Pulsus Paradoxus: Original Description by Kussmaul in the Year 1873.

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Translated excerpts from Berliner Klinische Wochenschrift published in 1873.

Prof. Dr. Adolf Kussmaul

There is a variety of chronic pericarditis well characterized both anatomically and clinically that one may call adhesive mediastino-pericarditis.

It is characterized anatomically by the unique localization of the inflammatory process and the character of its product. The inflammation affects not only the serosal layer but also the fibrous tissue of the pericardium (fibro-pericarditis of Gendrin) together with the mediastinal connective tissue, which extends from the reflection of the pericardium at the root of the great vessels upward to the arch of the aorta (mediastinitis). The products of the inflammation for this type of disease are fibrous structures, adhesive layers, dense cords and threads of connective tissue, together with fibrin and, more rarely, pus in various stages of desiccation and caseation, sometimes only in the pericardial sac or in the mediastinum. The pericardium becomes thickened, owing partly to the adhesive membranes and fibrous plaques which are sometimes on its inner or outer surface, and partly to the thickening of its fibrous sheet alone; and there exists an extraordinary tendency to obliteration of its cavity (adhesive or fibrous pericarditis). In the mediastinum, adhesive bands and thick fibrous masses develop, which decrease in size as they leave the pericardium and run upwards toward the aortic arch and the innominate veins. These bands strangle, narrow, pinch, and twist the roots of the great vessels, and the arch of the aorta, and especially draw them down against the pericardium and solder the roots of the great vessels directly to the sternum (adhesive and fibrous mediastinitis). One must distinguish between the adhesive inflammation of the mediastinum and the purulent, from which retrosternal abscesses arise; these much earlier and much oftener fasten the attention of physicians and surgeons than retrosternal adhesions.

Clinically, our attention is drawn to the symptoms of chronic inflammation of the pericardium and its obliteration, to which, as a diagnostic criterion of adhesive mediastinitis, there exists a unique phenomenon of the arterial pulse and a unique phenomenon of the veins of the neck. Since the fibrous and mediastinal adhesions to the sternum exert a restrictive pull on the aorta below and at the arch, the pulse of all the arteries becomes very small or disappears completely during inspiration, despite the regularly repeated movements of the heart; during expiration, the pulse returns immediately. I suggest naming this pulse paradoxical, partly because of the conspicuous discrepancy between the cardiac action and the arterial pulse, partly because the pulse, despite its apparent irregularity, actually waxes and wanes in a regular fashion. The restricting pull of the adhesive bands at the apex of each deep inspiration creates a noteworthy and remarkable dilatation rather than collapse of the cervical veins, especially the jugular bulb. These phenomena make possible a diagnosis of adhesive mediastinitis plus pericarditis with a more reasonable degree of certainty in cases where the history, the onset and course of the disease, the physical examination of the heart, and the disturbance of the circulation, to be sure, point toward pericarditis and compression of the heart, but, however, do not allow the diagnosis to be definite.

According to my knowledge, up to now only one case of this interesting disorder has been published; namely, Griesinger in 1854, observed and A. Widemann in 1856, published under the title: "Contribution to the Diagnosis of Mediastinitis" in a Tubingen Inaugural Dissertation.

At the Tubingen inpatient department, the incongruence of the small irregular pulse with the regular heart rhythm in a 22 year old man with many symptoms pointing toward pericarditis became conspicuous to a great degree. An imperfect graph of these pulses was made by Vierordt with his sphygmograph and is included in his well known work The Study of the Arterial Pulse (Baunschweig, 1885). Griesinger soon recognized that this disappearance of the pulse does not occur at indeterminate intervals but rather only during inspiration.

