congenital mitral valve disease in transposition of the great arteries

Congenital Mitral Valve Disease in Transposition ofthe Great Arteries

By GLENN C. ROSENQUIST, M.D., J. STARK, M.D., AND J. F. N. TAYLOR, M.D.

SUMMARYA spectrum of mitral valve disease was noted in 71 % of 163 specimens with transposition of the great

arteries. At one end of the spectrum were hearts with normal mitral valve, except that the free margin of theanterior leaflet was shortened; at the other end were specimens with underdevelopment of the space

between or behind the papillary muscles of the left ventricle. In additional specimens the anterior leaflet wasindented and attached by chordae tendineae to the ventricular septum.

Additional Indexing WordsMitral stenosisChordae tendineac

Double orifice mitral valve Papillary muscles, left ventricle

INCREASING NUMBERS of patients with trans-position of the great arteries (TGA) are undergoing

successful correction with Mustard's procedure.Although a success rate of over 90% has beenreported,'-' a knowledge of all associated lesions isnecessary if a high survival rate for the operation canbe expected.

In a recent paper we identified a spectrum ofanomalies of the mitral valve in specimens with coarc-tation of the aorta.' In the present study we report aspectrum in specimens from patients with TGA. Atone end of the spectrum were hearts with normalmitral valve, except that the free margin of theanterior leaflet was shortened. The other end of thespectrum was comprised of hearts with underdevelop-ment of the space behind or between the papillarymuscles of the left ventricle, i.e., formes frustes ofparachute mitral valve.' In additional hearts the in-dentation in the anterior leaflet adhered to the ven-tricular septum in the left ventricular outflow tract.

Materials and Methods

Heart specimens with TGA were reviewed from thecollections of the Hospital for Sick Children, London, andthe Johns Hopkins Hospital, Baltimore. In each caseMustard's operation might have been used for correctionsince specimens with endocardial cushion defect, single,

From the Departments of Pediatrics and Pathology, The JohnsHopkins School of Medicine, Baltimore, Maryland, and theThoracic Unit, The Hospital for Sick Children, London, England.

Supported bv grants HL 5984 and 16466 from the NationalInstitutes of Health and the British Heart Foundation.

Address for reprints: Glenn C. Rosenquist, M.D., The Helen B.Taussig Children's Cardiac Center, The Johns Hopkins Hospital,Baltimore, \MarvIand 21205.

Received September 9, 1974; revision accepted for publicationNovember 29, 1974.

Circulation, Volume 51, April 1975

hypoplastic, double outlet or double inlet ventricles, coarc-tation of the aorta, interrupted arch of the aorta, andtricuspid atresia were eliminated.The mitral valves were considered normal if their

anatomy was similar to that of the mitral valves in 18 normalhearts obtained from patients comparable in age to thosereported in this study (newborn to seven years of age-table 1), which met the following criteria:'-" In a normal leftventricle the two papillary muscles arise opposite eachother, from points about two-thirds of the way from thebasal to the apical end of the left ventricular wall, as con-tinuations of interlacing trabeculae carneae. Some protrudefreely into the left ventricular cavity with few or notrabecular attachments; in others trabecular bridges tetherthe muscle to the wall over most of its length, or the muscleis bifid, trifid, or even a row of muscles attached to the leftventricular wall over a wide base. The tip of each muscle isfreely differentiated from the wall and both papillarymuscles receive chordae tendineae from both mitral valveleaflets. The chordae tendineae are stout cords of fibroustissue that radiate away from the tip of the papillary muscleto attach it to the valve leaflets. The majority of the chordaetendineae branch either soon after their origin or just beforetheir insertion into the commissures or free edges of thevalve leaflets, or into the rough and basal zones on the un-derside of the leaflets. The mitral valve leaflets are mobilefibroelastic tissue fashioned into a cone-shaped funnel.Antero-lateral and postero-medial indentations extendingmost of but not all the way to the valve annulus divide it intotwo major leaflets. The semicircular anterior leaflet has fewor no indentations in its free edge; it is almost twice as wideas the posterior leaflet, which has more indentations in itsfree edge. Each leaflet normally has a central margin, free ofchordae tendineae attachments, which can balloon outwardsto allow free flow of blood in ventricular diastole; but thefree margin of the anterior leaflet is characteristically muchwider than that of the posterior leaflet.

