Brit. J. vener. Dis. (1976) 52, 366

Syphilitic aortic regurgitationAn appraisal of surgical treatment

WOLF GRABAU, RICHARD EMANUEL, DONALD ROSS, JOHN PARKER,AND M. HEGDEFrom the National Heart Hospital and Cardiothoracic Institute, London

SummaryDuring the 10 years from 1964 to 1973, fifteenpatients with severe syphilitic aortic regurgitationwere treated surgically at the National HeartHospital. In thirteen the valve was replaced and intwo it was repaired. In addition four had replace-ment of an aneurysmal ascending aorta with aDacron graft and seven some form of plastic repairto the coronary ostia. Three patients died within1 month of surgery and a further six during thefollow-up period which varied from 1 to 55 months(mean 25.5). The six survivors have been followed-up for an average of 33 months.

Factors contributing to this high mortality wereanalysed and it wasffound that the mean duration ofeffort dyspnoea was 22 months in the survivorscompared with 48 months in those who had died.Similarly the average duration ofnocturnal dyspnoeawas 4 months in the survivors compared with amean of 8 months in those who had died. Only sixout of the fifteen patients had angina; this waspresent in two of the survivors and in four of thefatalities. The pulse pressure, heart size, andhaemodynamic findings were similar in the twogroups. The prognostic value of an elevatederythocyte sedimentation rate was also examined.

It was concluded that preoperative investigationsshould include aortography, coronary arteriography,an assessment of left ventricular fimction, andwhenever possible myocardial biopsy.These data were interpreted as suggesting that

patients should be referred for surgery at an earlierstage in the disease-certainly before the onset ofcardiac failure-and that if this more aggressiveattitude was adopted, as it has been in non-syphiliticcases of aortic valve disease, the present highmortality in this group would be reduced.

Received for publication May 14, 1976Address for reprints: Dr R. Emanuel, Cardiothoracic Institute,2 Beaumont Street, London WlN 2DX

IntroductionCampbell and Shackle (1932) reported that 19 percent. of all cases of aortic valve disease were due tosyphilis. Since then there has been a dramatic reduc-tion in the incidence of cardiovascular syphilis dueboth to public health measures and to the introductionof penicillin therapy (Koulumies and Heinivaara,1957). The natural history of cardiovascular syphilishas also changed; the disease has become more benignand cardiac complications less common, and theprognosis, even in untreated cases, is now relativelygood. Heart failure due to syphilis used to be seenbetween the ages of 30 and 50 years. Now thiscomplication is uncommon and tends to occur adecade later (Prewitt, 1970).A proportion of syphilitic patients still develop

heart disease, which includes syphilitic aortitis, withor without aneurysms of the ascending aorta, aorticregurgitation, and coronary ostial stenosis. The ad-vent of open heart surgery has increased the availabletreatment, particularly for those with aortic regurgita-tion with or without coronary ostial stenosis. In spiteof this surprisingly few cases of aortic regurgitationdue to syphilis have been treated surgically (Michaud,Termet, Chassignolle, Dalloz, Age, Delaye, du Gres,and Champsaur, 1970; Samaan, 1973).

In this report we review fifteen cases from theNational Heart Hospital in which aortic valve re-placement (13) or repair (2) was undertaken forsevere syphilitic aortic regurgitation.

MaterialBetween the beginning of 1964 and the end of 1973 a totalof 850 patients had an aortic valve repair replacement atthe National Heart Hospital. Fifteen (1 -8 per cent.) ofthese had syphilitic aortic regurgitation. Seven were maleand eight female. The age at operation varied from 33 to60 years (mean 52). The postoperative follow-up periodranged from 1 to 55 months (mean 25 5). All had clinicalevidence of severe aortic regurgitation.A syphilitic aetiology was accepted on the following

grounds. In all cases the macroscopic appearance of theascending aorta at operation was compatible with syphilis.Twelve had positive results to serological tests (WR andVDRL), in nine of these histology of the aorta was

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Syphilitic aortic regurgitation 367

available and changes compatible with syphilis werepresent in eight. In all three cases with negative serologicaltests, the histology of the aortic wall was available andconfirmed the diagnosis of syphilis (Table I). A historyof primary syphilis was obtained in only three (Cases 10,12, and 14) and in each of these infection occurred whenthe patient was in the late 'teens. Case 12 was of particularinterest, syphilitic aortic regurgitation was diagnosed, andserology was positive, but histology of the aorta was notavailable. The aortic valve was successfully replaced but30 months later the patient developed acute dissection ofthe ascending aortic and died after an operation. Postmortem histological examination of the aorta showedmedionecrosis in addition to the changes of syphilis,a rare combination but one previously reported byHeggtveit (1964).

TABLE I Criteria for the diagnosis of syphiliticaortitis

Case no.

