Case Report Uncommon Type of Mitrallnsufficiency Caused by Perivalvular Communication Between Left Ventr icle and Left Atr ium H. D. S chulte. H. Gramsch-Zabe l, D. Horstkotte', and 8 . Losse' Dept . of Thoraeie and Cardiovascula r Surgery . I Cardlology. Pneumol ogy. and Angiology'c Helnrtch-Heine Universit y. Düss eldorf FRG

This case report descrlbes a patie nt with an uncomm on type of mitrat incomp ete nce ca used by a perivalvula r commu nicatio n betwee n th e Icft vcnt ricJc (LV) and the Ieft atrium (LAI mask ed by a considera ble flbr otic su bvalvular aorti c stenosts . ondoce rd itis an d con gestive hea rt Cailu re (CHF) . A 64 yea r old far mer with a history of a systolic murmur stncc childhood complaining ofi ncreasing fatiguc and dys pnoe. tcmperaturc ovcr 39 "C. a nd signs of eHF was admitted a nd tra nsferred to a ca rdiological unit. Invasive exa minatio n a nd continuing clinical deter ior at lon ca used ur gent transfe r far surgery undcr suspicion of a decomp ensat ed hypertrophie obst ructive ca rdiomyopathy. Clinical investigation revca led a decomp ensat ed subvalvula r aortic ste nosis a nd a mild mit ral insu fficiency. At surgcry the advanced flbrotic subvalvular stenosis was resectod. After coming olT bypass severe mitral insu fficiency was detected by intraoper ative a nalys is of the simulta ncous intracavita ry-pressure tra cings. A midsystolic maximum of a high v-wave of the Le-pressure tracing was suggestive of a n unusu al reaso n of the mitral insufficicncy. Heexploration indicated a periv alvula r broad communication from the LAgroovc to the LVwith a n otherwisc normal mitral valve. The communica tion was closed using buttr esse d mat tress-sutures .This un common type ofmitra l incom pete nce via a perivalvular LA-LV communication was proba bly ca uscd by endoca rditis a nd a n intr amyocar dial abscess in the l.Avwall which sub endoca rd ially led to a LV-LA communication. Key wo rds Subvalvular fibromuscula r aortic stenosis - Mitral Incompetence - Endoca rditis - Myocardial a bscess - Congesti vc heart failure

Introd uelion Infective endocarditis of nat ive heart valves and accornpan ying perivalvular intr arnyocardi al abscesses play a major role at the site of the aortic valve. Usua lly acute aortic insufficiency, continuing ra ised temperatures, embolization o f infected pa rt icles of veg etations m ake n ecessary ea rly

surgical interventi on including aortic valve replacement.

