alastair mcdonald,' alan harris, keith mcdonald386 mcdonald,harris,jefferson, marshall, andmcdonald...

British HeartJournal, I97I, 33, 383-387.

Association of prolapse of posterior cusp ofmitral valve and atrial septal defect

Alastair McDonald,' Alan Harris, Keith Jefferson, John Marshall,and Lawson McDonaldFrom the Cardiac Departments of The London and St. George's Hospitals,the National Heart Hospital, and the Institute of Cardiology, London

Eleven patients with fossa ovalis atrial septal defects and prolapse of the posterior cusp of themitral valve are described. Six patients had clinical evidence of mitral regurgitation, and in 2others the electrocardiogram was unusual for uncomplicated fossa ovalis atrial septal defects.The varied appearance of the prolapsed cusp was shown by left ventricular angiography. Theprincipal significance of this association is in its differentiation from atrioventricular defects.

The coincidence of prolapse of the posteriorcusp of the mitral valve with fossa ovalis atrialseptal defects has not previously been em-phasized though the association occurred in2 of go patients reported by Barlow et al.(I968) and 2 of 40 patients reported by Han-cock and Cohn (I966). Prolapse of the pos-terior cusp of the mitral valve is now describedin i I patients who had, in addition, fossaovalis atrial septal defects. A preliminaryaccount of this work was given at a meetingof the British Cardiac Society in London inNovember I969 (McDonald et al., I970).

Material and methodsThe ii patients were aged from 4 to 57 years;3 were male and 8 female. Electrocardiograms,chest radiographs, and phonocardiograms wereobtained in all patients and cardiac catheterization-and left ventricular angiography were performed.The findings at operation were available in 5patients.

F ResultsSymptoms Two patients were asympto-matic. Four had slight, and 4 moderate

,Fexertional dyspnoea; supraventricular tachy-cardia caused paroxysmal palpitation in 3, andventricular ectopic beats occurred in the otherpatient. A past history of rheumatic fever wasobtained in Case 6.

Received I October 1970.4 Address requests forreprintsto Dr. Alastair McDonald,Cardiac Department, The London Hospital, White-chapel, London E.1.

Signs Apart from the usual clinical findingsof an atrial septal defect (Cleland et al., I969),an abnormality of the mitral valve was sus-pected clinically in 6 patients (Table) whohad a mitral pansystolic murmur which wasassociated with a mid-systolic click in 2. Inone patient (Case 8) who had moderate pul-monary valvar stenosis and subvalvar stenosiswith reversal of the atrial shunt there wasmoderate central cyanosis and finger clubbing.The skeletal manifestations of Marfan's syn-drome were present in Case 2.

Electrocardiography Eight patients hadpartial right bundle-branch block and 3 com-plete right bundle-branch block (Table). Themean frontal QRS axis showed right axisdeviation (greater than +go9) in 7 patientsand was normal in 3. The frontal axis wasunusual for uncomplicated fossa ovalis atrialseptal defects in 2 patients. Abnormal leftaxis deviation (>-300) was present in onecase, and the axis was indeterminate in an-other, both having partial right bundle-branch block.

Radiology The findings in 1o patients weretypical of atrial septal defect with cardiacenlargement, dilatation of the pulmonaryartery, and pulmonary plethora. There wasabsence of pulmonary plethora in one patient(Case 8) who had moderate pulmonary steno-sis. In 2 patients (Cases 4 and 6) there wasenlargement of the left atrium suggesting amitral valvar lesion.

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384 McDonald, Harris, Jefferson, Marshall, and McDonald

TABLE Clinical findings and investigations in eleven patients

Case No., age, and sex Dyspnoea Palpitation Apical Mid- Mean Pulmonary Pulmonary Mitralpansystolic systolic frontal systemic vascular regurgitationmurmur click QRS axis flow ratio resistance on angio-

cardiography

I 4 F - - - - Right axis 2-5:I Moderatedeviation

2 i8 F - - + + Right axis i-8: I Normal Moderatedeviation

3 28 F + - + + Right axis 2.4:1 Slight Slightdeviation

4 28 F + + - + - Right axis 2-6: I Moderate Slightdeviation

5 3I F + + _ - Indeter- 2.3:1 Normalminate

6 34 F + + + + - Right axis i-8: I Moderate Slightdeviation

7 40 F + - _ - Left axis 2-0:I Slightdeviation

8 57 F + + - _ - Right axis I-0: I-4 Normaldeviation

9 27 M - - + - Normal 3-0:I Normal10 35 M - + _ - Right axis 3-0:I Normal Slight

deviationII 48 M + + + - Right axis 2-0: I Slight Moderate

deviation

+ present, - absent.