123456789101112131415

No. positive

Aorta

Serology Macroscopic Microscopic

+

Neg+

Neg+

+

++

Neg

+

+

+

12/15

+

+

+

+

+

_a

+

+

+

+

+

+

+

Neg

+

+

+ +

15/15 11/12

aHistology of valve only

ResultsWith the exception of one patient (Case 4) who wasasymptomatic, all had severely limited exercisetolerance with evidence of heart failure. Fourteen ofthe fifteen had effort dyspnoea with a mean durationof 40 months (range 2 to 162), twelve had nocturnaldyspnoea with a mean duration of 7 months (range1 to 24), and six had angina with a mean duration of44 months (range 1 to 144).

Evidence of severe aortic regurgitation included anincreased pulse pressure and cardiomegaly. All werein sinus rhythm, except Case 1 who had atrialfibrillation; the mean pulse pressure was 109 mm Hg(range 80 to 150); and the mean cardiothoracic ratioin the fourteen patients who had a preoperative chestradiograph was 59 per cent. (range 50 to 70). Pre-operative haemodynamic data were obtained ineleven patients, in whom the mean pulmonary arterypressure was 27 mm Hg (range 14 to 47) and themean left ventricular end diastolic pressure was20 mm Hg (range 5 to 38). There was inadequateinformation for a detailed study of left ventricular

function; only six patients had a left ventriculogrambut in others the degree of reflux into the left ventriclefrom the aortogram gave some information. AUappeared to have severe left ventricular dysfunctionin keeping with their symptoms and the raised leftventricular diastolic pressure. Coronary arteriographywas available in three (Cases 5, 7, and 10); in two itwas normal, but in Case 7 stenosis of both coronaryostia was shown. In a further six patients who did nothave preoperative coronary arteriography, ostialstenosis was noted at the time of operation and in anadditional two patients there was slight thickeningof the intima around the coronary ostia withoutstenosis. No myocardial biopsies were taken.The preoperative erythrocyte sedimentation rate

(Westergren) was available in fourteen patients andwas elevated in thirteen; the mean reading was47 mm/hr (range 5 to 120). In the three patients withpositive serological results who had not been treatedwith penicillin, the mean ESR was 106 mm/hr (range90 to 120) compared with a mean of 36 mm/hr(range 10 to 90) in the eight patients with positiveserological results who had received penicillin therapy(usually procaine penicillin 600,000 units daily for10 days) within the 12 months before surgery. Threepatients with negative serological results who had nothad any antibiotic treatment had a mean ESR of19 mm/hr (range 5 to 37).

In two patients the aortic valve was repaired andin thirteen it was replaced, a Starr-Edwards pros-thesis being used in ten, a Bjork-Shiley in two, anda frame-mounted fascia lata valve in one (Ionescu,Ross, Deac, Grimshaw, Taylor, Whitaker, andWooler, 1970). In addition four had replacement ofthe ascending aorta with a dacron graft, seven hadsurgery to the coronary ostia and one had both(Table II).The operative mortality (death within one month

of surgery) was 20 per cent. (3 patients) and the latemortality 40 per cent. (6 cases), making a total mor-tality of 60 per cent (Table III). Of the seven patientswho had surgical treatment before January, 1972, sixdied, whereas after that date there were only threedeaths. Two of the three operative deaths occurred inthe earlier period. Thus there was some evidence tosuggest that increasing experience reduced themortality. Post mortem examinations were obtainedin six cases but informnation on the coronary arterieswas available in only two (Cases 12 and 15).

DiscussionThe outstanding and disturbing feature in thesesurgically treated cases of syphilitic aortic regurgita-tion was the operative mortality of 20 per cent. andlate mortality of 40 per cent. at a time when themortality for aortic valve replacement in non-syphilitic cases at the National Heart Hospital was

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368 British J7ournal of Venereal Diseases

TABLE II Types of aortic prosthesis used andadditional operative procedures to the ascending aortaand coronary ostia

Case no. Valvea Aorta Coronary arteries

1 S.E -2 S.E Dacron graft3 S.E -4 S.E Dacron graft5 B-S -6 B-S -7 Repair Endarterectomy Endarterectomy8 S.E Dacron graft Endarterectomy9 Repair - Patch angioplasty10 F.L - Patch angioplasty11 S.E - Dilatation12 S.E -13 S.E - Endarterectomy14 S.E Dacron graft15 S.E - Endarterectomy

&S.E=Starr-Edwards; B-S =Bjork-Shiley; F.L =Fascia lata

TABLE III Surgical procedures, time, and cause ofdeath in the nine patients who died

Surgicalprocedure

S.EC.O.S

F.LC.O.s

S.EC.O.SA.G

S.EC.O.s

S.EC.O.S

RepairC.O.SA.E

S.EA.G

S.E

RepairC.O.s

Interval beforedeath Cause

10 days aGangrene of bowel? Embolus

10 days Cerebral embolus

14 days aSepticaemiaColiform organism

4 mths aSudden deathValve obstruction

7 mths Sudden death? Cause

8 mths aB.ECoag. neg. Staph.