In order to determine whether the mitral valve was small,its diameter was compared with that of the tricuspid valveand with the length of the left ventricle in the samespecimen. The diameters of the mitral and tricuspid valveannuli were determined by measuring the circumference ofeach annulus (d = c/7r). The size of the left ventricle was

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CONGENITAL MITRAL VALVE DISEASE IN TGA

recorded as the distance between the base of the right cor-onary cusp of the aortic valve and the apex of the left ventri-cle, measured along the ventricular septum. Since fixedspecimens consist largely of muscle, caution should be exer-cised in interpreting measurements because some hearts atautopsy are dilated, whereas others are contracted. This maynot only influence the measurements, but some crowding ofthe papillary muscles and chordae tendineae could con-ceivably be induced. In this determination we also recognizethat the relative size of a valve or chamber may dependupon the presence of associated lesions, particularly in-tracardiac and extracardiac shunts. Even so, a mitral an-nulus was considered small if its diameter was less than0.280 the length of the left ventricle. In relation to atricuspid annulus, the mitral annulus was considered small ifit was less than 0.786 the diameter of the tricuspid annulus,and large if it was more than 1.2.

Results

The 163 specimens of TGA were divided into fourgroups: those with normal mitral valves, those withnormally formed valves but small mitral annuli, thosewhose only abnormality of the mitral valve wasrestriction of the free margin of the anterior leaflet,and those with anomalies of the mitral valve. Thedifferences in ages of the patients that comprised thefour groups were not statistically significant (table 1).

A

TGA with Normal Mitral Valves

In 38 specimens (23%) the diameter of the mitralannulus in relation to the length of the left ventricularcavity was within normal limits (table 1, fig. 1A).There were no abnormalities of position ormorphology of papillary muscles, chordae tendineaeor valve leaflets. In three specimens (all with a ven-tricular septal defect) the mitral valve was consideredlarge in relation to the tricuspid valve (table 1).

TCA with Normally Formed Valves but Small Mitral Annuli

In nine specimens (6%) the morphology of themitral valve was normal but the annulus was small inrelation to the diameter of the tricuspid annulus andthe length of the left ventricular cavity (table 1, fig.iB). There were no abnormalities of position ormorphology of papillary muscles, chordae tendineaeor valve leaflets.

TGA with Normally Formed Valves Except for Restricted FreeMargin of the Anterior Leaflet.

In 54 specimens (33%) the mitral valve wasnormally formed, except that some chordae tendineaearising from each papillary muscle were attached atthe midline of the anterior leaflet at right angles to the

/

Figure 1

Diagram illustrating the status of themitral valve in 163 specimens with TGA.Specimens in A-C do not have mitral valveanomalies; D-F represent underdevelop-ment of space between the papillarymuscles. In G the space between thepapillary muscles and the ventricular wallis underdeveloped, which results in thepapillary muscles being indistinctfrom thewall of the left ventricle. H representsspecimens in which the anterior leaflet isindented and attached by chordae ten-dineae to the ventricular septum.


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plane of the annulus (table 1, figs. IC and 2) so thatthe free margin of the anterior leaflet was markedlyshortened. In 16 of these specimens the diameter ofthe mitral annulus was small in relation to the lengthof the left ventricle. Six specimens had small annulicompared to the tricuspid annuli and three (all with aventricular septal defect) had a mitral annulus thatwas considered large compared to the tricuspid an-nuluis (table 1).

TCA Mwith Mitral Valve Aniomalies

In 62 specimens (38C%o) the mitral valve anomalieswere of three basic types: 1) underdevelopment ofspace between the papillary muscles; 2) un-derdevelopment of space between the papillarymuscles and the ventrictular wall; an1d 3) indentationof the anterior leaflet of the mitral valve and attach-ment to the ventricular septum.

Itliiclerdci>elo/)nici it ofO Spaee l 1etwcriPaipillarii l sc le.sThis was the most common abnormality

encotuntered, being present in 56 hearts (table 1). Inno specimens had the space between the papillarymuscles been so poorly developed that the twoappeared fused together, as occurs in association withcoarctation of the aorta.' In 21 specimens thediameter of the mitral annulus was considered smallcompared to the length of the left ventricle, in 15specimens it vas considered small compared to the

Figure 2

Vicii of left cintricrle sliocitlig a sntia(ll mitral aniiilus ard a calicthat is riorrrial/ly formed except for a restricteil freccmiargin of thnaiiterior leofilet fatoron). ITPJ postcro lateral papillary mrnsce;A.PIt: aritero lateral papillary irins lc

diameter of the tricuspid annulus, and in fivespecimens it was considered large compared to that ofthe tricuspid annulus (table 1). Three of these fivespecimens had a ventricular septal defect, and onespecimen had a large patent ductus arteriosus.

