiers in the American Civil War identified a set of symptoms which has since been named after him as Da Costa’s syndrome. The symptoms included chest pains, palpitations, breathlessness, dizziness and fatigue, typically brought on by strenuous exertion. The pulse was always greatly and rapidly influenced by position and could be aggravated by stooping, or by lying on the left or right side in some cases, and on the back in others. He reported that the waist belt and the knapsack seemed to have something to do with it, and recommended that the soldiers did not wear restrictive clothing because it was liable to retard or prevent recovery. He gives the typical case of a man who was on active duty for several months or more and contracted an annoying bout of diarrhoea or fever, and then, after a short stay in hospital, returned to active service. The soldier soon found that he could not keep up with his comrades in the exertions of a soldier's life as previously, because he would get out of breath, and would get dizzy, and have palpitations and pains in his chest, yet upon examination some time later he looked like a man in sound condition. Although the digestive disturbance usually passed away, the tendency to develop the other symptoms, especially the abnormal response to exertion, would persist for short or long periods of time, with occasional cases of complete recovery. In considering the different types of predisposing causes, Da Costa came to the conclusion that many factors seemed to overlap, but close study revealed that it was "Fevers" 17%, "Diarrhoea" 30.5%, "Hard field service, particularly excessive marching" 34.5%, and finally, "Wounds, injuries, rheumatism, scurvy, ordinary duties of soldier life, and doubtful causes" 18%.

In 1919 Sir Thomas Lewis commented “ it is because these symptoms and signs are largely, and sometimes wholly, the exaggerated physiological response to exercise . . . that I term the whole the ‘effort syndrome’.”

In 1941 Paul Wood studied the respiratory function of 150 cases of DaCosta's syndrome, and found that although the cause of the left sided chest pain was elusive it was located in the muscular and fibrous structures in the anterior chest wall, and although "very few patients had pain while these measurements were being made" it was associated with poor upward movement of the diaphragm, and poor expansion of the chest, particularly the lower chest. Similar pains occurred in the right side of the chest, and in other parts of the chest wall, but less commonly. A more severe chest pain could be brought on by cranking a lorry engine, or lifting a heavy weight.

In 1946 Mandel.E. Cohen and his colleagues from the Massacheusetts General Hospital, Harvard Medical School, and the Fatigue Laboratory, Harvard Graduate School of Business Administration, reported on their comparative study of 20 men with N.C.A. and 20 healthy men of the same age and found that during hard muscular work, “N.C.A. patients consume less oxygen, have a lower ventilatory efficiency and show a higher blood lactate than do healthy control subjects of comparable age", and that the difference in oxygen consumption became greater with the duration of the exercise. Some of the tests were conducted on a larger number of patients. "The data are all consistent with the idea that aerobic metabolism in hard muscular work is abnormal in N.C.A. and suggests high oxygen debt." However this particular study did not determine if this was due to poor general health, poor fitness, or lack of training.

In 1947 M.E. Cohen, and P.D.White and their colleagues from the Massachusetts General Hospital, and the Harvard Medical School, presented more detailed research findings on the type of breathlessness seen in the effort syndrome, and they stated "In neurocirculatory asthenia, anxiety neurosis, or effort syndrome many respiratory symptoms occur in high incidence. This constitutes a characteristic and therefore diagnostic feature of the disorder; the absence of such symptoms makes the diagnosis of N.C.A. improbable. The complete mechanism of these symptoms is unknown but it is of interest that when respiration is investigated objective abnormalities are found, just as when other symptoms of N.C.A. are investigated with objective methods, which demonstrates that the abnormalities are not all in the subjective sphere". The breathlessness is more pronounced when the patient is required to wear a gas mask or when swimming. Also as a general observation there were not many differences while the patients were resting but respiratory rate is slightly greater, breathing is shallower, and sighing is more frequent than normal. In summarising the study of ventilation in relation to exertion, it was found to be higher in N.C.A. patients doing moderate work, compared to healthy people, and "it is higher as hard work begins but becomes lower as hard work progresses. Ventilatory efficiency is lower in N.C.A. than in healthy controls for moderate and hard muscular work”. The study also found that “the incidence and degree of breathlessness in N.C.A. is not only out of proportion to the amount of exercise, but also is out of proportion to the amount of ventilation and ventilation index". "The evidence of poor ventilatory efficiency corresponds interestingly, although it may not explain, another symptom which patients have which is that they 'can't get in enough air' or that 'air doesn't seem to do as much good as it should'". The concluding discussion mentioned that if this type of breathlessness is to be studied effectively the definition of the word “dyspnea” needs to mean the same thing to all who use it and . . . "it should be used uniformly to mean the same thing by all observers", and by implication the effort syndrome type should include the abnormal pattern of breathlessness which occurs in relation to strenuous exertion, which is characteristically distinct.

