Aortic Regurgitation Eponymythology

Eponymythology: The myths behind the history

Chronological review on the eponymythology of aortic regurgitation/insufficiency based on the 31 eponymous signs proposed by Ashrafian (2005) and Zacek (2018). We review the original publications for each sign and attempt to rationalise the eponyms in both their historical context and modern day application. To this end we have:

• Removed the early pulse descriptions, later formally described by Corrigan, such as the Al-Razi pulse; Vieussens pulse (1715); and Cumming sign (1822).
• Corrected the dates and references for 9 of the signs
• Combined duplicate signs e.g Dennison/Shelley; and Gerhardt/Sailer signs
• Updated the evidence against the use of signs with no pathognomic significance e.g. Quinke and Mayne’s signs

Corrigan pulse; Corrigan sign

Visible pulse of aortic insufficiency

Sir Dominic John Corrigan (1802 – 1880) described the exaggerated visible pulsations of head and upper extremity arteries, in addition to ‘Bruit de soufflet’ and ‘frémissement’ as the three physical signs of aortic regurgitation in the Edinburgh Medical and Surgical Journal. He also briefly mentioned the ‘full pulse’ of aortic regurgitation in passing.

When a patient affected by the disease is stripped, the arterial trunks of the head, neck, and superior extremities immediately catch the eye by their singular pulsation. At each diastole the subclavian, carotid, temporal, brachial, and in some cases even the palmar arteries, are suddenly thrown from their bed, bounding up under the skin…in the disease now under consideration, the degree to which the vessels are thrown cutis excessive. Though a moment before unmarked, they are at each pulsation thrown out on the surface in the strongest relief. From its singular and striking appearance, the name of visible pulsation is given to this beating of the arteries.

Corrigan Sign. 1832: 226

Watson water-hammer pulse

Palpable pulse of aortic insufficiency

The pulse of aortic regurgitation is, sometimes at least, very striking and peculiar: sudden, like the blow of a hammer, without any prolonged swell of the artery. This pulse always reminds me of the well-known chemical toy, formed by including a small quantity of liquid in a glass tube, exhausted of air, and hermetically sealed. On reversing the tube, the liquid falls from one end of it to the other with a hard short knock, as if it were a mass of lead. The sensation given to the finger by the pulse, when there is much regurgitation through the aortic valves, is very similar to this. It is as if successive balls of blood were suddenly shot along under the finger.

Watson. 1842: 647

In 1933, the eminent cardiologist Sir Thomas Lewis described the quality of the water-hammer pulse-wave of aortic incompetence.

If a globule of mercury is sealed into a glass tube 2 inches long, and this is held by its ends between thumb and little finger and tipped up, it delivers a pulse closely resembling that of free aortic regurgitation. The water-hammer was a similar toy.

Lewis 1933: 121

Duroziez sign

Double intermittent, systolic-diastolic (antegrade-retrograde) murmur produced by compression of femoral artery with a stethoscope as a sign of aortic insufficiency.

The double intermittent crural murmur always accompanies aortic insufficiency ; it reveals it in difficult and complicated cases ; it is its pathognomonic sign. Now this has not been said by any author – I am going to prove it

Paul Louis Duroziez (1826-1897)

The murmur is produced when firm pressure from the bell of the stethoscope is applied to the artery then altering the pressure proximally and distally. The diastolic component often becomes louder with pressure applied distal to the stethoscope. Duroziez sign is an unreliable indicator of severity of aortic regurgitation.

There are 2 ways of producing the double murmur, with the stethoscope or with the hand. [The first method] One gradually presses the instrument as if to obliterate the (femoral) artery; at a certain moment, the double murmurs appears; [The second method] Or else this, which can be done only when the diastolic component is easy to find; one can simply lay the stethoscope on the (femoral) artery; then in sequence, press with the hand, in succession, first upstream (2 cm above) and then downstream (2 cm below) of the stethoscope. The upstream pressure produces the systolic murmur and the downstream pressure produces the diastolic component, clearly proving that the diastolic component is produced by the blood flowing backwards from the artery of the leg.