During the entire duration of inspiration, the pulse would become uncommonly weak or would disappear entirely and would return once again when the thorax would begin to sink in expiration. The heart continues its function in rhythmic fashion, and its tones are as good and clear during inspiration as in expiration, except generally weaker. This dependence of the pulse upon respiration is found, as often as one looks for it, in all the arteries. The necropsy gave proof of the reason for the remarkable anomaly of the pulse. There was found along with "a purulo-fibrous pericarditis" a "fibrous mediastinitis." Dense cordlike exudates were in all connective-tissue sites near the great vessels of the thorax where they exit out of the pericardium, without spreading into the posterior mediastinum. The superior vena cava, the left innominate vein, and the azygous were considerably narrowed; the aorta in the ascending portion and in the arch was not only significantly narrowed but also kinked and partially twisted on its axis. When the thorax expanded with every inspiration accompanied by dyspnea, the sternum was raised anteriorly as much as possible. Similarly, through the medium of the exudate, the root of the aorta, which was adherent to the sternum, would move anteriorly as did also the stretched and shredded cords that went from the aorta to the left mediastinal border; and in this way with every inspiration the already narrowed aorta was significantly coarcted at the place of kinking, here and there perhaps completely pinched off. While the heart continued its contractions, much blood could no longer enter all the arteries of the body to produce a palpable pulse by the expansion of the arteries.

I was reminded of this observation of Griesinger's, when, during the course of this summer, two similar eases, one soon after the other, came under my care. Because of his observation, I made the diagnosis of adhesive mediastino-pericarditis both times, verified by necropsy. I have now altered my position and I am taking up Marey's sphygmographic pulse registration in order to follow the subject more exactly. Nevertheless, these three cases that I report in their essential aspects, I believe, demonstrate the special variety of pericarditis under the title that I have chosen.

Observation 1 (Griesinger, 1851):

A 22 year old wheelwright's apprentice had frequently pressed his sternum against a heavy drill for many years... The illness began in June 1854 with sticking pains in the chest, hard cough, hemoptysis, and fever, followed in 3 weeks by ascites and then edema of the feet. He was bled twice and cupped... He was pale, slightly cyanotic, with anasarca, including swelling of the scrotum ... The pulse was small, tremulous, rapid and disappearing... The incongruity of the arterial pulse and the cardiac rhythm was easily recognized. The heart tones were pure but weak; P2 was not accentuated. The neck veins were not especially distended... The disappearance of the pulse occurred during each inspiration...

Autopsy: The sternum is plastered to the pericardium by tough adhesions and exudate. The entire space between both sheets of the anterior mediastinum is full of dense... tissue that stretches from the diaphragm to the manubrium... The borders of the pericardium and the root of the aorta are not recognizable ... The wall of the aorta and its branches are thickened by the newly formed connective tissue which envelops it completely but nonetheless narrows its lumen. The lumen of the arch would admit only the little finger... The root of the superior vena cava is, to be sure, not so completely imbedded in the pseudomembranous exudate. However, it is also narrowed ... The site of origin of the internal jugular and subclavian veins cannot be found because of the very thickened and densely interwoven connective tissue.
The pericardium is covered on its outer surface with many layers of pseudomembranous plaques and has become so rigid that it can no longer be indented. It is for the most part adherent to the heart...; over the right ventricle is 6 oz. yellow-green pus.... The heart is flabby, its flesh pale, friable. The cavity of the right ventricle is considerably constricted. The left hemithorax is almost completely filled with serofibrinous effusion... The right pleural cavity contains one and a half to two lb. of exudate.
Ascites. Nutmeg liver of normal size. Both kidneys are enlarged with pale, dense cortex and smooth surface.

Observation 2 (Kussmaul, 1873).

A strong farmer, 21 years old, of healthy parents, became ill after a christening feast at the end of October 1872. He had fever, sweats, anorexia, cough productive of watery and slimy sputum at times streaked with blood...

The pulse rate was always rapid, never under 100, usually 100 - 120. The pulse at first was small and disappeared completely during inspiration and returned in expiration. After every second and sometimes after the third beat, the pulse completely disappeared. This could be demonstrated also at the carotid arteries as well as the radials: palpation of the femoral arteries was prevented by the edema. Respirations were between 27 and 30 and on exertion rose to and over 40 per minute. The apex beat was not palpable. The heart tones were pure, although distant. Neither murmurs nor accentuation of P2 could be heard... The neck veins, especially the right internal jugular, were very distended.

The initial diagnosis was chronic adhesive pericarditis and mediastinitis, upon remembering the observation of Griesinger... Anasarca and dyspnea ere progressive... The distended neck veins became even more distended with each inspiration, and with each inspiration one to two weak retrograde waves were visible...

