Congenital aortic valve disease with rupture ofmitral … · showedconsiderable...

British Heart Journal, 1976, 38, 665-673.

Congenital aortic valve disease with ruptureof mitral chordae tendineae

Simon Joseph, Richard Emanuel, Marvin Sturridge, and Eckhardt OlsenFrom The Middlesex Hospital; Cardiothoracic Institute; and The National Heart Hospital, London

A new clinical entity is described in which free aortic regurgitation from congenital aortic valve diseasecaused rupture of the chordae to the anterior leaflet of the mitral valve in 7 men aged 45 to 63years (mean52 years); 2 of the patients also had rupture of chordae to the posterior leaflet. Comparing these patients withthose with ruptured mitral chordae in association with rheumatic heart disease and patients with spontaneouschordal rupture, differences were evident. No patient had a history of rheumatic fever and none had activeinfection. The typical clinical presentation was of acute mitral regurgitation into a small left atrium, withsevere pulmonary oedema which was often resistant to medical treatment. The cause of chordal rupture in thesepatients was in part the result ofprogressive left ventricular dilatation, of direct trauma to the anterior cuspof the mitral valve, and possibly of a genetic factor. The anatomicalfeatures of both aortic and mitral valvesare described, and in 3 histology of the mitral valve was available; 2 had myxomatous degeneration similarto that seen in patients with spontaneous chordal rupture, and in 1 there was degeneration of collagen tissue.All patients were treated surgically but the mortality was high (5 out of 7, 70%). Early operation withreplacement of the aortic and mitral valves is recommended if this high mortality is to be reduced.

Chordal rupture without obvious cause was des- Bailey, Vera, and Hirose, 1969; Edwards, 1971;cribed 40 years ago (Frothingham and Hass, 1934) Cooley et al., 1972; Eisenmann, 1973; Goodman,but its importance has only become recognized Kimbiris, and Linhart, 1974; Aranda et al., 1975).relatively recently (Bailey and Hickam, 1944; Rare associations include pregnancy, left atrialSanders et al., 1965; Roberts, Braunwald, and myxoma, osteogenesis imperfecta, Ehlers-DanlosMorrow, 1966; Klughaupt et al., 1969; Eisenmann, syndrome, gargoylism, acute rheumatic fever,1973; Sutton, Chatterjee, and Caves, 1973). During scarlet fever, aneurysm of sinus of Valsalva, con-the past decade, it has been recognized as a common gestive cardiomyopathy, and hypertrophic cardio-cause of mitral regurgitation and is now believed to myopathy (Frew, 1931; Wigle, 1964; Childressaccount for between 20 and 30 per cent of all cases et al., 1966; Barrillon et al., 1967; Sanders et al.,of isolated mitral regurgitation (Ellis, Frye, and 1967; Hwang and Lam, 1968; Soulie et al., 1969;McGoon, 1966; Eisenmann, 1973). Previously Sloman, Hunt, and Hare, 1969; Edwards, 1971;rupture of mitral chordae was generally attributed Caves and Paneth, 1972; Lainee et al., 1972;to rheumatic heart disease with or without infective Ghahramani et al., 1972; Wood, Thomas, andendocarditis (Osmundson, Callahan, and Edwards, Braimbridge, 1973; Wise, 1974).1958; Barrillon, Maurice, and Lenegre, 1967; This report describes 7 patients with severeSanders et al., 1967; Littler, Epstein, and Coulshed, aortic regurgitation caused by congenital aortic1973; Eisenmann, 1973). valve disease and ruptured mitral chordaetendineae,Other causes of ruptured chordae include an association not previously reported. In these

coronary artery disease, Marfan's disease, trauma, patients there was no history of rheumatic fever orand the 'floppy valve syndrome' (Childress, evidence of rheumatic heart disease. One patientMaroon, and Genovese, 1966; Barrillon et al., only had had infective endocarditis. In all patients,1967; Simpson, Nora, and McNamara, 1969; chordae to the anterior mitral cusp were involved

and 6 of the 7 patients presented with acute heartReceived 1 December 1975. failure.