Duroziez 1861

Austin Flint murmur

A mid-diastolic to late diastolic or presystolic mitral murmur best heard at the apex that is low-pitched and rumbling in the presence of aortic regurgitation with no underlying mitral pathology

In some cases, in which free aortic regurgitation exists, the left ventricle becoming filled before the auricles contract, the mitral curtains are floated out and the valve closed when the mitral current takes place and under the circumstances, this murmur may be produced by current just named, although no mitral lesion exists. A mitral direct murmur, then, may exist without mitral contraction and without any mitral lesions, provided there be aortic lesions involving considerable aortic regurgitation. This murmur by no means accompanies aortic regurgitation lesions as a rule. The circumstances, which may be required to develop, functionally, the latter murmur, in addition to the amount of aortic regurgitation, remain to be ascertained. Probably enlargement of the left ventricle is one condition.

Flint A. 1862: 39

Lincoln sign

Forceful popliteal artery pulsation secondary to aortic regurgitation; exaggerated when the patient sits with legs crossed; and deemed positive if the elevated foot bobs up and down with each systolic contraction.

On Sunday, November 8, 1863 Abraham Lincoln posed for the famous “big foot” photograph, taken by Alexander Gardner. Lincoln noted that the outline of his left boot was blurred. The journalist Noah Brooks suggested that it was the throbbing of the arteries behind Lincoln’s knee that caused the leg to move almost imperceptibly.

One day we were looking at a photograph of the President, taken in a sitting position, with the legs crossed. Lincoln’s attention was attracted to the foot of the leg which was crossed above the other, and [Lincoln] said,

“Now, I can understand why that foot should be so enormous. It’s a big foot, anyway, and it is near the focus of the instrument. But why is the outline of it so indistinct and blurred ? I am confident I did not move it.”

I studied it for a moment, and told him that probably the throbbing of the large arteries in side of the bend of the knee caused an almost imperceptible motion. The President, very much interested in the discovery,’ as he called it, immediately took the position of the figure in the picture, and, narrowly watching his foot, exclaimed,

” That’s it ! that’s it! Now, that’s very curious, isn’t it.”

Noah Brooks 1878

Traube sign; Traube double tone

Ludwig Traube (1818-1876) described two distinct sounds heard over the femoral artery in a 28 year old Prussian veteran of the Schleswig-Holstein military campaign, suffering from aortic insufficiency associated with rheumatic heart disease. Traube described the Doppelton (double tone) phenomenon when applying the stethoscope lightly over the femoral artery, so that the diameter of the vessel was not modified by compression.

Traube sign is the first (systolic) sound caused by the rapid distension of the artery and likened to that of a pistol-shot. The Traube ‘double-tone’ is the second sound associated with the rapid change of tension of the arterial wall after the quick distension.

Traube pulse

Traube pulse (pulsus bigeminus) Traube coined the term ‘pulsus bigeminus’ in his classic paper presented to the Berlin Medical Society in 1871. Pulsus bigeminus is not pulsus bisferiens, and although this phenomenon is associated with heart disease (e.g. hypertrophic cardiomyopathy), it is not pathognomic of aortic insufficiency

…following every two pulses which originate in the aorta, a longer pause ensues. This phenomenon is differentiated from the pulsus dicroticus by the fact that in the latter there is only one contraction of the heart for every two beats of the pulse, while in pulsus bigeminus there are two contractions of the heart, which follow one another rapidly and are separated from the preceding and succeeding contractions by a longer pause.

Traube 1872

Pulsus bisferiens: defined as a wave pattern with two separate systolic peaks for each heart contraction and does have an association with aortic insufficiency (among other cardiac conditions…)

Debate concerning the nature and significance of pulsus bisferiens dates from the first century A.D. as recorded in Galen’s De Pulsibus (Latin edition, 1532). Broadbent was amongst the first to relate the pulse to aortic valve disease as visualised in pressure tracings of the the ascending aorta and carotid artery [Broadbent 1899]. The bisferiens pulse in severe aortic regurgitation has been attributed to a Venturi effect that occurs in the ascending aorta in mid-systole due to the high flow produced by ventricular ejection [Fleming 1957]

Quincke’s pulse

An alternate paling and flushing of the skin at the root of the nail while pressure is applied to the tip of the nail in aortic regurgitation.