Autopsy: Generalized edema... and ascites right and left hydrothorax... The pericardium is unattached in the area of the conus and the bordering left ventricle;... it is adherent to the right lung and to the left lung... and the chest-wall... (There are no abnormal adhesions between the sternum and the mediastinal organs as there was in Griesinger's ease). The pericardium is thickened... in places as much as 5 mm. and the pericardial space completely obliterated by adhesions. The heart is as large as a man's fist, it is flesh pale, red-brown, its cavities not narrowed, and the valves intact. The liver is very large, nutmeg, with innumerable miliary tubercles on the serosa.

Observation 3 (Kussmaul, 1873):

A 34 year old unemployed servant girl, whose mother had died of pulmonary disease, had suffered for many years each winter from a dry cough and for three years a constricting feeling which at times became dyspnea. At one time.., she coughed up blood. On admission, she appeared cachectic, with edema of the legs and ascites. The phenomenon of the arterial pulse with regular and constant action of the heart was observed at the initial examination. The pulse would become smaller with inspiration or would become totally impalpable on deep inspiration. On expiration it returned to its former amplitude. The amplitude was always diminished and the tension small; the rate was between 104-120. The disappearance or diminution of the pulse during inspiration was manifest in all palpable arteries... The infraclavicular fossa was retracted, and the supraclavicular fossa was filled by the bulb of the internal jugular vein; even the external jugular veins were very distended, with insufficient valves... The heart tones were clear but faint; the second pulmonary tone was loudest...

The diagnosis was chronic bronchitis, adhesive mediastinitis and chronic pericarditis...

Autopsy: Enormous anasarca... and ascites and bilateral pleural effusions. The pericardium is except for one small area anteriorly, completely covered With thick exudate and adhesions. It is adherent to the lungs, thoracic wall, diaphragm, vertebrae... manubrium and sternum. The pericardium is so thick (up to 1 cm.) that it cannot be folded over. The heart appears enlarged...; the valves are normal... There is a cavity in the left lung the size of a hen's egg. On the distal small bowel, there are many miliary tubercles.

The interesting phenomenon which these three cases of mediastinopericarditis present is the pulse. In all three, it was rapid, almost always more than 100, the impulse of low amplitude, the tension soft, and the rhythm paradoxical in two ways: (1) despite continuing action of the heart, the pulse disappeared for short intervals at the palpating finger, one or two heats completely or almost completely and then returned immediately for two or more beats; (2) the apparent irregularity was in actuality only the difference associated with the phases of respiration. The disappearance or diminution of the pulse returned with great regularity with each inspiration; and with each expiration, contrariwise, the pulse assumed its former amplitude. This unique phenomenon was manifest in all palpable arteries.

In Case 2, we were not able to obtain a graph of the pulse when there was significant pericardial effusion. However, once the paracentesis was done, we obtained some useful traces with Marey's sphygmograph. In Case 3, the graph of the pulse was also difficult to obtain and is not as satisfactory as the trace of Case 2.

Figures 1 and 2 are from the second case, and Figure 3 is from the third case. From these graphs it is seen that the pulse in actuality never completely disappeared. Even when it was impalpable, it was still present, albeit in a damped form. Griesinger believed that in his cases, the waning of the pulse during inspiration was owing to adhesions between the aortic root and the sternum.

The essential anatomic condition for the development of the paradoxical pulse appears to be the adherence between the pericardium and the sternum, and the adhesions of the pericardium to the aorta below and at the arch in the form of constricting hands, in the well known triangular area where normally in the adult there is a reflection of the pericardium from the sternum. What is not absolutely clear is the necessity of direct adhesion between the root of the great vessels and the sternum lying anterior to them, which we found in all 3 cases. Whether in my 3 cases the fact that more or less complete obliteration of the pericardial space plays a part must be clarified in future studies: it probably does not.

In Case 2, another phenomenon was observed in the neck veins that one might also attribute to adhesive mediastinitis. Its probable explanation is that on inspiration the same narrowing that tugs on the aorta also manifests itself on the inferior vena cava or even the right innominate vein. With every inspiration, the fully distended jugular veins swelled very markedly on the right side and less conspicuously on the left. At the same time, one could see one or two weak retrograde waves with each inspiration in a small subcutaneous vein that ran in the direction of the omohyoid muscle. It was not possible to register this phenomenon on a pulse tracing...