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666 J6oseph, Emanuel, Sturridge, and Olsen

Clinical data rheumatic fever. He had signs of severe aortic andTheclinicaldetails ofthe7patientsaredescre. mitral regurgitation, with clinical evidence of leftTheclmncal details of the 7 patients are described, and right ventricular hypertrophy and left heart

Haemodynamic data, and operative and patho- failure. The chest radiograph showed pronouncedlogical findings are summarized in the Table. car diomoary oed and*cardiomegaly with pulmonary oedema and theCase 1 electrocardiogram confirmed severe left ventricular

hypertrophy and atrial fibrillation.A man, known to have valvular heart disease since Emergency surgery without cardiac catheteriza-the age of 39 years, presented with acute left heart tion was undertaken. The aortic valve was calcifiedfailure at the age of 62. There was no history of and bicuspid, with calcification in the rudimentary

TABLE Clinical, haemodynamic, and pathological data

Case Sex Age Cardiac Duration Duration Additional Haemodynamic data (mmHg) AngiographicNo. at rhythm of offinal diagnosis assessment

opn symptoms illness PCV PCV PA PA LVmean 'v' wave mean edp AR MR

1 M 62 AF 23 yrs 3/12 24* 55* 55-60t/0* 20* - -

2 M 63 SR 7/12 7/12 AVS 20 35 55/12 - 20 2/4 2/4

3 M 50 AF 7/12 7/12 CAD 22 28 80/4 50 15 2/4 3/4

4 M 45 SR 18 yrs - - - - - 4/4

50 SR 12 18 34/6 18 9 2/4 3/4(second opn)

5 M 45 AF 12 yrs 9/12 - 18* 27* 42/17* 27* 25* 2/4 2/4

6 M 51 SR 23yrs 1/12 - - - - - 2/4

7 M 47 SR 6 yrs 4j/12 - 20 42 80/35 40 17 3/4 4/4

*Pressures obtained at operation.tRight ventricular pressure.Abbreviations: AF, atrial fibrillation: AML, anterior mitral leaflet; AR, aortic regurgitation; AVS, aortic valve stenosis; CAD, coronaryartery disease; LV edp, left ventricular end diastolic pressure; MR, mitral regurgitation; PA, pulmonary artery; PCV, pulmonarycapillary venous pressure; PML, posterior mitral leaflet; SR, sinus rhythm.Conversion from Traditional to SI Units: 1 mn!Hgu0-133 kPa.



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Congenital aortic valve disease 667

commissure; the non-coronary cusp was shortened, collagen tissue, which suggested aortic regurgitationallowing free regurgitation. The mitral valve of some duration. Myxomatous degeneration wasannulus was very dilated; the anterior cusp was a prominent. The mitral valve showed thickening oflittle thickened and chordae to its centre were collagen tissue and extensive myxomatous de-ruptured. The posterior cusp was normal and the generation in the centre of the anterior leaflet in thechordae intact. Both valves were replaced with position ofthe zona spongiosa. Similar changes wereStarr-Edwards prostheses. present in the ruptured chordae. There was no in-

Initial progress was satisfactory but four months creased vascularity.after operation he developed left heart failure anddied at home. Case 2

A 63-year-old man with no rheumatic historypresented with sudden onset of left heart failure,

Mitral valve Aortic valve and effort angina. He had features of aortic valve

Macroscopical Histology Macroscopical histology disease with stenosis and regurgitation. On the chestappearance appearance radiograph there was considerable cardiomegaly

with pulmonary venous congestion and aortic valveAML-2 rupt. Myxomatous Bicuspid Myxomatous calcification, and electrocardiogram showed severe

central chordae; degeneration; calcified degeneration; left ventricular hypertrophy. Cardiac catheteriza-PML-normal; fibroelastic fibroelastic tion confirmed aortic palv stnois andererita-

annulus dilated thickening thickening tion confirmed aortic valve stenosis and regurgita-AML-3-4 rupt. Collagenous Bicuspid Collagenous tion; and also showed important mitral regurgitation,

ant-lat. chordae; thickening and calcified thickening, without stenosis.PML-normal; degeneration degeneration, At operation the aortic valve was bicuspid and

annulus normal andcalcification heavily calcified with a transverse opening. The

AML-rupt. ant- - Bicuspid - chordae to the anterolateral part of the mitrallat. chordae; thickened anterior cusp were ruptured but the mitral valve