Heinrich Irenaeus Quincke (1842 – 1922) published his observation of capillary and venous pulsations aged 26 years as an assistant in the Medical Clinic in Berlin. However, as Quincke pointed out, the sign of capillary pulsation is simply a normally observable phenomenon present in most people which just happens to be especially clear in patients with aortic insufficiency…so not really very useful at all

As far as the capillary pulse is concerned, so can one see it best on his own finger nail, or better, on that of another, in the area between the whitish, blood-poor area and the red injected part of the capillary system of the nail-bed; in the majority of persons examined, there is, with each heart-beat, a forward and backward movement of the margin between the red and white part…

A large and rapidly falling pulse is seen especially in aortic insufficiency, and for this reason the capillary pulse is especially clear in this condition.

Quincke 1868

Becker sign

Prominent (spontaneous) pulsation of the retinal arteries.

Otto Heinrich Enoch Becker (1828-1890) originally described this phenomenon in association with aortic insufficiency in 1871. Becker then proceeded to evaluate and document the phenomenon in 21 patients published in 1872. He turned his attention to the correlation of spontaneous retinal pulsation associated with exophthalmic goitre in 1873, and this association is the one most oft quoted .

…in allen Fällen von nicht mit anderen Klappen fehlern complicirter Insuffieienz der Aortenklappen die spontane Arterienpulsation beobachtet wird, und zwar um so deutlicher, je stärker gleichzeitig die Hypertrophie des linken Ventrikels entwickelt ist.

In all cases of incompetent insufficiency of the aortic valves, which are not accompanied by other valves, the spontaneous arterial pulsation is observed. The more pronounced the pulsations, the greater the degree of assoicated left ventricle hypertrophy. [Becker 1871]

Note: Becker attributes the original finding to Quincke who had previously described in relation to aortic regurgitation in 1868.

Lately I had an opportunity of observing the capillary pulse in still another place besides the finger nails, namely in the retina, and it was in one of the two cases of aortic insufficiency already mentioned, in a man who is still under my observation

Quincke 1868

de Musset sign

Rhythmic bobbing or nodding of the head synchronous with each heart beat

As a young man, Alfred de Musset contracted syphilis, which led to aortic aneurysm and aortic insufficiency. He died in a sleep in 1857 at the age of 47, as a result of heart failure secondary to alcoholism, long-standing aortic regurgitation and tertiary syphilis. Alfred’s brother Paul Edme de Musset published his biography in 1877. In 1900, Armand Delpeuch reviewed the biography and proposed the eponym.

Un matin du mois de mars, pendant le déjeuner, je m’aperçus que mon frère, à chaque battement du pouls, éprouvait un petit hochement de tête involontaire…nous venions de remarquer le premier symptôme d’une affection grave , à laquelle il devait succomber quinze ans plus tard. C’était une altération des valvules de l’aorte.

At breakfast one morning in March, I noticed that my brother’s head was bobbing involuntarily with every pulse beat…we had just observed the first symptom of a grave malady, to which he was to succumb to 15 years later. It was an malformation of the valves of the aorta.

Rosenbach sign

Pulsatile liver with aortic regurgitation

ln jüngster Zeit habe ich wiederum einen Fall von insufficienz der Aortenklappen allerhöchsten Grades beobachtet, in welchem exquisite systolische Leberpulsation bestand, ohne dass Zeichen einer Insufficienz der Tricuspidalis vorhanden.

Recently I have again observed a case of incompetence of the aortic valves of the highest degree, in which exquisite systolic liver pulsation existed, without signs of tricuspid insufficiency.

Gerhardt sign

Pulsatile spleen (usually in the presence of splenomegaly).

The pulsating spleen was first described by Nicholaes Tulpii in 1641 as ‘lien verberans’ , without a defined cause. Carl Gerhardt (1833–1902) presented three cases of patients with aortic insufficiency and concomitant fever. Gerhardt regarded the pulsatile spleen as a characteristic sign caused by the abnormal blood-pressure conditions of aortic insufficiency with the relaxation of the blood vessels produced by the fever.

Entsteht bei Kranken mit Insufficienz der Aortenklappen ein tastbarer Milztumor, so kann man pulsatorische Bewegungen daran wahrnehmen.