27 mths

30 mths

36 mths

aB.EStaph.

aAortic dissection

Cardiac failure A.R

S.E=Starr-Edwards; F.L=Fascia lata; C.O.S=Coronary ostiasurgery; A.G=Aortic graft; A.E =Aortic endarterectomy; B.E=Bacterial endocarditis; A.R =Aortic regurgitation.aConfirmed at necropsy

not higher than 13 per cent. and is currently 3 per

cent. Over the same period the late mortality, in-cluding the early cases operated on in 1963, has notexceeded 24 per cent.

Part of the explanation for the high mortality be-came evident when data from the six survivors(Cases 1 to 6) was compared with that from the ninewho had died (Cases 7 to 15). Regarding symptoms,all except one (Case 4) were severely limited by

dyspnoea, but in the survivors the mean duration ofeffort dyspnoea was 22 months (range 0-60) com-pared with a mean of 48 months (range 2 to 162)in those who had died. Similarly, the average durationof nocturnal dyspnoea in the survivors was 4 months(range 0 to 6) compared with a mean of 8 months(range 0 to 24) in those who had died. Only six ofthe fifteen patients had angina, which occurred intwo of the survivors and in four of those who died(Table IV).

TABLE IV Comparison of symptoms in the sixsurvivors and nine fatalities

Duration of symptoms (mths)

Fate Case no. Effort dyspnoea P.N.D. A.P.

1 12 1 -2 6 6 6

Alive 3 24 - -

4 - _ _5 6 6 -6 60 2 60

Average 22 4

7 144 24 1448 18 3 -9 48 2 48

Dead 10 18 15 611 12 12 112 24 - -13 2 2 -14 24 1 -15 162 6 -

Average 48 8

P.N.D =Paroxysmal noctural dyspnoea; A.P =Angina pectoris

The physical findings, chest radiograph, andhaemodynamic state as assessed from the pulmonaryartery pressure and left ventricular end diastolicpressure did not differ significantly in the two groups,although in the three survivors for whom data wereavailable the mean left ventricular end diastolicpressure was 11 mm Hg (range 5 to 16) comparedwith 24 mm Hg (range 5 to 38) in those who died.A high ESR appeared to be a bad prognostic sign.In those who survived the mean ESR was 25 mm/hr(range 5 to 56) compared with a mean of 60 mm/hr(range 10 to 120) in those who died (Table V).Leonard and Smith (1957) reported that the prog-

nosis in patients with symptoms of heart failuretreated medically was poor with a 15 per cent. survivalat 5 years and a mean survival time after the onset ofcardiac failure of 18 months. Comparing our datawith these findings and accepting that the onset ofeffort dyspnoea in the presence of severe aorticregurgitation was a manifestation of heart failure, inboth groups the duration of symptoms exceeded themean expected survival time quoted by the aboveauthors.

Case no.

15

10

8

11

13

7

14

12

9

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Syphilitic aortic regurgitation 369

TABLE V Comparison of erythrocyte sedimentationrate, pulse pressure, cardiothoracic ratio, andhaemodynamic findings in the six survivors and ninefatalities

Objective findings

PulseFate Case ESR pressure C. T.R P.A.P L.V.E.D.P

no. (mm/hr) (mm/Hg) (mm/Hg) (mm/Hg)

1 - 110 - - -

2 15 125 67 16 12Alive 3 29 130 65 - -

4 5 120 52 31 165 20 90 55 25 56 56 100 56 - -

Average 25 112 59 24 11

7 25 100 50 14 58 90 135 62 47 359 37 100 64 - -10 120 150 52 18 22

Dead 11 25 80 70 30 2012 10 80 53 28 2513 110 110 64 22 3014 36 110 56 45 3815 90 100 57 16 15

Average 60 107 59 28 24

ESR =Erythrocyte sedimentation rate; C.T.R = Cardiothoracicratio; P.A.P =Pulmonary artery pressure; L.V.E.D.P=Left ven-tricular end diastolic oressure

The duration of symptoms is not the sole factorinfluencing mortality, but our information on otherparameters is incomplete-particularly left ventricu-lar function. In syphilitic aortic regurgitation thereare at least three factors which may impair leftventricular function:

(a) The degree of reflux, which in all our cases wassevere.

(b) The degree of coronary ostial stenosis and thestate of the coronary arteries. In only three ofthe patients were coronary arteriograms obtainedbefore surgery; these were normal in two andshowed coronary ostial stenosis in the third.Coronary ostial stenosis however was found atsurgery in a further six patients and in an addi-tional two there was slight thickening of theintima around the coronary ostia without stenosis.Endarterectomy was performed in four, patchangioplasty in two, and dilatation of the ostia inone. None of these patients was a long-termsurvivor.