Ein Patient mit eine r ung ewöhnli chen f orm einer häm odyna misch wirksame n MitraIins uffizienz. bedin gt du rch eine linksventrikulä r Iinksatr iale Kommunikation bei inta kten Mitralklappe nsegeln und Aufhängeappara t. wird vorgestellt. Das klinische Bild wurd e überdeckt durch eine hochgradige. Ilbrct isehe. subvalvulä re Aortenstenose. eine a bgela ufene Endokardi tis und eine globale Herzinsuffizienz. Ein 64jä hriger Landwi rt mit einem seit Kindheit beka nnte n systolischen Gerä usch wurde wegen zune hme nder Müdigkeit und Dysp noe. Temp eratur en über 39 °C und Zeiche n der Herzins uffizienz a ufgenomme n und kard iologisch unter su cht. Unte r der Verd achtsdia gnose einer dekomp ensierten hypertroph isehen obstruktiven Kardiom yopath ie wurd e der Patient zur dringlichen Operatio n verlegt. Es erga b sich der Verdac ht auf eine s ubvalvuläre Aorte ns tenose und eine geringgradige Mitralklapp eninsuffizienz. Bei der Oper ation fand s ich eine hochgradige. flhrotlsche, subvalvulä re Ausflußb a hnste nose links mit Einbez iehung des aortal cn Muralsegels in de n narbigen Proze ß. Die Simultand ruckmessung im linken Vorho fund linken Ventrikel nach EKZerga b eine erhebliche Mitralinsuffizien z, die eine sofortige Revision der Mitra lklappe er forderte. Es fand sich eine fast Zcm breite Kommunikati on zwischen linkem Vorhof und linkem Ventrik el durch Ablösu ng der Kla ppe vom Klapp enring und von de r LV- llinterwand. Ober Teflonstreifen wu rde die Kommu nikati on mit Mat ratzen -U-Näht en vers chlossen . Der weitere Verlauf wa r kompl ikat ionslos. Diese ungewöhn liche Form einer Mitralinsu ffizienz durch Entwicklung einer perivalvulären Verb indung zwischen Vorh of und Ven trikel wa r vermutlich verursacht du rch eine Endoka rdltis mit Abszeßbildung im Bereich der Klappenb asis und sekun där em Anschlu ß an den linken Ventrikel. Die ..Mitraiin suffizien z" wurd e durch die subvalvul äre Ausflußb ahn stenose maskiert und durch Druckkurv enanalyse nach der Korrektur in ihrer Auswi rkung entdeckt und effektiv korrigiert.

und a bscesses can ca use difficult surgical pr oblems both during and after surgery. Similar alteratio ns ca n be seen at th e mitral va lve , wh ere a cute inte rve ntio ns a re n ot so fre -

quent. However , extended perivalvular calcifications of the valve rin g. atri al and ventricula r m yocardium , a re p robabl e

signs of ea rlier local infections and a bscesses. This is a report on the c1inical and surgical treatm ent of

Simultan eou s s u rgical treatment of p e riv al vula r in fec tio ns

a n un common ty p e of mi tral inco m pc tc nce ca used by a ventricu lo-atria l p e r ivalvu lar com m u nica tio n .

Thorac. cardiovasc . Surgoon 38 (1990) 33-35

Hecei ved for »u hlication : Sep te mbe r 2Y. lf)S9

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Geo rg Thlemo Verlag Stuttgart -NewYork

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Summary

Unge wö hnliche For m eine r ~1i tra li ns um7.ie n7.. a usg elöst durch eine perivalvuläre Ver bindu ng zwische n link em Vent rik el und linkem Vorhof

TlIOW c.

11. l). Sc hulte. 1I. Gramsch-Zabet. Lutlors tk otte. IJ. Löss e

m rdiom sc. S' llrg('(U/ 38 (1 990)

Case fIeport A 64 yea r old farmer 072 em. 92k g body we ightl was sent to his loeal hospital beeaus e of inercasing dyspnoea , fatigue, loss of 4 kg body we ight, temperatures over 39 °C. and cJ inieal signs of conges tive heart faHure. Since childhood a cardiac defect lsystolic rnur mur) was know n of but nevor followe d up because of the patient's good eondition. Two weeks after admission a nd after ge ne ral and a ntibiotic therapy the patient was transferred to a eardiological un tt for furt her invasive diagnosis and therapy. A positive blood cultu re (Staph. aurcus) could be obtained. The septiecm ia was treated with tested antibioties. Do ppler-sonography revealed a minimal mitral regurgitation. The preliminary diagnosis were : congest lve heart Iailure. congestlve pneumonia, and susp icion of deeompensated hypert ruph ic obstructive cardiomyopathy (IIDC~tJ , mitral insufficiency . pulmonary hypertension . After eatheterization and angioeardiography the patlent deteriorated cJinically despite intensive medieal therapy. Under the suspicion of a deeompen sated I IDeM the patient was transferred for urgent surgica l therapy