If a palpable splenomegaly develops in patients with insufficiency of the aortic valves, it is possible to perceive pulsatile movements

Also known as Sailer sign folowing Joseph Sailer’s 1928 review of the pulsatile spleen related to aortic insufficiency

Bozzolo sign

Visible pulsation of the arteries within the nasal mucosa.

Camillo Bozzolo (1845 – 1920) initially described nasal mucosal pulsations with thoracic aortic aneurysm and later associated with aortic regurgitation

Müller sign

Pulsatile uvula, soft palate and tonsils with associated redness and swelling

Friedrich von Müller (1858 – 1941) treated a 22 year old female (Luise B) who suffered from aortic incompetence and recorded:

…a rhythmic movement of the palate was observed…the reddened tonsils, as well as the anterior and posterior palatal arches, moved somewhat median with each pulse and the uvula was moved downward.

A further six cases of aortic insufficiency were observed with attention paid to possible pulsation of the palate…this phenomenon was noted in three of them…In two of the cases the diagnosis of aortic insufficiency was confirmed at autopsy

Müller surmised that the pulsation of the palate in the case of aortic incompetence was caused by a similar mechanism to the previously observed capillary pulse in the nail bed (Quinke’s pulse, 1868); facial flushing (Morton-Mahon sign, 2002); and inflammatory processes of the skin such as erysipelas or urticaria (Penny sign, 1988). He concluded:

Pulsation of the soft palate is a common symptom of aortic incompetence, as is evident from the fact that the same was found four times among seven subsequently examined cases.

Müller, 1889

Drummond sign

Audible, pulsatile systolic expulsion of air from the trachea; original defined with relation to intrathoracic aortic aneurysm.

Tracheal whiff may be heard either in the trachea or in the mouth; it is usually systolic, but may be double, and is loudest when the patient is expiring slowly after a deep inspiration with the mouth wide open.

Drummond 1908

This had been previously described by Dr Arthur Ernest Sansom in 1896:

A systolic murmur, too feeble to be easily recognised in the ordinary manner, may be rendered distinctly audible by listening with a binaural stethoscope, the chest piece of which is placed in the patient’s mouth with his lips closed over it. On auscultating thus, the observer may hear a distinct or loud systolic murmur in the case of a thoracic aneurysm, the vibrations being communicated to the trachea, and thence directly by the air-column to the ears.

Sansom 1896

Landolfi sign

Pulsatile pupils: systolic contraction and diastolic dilation of the pupil

Michele Landolfi (1878 – 1959) examined twenty-four patients with grave aortic insufficiency, and found in one circulatory hippus. The patient suffered from arteriosclerosis with aortic insufficiency and hypertrophy of the left ventricle. A rapid alternate contraction and dilatation of pupil was observed.

Landolfi was able to produce this sign in three other patients after giving fifteen drops of tincture of digitalis a day for four days; and observed the circulatory hippus in a dog with experimental aortic insufficiency after the administration of heart stimulants. Believed to be an exaggeration of the physiologic circulatory hippus in the iridal vessels due to high pulse pressure and large stroke volume.

Hill’s sign

Increased systolic arterial pressure in lower (popliteal) compared to upper (brachial) limbs in cases of aortic insufficiency.

Sir Leonard Erskine Hill (1866 – 1952) described his sign in 10 cases of aortic insufficiency (1909), and later confirmed the findings (1912) with an in depth explanation of the systolic popliteal-brachial gradient in aortic regurgitation.

A difference between arm and leg systolic readings is most marked in all cases of aortic regurgitation, and when such patients are lying quiet in bed this difference is a diagnostic sign of aortic regurgitation.

…we found that in all cases of aortic regurgitation there is this marked difference, which is not abolished by the method of oscillating the pressure up and down near the obliteration pressure. So marked is this difference that we believe we could pick out cases of aortic regurgitation by it alone

Hill 1909

Blumgart-Ernstene murmur

Duroziez-type murmur observed with the patients arm subjected to various temperatures of water and by applying a subdiastolic pressure below the auscultation site, to help differentiate between aortic insufficiency and peripheral vasodilatation.

In patients with peripheral vasodilatation (thyrotoxicosis, anemia, fever, normal subjects with increased local blood flow due to immersion of the limb in hot water), the diastolic arterial murmur is due to an increased forward flow of blood toward the periphery during diastole. The diastolic murmur is increased by pressure with the upper edge of the stethoscope, while the murmur is abolished by immersion of the limb in cold water or by application of subdiastolic pressure distal to the site of auscultation.