In 1947 Stewart Wolf of New York presented his findings at the Proceedings of the Thirty-Ninth Annual Meeting of The American Society For Clinical Investigation held in Atlantic City, N.J. on May 5; He studied a type of "respiratory distress characterized by inability to get a full breath": Flouroscopic observations showed that the diaphragm function was abnormal in patients who were anxious, and others who were not . . . and when some of them were put in discussions of situational conflict the changes in inspiration and expiration were evident, and when the diaphragms contractile state during inspiration was such that adequate inspiration was no longer possible, breathlessness occurred with a feeling of inability to take a full breath. The spasm of the diaphragm was often accompanied by pains in the chest and shoulder, occlusion of the lower end of the esophagus, and difficulty swallowing.

In 1950 Edwin Wheeler and his colleagues from the Massachusetts General Hospital and the Harvard Medical School presented their report of a 20 year follow-up study of 173 patients with the Effort Syndrome in J.A.M.A. They found that the condition generally takes a variable course, and also varies from person to person. Most subjects completed questionnaires and of the 60 who attended medical examinations, 11.7% were well, 35% had symptoms, 38.3% had mild disability, and 15% had severe disability, and there are notes where the subjects led quiet or moderate lifestyles, and when changing to more strenuous and sustained activities their health deteriorated, so they returned to the quiet life and recovered. The periods of recuperation from the severe episodes varied from several days, and up to six weeks, and in one case for a year. In some cases this occurred two or three times in their life before they recognised the necessity and value of limiting their activities. Co-morbidity and life expectancy were better than average. An abstract attached to the article referred to the typical features of thin physiques, and long, narrow chests, and the vasomotor responses were below normal with delayed blood pressure and pulse in response to standard exertion, and there was high blood lactate concentration and low oxygen consumption associated with strenuous exercise.

After 1950

In 1951 the fourth edition of Paul Dudley White’s book “Heart Disease” contained a chapter on “Neurocirculatory Asthenia”, because, as he explains, the symptoms are similar to heart disease, but are not the same, and he adds, that they are also similar to, but can occur in the absence of anxiety, and therefore need to be discussed separately. He gives the definition of N.C.A. as the typical group of symptoms of breathlessness, often with sighing, palpitations, precordial aches and pains, exhaustion, and related symptoms such as dizziness and faintness, which are precipitated by excitement or effort, and “it constitutes a kind of fatigue syndrome” . . . and in some cases . . . “it is more or less a chronic condition“ . . . and . . . “That such a state of ill health exists there can be no doubt, no matter what its pathogenesis or exciting factors.” and “the symptoms are not exactly like those produced by effort in a normal healthy person. In some patients the neurocirculatory symptoms are prominent but in others it is gastrointestinal or cerebral symptoms, but the reason for those differences “has not been explained”. The general causes of the condition appear to include such strains as worry over business, social, or family matters, emotional conflicts, physical or nervous fatigue, and exhaustion from acute infections or illnesses. The organic basis is not known although it may involve a disorder of the autonomic nervous system. Other possibilities which have been considered in the past 25 years, include thyrotoxicosis, low-grade infection, adrenal hyperactivity, hyperventilation, and lack of salt, but none have been confirmed, However many of the patients have thin physiques with an “unusually vertical position of the heart”, and “Another interesting finding is abnormality of shape of the capillary loops at the base of the nail”. . . “It is common to find that close relatives have had similar problems, and “Recent studies have suggested that neurocirculatory asthenia belongs to the Mendelian dominant group of inherited disorders.” It was common in World War 1, occurred in civilians as well as soldier’s, and it is generally seen in young adults, but can occur at any age, and is more common in women than men. The symptom of frequent sighing distinguishes the condition from heart disease, and the fatigue sometimes produces more incapacity than heart disease, and in some cases results in complete disability. “It is a real and not an imaginary incapacity, even though at first glance it may have appeared imaginary in World War 1 (1914-1918) when it was sometimes labelled ‘malingering,’ and even though in civilian practice it has frequently been diagnosed as ‘mere nervouseness’.” It is milder in civilian life than in war and it is so commonly associated with psycho-neurosis of the anxiety type “that the two conditions have sometimes been confused one for the other or considered to be synonymous, the term anxiety neurosis having come to mean for many the same collection of symptoms which identify neurocirculatory asthenia.” Treatment involves rest for days or months or as long as required, and elaborate psychotherapy is generally not needed. “In fact, since this condition is neither heart disease nor mental disorder, both cardiologist and psychiatrist are well kept away after the diagnosis has been established, so that the patient may not develop unnecessary fears about either heart or mental state.” “The condition must be discussed seriously, not lightly as if it was of no importance”, and it is equally wrong to dismiss it as negligible or imaginary, as it is to to regard it as dangerous or serious and a threat to life which demands bed rest. Careless disregard will alienate patients and have them seeking advice from charlatans. “The plan of life for the patient needs to be worked out with care” where usually normal but quiet work and play are required, with the avoidance long working hours or new and burdensome tasks. “Often the patient himself is aware of this necessity, but he has perhaps disliked to humor his symptoms or to fall behind his fellows in strenuous living in the business, professional, or social world. With clear medical advice, however, he realizes the wisdom of doing so, and gradually he adjusts himself to suit his symptoms, and is surprised at recapturing a feeling of well being.”