Phonocardiography The records con-firmed the auscultatory findings; an illustra-tive sound recording from Case 2 is shown inFig. i.

Cardiac catheterization The presence ofan atrial septal defect was shown in all pa-tients. In IO patients the atrial shunt was fromleft to right, the pulmonary/systemic flowratio varying from 3 to I7 (Table). In thepatient with pulmonary stenosis there was a

PA.------ - 1111-- -l CL

C* R ' .f |...

FIG. I Sound recording, Case 2. H.F., highfrequency; P.A., pulmonary area; M.A.,mitral area; CAR., external carotid pulse;L2, lead 2 of electrocardiogram; INSP.,inspiration; C, systolic click; A2, aortic com-ponent of second sound; P2, pulmonary com-ponent of second sound.

right-to-left shunt at atrial level resulting inmoderate arterial desaturation. The pulmon-ary vascular resistance was normal in 7patients and there was a slight to moderaterise in resistance in 5.

Angiography Left ventricular biplane an-giograms (Elema-Schonander), or cineangio-grams in the right anterior oblique were per-formed. Mitral regurgitation was seen in 6patients; it was slight in 4, and moderate in 2(Table). Prolapse of the posterior cusp ofthe mitral valve was shown in all. The angio-graphic appearance of prolapse varied particu-larly with regard to the size and the directionof the prolapse. In the anteroposterior planethe prolapse appeared in some cases as a bal-looning deformity at the right border of theleft ventricle (Fig. 2), in others the prolapseoccurred to either side of the mitral ring(Fig. 3). In a few the ballooning cusp wasmainly directed posteriorly (Fig. 4) and wasnot well seen in the anteroposterior plane.The prolapse into the left atrium occurred insystole; in some cases a little medium wasseen to be caught among folds of redundantcusp tissue, as the posterior cusp swung intothe left ventricular cavity during diastole.The size of the prolapse was not related to thedegree of mitral regurgitation. For example,in Case 5 (Fig. 4 and 5) a large prolapse wasshown but the mitral valve remained fullycompetent.

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Prolapse of the mitral valve with atrial septal defect 385

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FIG. 2 Left ventricular angiogram, antero-

posterior view, Case I I. The arrow indicatesthe ballooning posterior cusp of the mitralvalve outlined by contrast in the left atriumand left ventricle.

F I G. 3 Left ventricular angiogram, antero-posterior view, Case 7. The arrows indicateprolapse to either side of the mitral valve ring.

Surgery Surgery was undertaken in 5 pa-. tients and a fossa ovalis defect closed by directsuture. The appearances of the mitral valves

% at operation varied. In one patient (Case 6)who had a past history of rheumatic fever

4,slight mitral stenosis was found and mitralvalvotomy was performed, and no significantabnormality of the posterior cusp or chordae

was noted. In 2 patients (Cases 3 and i i) re-dundant posterior cusp tissue was found andin the latter patient (Case ii), who had moder-ately severe mitral regurgitation, this was par-ticularly noticeable and a posterior cuspvalvuloplasty was performed. This procedureappeared to reduce the amount of regurgita-tion, yet after operation the mitral pansystolicmurmur became a late systolic murmur. Inthe remaining 2 patients (Cases 4 and IO), inwhom slight mitral regurgitation was shown,no significant abnormality was noted onviewing the mitral valve from the atrium. Itmay be impossible with a non-beating emptyheart to show the dynamic abnormality of aprolapsing posterior cusp of the mitral valve,and this seems likely to apply to Cases 4, 6,and IO.