In 25 specimens the papillary muscles were adja-cent to each other, but not in apposition (fig. IF). Inall but four of these specimens the free margin of theanterior leaflet was restricted as described above. In31 specimens the two papillary muscles remainedseparate but were apposed. Since their bases aroseadjacent to each other along the lateral wall of the leftventricle (figs. ID, IE, 3) all 31 cases could be con-sidered examples of forrnesfrustes of parachute mitralvalve.' In 23 of these 31 specimens accessory papillarymuscles guarded the inflow tract of the left ventricle(fig. IE); this indicates that one or both of the apposedpapillary muscles were probably originally bifid ortrifid. In many cases it was impossible to identify withcertainty the origin of each column of muscle. In all31 specimens in this group, free margins were presentat tw) or more positions along the edge of the anteriorleaflet; each would have permitted some unrestrictedflow of blood from left atrium to left ventricle in ven-tricular diastole (fig. 3).

l tiderleteeloi)tynent of .Spiace Betta yee the Papillary Ju.scls and theVSentricular W`all

In two specimens (table 1), it was not possible to

Figure 3

uderderUeloped aspce betweeii pap)illariy minscies. Veten of lefttciiitri(c sh/totting restriction oj* the free rmargin of the anitterior

leaflu of i/ie mtitral value (middle arrow.) Additional free marginsare prcsentt btwceeii the bifid rniscular coiltrnrris ojl the postero-niedial p/apillaii/ rmscleic ,( )2and thic aiitero lateral papillary m712as-tIlc (3 .


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pass a probe behind either papillary muscle except at right subelavian artery passing behind the esophagus,their basal tips because they were tightly bound to the bicuspid aortic valve, anomalous pulmonary venousventricular wall (fig. IG). Although muscular columns return to superior vena cava, interrupted aortic arch,or bellies could be outlined in both specimens they and redundant tricuspid tissue causing subaorticwere fused to the wall of the left ventricle. Both stenosis each occurred in one specimen.specimens had a mitral valve annulus that was smallin relation to the length of the left ventricle and nlinicopathological Correlationsdiameter of the tricuspid annulus (table 1). In no case as mitral valve disease suspected on

,Xttiaclhitect of .\nfte(rior Icra/t of tlhc .Mitral Val/u to f/ic

In four specimens (table 1) an indentation in theanterior leaflet of the mitral valve was attached bychordlae tendineae to the ventricular septum along themargiins of a ventricular septal defect (figs. 1H, 4A andB). These indentations were similar to those noted insome specimens with ostium primum atrial septaldefect except that the point of attachment for theaccessorv commissure was more anterior along theseptum. Three of these four specimens were probablylike those with T(-IA classified by Layman andEdwards" as adherence of the anterior leaflet to theventricular septum, the specimens with mitral valveadherent to the ventricular septum reported by Elliottet al.' and those reported without TGA as straddlingor displaced atrioventricular orifice by Liberthson etal. 33The specimen in which the posterior papillarymuscle was unrelated to the valve orifice (fig. 4A) maybe comparable to one reported by Layman andEdwards" as counterclockwise rotation of the valve.The accessory mitral orifice in a fourth specimen (fig.4B) has been previously described also." Two of thefour specimens had a mitral valve annuilus that wassmall in comparison to the length of the left ventricleand three specimens had a mitral annulus that wassmall in relation to the tricuspid annulus (table 1).

