Br J: first published as 10.1136/hrt.38.7.665 on 1 July 1976. Downloaded from

British Heart Journal, 1976, 38, 665-673. Congenital disease with rupture of mitral chordae tendineae

Simon Joseph, Richard Emanuel, Marvin Sturridge, and Eckhardt Olsen From 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 disease caused rupture of the chordae to the anterior leaflet of the in 7 men aged 45 to 63years (mean 52years); 2 of the patients also had rupture ofchordae to the posterior leaflet. Comparing these patients with those with ruptured mitral chordae in association with rheumatic heart disease and patients with spontaneous chordal rupture, differences were evident. No patient had a history of and none had active infection. The typical clinical presentation was of acute mitral regurgitation into a small left , with severe pulmonary oedema which was often resistant to medical treatment. The cause ofchordal rupture in these patients was in part the result ofprogressive left ventricular dilatation, of direct trauma to the anterior cusp of the mitral valve, and possibly of a geneticfactor. The anatomicalfeatures of both aortic and mitral valves are described, and in 3 histology of the mitral valve was available; 2 had myxomatous degeneration similar to 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 with replacement of the aortic and mitral valves is recommended if this high mortality is to be reduced. http://heart.bmj.com/

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 atrial Sanders et al., 1965; Roberts, Braunwald, and myxoma, osteogenesis imperfecta, Ehlers-Danlos Morrow, 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- on September 28, 2021 by guest. Protected copyright. 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; Childress account 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, and (Osmundson, Callahan, and Edwards, Braimbridge, 1973; Wise, 1974). 1958; Barrillon, Maurice, and Lenegre, 1967; This report describes 7 patients with severe Sanders et al., 1967; Littler, Epstein, and Coulshed, aortic regurgitation caused by congenital aortic 1973; 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 or and the 'floppy valve syndrome' (Childress, evidence of rheumatic heart disease. One patient Maroon, 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 heart Received 1 December 1975. failure. Br Heart J: first published as 10.1136/hrt.38.7.665 on 1 July 1976. Downloaded from 666 J6oseph, Emanuel, Sturridge, and Olsen

Clinical data rheumatic fever. He had signs of severe aortic and mitral TheTheclmncalclinicaldetails ofthe7patientsaredescre.of the 7 patients are described, and rightregurgitation,ventricularwithhypertrophyclinical evidenceand leftofheartleft Haemodynamic data, and operative and patho- failure. The chest radiograph showed pronounced oed logical findings are summarized in the Table. *cardiomegalycar diomoarywith pulmonary oedema andthe electrocardiogram confirmed severe left ventricular Case 1 hypertrophy and atrial fibrillation. A man, known to have since Emergency surgery without cardiac catheteriza- the age of 39 years, presented with acute left heart tion was undertaken. The aortic valve was calcified failure 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) Angiographic No. at rhythm of offinal diagnosis assessment opn symptoms illness PCV PCV PA PA LV mean '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 http://heart.bmj.com/

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 on September 28, 2021 by guest. Protected copyright.

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, coronary artery disease; LV edp, left ventricular end diastolic pressure; MR, mitral regurgitation; PA, ; PCV, pulmonary capillary venous pressure; PML, posterior mitral leaflet; SR, sinus rhythm. Conversion from Traditional to SI Units: 1 mn!Hgu0-133 kPa. Br Heart J: first published as 10.1136/hrt.38.7.665 on 1 July 1976. Downloaded from

Congenital aortic valve disease 667

commissure; the non-coronary cusp was shortened, collagen tissue, which suggested aortic regurgitation allowing free regurgitation. The mitral valve of some duration. Myxomatous degeneration was annulus was very dilated; the anterior cusp was a prominent. The mitral valve showed thickening of little 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 the chordae intact. Both valves were replaced with position ofthe zona spongiosa. Similar changes were Starr-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 and died at home. Case 2 A 63-year-old man with no rheumatic history presented 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 chest appearance appearance radiograph there was considerable cardiomegaly with pulmonary venous congestion and aortic valve AML-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 and calcification heavily calcified with a transverse opening. The AML-rupt. ant- - Bicuspid - chordae to the anterolateral part of the mitral lat. chordae; thickened anterior cusp were ruptured but the mitral valve PML-voluminous; was otherwise normal. Both valves were replaced atulus with Starr-Edwards prostheses. The patient made AML-2rapt. - Bicuspid Thickened and an uncomplicated recovery apart from the onset of