(Tablc 1) . Clinical Fiudi nqs After admission to our intens ive care unit the patient was hypotonie (80/ 50 mmHg) and hypovolem ic. He had low pota ssium (3.6 mm ol/Ll, sod ium (139 mrnol/L), a nd hem oglobin (100 g/ L) values . The cent ra l ven ous pr essure was 2 mm ll g. With substitution he slow ly recovered. At examination his condition wa s reduced without peripheral ederna, venous inflow congestion. and dyspnea. There wer e a holosysto!ic pr ecordia l murrnur. a heart rat e of7 5/ min , a blood pr essore of BO/90 mm llg, sin usrhyth m, an incom plete left bundle bra nch block, a nd signs of 1eft ventricular hyper tro phy. Echocardiogra phy show ed a wa ll and se ptum hypertrophy, and a mid ventricular obstruction , but no sys to!ic an terio r movem ent of the anterior mitr al leaflet, and the suspicion of a subvalvular fibrotic membr ano us steno sis was mad e, In phonoca rd iogra phy-tra cings th ere was a holosystolic murmur with an inconstant double maximum above the ERB point. Also tbe caro tid pulse traeings were completely atypical for HOCM. Because of the unel ear c!inical situa tion recatheterization was performed, dem oristrating a subvalvular fibrous and mid ventricular LVobstru ction with a low-grade rnitral ins ufficiency,

Before Surgery

RA RV

PA

LA

LV

Apex Aorta LVp." Aop." Clinical dtagnosis

4

54/0 -6 55/29

28{PJ 119/0- 37 176/5 -33 113172 186/0 Brockenbrough + HOCM (atyplC,l) Mitrahnsufficiency1° Pulmonary hypertension

lntraop. before correctlon

un usu al mid systo lic V-wave peak of about 23 mmll g in the LA-pressure tracings . The inspection of the aortic valve dem onstrat ed ba sic calcifica tions of the left coro nary and non- cor ona ry eusps and a eonsiderable subvalvular fibrotic membran e attached to them and extending to th e aorti c mitral leaflet , causing cons iderable thickening and reduced mob ility of it. No signs of an acute infection could be obse rved. The complete fibrotic partly-m em bran ous tiss ue wa s ca utiously removed both from th e ao rt ic cusps and the aort ic mitra l lea flet thus open ing up th e subvalvular a rea, freeing the ao rtic cusps an d the othe rw ise norm al-Iooking anterior mitral lea flet. However, there was a severe muscula r se pta l hype rt rophy obs truc tin g the LV outflow tr act. Th erefor e a transaortic subvalvul ar se pta l myectomy wa s perform ed . After rep eated rinsing of the LV ca vity and under rewarming the aortotomy was closed by continuous suture. Reperfusion was continued for 20 minutes and the pati en t wea ned ofT ECC u nder LA-pres su re control, 1J 0wever, in adequate cardiac output the LA pressure cons ide rably increas ed. Simultaneo us pressure-tracings after ECC dem onstrat ed a good relief of the left ventricular outflow tract. Howeve r , the tr aeings of th e left atr ial and left ventricular pressures demon strated a severe mitral incompetence with a midsystoli c pea k ofnearl y 40 mm Hg. These findings lead to a furth er exa mina tion of th e mitral valve via the LAafter going on bypass again. Insp ection of th e mitral valve showed light fibro tic thickening of all st ructures (lea flets , chordae) but no signs respo nsible for mitral insufficicncy i. e. prolonged chordae , bulgin g leaflets , Parallel to th e mitra l va lve ring along the mid -pa rt of th e mu ral mitralleaflet a elea n 2 cm long and 8 mm deep groove was found wb ich seem ed to be the res ult of an intramyocardial now- was hed-out and cleaned abscess . After cautions lifting of the thickened endoca rdial layer towa rd s the mitral valve ring (ab out 15 mm distan cel a bro ad subvalvula r communication to the LV was visib le. As th e mitra l valve leaflets an d the chordae were normal the elosu re of the subvalvular LA-LV comm unica tion was performed by using 5 Il-mattre ss-sutures secured by two teflon strips with out a ny imp airment oft he mit ral valve function. Th e postoperativ e course was com pletely uneven tful a nd tbe patient wa s retransferred to his local hospital 16 days later an d after 3 ad ditiona l weeks of elinical reh abilitati on he went horne in good physica l and elinical condition.