DiscussionProlapse of the posterior cusp of the mitralvalve was shown by left ventricular angio-graphy in all ii patients, but in only 6 patientshad a mitral valve abnormality been clinicallydiagnosed by the presence of a mitral pan-systolic murmur. In the other 5 patientsthere were no auscultatory features suggestinga mitral valvar lesion. An unusual meanfrontal QRS in 2 of these patients was con-sidered a possible indication that an atrio-ventricular defect might be present. Thecharacteristic deformity of the left ventricularoutflow tract in atrioventricular defects (Baronet al., I964; Somerville and Jefferson, I968)was not found on angiography. In the remain-ing 3 patients the prolapse was an incidentalfinding at angiography. A late systolic mur-mur has been shown to be due to mitralregurgitation (Barlow et al., I963; Segal andLikoff, I964; Stannard et al., I967) and leftventricular angiography has shown that theregurgitation is related to prolapse of theposterior cusp of the mitral valve (Barlow andBosman, I966; Criley et al., I966). A mid orlate systolic click is not uncommon and hasbeen considered to be due to sudden chordaltension (Reid, I96I). The patients describedhere underline that prolapse of the posteriorcusp need not occasion any abnormal physicalsigns, if mitral regurgitation is lacking, evenwhen the size of the prolapse is considerable.

This type of deformity of the mitral valvehas been noted in Marfan's syndrome (Readet al., I964; Criley et al., I966; Barlow et al.,I968), and one patient in this series had theskeletal manifestations of Marfan's syndrome.In most instances, however, no definite aetio-logical factor is identified. Rheumatic endo-carditis is not infrequently noted (Barlowet al., I968), as in one case in this series, but it



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386 McDonald, Harris, Jefferson, Marshall, and McDonald

FIG. 4 Left ventricular angiogram, lateralview, Case 4. Arrow indicates large prolapseof posterior cusp of the mitral valve.

FIG. 5 Left ventricular angiogram, antero-pQsterior view, Case 4. Arrow indicatesprolapse.

cannot be concluded that this abnormalitywas the consequence of rheumatic endocar-ditis. A familial incidence has been noted(Stannard et al., I967; Hunt and Sloman,I969). In two series (Hancock and Cohn,I966; Barlow et al., I968) two cases havebeen reported where prolapse was associated

with atrial septal defects of secundum type.In our own experience and that of Barlow etal. (I968), prolapse has rarely been found withother congenital lesions such as persistentductus arteriosus, ventricular septal defect,atrioventricular septal defect, and pulmonarystenosis. The familial tendency, associationwith congenital cardiac lesions, and its occur-rence in children suggest that this abnormalityof the mitral valve may be congenital. Thecoincidence of fossa ovalis atrial septal defectswith prolapse could be a random associationof two fairly common abnormalities. How-ever, the authors have performed many leftventricular angiograms in patients with ven-tricular septal defects and have found theassociation of a ballooned-posterior cusp to beextremely rare, suggesting that the associationwith fossa ovalis atrial septal defects may bemore specific. There is little pathologicalinformation. A voluminous posterior cuspwith elongated chordae has been found atnecropsy in one case (Barlow et al., I963), andreference to a localized expansion of the cusphas also been made (Hunt and Sloman, I969).In the latter report the findings at operation intwo patients with symptomatic mitral regur-gitation were of ruptured chordae and largeposterior mitral cusps, and others have re-ported similar findings (Marchand et al.,I966). In Marfan's syndrome the chordaehave been found to be unduly lax (McKusick,i955; Raghib et al., I965). These findingssupport the contention that the primary ab-normality is chordal in origin leading to aprogressive ballooning of the cusp (Barlow etal., I968). The natural history of prolapse ofthe posterior cusp of the mitral valve is notknown. It is possible that the severity of themitral regurgitation may increase in time andcause significant symptoms. The risk of infec-tive endocarditis in atrial septal defect of thefossa ovalis type is exceedingly small (Bed-ford, Papp, and Parkinson, I941), but it mayoccur on the abnormal mitral valve in patientswith atrioventricular defects. In patients withprolapse of the posterior cusp infective endo-carditis has been found (Linhart and Taylor,I966) and prophylactic antibiotic therapy atthe time of septic hazard is indicated. Whenatrial septal defects of fossa ovalis type areassociated with prolapse of the posterior cusp,especially where there are signs indicatingmitral regurgitation, it is important to distin-guish this combination from atrioventriculardefects. The left ventricular angiographicfeatures are distinct.