chordae; volumi- calcified fibrosed atrial fibrillation. http://heart.bmj.com/ nous; Microscopically the aortic valve leaflets were PML-voluminous; thickened, particularly towards the free edge. This AMLusame Myxomatous - thickening consisted of collagen tissue and foci of chordae rupt.; degeneration; calcification. There was also some degeneration of voluminous; collagenous the collagen tissue. Vascularity was not increased. PML-thickened; thickening and The mitral valve showed uniform thickening caused voluminous; degeneration; annulus dilated increased by collagen tissue, which, particularly in the central vascularity portion of the valve leaflet, was associated with ex- on September 28, 2021 by guest. Protected copyright. AML-rupt. - Bicuspid Nodular tensive degenerative changes; again the vascularity chordae at med. calcified calcific commissure; fibrosis was not increased. PML-rupt. chordae at med. Case 3 commissure; anulus dilated A 50-year-old man with no previous cardiac or AML-rupt. Bicuspid - rheumatic history presented with sudden onset of chordae to med. calcified left heart failure. Clinically he had the signs of half; 'floppy, PML.-l rapt. chorda; congestive cardiac failure, mitral regurgitation, and annulus normal atrial fibrillation. Radiographic information is not AML-1 rupt. Bicuspid available. Cardiac catheterization confirmed mitral central chorda; regurgitation but aortography was not performed. annulusdilated He was discharged on medical treatment but 4 a______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 showed fibroelastic tissue with foci of calcification. Super- considerable cardiomegaly. The electrocardiogram imposed on the deformed face was a layer of confirmed atrial fibrillation and left ventricular Br Heart J: first published as 10.1136/hrt.38.7.665 on 1 July 1976. Downloaded from 668 Joseph, Emanuel, Sturridge, and Olsen

hypertrophy. A second cardiac catheterization con- years when he was readmitted in extremis. At firmed severe mitral regurgitation, pronounced operation, which was undertaken as a matter of pulmonary hypertension, and important aortic urgency, the anterior mitral leaflet was ballooned regurgitation which had been suspected clinically. with thickening of the free margin. The repaired In view of the abrupt onset of symptoms the pos- chordae of the anterior leaflet had ruptured and sibility of ruptured mitral chordae was considered. most of the unruptured chordae were slender At 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 thickened thickened with rolled edges, and the valve was and ballooned into the left atrium but all the clearly regurgitant. The mitral annulus was very chordae were intact. There was moderate aortic large; most chordae to the anterolateral end of the regurgitation resulting from prolapse of one cusp anterior cusp had ruptured allowing free mitral of the pulmonary autograft, and a perforation of a regurgitation. The posterior cusp was voluminous, second suggested previous infection. Degeneration but the chordae were intact. Both valves were re- of the pulmonary homograft had produced free placed with Starr-Edwards prostheses. pulmonary regurgitation. The was Postoperative progress was uneventful but in replaced with an aortic homograft and the mitral spite 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 the transient 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 with myxomatous degeneration, as well as degeneration of collagen tissue. The chordae showed similar Case 4 changes. 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 the fever developed acute left ventricular failure. He anterior mitral valve leaflet, thick-walled small was treated medically until the age of 37 years when vessels were identified. In view of the patient's he deteriorated and was admitted to hospital. previous operation, this increased vascularity could severe aortic which Clinically he had regurgitation have been the result of the previous surgical inter- http://heart.bmj.com/ 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 this medically but deteriorated progressively. At the age and no vegetations were seen. The pulmonary of 45 years when readmitted the chest radiograph homograft showed some degenerative changes of showed considerable cardiomegaly, with pulmonary collagen tissue and the body of the leaflet was venous congestion, and electrocardiogram confirmed totally devoid of nuclei, but the surfaces were sinus 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 no namic findings, a second cardiac catheterization evidence of infection. The pulmonary autograft on September 28, 2021 by guest. Protected copyright. was not performed. showed uniform thickening, together with mild At 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 the was caused by 2 ruptured chordae to the anterior position of the non-coronary cusp showed total mitral 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 is valve with shallow ulceration. A mitral annuloplasty not available. was performed and the chordae were sutured; the aortic valve was replaced with a pulmonary auto- graft (Gonzalez-Lavin et al., 1970a) and a pul- Case 5 monary homograft was used to replace the pul- This man had no history of rheumatic fever, and monary valve. He made a satisfactory recovery but heart disease was first diagnosed at the age of 33 he developed signs of mitral regurgitation 19 years. He presented at the age of 42 years with months later; at this time cardiac catheterization moderate aortic regurgitation, having deteriorated showed mild aortic regurgitation and moderate with the onset of atrial fibrillation, but there was no mitral regurgitation. He developed progressive other evidence of mitral valve disease. At the age of symptoms but again refused surgery for a further 4 44 he developed severe acute left ventricular failure Br Heart J: first published as 10.1136/hrt.38.7.665 on 1 July 1976. Downloaded from