Associated Lesions

At least one of the following atrial communicationswas present in each of the 163 specimens: patentforamen ovale, ostium secundum, balloon septostomyor Blalock-Hanlon septectomy. The ductus arteriosuswas patent in 100 specimens (61%), and ventricularseptal defects were found in 72 hearts (44%r) with the _highest incidence in specimens with underdeveloped Figure 4space between the papillary muscles and the ven-tricular wall (2 of 2, table 1). The distribution of the In AS ti anterior leaflet of f/ic niitral vale is iidcntced and attachedother anomalies within each group was not a it ide traj of elordae-like tissue to n trenlar sept 11

I1 {>/(JI~alog f/ic inlferior bo(rdeUr of a1 Xrl). I/icJ arroew poinlrts to f/ic ontfion.statistically significant (table 1). Thus pulmonary tract and /)widmonarii value. F'he posterinor papillary niiusc (PI'M)stenosis, due either to bicuspid or stenotic valve oc- aura /ichs directry to tfli va/lc neair tfi aLniiilis. B) In this sptei mcurred in 18 of the 163 specimens; subpulmonary f/ic inc/entfocI anft nor le(ifict is attaclhed by clordae tendlidnac to i/t'lstenosis in 23; coaretation of the aorta in 17; aneurysm rinterior margin of ri VS1). A bridge of val/un/ar tissue (arirot£c iso-

of the membranous ventricular septum in 6;jux, talts a portion of tJis in/entatioi as ani orijic e C)] lit mitraltapoesed atrial appendage in 6 (subject of a separate va/vt'l Ic postfcrnor paipil/ary nmis/cl P(PSI is inore laterallyiplaccdtaposed i/i~~~~~~~~~~~~~~~~~tannormnal aloong f/li'frt,t' wucll of i/t' 1efi vc'ntricle anid i/ic anteriorreport'4); single coronary artery in 4; right aortic arch papillary inusc/e is not s/lotn. nXtIailcrior lcaflet, iiiriat/lin 3; persistent left superior vena cava in 2; anomalous ia/it', . = left aitrial appendlagcCirculation, Volume 51, April 1975

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physical examination, nor were echocardiograms per-formed. Patients who had left heart catheterization tomeasure pulmonary artery pressure had no pressuredifference recorded across the mitral valve; angi-ography was reported as normal. Decisions to place aBlalock-Hanlon septectomy (74 patients) or balloonseptostomy (24), Potts (1) or Blalock-Taussig (2)anastomosis, and Glenn (2) or Waterston (1) shuntwere made on the basis of poor mixing of thepulmonary and systemic circulations or attempts to in-crease pulmonary blood flow in cases of pulmonarystenosis, rather than per se poor flow through themitral valve. There was no statistical difference in theincidence of palliative procedures between groups(table 1). Finally, no specimens had dilated orsclerotic left atria which would indicate the presenceof significant mitral stenosis or regurgitation. All thissuggests that the spectrum of mitral valve abnor-malities reported here did not result in clinicallysignificant obstruction during life.

Discussion

The incidence of mitral valve anomalies reportedhere is higher than that found in previous studies ofTGA. ' 12, 15 Although Lev et al.15 noted mitralstenosis or atresia in five of 147 specimens, theirspecimens also had common or single ventricle, andthus are not comparable to ours. Layman andEdwards" found the following mitral anomalies in 13of 88 hearts with TGA (15%): cleft anterior leaflet,adherence of the anterior leaflet to the ventricular sep-tum, counterclockwise rotation of the annulus andmalposition of the papillary muscles, accessoryorifices, parachute malformation, and filigree mitralvalve. The specimen reported by Elliott et al.'2 from agroup of 60 hearts with TGA had two cusps attachedto the ventricular wall like that observed in cases ofEbsteins' malformation and an anterior leaflet that in-serted into the ventricular septum, resulting in sub-pulmonary stenosis.What is the etiology of congenital mitral valve dis-

ease in TGA? In nontransposed specimens the high in-cidence of coarctation and aortic stenosis has im-plicated left ventricular outflow tract obstruction duringfetal life. Although valvular or subvalvular pulmonarystenoses were common in our specimens (25%, table1), their incidence was not increased in hearts withmitral valve disease. Another possible etiology isreduction of blood flow into the left ventricle duringfetal life so that the apex does not expand, thepapillary muscles are apposed, and the free margin ofthe anterior leaflet is restricted. Although un-derdevelopment of the space between or behind thepapillary muscles could follow a reduction in suchflow during the stage of systolic undermining of

presumptive valve tissue,'6 17 it is not clear whetherchanges in flow alone could account for the adherenceof the indented anterior leaflet to the ventricular sep-tum.