As I am writing these pages, I have had the opportunity to observe a new case, the fourth one, of paradoxical pulse that is doubtless due to mediastino-pericarditis. I admit that if this case is lethal, we will not be able to confirm the diagnosis by autopsy because the patient is Jewish and the Israelites in this country do not permit autopsies on their relatives. In this case the inspiratory distension of the neck veins is especially well seen.

Observation 4:

A 35 year old tradesman of healthy family and muscular build was never previously ill. The cause of his present illness is not known, although he had often been exposed to inclement weather. He developed a left-sided goiter the size of a hens egg, which descended under the sternum...,

I examined him in consultation, finding a cyanotic, orthopneic. suffering man with slight edema of the face, enormous protuberance of the neck, marked edema of the hands and forearms, edema of the skin of the buttocks, marked swelling of the scrotum and lower extremities, massive ascites, large left hydrothorax. Precordium somewhat prominent, cardiac dullness rather widened, apex beat barely palpable (at the left nipple), heart tones weak and distant. second pulmonary sound rather loud... With inspiration, the pulse at the wrists, the carotids. and the temples disappeared despite the regular contractions of the heart (over 100 beats per minute). Both internal jugular veins and the subcutaneous veins of the neck were enormously dilated, but neither undulating nor pulsating; the jugular bulb swelled with each inspiration and collapsed with each expiration. Urine light yellow; no albumin.

Except for Griesinger, it appears that up to now the paradoxical pulse was observed only by Thos. Fox. Unfortunately, I find in my reference only the following short communication (Schmidt's Yearbook, 152: 200; the original report is in the St. Louis Journal, N.S., 6: 217, 1869):

"In a phthisical subject, whose right lung was affected, there was found the disappearance of the pulse every sixth or seventh beat, without the examination revealing the cause. The heart was completely normal and pulsated without intermission. At autopsy, there was found in addition to the phthisical changes, behind the ascending aorta a swelling that originated from the dilated right main bronchus. The dilatation of the bronchus was so great that a hand could almost be slipped into it, whereas the left bronchus would accept only the finger. A controlled experiment demonstrated that, by closing the mouth and one nostril and blowing air into the other, this bronchus became so inflated that the aorta was significantly compressed."

The reporter (Neubert) noted justifiably that it is difficult to explain how even with a powerful inspiration such a degree of air pressure could be achieved at the area of dilatation to exceed the blood pressure in the aorta; it would be necessary in such a situation to have some type of valve that would prevent the back- flow of air out of the bronchus. With that, however, the respiration would have completely stopped on the right side, but respiratory sounds of the right lung are specifically men- tioned by the author...

At any rate, we learn from this observation that the paradoxical pulse may also occur without pericarditis. This sign is consistently a result of mediastinal disease, and we may only assume that it is mediastino-pericarditis when the history, onset, and course of the disease present as additional criteria for the diagnosis of pericarditis.

In conclusion, I must point out my conviction that adhesive mediastino-pericarditis is not such a great rarity as perhaps appears, and that it has more significance than just an interesting curiosity. I have been greatly impressed by finding three cases of this disease in the past 6 months, in two of which the correctness of the diagnosis could be confirmed at autopsy. I do not doubt that I have already come across similar cases of the paradoxical pulse at whose autopsies the fibrous cords of the mediastinum were overlooked. The paradox of the pulse may be easily missed, if as above, in the later stage of the disease the pulse is very small and rapid and the artery is more difficult to palpate because of edema, and if the heart beat is hardly palpable, and the heart sounds very weak. So it would have happened to me, probably, in Cases 3 and 4, for example, if I had not previously observed Case 2 in which these phenomena were more conspicuous. Also in this case, I would not have recognized its significance had I not by accident remembered Widemann's dissertation which, although very stimulating, did not receive much attention. However, in our best and newest articles about the arterial pulse and physical diagnosis, this peculiarity of the pulse, first noted by Griesinger, is not even considered. The mediastinal adhesions were not easily overlooked when they appeared in such great abundance, as in Griesinger's case. However, it is very possible in cases such as the category to which mine belongs, there is present only a thickened pericardium that continued upward in the mediastinum as fibrous cords and thickened strands of connective tissue, whose presence could be established only by very careful dissection.

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