PML-voluminous; was otherwise normal. Both valves were replacedatulus with Starr-Edwards prostheses. The patient made

AML-2rapt. - Bicuspid Thickened and an uncomplicated recovery apart from the onset ofchordae; volumi- calcified fibrosed atrial fibrillation.nous; Microscopically the aortic valve leaflets were

PML-voluminous; thickened, particularly towards the free edge. ThisAMLusame Myxomatous - thickening consisted of collagen tissue and foci of

chordae rupt.; degeneration; calcification. There was also some degeneration ofvoluminous; collagenous the collagen tissue. Vascularity was not increased.

PML-thickened; thickening and The mitral valve showed uniform thickening causedvoluminous; degeneration;

by collagen tissue, which, particularly in the centralannulus dilated increasedvascularity portion of the valve leaflet, was associated with ex-

AML-rupt. - Bicuspid Nodular tensive degenerative changes; again the vascularitychordae at med. calcified calcificcommissure; fibrosis was not increased.

PML-rupt.chordae at med. Case 3commissure;anulus dilated A 50-year-old man with no previous cardiac or

AML-rupt. Bicuspid - rheumatic history presented with sudden onset ofchordae to med. calcified left heart failure. Clinically he had the signs ofhalf; 'floppy, congestive cardiac failure, mitral regurgitation, andPML.-l rapt. chorda;annulus normal atrial fibrillation. Radiographic information is not

AML-1 rupt. Bicuspid available. Cardiac catheterization confirmed mitralcentral chorda; regurgitation but aortography was not performed.annulusdilated He was discharged on medical treatment but 4a______ulus ____________clilated____________________ months later was readmitted with further cardiac

failure and renal failure that later required haemo-Histological examination of the aortic valve dialysis. He was transferred to London 3 months

showed thickening of the valve leaflets caused by later and at this time the chest radiograph showedfibroelastic tissue with foci of calcification. Super- considerable cardiomegaly. The electrocardiogramimposed on the deformed face was a layer of confirmed atrial fibrillation and left ventricular



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668 Joseph, Emanuel, Sturridge, and Olsen

hypertrophy. A second cardiac catheterization con- years when he was readmitted in extremis. Atfirmed severe mitral regurgitation, pronounced operation, which was undertaken as a matter ofpulmonary hypertension, and important aortic urgency, the anterior mitral leaflet was balloonedregurgitation which had been suspected clinically. with thickening of the free margin. The repairedIn view of the abrupt onset of symptoms the pos- chordae of the anterior leaflet had ruptured andsibility of ruptured mitral chordae was considered. most of the unruptured chordae were slenderAt operation the aortic valve was bicuspid with a though two, adjacent to the commissures, had

calcified rudimentary commissure, the cusps were thickened. The posterior leaflet was also thickenedthickened with rolled edges, and the valve was and ballooned into the left atrium but all theclearly regurgitant. The mitral annulus was very chordae were intact. There was moderate aorticlarge; most chordae to the anterolateral end of the regurgitation resulting from prolapse of one cuspanterior cusp had ruptured allowing free mitral of the pulmonary autograft, and a perforation of aregurgitation. The posterior cusp was voluminous, second suggested previous infection. Degenerationbut the chordae were intact. Both valves were re- of the pulmonary homograft had produced freeplaced with Starr-Edwards prostheses. pulmonary regurgitation. The pulmonary valve was

Postoperative progress was uneventful but in replaced with an aortic homograft and the mitralspite of adequate anticoagulation he developed a and aortic valves with Starr-Edwards prostheses.cerebral embolism 14 months after operation, with The patient deteriorated because of infection of thetransient dysphasia and hemiparesis. Two years homograft and died 2 weeks after the second opera-later he suffered an anteroseptal myocardial in- tion from cardiac and renal failure.farction but made a satisfactory recovery. Histology of the mitral valve showed uniform