In patients with aortic regurgitation, the diastolic arterial murmur elicited by pressure on the artery with the stethoscope is due to a backward flow of blood during diastole toward the heart. The diastolic murmur may be strikingly accentuated by pressing predominantly with the lower edge of the stethoscope bell, by immersing the arm in cold water, or by applying a cuff inflated to subdiastolic pressure to the limb below the site of auscultation.

Cole-Cecil murmur

Early diastolic murmur of aortic insufficiency radiating with axillary radiation.

Rufus Cole (1872-1966) and Arthur Bond Cecil (1885-1967) examined 17 patients with provisional diagnosis of aortic insufficiency and mapped the site of maximal intensity and axillary radiation of the early diastolic murmur.

Having our attention drawn to the localization of an aortic diastolic murmur in the axilla we began to pay special attention to the occurrence of this murmur, and decided to make an accurate study of the distribution of the diastolic murmur in a number of cases of aortic insufficiency.

We foresee that the chief objection that will be raised to our description of the axillary aortic diastolic murmur will be that we have been listening to the diastolic mitral murmur (either a true stenotic murmur or a Flint murmur) which is transmitted into the axilla. We feel convinced, however, that such objections are not valid. The fact that the murmur described has been of exactly the same kind and quality as that heard at the base makes it seem almost certain that both have an identical origin.

Cole and Cecil 1908

Note: Original paper from 1908 and not 1936; and the cause of aortic insufficiency in the 17 cases included rheumatic fever, alcoholism, and syphillis and thoracic aortic aneurysm and not simply syphilitic aortitis as widely quoted

Mayne’s sign

Decrease in diastolic blood pressure (>15mmHg) on raising the upper extremity, as a sign of severe chronic aortic regurgitation

This physical sign, which is extremely simple in its concept, merely consists in taking the blood pressure with the arm elevated…In normal health, the diastolic pressure taken in this position is usually found to be 10 mmHg. below that found with the arm resting horizontally; but in aortic regurgitation, a much larger drop is commonly found…It is suggested that if the diastolic pressure, as measured with the arm elevated vertically, is more than 15 mmHg. lower than that measured with the arm in the conventional position, aortic regurgitation is probably present

Mayne 1953

However, this finding was proposed prior to the widespread use of Doppler echocardiography to detect the presence and severity of valvular heart disease. Happes et al evaluated the relationship between changes in DBP (ΔDBP) induced by arm raising and the degree of AR assessed by echocardiography. They conclude:

ΔDBP and Mayne’s sign unrelated to the presence or severity of AR…Greater decreases in DBP upon arm raising appear to be related to younger age but not to the presence or severity of AR. Therefore, Mayne’s sign should not be considered a reliable finding of regurgitant aortic valve flow.

Happes 2010

• Mayne B. On aortic regurgitation: a new physical sign. Irish Journal of Medical Science 1953; 28(2): 80–81.
• Abbas F, Sapira JD. Mayne’s sign is not pathognomonic of aortic insufficiency. South Med J. 1987 Aug;80(8):1051-2.
• Happes M et al. Mayne’s sign is mainly related to younger age and not to aortic regurgitation. Blood Pressure Monitoring. 2010; 15(6): 305-307

Shelley sign

Pulsatile cervix.

Most commonly described as either Dennison sign (1959) or Shelley sign (1964). However it was Dennison who published the sign and attributed the original description to his resident, Dr. Richard Shelley in 1959.

We have hoped to make a young aspirant in the field of obstetrics and gynecology quite famous by placing his name in the historical stream of eponyms. Dr. Richard Shelley, our outgoing resident in obstetrics and gynecology, was surprised and perplexed to note a vigorously pulsating cervix as he peered at this organ through the open speculum. He summoned the resident in medicine for consultation, demonstrated the pulsating cervix, and after the astute resident in medicine had listened to the precordium and noted free aortic insufficiency, it became quite apparent that the vigorous pulsation was isochronous with the heart beat. Such comments may not bring fame and fortune to this young man, but the ensuing jocularity has made the game of medicine more fun.