Intraop. after myectomy

Intraop.

10/3 4011-4/10

12/3 43/0- 3/ 10

21/3 126/0 - 19

39119 8010-20

16/3 92/0 - t 8

74/44 138/0 64/36

68/32

88132 9010 80/26

12/0 4013-8

Severemitral mcornpetence

after MV reconstr.

Pre-and intraoperative hemodynamicdata (mmHg)

Table 1

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:~ 4

Uneammen Typ e 01 Mltm l Insufficiencu Caused by Periva lvular Communtcatton

Mitral insufficiency of different degree in pati ents with hypert rop hie obstructive cardiomyopat hy can be demonstrated in most ofthe patients angiographically, which usually affords no additional thera py and only exceptiona lly makes additiona l surgical treatm ent necessa ry. In pat ien ts with subvalvular fibr omuscular or mem br an ous ao rtie stenosis the extens ion of the fibrotic str uctures to the an ter ior mitral leaflet may cau se a considera ble redu ction of leaflct-mobility and thereby an incomplete systolic elosure oft he mitr a I valve. An acute endoca rditis may aiso exten d to the mitr al valve and cause leaflet or chor dae related insufficicncy.ln our patient the systolic murrnur caused by su bvalvular fibr omembran ous stenosis was known since childhood but never followed up or investigated because the patient always was in good physical condition. Most patients become elinical relevant by the age of3 0 yea rs or at over 60 years as in our case (8, 9, 13). In case of acute infective endoca rditis of the a ortic valve perivalvular a nd myocardi al a bscesses may develop and are often very resistant to antib iotic therapy (I, 3, 5, 12, 13). Sometimes rhythm disturban ces such as different types of intermittent av-blocks indicate ea rly intramyocardi al abscesses . In othe r cases angiogra phy or two-dimensiona l echoca rdiogra phy demonstrat e perivalvular excavations or sinus of valsalvae perforations to the right atrium or right ventricle includin g extension of the endocarditis to the tricupid valve (I , 6, 7, 10, 11, 12). Similar acute destructions of the perivalvular region of the mitral valve seem to be very ra re. Amett and Roberts (2) reportcd findings of 74 necro psy patients with infective endoca rditis includin g 22 aorti c and only 2 mitr al ring abscesses. Recently Borouisk i et al. (4) published a c1inical case of asymptomatic myocardi al abscess in the posterior LA wall ncar the mitral valvc ring. Other complications such as rup tur e and pericarditis, bleeding and pericardi al ta mponad e, and heart failure or sepsis were report ed as single cases (7, 14, 15). But a communication between the LV and LA following a myocardi al abscess of infective endocarditis as demonst ra ted in our case was not publish ed as far as we know. Only Horstkott e et al. (8) mentioned the possibility of partial mural mitral valve detachment from the valve ring du e to infective endocarditis or myocardi al ab scess. The clinical and intr aoper ative course of this case demonstrates the necessities to clear up preoper atively history, symptoms and hemodynamics accur ately and to control intraoperatively before and after correction the hemod ynamic condition of the pati ent by accura te simulta neous pressure tr acings of the right and left hea rt cavities .