(c) The possibility of a syphilitic myocarditis, al-though previously considered rare (Ince andMahabir, 1974), has to be reconsidered and withthe increasing use of left ventricular myocardialbiopsies this information should not be difficultto obtain (Brooksby, Jenkins, Coltart, Webb-Peploe, and Davies, 1974). In this context Case 14

was of particular interest, for after successfulreplacement of the aortic valve with a Starr-Edwards prosthesis and replacement of ananeurysmal ascending aorta with a dacron graft,the patient developed progressive cardiomegalyand first-degree heart block with left bundlebranch block 18 months after surgery. Thecoronary ostia appeared normal at surgery, butthere was no pre- or postoperative coronaryarteriogram. The patient died from infectiveendocarditis 27 months after operation. Althougha post mortem examination was obtained, there wasno information on the coronary arteries and nohistological examination of the myocardium.

We have inadequate data to make any valid cor-relations between angina, coronary ostial stenosis,coronary artery disease, and the degree of elevationof the ESR. Perhaps, however, it should be notedthat, of the ten patients in whom coronary ostialstenosis or slight thickening of the intima around thecoronary ostia was found at operation, five hadangina, whereas of the five patients with normalcoronary ostia only one had angina. The coronaryarteries themselves were normal in the three patients(Cases 5, 7, and 10) who had preoperative coronaryarteriography, but in one of these (Case 7) ostialstenosis was demonstrated angiographically. Thetechnique of selective coronary arteriography is such,however, that ostial stenosis, even of moderateseverity, can be missed as the catheter is introducedinto the proximal part of the artery. The ten patientswith ostial stenosis had a mean ESR of 65 mm/hr(range 25 to 120) compared with a mean ESR of16 mm/hr (range 5 to 29) in those with normal ostia,but in the former group six had had treatment withpenicillin compared with three in the latter group;this makes it impossible to correlate the eJevation ofthe ESR with coronary ostial stenosis although it isconceivable that adequate penicillin treatment givenearly in the disease might prevent this complication.Post mortem data of the coronary arteries were limitedto two patients (Cases 12 and 15). The coronaryarteries were normal in one and showed generalizedatheroma with calcified plaques in the other.

Reviewing the causes of death (Table III), theredid not appear to be anything in the syphiliticaetiology itself to account for the high mortality.Although none of the patients who had any form ofcoronary surgery (endarterectomy, patch angioplastyand ostial dilatation) was amongst the six long-termsurvivors, there was no indication that these tech-niques contributed to either the operative mortalityor the late deaths. A study of the pre- and post-operative electrocardiograms showed no evidenceof intraoperative myocardial damage. On theoreticalgrounds saphenous vein bypass grafts should besatisfactory for relieving syphilitic ostial stenosis, and

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since the close of this series one case, similar to thosereported here, has been successfully treated (J.P.)with a combination of aortic valve replacement anda saphenous vein graft for left coronary ostial stenosis.

It is concluded that aortic valve replacementcorrectly timed is probably the best treatment forsevere syphilitic aortic regurgitation and that casesshould be referred for operation before the onset ofcardiac failure. This view is supported by the poorprognosis with medical treatment.

In the past too much attention has been given tothe degree of aortic regurgitation and insufficientattention to left ventricular function, coronary ostialstenosis, and the state of the coronary arteries. Pre-operative investigations should include aortography,coronary arteriography, an assessment of left ventri-cular function, and possibly myocardial biopsy. Thepresent series is too small to determine the place ofpreoperative penicillin. The operative procedure inthese cases may not be confined to aortic valve re-placement but may include resection ofan aneurysmalascending aorta and repair, disobliteration, or by-passing of the coronary ostia. The postoperativecourse in the six long-term survivors (averagefollow-up 33 months) surpasses anything that can beachieved with medical treatment alone and earlierreferral for surgery should reduce the present highmortality.

AppendixCase reportsCase 1, a 58-year-old white female with a 1-yearhistory of effort dyspnoea, presented in 1971 with heartfailure. Clinically she had aortic regurgitation. TheVenereal Diseases Reference Laboratory (VDRL) testwas positive, Reiter protein complement-fixation (RPCF)test positive, fluorescent treponemal antibody (absorbed)(FTA-ABS) test positive. She was given a course ofpenicillin. One month later she developed cardiorespiratoryarrest and was transferred to the National Heart Hospital(NHH) for emergency surgery. She had gross pulmonaryoedema with atrial fibrillation and was already on a

ventilator. The electrocardiogram (ECG) showed leftventricular hypertrophy and right bundle branch block.At operation (25.3.71), which was carried out without adetailed haemodynamic assessment, there was gross aorticregurgitation from prolapse of the right coronary cusp.The other two cusps appeared normal. There was slightdilation of the ascending aorta and both coronary ostiawere small. The aortic valve was replaced with a No. 10composite seat Starr-Edwards prosthesis. Postoperativeprogress was uneventful apart from Klebsiella pneumoniawhich was treated with penicillin and she remains wellwith a competent aortic valve 4 years and 7 months aftersurgery.