Valve histology is not available. thickening and a prominent zona spongiosa withmyxomatous degeneration, as well as degeneration

Case 4 of collagen tissue. The chordae showed similarchanges. Both the degenerative areas gave a positive

A man aged 27 years without a history of rheumatic reaction with alcian blue stain. In the centre of thefever developed acute left ventricular failure. He anterior mitral valve leaflet, thick-walled smallwas treated medically until the age of 37 years when vessels were identified. In view of the patient'she deteriorated and was admitted to hospital. previous operation, this increased vascularity couldClinically he had severe aortic regurgitation which have been the result of the previous surgical inter-was confirmed by aortography. Surgery was advised vention or, secondly, of inflammation such as in-but the patient declined. He was again treated fective endocarditis, but there was no history of thismedically but deteriorated progressively. At the age and no vegetations were seen. The pulmonaryof 45 years when readmitted the chest radiograph homograft showed some degenerative changes ofshowed considerable cardiomegaly, with pulmonary collagen tissue and the body of the leaflet wasvenous congestion, and electrocardiogram confirmed totally devoid of nuclei, but the surfaces weresinus rhythm with left atrial and severe left ventri- covered with fibroblasts. One leaflet (anterior cusp)cular hypertrophy. In view of the earlier haemody- showed recent fibrin superimposition. There was nonamic findings, a second cardiac catheterization evidence of infection. The pulmonary autograftwas not performed. showed uniform thickening, together with mildAt surgery the aortic valve was bicuspid, calcified, degenerative changes of collagen tissue, and in these

and grossly regurgitant. Severe mitral regurgitation areas the nuclei were reduced. The leaflet in thewas caused by 2 ruptured chordae to the anterior position of the non-coronary cusp showed totalmitral cusp and the annulus was dilated. There was destruction of normal architecture.also a jet lesion on the anterior cusp of the mitral Pathology of the patient's original aortic valve isvalve with shallow ulceration. A mitral annuloplasty not available.was performed and the chordae were sutured; theaortic valve was replaced with a pulmonary auto-graft (Gonzalez-Lavin et al., 1970a) and a pul- Case 5monary homograft was used to replace the pul- This man had no history of rheumatic fever, andmonary valve. He made a satisfactory recovery but heart disease was first diagnosed at the age of 33he developed signs of mitral regurgitation 19 years. He presented at the age of 42 years withmonths later; at this time cardiac catheterization moderate aortic regurgitation, having deterioratedshowed mild aortic regurgitation and moderate with the onset of atrial fibrillation, but there was nomitral regurgitation. He developed progressive other evidence of mitral valve disease. At the age ofsymptoms but again refused surgery for a further 4 44 he developed severe acute left ventricular failure



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with considerable cardiomegaly and the signs of In spite of an adequate cardiac output immediatelyaortic and mitral regurgitation. The electrocardio- after the operation, his circulation deteriorated andgram and chest radiograph confirmed severe left he developed acute renal failure. Medical treatment,ventricular enlargement, and the diagnosis of aortic including peritoneal dialysis, failed to prevent hisand mitral regurgitation was confirmed by angio- death on the fourth postoperative day.graphy. Valve histology is not available.

Surgery was initially refused but was undertakenas a matter of urgency 7 months later after a re- Case 7currence of heart failure with tricuspid regurgita-tion. The aortic valve was bicuspid with massive A male patient had no rheumatic history but anodular calcification and fibrosis. It was replaced cardiac murmur was heard during childhood.with a Starr-Edwards prosthesis. The mitral Aortic regurgitation was diagnosed at 41 years andannulus was grossly enlarged with pliable cusps and he presented at the age of 44 years with suddenruptured chordae at the medial end of both onset of left and right heart failure. He had signsanterior and posterior leaflets; an annuloplasty was of severe aortic and mitral regurgitation. The chestperformed with imbrication at the level of the radiograph showed considerable cardiomegaly andruptured chordae; the valve was subsequently pulmonary venous congestion, and an electrocardio-competent. The tricuspid ring was also dilated and gram confirmed left and right ventricular and leftrequired an annuloplasty. After the operation severe atrial hypertrophy. Angiography confirmed theneurological complications developed and he died diagnosis.9 days later. At surgery the aortic valve was bicuspid and

dayslater.goteirvlisoaib. uncalcified and the root large with a diameter ofHistology of the mitral valve is not available. 3*3 cm; regurgitation was the result of cusp attenua-tion and insufficiency. The mitral ring was also