In 1956 Paul Wood’s 2nd edition of Diseases of the Heart and Circulation included a chapter on the effort syndrome . He described how "The syndrome is characterised by a group of symptoms which unduly limit the subject's capacity for effort" and recorded that " The cardinal symptoms" of effort syndrome, neurocirculatory asthenia, irritable heart, soldier's heart, disordered action of the heart (D.A.H.), etc. are "breathlessness (93%), palpitations (89%), fatigue (88%), left inframammary pain (78%), and dizziness (78%), or syncope (fainting) (35%)". He also suggested a variety of methods for diagnosing the difference between the symptoms and those of heart disease. For example (the) “Left inframammary pain (in the lower rib area) is commonly described as aching or as sharp and stabbing in quality” and “It may be initiated” “by fatigue or strain of respiratory muscles” caused by such things as “incessant minimum trauma from” “faulty posture" . . . and with regard to the breathlessness "It is not only a question of breathlessness on effort, but patients will say they are not able to obtain a satisfying breath, and may take "frequent deep sighs". This can sometimes occur at night when it "may be confused with bronchial asthma or paroxysmal cardiac dyspnea". and "A simple and illuminating test" for the symptom involves forced hyperventilation where "The patient is asked to breath deeply and rapidly for one minute." When a healthy person is asked to stop he feels breathless for about 20 seconds, but a patient with Da Costa's syndrome "continues forced breathing, explaining later that he felt breathless." i.e. there is "Dyspnoea instead of apnoea after forced breathing", and "Normal subjects have no difficulty holding the breath for at least 30 seconds, but patients with Da Costa's syndrome usually give up very quickly, 30 per cent of them in less than 10 seconds; moreover, in contrast to controls, they show little distress when the reach the breaking-point." . . . With regard to the fatigue the patients often do not feel refreshed when they wake up in the morning, as if their sleep has been of no value, and they may "feel tired and listless during the day, and are unduly fatigued by effort. " . . . and . . . Orthostatic dizziness is related to orthostatic hypotension and "The effort-tolerance test (for effort-intolerance) consists of stepping on and off a chair ten times, and counting the pulse rate before, immediately after, and subsequently at minute intervals until the resting speed is regained. The deceleration time is abnormal (over 2 minutes) in 33% of these patients." and "Physical signs of autonomic dysfunction are helpful in” “assessing the severity of the case." The photo of a painting of a typical round shouldered, thin chested, kyphotic patient is included on page 941.

In 1980 Soviet researcher V.S.Volkov presented his report on a comparative study of the exertional capacity of 228 patients which distinguished three stages of the effort syndrome (which he referred to as neurocirculatory dystony - NCD). For healthy men the average was 1176 kgm/min, and the three stages of NCD were 1161, 940 & 591 respectively, and for healthy women was 834, and the stages of NCD were 854, 621 & 420 kgm/min, indicating that the severity of the condition was related to circulatory efficiency and exertional capacity. 87.2% tolerated levels of 600 kgm/min or more, and 14 of the others had to stop because of overwhelming radiating chest pain, fatigue, and “fear for their hearts”, and another 14 stopped their test prematurely because of changes in their heart rates which reached sub-maximal levels.

Related conditions

Some researchers have described similarities between orthostatic intolerance, chronic fatigue syndrome, mitral valve prolapse syndrome, and the observations of Da Costa.