Histological examination of the aortic valve cusps

We wish to thank the physicians of the National HeartHospital for permission to study their cases, and Mr.Lorenzo Gonzales-Lavin and Mr. Keith Ross whooperated on Cases 2 and 12 respectively.

ReferencesBROOKSBY, I. A. B., JENKINS, B. S., COLTART, D. J.,

WEBB-PEPLOE, M. M., and DAVIES, M. J. (1974)Lancet, 2, 1222

CAMPBELL, M., and SHACKLE, J. W. (1932) Brit. med.J_., 1,328

HEGGTVEIT, H. A. (1964) Circulation, 29, 346INCE, W. E. and MAHABIR, B. S. (1974) Brit.J_. vener. Dis.,

50, 97IoNEscU, M. I., Ross, D. N., DEAC, R., GRIMSHAw,

V. A., TAYLOR, S. H., WHITAKER, W., and WOOLER,G. H. (1970) Thorax, 25, 46

KOULUMIES, R., and HEINIVAARA, 0. (1957) Acta med.scand., 159, 453

LEONMD, J. C., and SMITH, W. G. (1957) Lancet, 1, 234MICHAUD, P., TERMET, H., CHASSIGNOLLE, J., DALLOZ, C.,

AGE, C., DELAVE, J., DU GRgs, B., and CHAMPSAUR, G.(1970) Arch. Mal. Coeur, 63, 674

PREWITT, T. A. (1970) J. Amer. med. Ass., 211, 637SAMAAN, H. A. (1973) J. cardiovasc. Surg. (Torino), 14,

247

showed fibrous thickening with distinct rolling of the freemargins. Histology of the aortic wall was not available.

Case 2, a 57-year-old white female presented inJanuary 1972 with a 6-month history of paroxysmaldyspnoea, effort dyspnoea, and angina. Clinically therewas severe aortic regurgitation. The VDRL, RPCF,FTA-ABS, treponemal immobilization (TPI) tests wereall negative, but a chest radiograph showed cardiacenlargement with a dilated calcified ascending aorta andthe electrocardiogram indicated left axis deviation and leftventricular hypertrophy with strain. Aortography showedsevere aortic regurgitation with aneurysmal dilatation ofthe ascending aorta. At operation (24.3.72) the aneurysmof the ascending aorta and the dilated aortic root wereconfirmed. The coronary ostia were normal. The valveand ascending aorta were replaced using a No. 10 Starr-Edwards prosthesis and a 35 mm Dacron graft. The post-operative course was uneventful and the patient hasremained well with minimal effort dyspnoea for the last3 years. Histology of the aorta confirmed the presence ofsyphilis and the aortic cusps were normal.

Case 3, a 52-year-old white male, presented inSeptember 1972 with a 2-year history of effort dyspnoea.

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Syphilitic aortic regurgitation 371

He had been given a course of penicillin for syphiliticaortic regurgitation 6 months previously. Clinical examina-tion showed severe aortic regurgitation. The Wassermannreaction (WR), and the RPCF and FTA-ABS tests werepositive. A chest radiograph showed cardiac enlargementwith dilatation of the aorta and the ECG indicated leftatrial hypertrophy and left ventricular hypertrophy withstrain. Aortography confirmed aortic regurgitation. On29.9.72 the VDRL test was positive 1:128, and the RPCFand FTA-ABS tests were positive. At operation (6.10.72)there was a dilated ascending aorta with thin valvecusps and normal coronary ostia. The valve was replacedwith a No. 11 Starr-Edwards prosthesis. The post-operative course was uneventful and the patient hasremained well with minimal effort dyspnoea for the last2 years and 9 months. Histology of the aorta confirmedthe presence of syphilis.

Case 4, a 56-year-old asymptomatic white male,was referred for assessment of a heart murmur discoveredat routine medical examination. Clinically there was severeaortic regurgitation, a chest radiograph showed cardiacenlargement with a prominent ascending aorta, and theelectrocardiogram left axis deviation, left atrial hyper-trophy, and left ventricular hypertrophy with strain.Syphilis was not suspected, and the serology was notchecked until after the operation, when the VDRL andFTA-ABS tests were negative and the RPCF test doubtful.At operation (17.5.73), there was aortic regurgitation withthickened and slightly shortened valve cusps. Theascending aorta showed aneurysmal dilatation and thecoronary ostia were normal. The valve was replaced by aNo. 10 Starr-Edwards prosthesis and the aorta by a30 mm Dacron graft. The postoperative course wasuneventful and the patient has remained well for 2 yearsbut has essential hypertension (205/110). Histology of theaorta confirmed a syphilitic aetiology.