Case 6 grossly dilated with a diameter of about 8 cm;A man developed breathlessness at the age of 37 the valve cusps were thin and attenuated and oneyears and had an attack of infective endocarditis at chorda to the central part of the anterior cusp wasthe age of 38 years when heart disease was first ruptured. Both valves were replaced with Starr-diagnosed. He had further attacks of infective Edwards prostheses but the patient died from un-endocarditis at 43 and 47 years. He presented at the controllable haemorrhage from the left coronaryage of 51 years with evidence of moderate aortic artery.regurgitation, confirmed by aortography and slight Valve histology is not available.mitral regurgitation. Chest radiograph at that timeshowed mild cardiomegaly with no evidence of left Discussionatrial enlargement, and electrocardiogram confirmedsinus rhythm with moderate left ventricular hyper- The clinical features of acute mitral regurgitationtrophy. He resronded initially to medical treatment from ruptured chordae tendineae have been re-and was then lost to follow-up for 7 years. He re- viewed recently (Roberts and Perloff, 1972; Littlerturned at-the age of 58 years; surgery was advised et al., 1973; Sutton et al., 1973). Some of the pointsbut he refused operation in spite of progressive which help to distinguish it from rheumatic mitralsymptoms. regurgitation with intact chordae are the shortTwo years later at the age of 60 years he developed history, persistence of sinus rhythm, high left atrial

acute left ventricular failure. Chest radiograph pressure caused by a small non-compliant leftshowed further increase in heart size with pul- atrium (Baxley et al., 1973) and the unusual radia-monary oedema, and electrocardiogram confirmed tion of systolic murmurs. Before the syndrome be-progressive left ventricular hypertrophy. Emergency came well recognized it was often mistaken forsurgery was undertaken without prior cardiac aortic stenosis because of the radiation of the mitralcatheterization. systolic murmur to the base of the heartThe aortic valve, which was congenitally bicuspid, (Osmundson et al., 1958). This was particularly so

was large and 'floppy'. There was minimal calcifica- when the chordae to the posterior mitral cusp weretion, with prolapse of the non-coronary cusp. The involved. When chordae to the anterior cusp weremitral valve was grossly regurgitant because of ruptured the radiation of the murmur was morerupture of the chordae from the medial half of the typical of rheumatic mitral regurgitation. Someanterior cusp. In addition there was a single authors, however, have found that the radiation ofruptured chorda from the posterior cusp. Both the murmur was an unreliable guide to the nature ofvalves were replaced with Starr-Edwards prostheses. the regurgitation (Sutton et al., 1973; Littler et al.,



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1973). A further difficulty arises with the systolic carditis seemed unlikely as the normal architecture,ejection murmur which usually accompanies florid though distorted by thickening, was not destroyed.aortic regurgitation; this may mask the murmur Infective endocarditis also seemed improbableof mitral regurgitation, especially when the latter is because of the absence of recent or healed vegeta-conducted to the base. tions. Histological examination of the 3 mitralRuptured chordae in association with rheumatic valves available (Cases 1, 2, and 4) showed myxo-