35

References Abe/a. G. S.. B. Majmudar, and J. M. Pelner : Myoca rdial ab scess unassociated with Infect lve endoca rdiüs . South. med . J. 74 (19 81) 432 -43 4 2 ArT/eU. E. N.. a nd W. C. Roberts: Acute infecüve endocarditis: A clinicopat hological a nalysis of 137 nccropsy paüe nts . Curr. Probl. Cardiol. 1 (1976) 3 3 ArT/ eU, E, N. , and W C. Roberts: Valve ring a bsccss in active infective endoca rditls: Fre qu en cy. loca tion. and clu es to clinical dia gnosis from the study of95 ne cropsy patients . Circ ulati on 54 (1976) 140 ~ Borkow ski, 11. . 11. Korb. H. voth. a nd H. H. de Vivie: Asymptc matic myocard ial a bsc ess . Th orac. Cardiova sc. Surgeon 36 (19 88 ) 338-340 5 Buchbinder. N. 11., a nd W C. Ronens: Left-sided valv ular acti ve endoca rd itis. Am . J . M ed . 5 3 (19 72) 20 b Fin/ey. H. W , a nd J. 1. Mar c: Ana e robic myocardia l a bscess following myo card ial infarction . Am. J. Med. 78 (19 75) 513 - 514 Gladd en. J.: Myocardial a bsc ess with perfo ratio n of thc hea rt fcl lowin g sta phy lococcal pyemia . Am. J . Surg. 60 (1943 ) 227-2 85 l:I Gross, H.. M. Roth kopf a nd If. Chm e/: Myocardial a bscess ca used by Stre ptoco ccus agalaetiae : Success ful d iag nosing a nd trea tment. South. med. J. 74 (198 1) 1001 -1002 v Horst koue. D.. F. Loog en. a nd W. Bircks: Erw or ben e Her zkla ppen feh ler . Ur ban- Schwa rze n berg. Münch en -wle n -Haltimo re 1987. Infektiöse Endokard itis . pp . 149 - 160 10 Karp . H. B.: Role of surgery in infective cndoca rditis. In; C. D. McGoon IEd. ]. Card iova sc . Clinics 12/3 Ca rd iac Surge ry. F. A. lJavis Com pa ny Philade lphia 1982. pp . 157-1 75 11 Nakamura, K. . S . S uzuki, G. S ato mi. 11. lIayashi. a nd K. t llrosau:a: Detecti on of mitr a l ring absccss by two dimens iona l echoca rd iogra phy. Circula tion 65 (1982) 816-8 18 12 Robert s. W. c.. a nd N. 11. Buchbinder: Hight sid ed valvula r infective endoca rditis. Am. J. Med . 5 3 (19 72 ) 7 13 Roberts. W. c.. a nd N. A . Buchb ind er. lIeal ed left-sided infoctive endoca rditt s : A clinicopathological study of 59 patients. Am . J. Card iol. 40 (19 76 ) 8 76 1~ St ier te. 1. Pot nüz, D. Meyer. 11. Sheikttzadeh, a nd K. W. lJiedrich: Valvulä re Herzerkran ku ngen. IV. Die valvu lä re Aortenstenose • Teil 1. He rz/K reisla uf 19 (987) 58 5-59 0 15 Varqa s-Borron . .I.. G. Pop, C.Keims, F. Auie. a nd 1. Bsc uioai-Aotla: Two d ime nsional echocardiographic dia gnosi s of myoca rdial a bsces s com plica ting ventricular se pta l defcct . Am. Ilea rt J. 109 (1985 ) 1109-11 10 16 Weiss, S.. a nd R. W w ilkins: Myoca rdial a bsc ess a nd perfor anon of the heart. Am . J . Med. 194 (19 27) 199-205 1

u..

Prof Dr. med.1l. D. Sc hulte Chirurgisc he Univ .-Klinik Abte ilung Tho rax- und Ka rdiova sk ula rch irurgie Moorenstraße 5 D-400 0 Düsseldorf

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TllOrac. cardiovasc. S urgeo1l38 (l9 90)

Uncommon type of mitral insufficiency caused by perivalvular communication between left ventricle and left atrium.

This case report describes a patient with an uncommon type of mitral incompetence caused by a perivalvular communication between the left ventricle (L...
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