Case 5, a 57-year-old white female known to have asystolic murmur, developed infective endocarditis andheart failure in 1973. Blood cultures grew Streptococcusviridans. Subsequently there was clinical evidence ofaortic and mitral regurgitation. She was given a 6-weekcourse of penicillin, and the blood cultures becamenegative but cardiac failure persisted. A chest radiographshowed cardiac enlargement with calcification of theascending aorta and the ECG showed first-degree heartblock and indicated left ventricular hypertrophy withstrain. Syphilis was not suspected, but after operationserological tests showed VDRL positive 1:16 and FTA-ABS positive. Cardiac catheterization, left ventricularangiography, and aortography confirmed severe aorticregurgitation and moderate mitral regurgitation. Coronaryarteriograms were normal. At operation (3.10.73) eachaortic cusp was perforated and there was a 6 mm perfora-tion in the anterior cusp of the mitral valve. The ascendingaorta was calcified and the coronary ostia were normal.The aortic valve was replaced with a 21 mm Bjork-Shileyprosthesis and the mitral valve repaired. The postoperativecourse was uneventful and the patient has remained wellfor 2 years. Histology of the aorta confirmed the presenceof syphilis.

Case 6, a 50-year-old white male with a 5-yearhistory of effort dyspnoea and angina, presented in 1973with left ventricular failure of 2 months' duration. In1955 he had developed a urethral discharge and the WRwas then negative. When he developed heart failure theWR was positive. Subsequently he was given a course ofpenicillin. Clinically, there was severe aortic regurgitation.The chest radiograph showed cardiac enlargement withcalcification of a dilated ascending aorta and the ECGindicated left ventricular hypertrophy with strain.Haemodynamic studies were not performed. On 24.9.73serological tests were positive (VDRL 1:16, TPHA, FTA-ABS) and he was given a further course of procainepenicillin 600,000 units daily for 21 days before surgery.At operation (10.10.73) there was aortic regurgitationwith thickened and shortened valve cusps. The ascendingaorta was calcified and showed changes typical of syphiliticaortitis, the right coronary ostium could not be located,and the left was obscured by calcium in the aortic sinus.The valve was replaced with a 21 mm Bjork-Shileyprosthesis using left coronary perfusion. The post-operative progress was uneventful and the patient hasremained well with no symptoms for 2 years. Histologyof the aorta confirmed the presence of syphilis.

Case 7, a 57-year-old white female originally presentedin 1951, 12 years before operation, with angina and effortdyspnoea. Syphilitic aortic regurgitation was diagnosed(WR and Kahn test positive) and she was given a courseof penicillin. Left ventricular failure developed in 1963,one year before surgery. Clinically, there was severeaortic regurgitation and cardiac failure. The WR andTPI test were positive, and the RPCF test weakly positive.A chest radiograph showed an enlarged heart with acalcified ascending aorta and the ECG showed moderateleft ventricular hypertrophy. Cardiac catheterization andaortography confirmed severe aortic regurgitation andcoronary arteriography showed partial occlusion of theleft coronary ostium but failed to demonstrate a rightcoronary ostium or right coronary artery. At operation(3.4.64) there was aortic regurgitation with thickening ofthe aortic cusps. The aorta was calcified and the macro-scopic appearances were compatible with syphilis. Neithercoronary ostium was visualized. The aortic valve wasreconstructed using a pericardial patch, and a left andright coronary endarterectomy was performed. Thesymptoms improved but signs of moderate aortic regurgi-tation persisted postoperatively. She was given crystallinepenicillin 1 m.u. 6-hrly for 10 days, but 3 months aftersurgery she developed coagulase-negative staphylococcalendocarditis and died. Surgical and post mortem histologyof the aorta confirmed the presence of syphilis. There wasno information on the state of the coronary arteries.

Case 8, a 33-year-old white female presented in 1965with a 3-month history of paroxysmal dyspnoea precededby effort dyspnoea for 15 months. The WR was positive,syphilitic aortic regurgitation was diagnosed, and she wasgiven a course of penicillin. Clinically there was severeaortic regurgitation with cardiac failure. The chest radio-graph showed a large heart with pulmonary oedema andthe ECG indicated left ventricular hypertrophy withstrain. In October 1966 the WR was positive 1: 320, and

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the VDRL and TPI tests were positive. Cardiac cathe-terization confirmed severe aortic regurgitation in additionto mild mitral regurgitation and pulmonary hypertension.Before surgery peritoneal dialysis was carried out forintractable cardiac failure, but she did not respond. Atoperation (30.1.67) there was aortic regurgitation withthickening of the aortic wall extending into the valvecusps. The valve was replaced with a No. 9 Starr-Edwardsprosthesis after the outflow tract had been enlarged by theinsertion of a Dacron patch. Endarterectomy of the leftcoronary ostium was also performed. Postoperatively shewas in low output cardiac failure and developed a coliformsepticaemia. She died 2 weeks after surgery duringhaemodialysis for renal failure. Surgical and post mortemhistology of the aorta confirmed the presence of syphilis.There was no information on the state of the coronaryarteries.