mitral and aortic valve disease with and without matous degeneration together with degenerativeinfective endocarditis is well recognized (Sanders changes in collagen tissue (Fig.), the latter beinget al., 1967; Shine et al., 1968; Gonzalez-Lavin, the only change seen in Case 2. The elastic tissueLise, and Ross, 1970b). Other cases with aortic was often fragmented. These histological appear-regurgitation have been recorded, but the nature of ances were similar to those described in cases ofthe valve lesions in these reports was not mentioned spontaneous or idiopathic chordal rupture (Bailey(Barrillon et al., 1967; Manhas et al., 1971). and Hickam, 1944; Shine et al., 1968; GerbodeRupture of mitral chordae associated with severe et al., 1969; Caulfield et al., 1971; Caves, Sutton,aortic regurgitation from a congenitally bicuspid and Paneth, 1973). Similar pathology is probablyaortic valve has not previously been reported. responsible for the floppy valve syndrome whereAcute mitral regurgitation from chordal rupture from 9 to 20 per cent may develop ruptured chordae

frequently presents as unexpected left heart failure. (Read, Thal, and Wendt, 1965; Pomerance, 1969;This was so in 6 of our 7 patients. Four were in Cooley et al., 1972). The 'floppy' or 'ballooning'sinus rhythm and 4 had a small left atrium. As left mitral valve is probably responsible for the systolicheart failure in these cases was primarily mecha- click-late systolic murmur syndrome in whichnical, the result of ruptured chordae with subse- chordal rupture has also been reported (Goodmanquent prolapse of the anterior cusp and a dilated et al., 1974).mitral annulus, it was not surprising that they failed The macroscopical appearances of the normalto respond to medical treatment. In 6, surgery was mitral valve have been described in detail byundertaken within 7 months of the onset of symp- Ranganathan et al. (1970). The anterior leaflettoms. In spite of this, 4 were emergency procedures consists of rough and clear zones, while the posteriorand the operative mortality was high; 5 out of the 7 leaflet, like the tricuspid valve, has an additional(70%) died. If the mortality is to be reduced, the basal zone. On average 25 chordae are present, 9malignant course of mitral regurgitation from are inserted into the anterior leaflet, the majorityruptured chordae must be recognized and surgery into the rough zone, while 14 usually form part ofundertaken as soon as the diagnosis is made. the posterior leaflet. The remaining 2 chordae are

All 7 patients had bicuspid aortic valves; 6 were inserted at the commissures (Lam et al., 1970).thickened and 4 were calcified. One valve was Basically the valve leaflets have two layers. The'floppy' (Case 6) and aortic regurgitation in this case 'deformed' face which consists of elastic tissue, andwas caused by cusp prolapse. Histology was avail- the 'holding face' of collagen tissue. Not infre-able on 4 of the aortic valves, one showed myxo- quently, an additional zone can be identifiedmatous degeneration (Case 1), while in another between these two layers, the so-called zona(Case 2) there were degenerative changes in the spongiosa (Gross and Kugel, 1931). When myxo-collagen tissue. In the remaining 2, non-specific matous degeneration is prominent, it is in a centralfibrosis and calcification were found. position, so that it suggests an increase in the zona

In 5 cases rupture of mitral chordae was confined spongiosa. Frequently, however, degenerativeto the anterior leaflet; in the remaining 2, chordae to changes in collagen tissue are also present. It mayboth anterior and posterior cusps were involved. be that a prominent zona spongiosa is a geneticallyFive patients had considerable dilatation of the determined component which, in some cases,mitral annulus and in 2 there was excessive cusp degenerates in later life, giving rise to the 'floppytissue involving both leaflets in one and the posterior valve' syndrome.cusp in the other. There was no evidence in our Caulfield et al. (1971) considered myxomatouspatients that chordal rupture was caused by the degeneration to be enzymatic destruction of normalmore common aetiologies of rheumatic fever or collagenous and elastic structure. The sameinfective endocarditis, except possibly in Case 6 authors noted the presence of blood vessels inwhere there was a history of bacterial endocarditis. normal chordae but reported fewer in degeneratedIn Case 4 the anterior leaflet of the mitral valve zones. There is increasing evidence that ischaemia,showed increased vascularity with thick walled which is known to cause papillary muscle dysfunc-vessels, which was thought to be caused by the tion and rupture, is also a factor in chordal ruptureprevious mitral annuloplasty. Rheumatic endo- (Sanders et al., 1967; Barrillon et al., 1967; Gerbode