We would like to thank Drs. Wallace Brigden,Aubrey Leatham, and Edgar Sowton for permis-



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Prolapse of the mitral valve with atrial septal defect 387

sion to report the findings in patients under theircare. Mr. Charles Drew operated on Cases 4, 6,and io, Mr. Keith Ross on Case 3, and Sir ThomasHolmes Sellors on Case ii.

ReferencesBarlow, J. B., and Bosman, C. K. (I966). Aneurysmal

protrusion of the posterior leaflet ofthe mitral valve.American Heart Journal, 71, i66.

Barlow, J. B., Bosman, C. K., Pocock, W. A., andMarchand, P. (I968). Late systolic murmurs andnon-ejection ('mid-late') systolic clicks. An analysisof go patients. British HeartJournal, 30, 203.

Barlow, J. B., Pocock, W. A., Marchand, P., andDenny, M. (I963). The significance of late systolicmurmurs. American Heart Journal, 66, 443.

Baron, M. G., Wolf, B. S., Steinfeld, L., and VanMicrop, L. H. S. (I964). Endocardial cushion de-fects. Specific diagnosis by angiocardiography.American Journal of Cardiology, 13, 162.

Bedford, D. E., Papp, C., and Parkinson, J. (I941).Atrial septal defect. British HeartJournal, 3, 37.

Cleland, W., Goodwin, J., McDonald, L., and Ross,D. (I969). Medical and Surgical Cardiology.Blackwell Scientific Publications, Oxford.

Criley, J. M., Lewis, K. B., Humphries, J. O'N., andRoss, R. S. (I966). Prolapse of the mitral valve:clinical and cine-angiocardiographic findings.British Heart_Journal, 28, 488.

Hancock, E. W., and Cohn, K. (I966). The syndromeassociated with midsystolic click and late systolicmurmur. American journal of Medicine, 41, I83.

Hunt, D., and Sloman, G. (I969). Prolapse of the pos-terior leaflet of the mitral valve occurring in elevenmembers of a family. American Heart Journal, 78,I49.

Linhart, J. W., and Taylor, W. J. (I966). The lateapical systolic murmur. Clinical, hemodynamicand angiographic observations. AmericanJournal ofCardiology, ig, I64.

McDonald, A., Harris, A., Jefferson, K., Marshall, J.,and McDonald, L. (I970). Association of prolapseof posterior cusp of mitral valve and atrial septaldefect. In Proceedings of the British CardiacSociety. British Heart_Journal, 32, 554.

McKusick, V. A. (i955). The cardiovascular aspect ofMarfan's syndrome: a heritable disorder of con-nective tissue. Circulation, II, 321.

Marchand, P., Barlow, J. B., du Plessis, L. A., andWebster, I. (I966). Mitral regurgitation with rup-ture of normal chordae tendineae. British HeartJ7ournal, 28, 746.

Raghib, G., Jue, K. L., Anderson, R. C., and Edwards,J. E. (I965). Marfan's syndrome with mitral in-sufficiency. American_Journal of Cardiology, I6, I27.

Read, R. C., Thal, A. P., Wolf, P. L., and Wendt,V. E. (I964). Symptomatic valvular myxomatousdegeneration: floppy valve syndrome. (Abstract.)Circulation, 30, Suppl. 3, I43.

Reid, J. V. 0. (I96I). Mid-systolic clicks. SouthAfrican Medical Journal, 35, 353.

Segal, B. L., and Likoff, W. (I964). Late systolic mur-mur of mitral regurgitation. American Heart Jour-nal, 67, 757.

Somerville, J., and Jefferson, K. (I968). Left ventricu-lar angiocardiography in atrioventricular defects.British Heart J7ournal, 30, 446.

Stannard, M., Sloman, J. G., Hare, W. S. C., andGoble, A. J. (I967). Prolapse of the posterior leafletof the mitral valve: A clinical familial, and cine-angiographic study. British Medical Journal, 3,71.



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