Case 9, a 51-year-old female with a 4-year history ofeffort dyspnoea and angina, presented early in 1967 wthparoxysmal noctural dyspnoea and congestive heartfailure. Clinically there was moderate aortic regurgitation.A chest radiograph showed a large heart with a dilatedand calcified ascending aorta and the ECG showed leftbundle branch block and left ventricular hypertrophy.On 18.4.67 the WR, Kahn test, and RPCF test werenegative. Serology was negative. Aortography confirmedan aneurysm of the ascending aorta with moderate aorticregurgitation. At operation (21.4.67) there was aorticregurgitation with normal valve cusps and a dilatedascending aorta with macroscopic appearances compatiblewith syphilis. The right coronary ostium was reduced to apinhole but the left was normal. The valve was repairedwith three 'x'-sutures to elevate the commissures and theright coronary artery was repaired with a pericardialpatch. There was no improvement after operation and boththe aortic regurgitation and heart failure persisted. InFebruary 1968 she was given procaine penicillin 600,000units daily for 21 days. She died 3 years later havingrefused a second operation. Histology (surgical material)of the aorta confirmed the presence of syphilis. A postmortem examination was not performed.

Case 10, a 55-year-old white male with an 18-monthhistory of effort dyspnoea and a 6-month history of angina,presented in January 1968 with left ventricular failure.lie had contracted primary syphilis at the age of 18 years.Clinically there was severe aortic regurgitation. The chestradiograph showed cardiac enlargement with wideningof the ascending aorta and the ECG showed left atrial andleft ventricular hypertrophy with strain. Aortographyconfirmed severe aortic regurgitation; the coronaryarteriogram was normal. The WR, VDRL, and RPCTtests were positive and he was given a course of 1 m.u.penicillin twice daily for 21 days in January 1968. InJuly 1969 he was given a preoperative course of procainepenicillin 600,000 units daily for 21 days. At operation(6.8.69) there was aortic regurgitation and the valve cuspswere retracted with rolled edges. The left coronary orificewas normal, and the right was partially occluded, butbilateral coronary artery perfusion was possible. Theaortic valve was replaced with a fascia lata graft mountedon an Ionescu frame and a right coronary patch angio-plasty was performed. Postoperatively the patient was

hypotensive and failed to regain consciousness. It wasthought that he might have suffered a cerebral embolism.He died 10 days after operation. Apost mortem examinationwas not performed. Histology (surgical material) of thevalve showed only simple fibrous thickening; the aortawas not examined.

Case 11, a 52-year-old white female, presented in1968, 2 years preoperatively with a pulsatile swelling inthe root of the neck. The WR was positive and syphiliticaortic regurgitation was diagnosed. She was given a courseof penicillin lasting 3 weeks. Subsequently she developedcardiac failure and angina with dysphagia thought to bedue to compression of the oesophagus from an aorticaneurysm. Clinically there was severe aortic regurgitation.The chest radiograph showed cardiac enlargement withan aneurysm of the ascending aorta and the ECG showedleft axis deviation and left ventricular hypertrophy withstrain. Aortography confirmed severe aortic regurgitationand diffuse dilatation of the ascending aorta. The VDRLtest was positive and she was given a further course ofprocaine penicillin 600,000 units daily for 21 days beforesurgery. At operation (11.12.70) there was severe aorticregurgitation with thickening and retraction of the valvecusps. The aorta showed aneurysmal dilatation and therewas severe stenosis of the right coronary ostium. The leftostium was normal. The aortic valve was replaced with aNo. 9 Starr-Edwards prosthesis. The postoperative coursewas uneventful, and the patient improved, but diedsuddenly 4 months after operation. Post mortem examina-tion showed that the valve was obstructed by fibrin clotalthough the patient had been on anticoagulants sinceoperation. Neither the aorta nor the coronary arteries wereexamined. Histology (surgical material) of the valveshowed only fibrous thickening.

Case 12, a 47-year-old white male with a history ofurethritis at the age of 19 years, presented in 1971 with a2-year history of effort dyspnoea. Clinically there wassevere aortic regurgitation. The chest radiograph showedcardiac enlargement with dilatation of the ascendingaorta and no obvious calcification in the aortic wall. TheECG showed left ventricular hypertrophy. Aortographyconfirmed severe aortic regurgitation with dilatation ofthe ascending aorta. The WR and RPCF test werenegative, the VDRL test weakly positive, and the FTA-ABS test positive. He was given procaine penicillin600,000 units daily for 14 days in May 1971. At operation(10.8.71) there was severe aortic regurgitation due todilatation of the aortic root; the valve cusps were thin.The ascending aorta was diffusely dilated with plaquesof calcium in the wall but the coronary ostia were normal.The valve was replaced with a No. 11 Starr-Edwardsprosthesis. Histology (surgical material) of the valveshowed fibrous rolling; the aorta was not examined.The patient made satisfactory progress after the operation

and improved for 1 year, but then developed recurrentgastro-intestinal haemorrhages due to acute gastricerosions. Investigations also showed polycystic kidneyswith impaired renal function. Anticoagulants werestopped for a time but were restarted after he developedmultiple cerebral emboli. A pyrexial illness developed2j years after operation and a chest radiograph showedwidening of the ascending aorta. There was no pain.