Congenital aortic valve disease 669 with considerable cardiomegaly and the signs of In spite of an adequate cardiac output immediately aortic and mitral regurgitation. The electrocardio- after the operation, his circulation deteriorated and gram 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 his and mitral regurgitation was confirmed by angio- death on the fourth postoperative day. graphy. Valve histology is not available. Surgery was initially refused but was undertaken as a matter of urgency 7 months later after a re- Case 7 currence of heart failure with tricuspid regurgita- tion. The aortic valve was bicuspid with massive A male patient had no rheumatic history but a nodular 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 and annulus was grossly enlarged with pliable cusps and he presented at the age of 44 years with sudden ruptured chordae at the medial end of both onset of left and right heart failure. He had signs anterior and posterior leaflets; an annuloplasty was of severe aortic and mitral regurgitation. The chest performed with imbrication at the level of the radiograph showed considerable cardiomegaly and ruptured 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 left required an annuloplasty. After the operation severe atrial hypertrophy. Angiography confirmed the neurological complications developed and he died diagnosis. 9 days later. At surgery the aortic valve was bicuspid and dayslater.g oteirvlisoaib. uncalcified and the root large with a diameter of Histology 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 one years and had an attack of infective endocarditis at chorda to the central part of the anterior cusp was the 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 coronary http://heart.bmj.com/ age 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 time showed mild cardiomegaly with no evidence of left Discussion atrial enlargement, and electrocardiogram confirmed sinus rhythm with moderate left ventricular hyper- The clinical features of acute mitral regurgitation trophy. 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; Littler on September 28, 2021 by guest. Protected copyright. turned at-the age of 58 years; surgery was advised et al., 1973; Sutton et al., 1973). Some of the points but he refused operation in spite of progressive which help to distinguish it from rheumatic mitral symptoms. regurgitation with intact chordae are the short Two years later at the age of60 years he developed history, persistence of sinus rhythm, high left atrial acute left ventricular failure. Chest radiograph pressure caused by a small non-compliant left showed 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 for surgery was undertaken without prior cardiac aortic stenosis because of the radiation of the mitral catheterization. systolic murmur to the base of the heart The 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 were tion, with prolapse of the non-coronary cusp. The involved. When chordae to the anterior cusp were mitral valve was grossly regurgitant because of ruptured the radiation of the murmur was more rupture of the chordae from the medial half of the typical of rheumatic mitral regurgitation. Some anterior cusp. In addition there was a single authors, however, have found that the radiation of ruptured chorda from the posterior cusp. Both the murmur was an unreliable guide to the nature of valves were replaced with Starr-Edwards prostheses. the regurgitation (Sutton et al., 1973; Littler et al., Br Heart J: first published as 10.1136/hrt.38.7.665 on 1 July 1976. Downloaded from 670 Joseph, Emanuel, Sturridge, and Olsen

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 improbable of 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 mitral Ruptured 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 degenerative infective endocarditis is well recognized (Sanders changes in collagen tissue (Fig.), the latter being et al., 1967; Shine et al., 1968; Gonzalez-Lavin, the only change seen in Case 2. The elastic tissue Lise, 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 of the 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; Gerbode Rupture 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 probably aortic valve has not previously been reported. responsible for the floppy valve syndrome where Acute 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 systolic heart failure in these cases was primarily mecha- click-late systolic murmur syndrome in which nical, the result of ruptured chordae with subse- chordal rupture has also been reported (Goodman quent 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 normal to respond to medical treatment. In 6, surgery was mitral valve have been described in detail by undertaken within 7 months of the onset of symp- Ranganathan et al. (1970). The anterior leaflet toms. In spite of this, 4 were emergency procedures consists of rough and clear zones, while the posterior and the operative mortality was high; 5 out of the 7 leaflet, like the , has an additional (70%) died. If the mortality is to be reduced, the basal zone. On average 25 chordae are present, 9 malignant course of mitral regurgitation from are inserted into the anterior leaflet, the majority http://heart.bmj.com/ ruptured chordae must be recognized and surgery into the rough zone, while 14 usually form part of undertaken 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, and was 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 identified