• Dennison AD Jr. Aortic regurgitation; multiple eponyms, physical signs and etiologies. Journal of the Indiana State Medical Association. 1959; 52: 1283-9.

Morton-Mahon sign

Facial flushing and blanching corresponding to head and neck arterial pulsations.

Morton and Mahon reported the case of a 79 year-old patient with severe chronic aortic regurgitation presenting for elective aortic valve replacement:

…we noted that with every systolic heartbeat his face would flush red and then immediately blanch. This facial flushing or
flashing was continuous and correlated to the upstroke of the arterial waveform and pulse oximeter; it would also blanch with the down stroke of the arterial waveform.

• Morton G, Mahon S. The ‘facial flashing’ of aortic regurgitation. Anaesthesia. 2002 May;57(5):501-2.

Penny sign

William J. Penny reported the case of a 55 year-old patient with severe chronic aortic regurgitation whi developed an urticalrial rash on her face and trunk after an aortogram.

A well-circumscribed wheal developed on her right cheek, with a blanched center and surrounding ring of erythema (“target” lesion). Much to the fascination of the staff, this lesion visibly pulsated. Extreme dilatation of dermal capillaries occurs in a wheal, providing the ideal setting for visible capillary pulsation in the presence of aortic regurgitation. We lay claim to be the first to have witnessed and described the sign of the “flashing wheal”

Penny 1988

Capillary pulsation may also be visualized at other sites and it has been termed the “lighthouse” sign when it emanates from the forehead

• William J Penny, American cardiologist
• Penny WJ. The sign of the flashing wheal in aortic regurgitation. Am J Cardiol. 1988 May 1;61(13):1158-9.

Sherman sign

Prominent dorsalis pedis artery pulsations in elderly patients with chronic aortic regurgitation.

The easily or quickly palpable dorsalis pedis pulse occurs in older adults who have chronic aortic regurgitation. Any adult age > 75, whose dorsalis pedis pulse is quickly located and is unexpectedly prominent, has chronic aortic regurgitation or insufficiency until proven otherwise. The key descriptors are: 1) “quickly” located, ie, appreciated within 1 to 2 seconds of palpating the dorsum of the foot; and 2) “unexpectedly” prominent with a rapid arterial upstroke.

Sherman 2004

…but, as with many things in medicine…“What has been will be again, what has been done will be done again…”

…it is said that Oppolzer won his professorship at Vienna by casually making a diagnosis of aortic insufficiency while walking down the wards of the hospital and merely resting his hand upon the dorsum of the patient’s foot.

Hirschfelder 1918

Ashrafian pseudo-proptosis

Pulsating pseudo-proptosis secondary to an increased ocular pulse amplitude noted in aortic regurgitation.

Facial examination and palpation revealed subtle rhythmical anterior propulsion of both eyes that followed the pattern of systole, which did not fulfil the criteria for proptosis when applying a Hertel exophthalmometer. We termed this as pulsatile pseudo-proptosis. Visual acuity and field were normal, but on closer examination, the retinal arterioles and pupils were pulsatile.

• Ashrafian H. Pulsatile pseudo-proptosis, aortic regurgitation and 31 eponyms. Int J Cardiol. 2006 Mar 8;107(3):421-3.

References

• Sapira JD. Quincke, de Musset, Duroziez, and Hill: some aortic regurgitations. South Med J. 1981;74(4):459–67.
• Mehta NJ, Mehta RN, Khan IA. Austin Flint: clinician, teacher and visionary. Tex Heart Inst J 2000;27:386–9.
• Babu AN, Kymes SM, Carpenter Fryer SM. Eponyms and the  diagnosis of aortic regurgitation: what says the evidence? Ann Intern Med 2003;138:736– 42.
• Mehta NJ, Khan IA. Original descriptions of the classic signs of aortic valve insufficiency. J Emerg Med. 2003;24(1):69–72.
• Cheng TO. Twelve eponymous signs of aortic regurgitation, one of which was named after a patient instead of a physician. Am J Cardiol. 2004;93(10):1332–3
• Ashrafian H. Pulsatile pseudo-proptosis, aortic regurgitation and 31 eponyms. Int J Cardiol. 2006;107(3):421–3.