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Aortography showed aneurysmal dilatation of the ascend-ing aorta. Operation (January 1974) showed a dissectionof the ascending aorta; a Dacron graft was inserted but thepatient died from haemorrhage. Post mortem examinationof the aorta showed medionecrosis suggesting Marfan'ssyndrome. The coronary arteries were normal.

Case 13, a 51-year-old Srilankan male, presented2 months before operation with an attack of acute leftventricular failure followed by effort dyspnoea. There wassevere aortic regurgitation and a chest radiograph showedcardiac enlargement with dilatation of the ascendingaorta. There was left ventricular hypertrophy on theelectrocardiogram. Aortography confirmed severe aorticregurgitation with an aneurysm of the ascending aorta.Syphilis was not suspected and the WR which waspositive was not checked until after surgery. At operation(24.4.72) there was severe aortic regurgitation with thinand retracted valve cusps. The aorta was thickened anddilated with appearances typical of syphilis. The aorticvalve was replaced by a No. 11 Starr-Edwards prosthesis,and a right coronary ostial endarterectomy was performed.The patient was given procaine penicillin 600,000 unitsdaily for 21 days after the operation. The immediatepostoperative course was uneventful but he died suddenly7 months later. An embolus was suspected, in spite ofanticoagulants since the time of operation. A post mortemexamination was not made. Histology (surgical material)of the aorta confirmed the presence of syphilis.

Case 14, a 51-year-old white male with a 2-year historyof effort dyspnoea, developed left ventricular failure in1972. He had had primary syphilis when aged 19. Clinic-ally there was severe aortic regurgitation. A chest radio-graph showed cardiac enlargement with a moderatelydilated ascending aorta and the ECG showed leftventricular hypertrophy with strain. Cardiac catheteriza-tion and aortography confirmed severe aortic regurgitationwith raised left and right heart pressures. The VDRL testwas positive 1: 64 and the RPCF and FTA-ABS testswere also positive. He was given procaine penicillin 600,000units daily for 21 days before surgery. At operation(28.11.72) there was severe aortic regurgitation withthickening and shortening of the valve cusps. Theascending aorta was aneurysmal and there was slightthickening of the intima at the site of the coronary ostia.

The valve and aorta were replaced with a No. 10 Starr-Edwards prosthesis and a 30 mm Dacron graft. Post-operative progress was initially uneventful and the patientimproved. After 18 months there was an increase in heartsize confirmed by the chest radiograph and the ECGshowed first-degree heart block and left bundle branchblock. The patient developed staphylococcal endo-carditis and died 27 months after the operation. Histology(surgical material) of the aorta confirmed the presence ofsyphilis. Post mortem examination showed vegetationinvolving the suture line of the aorta prosthesis. Leftventricular hypertrophy was confirmed but there was nomention of the state of the coronary arteries.

Case 15, a 60-year-old white female, presented in1959 with effort dyspnoea due to syphilitic aortic regurgi-tation and was given a course of bismuth. The WRand RPCF test were positive. In April 1973, 6 monthsbefore operation, she developed left ventricular failure.There was a past history of duodenal ulcer and hypo-thyroidism. Clinically she had severe aortic regurgitation;a chest radiograph showed cardiac enlargement withcalcification and slight dilatation of the ascending aorta,and the ECG showed left ventricular hypertrophy. Leftventricular angiography and aortography confirmedsevere aortic regurgitation and mild mitral regurgitation.The VDRL was positive 1: 4, the RPCF test negative,and the FTA-ABS test positive. She was given procainepenicillin 600,000 units daily for 21 days before surgery.At operation (8.10.73) there was severe aortic regurgitationwith thickened and retracted valve cusps. The aorta wascalcified and there was stenosis of the left coronary ostiumwith a normal right coronary ostium. The valve wasreplaced with a No. 11 Starr-Edwards prosthesis and aleft coronary endarterectomy was performed. Post-operatively renal failure developed followed by ileus andperitonitis. Laparotomy 10 days after the original opera-tion showed a gangrenous bowel from duodenojejunalflexure to rectum. This was thought to be due to embolialthough the patient had been on heparin since the valvereplacement. Histology (surgical material) of the valveshowed slight thickening. A post mortem examination ofthe aorta showed macroscopic and histological evidenceof syphilis. The coronary ostia were normal blut there wasuniform thickening with atheromatous calcified plaquesinvolving all major coronary arteries. None was occluded.

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