matous degeneration (Case 1), while in another between these two layers, the so-called zona on September 28, 2021 by guest. Protected copyright. (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 central fibrosis 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, degenerative to the anterior leaflet; in the remaining 2, chordae to changes in collagen tissue are also present. It may both anterior and posterior cusps were involved. be that a prominent zona spongiosa is a genetically Five 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 'floppy tissue 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 myxomatous patients that chordal rupture was caused by the degeneration to be enzymatic destruction of normal more common aetiologies of rheumatic fever or collagenous and elastic structure. The same infective endocarditis, except possibly in Case 6 authors noted the presence of blood vessels in where there was a history of bacterial endocarditis. normal chordae but reported fewer in degenerated In 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 dysfunc- vessels, which was thought to be caused by the tion and rupture, is also a factor in chordal rupture previous mitral annuloplasty. Rheumatic endo- (Sanders et al., 1967; Barrillon et al., 1967; Gerbode Br Heart J: first published as 10.1136/hrt.38.7.665 on 1 July 1976. Downloaded from

Congenital aortic valve disease 671

...~~~~~~~~~~~~~~~~~......

FIG. Photomicrograph (Weigert's elastic van Gieson. x 12) from Case 4 showing distal portion of the anterior mitral leaflet and severed chordae. Areas of myxomatous degeneration in 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, in http://heart.bmj.com/ generally agreed that in the physiological state it is which they are parallel to the inflow tract, thus the 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 by Thompson, 1956). So it is somewhat surprising that increasing the diastolic volume, but the regurgitant in cases of spontaneous chordae rupture (i.e. without jet may impinge directly onto the anterior cusp of other obvious pathology) it is usually the posterior the mitral valve causing local trauma to the valve mitral 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 of on September 28, 2021 by guest. Protected copyright. Goodwin, 1966; Acar et al., 1968; Selzer et al., aortic regurgitation, it is usually the chordae to the 1972). In the normal valve mechanical stress on the anterior cusp of the mitral valve which are ruptured mitral chordae during ventricular is probably (Menges, Ankeney, and Hellerstein, 1964; Ellis not 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 the threads used when they were exteriorized through rapid recoil of the left in early diastole the left ventricular wall. Similar views were ex- contributed to the mechanical stress to which the pressed 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 for diminished after aortic valve opening and before the increasing chordal stress were present; in addition development of peak systolic pressure. Further- there may have been a congenital fault in the mitral more, 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 clinical peak 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 Br Heart J: first published as 10.1136/hrt.38.7.665 on 1 July 1976. Downloaded from 672 Joseph, Emanuel, Sturridge, and Olsen of the chordae to the anterior leaflet. The classical Frew, H. W. 0. (1931). Rupture of the chordae tendineae mitral into a small picture is of acute regurgitation following scarletC., andfever.Hass,GlasgowG. M. (1934).MedicalRuptureJournal,of115,normal195. leftleftatrium,atwithwFrothingham,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, J. (1968). Surgical correction of mitral insufficiency valve is indicated. W.due 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., and permission to publish data from Case 2 and to Dr. Michael Puryear, G. H. (1969). Surgicaltreatment of non-rheumatic Honey for Case 7. mitral insufficiency. Journal of Cardiovascular Surgery, 10, 103. Ghahramani, A. R., Arnold, J. R., Hilder, F. J., Sommer, References L. S., and Samet, P. (1972). Left atrial myxoma: hemo- dynamic and phonocardiographic features. American Acar, J., Caramanian, M., Perrault, M., Luxereau, P., and Journal of Medicine, 52, 525. Arnaud, J. C. (1968). Les insuffisances mitrales par Gonzalez-Lavin, L., Geens, M., Somerville, J., and Ross, rupture de cordages d'origine degenerative. Archives des D. N. (1970a). Autologous pulmonary valve replacement Maladies du Coeur et des Vaisseaux, 61, 1724. of the diseased aortic valve. Circulation, 42, 781. Aranda, J. M., Befeler, B., Lazzara, R., Embi, A., and Gonzalez-Lavin, L., Lise, M., and Ross, D. (1970b). The Machado, H. (1975). and coronary importance of the 'jet lesion' in bacterial endocarditis artery disease: clinical, hemodynamic and angiographic involving the left heart. Journal of Thoracic and Cardio- correlations. Circulation, 52, 245. vascular Surgery, 59, 185. Bailey, C. P., Vera, C. A., and Hirose, T. (1969). 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