Mitral valve involvement in patients with TGA andleft ventricular outflow obstruction has been identifiedby angiography18 and at operation'9 and should be in-creasingly possible with echocardiography. Clinicalrecognition of this spectrum of anomalies would beparticularly useful in the management of infants withTGA who do not develop significant bidirectional mix-ing at the atrial level after balloon septostomy oratrioseptectomy, since it may further justify earlyprimary correction by Mustard's procedure.3' 20

Acknowledgments

The authors thank Lauren Sweeney for help in standardizingmeasurements and interpreting results, and for figure 1, and GarySterner for the photographs in figures 2-4.

References1. D xNIELSON- GK, MAix DD, ONGLEY PA, WALLAE RB, McGooN

DC: Repair of transposition of the great arteries bytransposition of venous return. J Thorac Cardiovasc Surg 61:96, 1971

2. ClA\KSON PM, BARRATT-BoYES BG, NEVTZE JM, LoMXXFE JB:Results over a ten year period of palliation followed by cor-rective surgery for complete transposition of the greatarteries. Circulation 45: 1251, 1972

3. STA.1K J, I)ELEV AL MR, WAJERSION DJ, GRAHAM GR, BON-HAMI-CARTER RE: Corrective surgery of transposition of the greatarteries in the first year of life. J Thorac Cardiovasc Surg 67:673, 1974

4. Cus\Ii.ii CGL, SOKOL DM, TiUSLER GA, MUSTARI) WT:Repair of transposition of the great arteries in 123 pediatricpatients. Circulation 47: 1032, 1973

5. RosENQis.ri GC: Congenital mitral valve disease associatedwith coarctation of the aorta: A spectrum that includesparachute deformity of the mitral valve. Circulation 49: 985,1974

6. DAVA(CIi F, MOLLEIR JH, EDovARDoS JE: Diseases of the mitralvalve in infancy. An anatomic analysis of 55 cases. Circula-tion 43: 656, 1971

7. LxAM JHC, RANGANATHAN N, WIcLE ED, SILVER MD:Morphology of the human mitral valve. I. Chordae ten-dineae: A new classification. Circulation 41: 449, 1970

8. RANGxNAIHAN N, LANI JHC, WICLE ED, SILVER MD:Morphology of the human mitral valve. II. The valveleaflets. Circulation 41: 459, 1970

9. ROBERTS WC, COHEN LS: Left ventricular papillary muscles.Description of the normal and a survey of conditions causingthem to be abnormal. Circulation 46: 138, 1972

10. RUSTlED IE, SCHIEFLEY CH, EDxx ARDS JE: Studies of the mitralvalve. I. Anatomic features of the normal mitral valve andassociated structures. Circulation 6: 825, 1952

1 1. Lx'.\IAN TE, EnxVARDS JE: Anomalies of the cardiac valvesassociated with complete transposition of the great vessels.Am J Cardiol 19: 247, 1961

12. Ei Liorir LP, NEIFELD HN, ANDERSON\ RC, ADAMS P JR,Eox ARDS JE: Complete transposition of the great vessels. I.An anatomic study of sixty cases. Circulation 27: 1105, 1963

13. LiBEIATI1SON RR, PAUL MH, MUSTER AJ, ARCILLA RE, ECKNERFAO, LEv M: Straddling and displaced atrioventricular


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orifices and valves with primitive ventricles. Circulation 43:413, 1971

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16. VAN MIEROP LHS, ALLEY RD, KAUSEL HW, STRANAHRA- A: Theanatomy and embryology of endocardial cushion defect. JThorac Cardiovasc Surg 74: 71, 1962

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18. SILOV!E ED, TAYLOR JFN: Angiographic and anatomicalfeatures of subvalvar left ventricular outflow obstruction intransposition of the great arteries. The possible role of theanterior mitral valve leaflet. Pediatr Radiol 1: 87, 1973

19. STARAK J: Primary definitive intracardiac operations in infants:Transposition of the great arteries. In Advances in Car-diovascular Surgery, edited by Kirklin JW. New York, Grune& Stratton 1973, p 101

20. TYN AN NI: Survival of infants with transposition of greatarteries after balloon atrial septostomy. Lancet 1: 621, 1971


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G C Rosenquist, J Stark and J F TaylorCongenital mitral valve disease in transposition of the great arteries.

Print ISSN: 0009-7322. Online ISSN: 1524-4539 Copyright © 1975 American Heart Association, Inc. All rights reserved.

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congenital mitral valve disease in transposition of the great arteries

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