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FIG. Photomicrograph (Weigert's elastic van Gieson. x 12) from Case 4 showing distalportion of the anterior mitral leaflet and severed chordae. Areas of myxomatous degenerationin both the valve leaflet and chordae are marked with arrows.

et al., 1968; Klughaupt et al., 1969; Eisenmann, postulated that left ventricular dilatation from what-1973; Caves et al., 1973; Aranda et al., 1975). It is ever cause altered the normal lie of chordae, ingenerally agreed that in the physiological state it is which they are parallel to the inflow tract, thusthe chordae to the anterior leaflet which are sub- increasing the stress to which they were subjected.jected to the greater stress (Chiechi, Lees, and Aortic regurgitation not only has this effect byThompson, 1956). So it is somewhat surprising that increasing the diastolic volume, but the regurgitantin cases of spontaneous chordae rupture (i.e. without jet may impinge directly onto the anterior cusp ofother obvious pathology) it is usually the posterior the mitral valve causing local trauma to the valvemitral leaflet that is affected (Sanders et al., 1965; leaflet and chordae (Gonzalez-Lavin et al., 1970b).Marchand et al., 1966; Raftery, Oakley, and This probably explains why, in the presence ofGoodwin, 1966; Acar et al., 1968; Selzer et al., aortic regurgitation, it is usually the chordae to the1972). In the normal valve mechanical stress on the anterior cusp of the mitral valve which are rupturedmitral chordae during ventricular systole is probably (Menges, Ankeney, and Hellerstein, 1964; Ellisnot great. Marchand et al. (1966) when repairing et al., 1966; Littler et al., 1973; Eisemann, 1973).ruptured chordae, noted little tension on nylon In addition, Shine et al. (1968) suggested that thethreads used when they were exteriorized through rapid recoil of the left ventricle in early diastolethe left ventricular wall. Similar views were ex- contributed to the mechanical stress to which thepressed by Salisbury, Cross, and Rieben (1963) who chordae of the anterior mitral leaflet were sub-found that stress borne by normal mitral chordae jected. In the 7 patients reported here all factors fordiminished after aortic valve opening and before the increasing chordal stress were present; in additiondevelopment of peak systolic pressure. Further- there may have been a congenital fault in the mitralmore, rupture of normal chordae is not particularly valve tissue.common in conditions such as aortic stenosis where We consider that this syndrome is a new clinicalpeak left ventricular pressure may be abnormally entity in which free aortic regurgitation from con-high. genital aortic valve disease causes progressive left

Pre-existing abnormalities of the left ventricle ventricular dilatation and direct trauma to the an-and the mitral valve almost certainly increase terior cusp of the mitral valve. These factors com-chordal stress. Edwards and Burchell (1958) bine, generally without infection, and lead to rupture



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of the chordae to the anterior leaflet. The classical Frew, H. W. 0. (1931). Rupture of the chordae tendineaepicture is of acute mitral regurgitation into a small following scarlet fever. Glasgow Medical Journal, 115, 195.leftatwFrothingham, C., and Hass, G. M. (1934). Rupture of normalleft atrium, with severe pulmonary oedema which is chordae tendineae of the mitral valve. American Heart

often resistant to medical treatment. Early surgical Journal, 9, 492.treatment with replacement of aortic and mitral Gerbode, F., Hill, J. D., Kelly, J. J., Selzer, A., and Kerth,valve is indicated. W. J. (1968). Surgical correction of mitral insufficiencydue to ruptured chordae tendineae. Circulation, 37-38,

Suppl. 2, 119.We are indebted to Drs. Raphael Balcon and Brian Heard for Gerbode, F., Kerth, W. J., Hill, J. D., Sanchez, P. A., andpermission to publish data from Case 2 and to Dr. Michael Puryear, G. H. (1969). Surgicaltreatment of non-rheumaticHoney for Case 7. mitral insufficiency. Journal of Cardiovascular Surgery,

10, 103.Ghahramani, A. R., Arnold, J. R., Hilder, F. J., Sommer,

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