Treatment

The reports of DaCosta, and Wheeler show that patients recovered from the more severe symptoms when removed from the strenuous activity or sustained lifestyle that caused them. In many cases relapses were prevented by determining the limits of exertion and lifestyle and keeping within them. The limits were related to abnormalities in respiration and circulation. Other treatments evident from the previous studies were improving physique and posture, appropriate levels of exercise where possible, wearing loose clothing about the waist, and avoiding postural changes such as stooping, or lying on the left or right side, or the back in some cases, which relieved some of the palpitations and chest pains, and standing up slowly can prevent the faintness associated with postural or orthostatic hypotension in some cases.

See also

• Exercise intolerance
• Orthostatic intolerance
• Hyperventilation syndrome
• Chronic fatigue syndrome
• Soldier's Heart (novel)

References

1. ^ Paul O (1987). "Da Costa's syndrome or neurocirculatory asthenia". Br Heart J. 58 (4): 306–15. PMID 3314950.
2. PBS Frontline: The Soldier's Heart
3. "vlib.us". Retrieved 2007-12-18.
4. Engel CC (2004). "Post-war syndromes: illustrating the impact of the social psyche on notions of risk, responsibility, reason, and remedy". J Am Acad Psychoanal Dyn Psychiatry. 32 (2): 321–34, discussion 335–43. PMID 15274499.
5. Pain And Depression: An Interdisciplinary Patient-centered Approach (Advances in Psychosomatic Medicine). Not Avail. 2006. p. 104. ISBN 3-8055-8184-X.
6. A Dictionary of the History of Medicine, Anton Sebastian, Informa Health Care, ISBN 1850700214
7. "Da Costa's syndrome (www.whonamedit.com)". Retrieved 2007-12-18.
8. Edmund D., MD Pellegrino; Caplan, Arthur L.; Mccartney, James Elvins; Dominic A. Sisti (2004). Health, Disease, and Illness: Concepts in Medicine. Washington, D.C: Georgetown University Press. p. 165. ISBN 1-58901-014-0.{{cite book}}: CS1 maint: multiple names: authors list (link)
9. World Health Organization (1992). Icd-10: The Icd-10 Classification of Mental and Behavioural Disorders : Clinical Descriptions and Diagnostic Guidelines. Geneva: World Health Organization. p. 168. ISBN 92-4-154422-8.
10. Lewis T. (1919) The soldier’s heart and the effort syndrome, Paul B. Hoeber, New York.
11. Wood P. (1941) DaCosta's syndrome, The British Medical Journal, May 24th 1941, Vol.1, p.767-772.
12. Cohen, Mandel E. (1946 Nov.). "Low oxygen consumption and low ventilatory efficiency during exhausting work in patients with neurocirculatory asthenia, effort syndrome, anxiety neurosis". Journal of Clinical Investigation. 25 (6): 920. {{cite journal}}: |access-date= requires |url= (help); Check date values in: |date= (help); Unknown parameter |coauthors= ignored (|author= suggested) (help)
13. Cohen, Mandel (May 1947). "Studies of Breathing, Pulmonary Ventilation and Subjective Awareneess of Shortness of Breath (Dyspnea) in Neurocirculatory Asthenia, Effort Syndrome, Anxiety Neurosis". The Journal of Clinical Investigation. 26 (3): 520–529. Retrieved 2008-02-04. {{cite journal}}: Unknown parameter |coauthors= ignored (|author= suggested) (help)
14. Wolf, Stewart (1947 Nov.). "Sustained Contraction of the Diaphragm, the Mechanism or a Common Type of Dyspnoea and Precordial Pain". The Journal of Clinical Investigation. 26 (6): 1201. {{cite journal}}: |access-date= requires |url= (help); Check date values in: |date= (help); Cite has empty unknown parameter: |coauthors= (help)
15. Wheeler E.O. (1950), Neurocirculatory Asthenia et.al. - A Twenty Year Follow-Up Study of One Hundred and Seventy-Three Patients., Journal of the American Medical Association, 25th March 1950, p.870-889 (Contributors to the study: Edwin O.Wheeler, M.D., Paul Dudley White, M.D., Eleanor W.Reed, and Mandel E.Cohen, M.D.)
16. White, Paul Dudley (1951). Heart Disease. New York, New York: MacMillan. pp. 578–591. {{cite book}}: Cite has empty unknown parameter: |coauthors= (help)
17. Volkov V.S. (1980) Psychosomatic Interrelations and their clinical importance in patients with cardiac type NCD, Soviet Medicine (11) p.9-15 English Abstract (and a translation)
18. Online Mendelian Inheritance in Man (OMIM): Orthostatic Intolerance - 604715

• Anxiety disorders