Case Report Right-Ventricular Hemangioma Ca using Ser ious OutflowTra ct Obstruction 1\'. Reifll . P. Theisst',,;'. arid W t eoux de Lacroir I Clinic rur Il..a n Su rXO'I)-

Insuun.. OrOi nica la nd Expertmerual S udear :\I,"did n,' ) (),'pannlf'1I1 ofP ath " IrIlO". lI niw· rs it,· orCologn f". r....Iogn.,. (~'rmany l

:'\iah ez u totale Obstruk tion dt·s r ectu cn ,\usnuBtr akl es

" 2S-yl'a r-old woman ha d a pa rt ially nc nexertio na l dyspnea and a mu rm ur of pulmona ry ste nosis. Echocard iograp hy a nd magnetic res onance imaging revealed a mas s ca using a nea rly completely pulmon a ry ouulow -rract ob st rucuc n. The mass wa s success fully resected and the pa tho!ol!:ical diagnos is of a mixed ca pillary a nd caverno us pulypuid hem angi oma was made. Thi s case rep rese nts one of the very rare cas es of a ngh t-ventr tcular hemangioma in ad ults in which obst ruction of the outflow-tract was initially evident. Non-in vas ive diagnosis a nd a n explora tory operation with re section is th e proced ure of choice. 1' (') ' words

Card iac hema ngioma - Rill:ht-venl ricula r ou mow-tracr obstruction

Introd uct ion Hem an giom as a re the co mmonest vascular tumo rs a rising from th e heart but acco unt for only a pproxima tely 5% of all be nign ca rd iac tu mor s in adults 11, 6). Fewer than (ifty cases have been descr ibed in the litera ture 0 , 2, 5, 7, 8), the majority having been diagnosed at auto psy. The nu mber of re port ed right-ventricular hem ang iomas is very ra re; in a se ries of 56 prima ry tu mors of the right ventricle that could be identified histologically only two (3.6 %) we re hemangiomas (2). We report a case of an he man giom a causing r ightventricular outflow obs truction in a zx-yoar-old woman. According to the literature this is one orthe very ra re cases of a righ t-ve ntricula r hemangioma in a dults, in which at the time of diagnosis major outflow-tra ct obst ruction was present.

The pa tient w as adm itted to the University lIospita l for evaluation of a ca rd iac murmur referable to a pulmonary ste nos is. The murmur was first noted I liZ years previously in a routi ne examination . On admission the patie nt reported shortness of breath ener exeruon a nd the recent onset of nonexeruonal dyspnea. On au scultation there w as a low first sound and a nonna lly sp lit seco nd sound. furt her a long grade 3-4/6 systolic murmu r at the l('fi uppe r ste mel border . The remai ning physica l exa mination revealed no rmal cond itions. Houtine la borat ory values we re within normal limits . Chesl radiogra phs de monstrated a norm al -sized hea rt a nd normal pulmon a ry cond itions. the etcc troca rdtogra m showed a regular rhythm a nd no pat hological cha nges . Thorac. ca rdiovasc. Surgeon 39 (I 99 11234 - 23() (l Grnr" Th il' IllI'

Verlag Stuttgart · New York

du reh efn Hamagtom

Elne 2Hjiih rige Pauentin bot die Sym pto me ein or teilw eise bela stungs unabhiingigen Dyspnoe und ein mit etner Pulm onals te nose zu vere tnbarendes lle rzger au sch . Erhoka rdiugra phle und Kem spmtomograp hie zclgte n cine Masse im rectn c n v e ntnkel. dte zu em or nahezu kom pletten Obslruktion des re cht en AusnuBbah ntra kles Iiih rte . Die Ma sse wurde erfolgreich resezte rt und es wurde die patho logische (Jiagn oS\' etnes gr mischt kapilla r ·kav('rn oS('n Ham angtom s gl·std lt. Dieser Fall stellt eln en der aull('rst selte nc n Faile einos Iliimangioms im Frwac bsenenalter da r, b('i dem zum Zeitpunkt der Dlagnose bere tts r-ine na hez u vollig(' verlegung drs rechwn Aus nuBbah ntraktf>S vorla g. x tcht .mvastvc Diagnost ik und etnc explora tive Operation mit Hesckuo n ist das Vcrgehen dcr Wahl.

2 ·lJ Echocardiographg, conti nuous uore and color-coded Doppler 2-1l Echoca rd iogra phy IFig. 1) showed an enddiastolicallycnlarged

righ t ve ntn clc with mcre esed wall th ickn('Ss an d mar kedl y decrea sed wa ll motion . In the right-ventricular cu ulow-t rac r m VOTI IIl' fi-\'en tricula r sha n ax is) a n Intracardlac ma ss tdirnen sions 3 x Scm ! could be imaged. This mass occluded the RVOT in systole. It wa s not poss ible to defineth e r elationship between pulmo na l valve a nd the RVOT-mass . In continuous -wa ve lCW} mea sur eme nts a r ight-ven tricula r pressure of xnmmt [g could be deter mined . In color-coded Doppler a regurgitation on tricus pid valve of K ra d l~ I I- I[] was observed. All the oth er ellrd inc structures exhibited normalflndtngs .

I1ipla1le tronsesophoaeol echocardiography Biplane trenseso phageal echoea rdiog ra phy s howed a pro nou nced ('nlarg('d enddtastolic rig ht ventricle with Increased wa ll thickness a nd decreased wa lt contractility. Th e RVOT was nearly completely occluded by a tumorous mass. Th is mass infiltra ted th e proximal third of the interve ntr icula r septum UVSI with a basis of abou t 2 x Zcm . The pulmonal valve seemed not a nccted .

Magnetic Resonance Imaging (,' fR/J In MHI IFig. 2 un d 3) a low-grade insufflciency orthe lri cus pid valve was obse rved. the right at rium and the right ven tricle were mod erat('l)' dilated. Punherm ore. in the right vent ricle a mass with a max imal radius of 30 mm originat ing from the IVS could be Idc ntifil'd. Durin g sys tole th e lume n of the HVOT was nearly occluded a nd tu rbule nces in the tr uncus pulmonalis we re evide nt.

Bt'l ·t'iH'd fur I'ubl k a ti u n : Ilf·t·f·m bt'r 17, I t)t)O

Downloaded by: National University of Singapore. Copyrighted material.

Sum ma r)"

TIIome . C1I rdiol'Gsc. Surgeon .1') (199 1)

Fig. 1 2-0 EchocardiographyshowInga mass obstructingthe right-ventricular cuttlcw.tract

S urgical procedu re After media n ste rnotomy the pericardium was opened. The heart in total wa s of nor ma l size, whereas th e right ventricle appeared qui te hypertrophied and showed a bulge in the region of the IIVOT. Total ca rdiopulmo na ry bypass was initiated with systemic hypoth ermia . The aorta was cross-cla mped a nd cardioplegia was given . An explora tion of the right ventricle was made via the righ t atrium . A mass with a smooth surface measuring ap pr oximately 30 x 30 x 3Smm wa s see n arising from the centre of the ventr icle sept um. The tumor's pedicle of attachment measured 20 x 20mm. The mass was completely excised together w ith a margin of Smm of healthy tissue from the septum. The a rea ap pearing extremely thin after rese ction wa s covered with a da cro n pa tch , The patien t recove red well from the surgic al procedure a nd rem ained asy mptomatic.

Fig. 2 Nuclear-magnetic-resonance imaging ab antercbaset myocardialsegmentof lett ventricle TU tumor Ao aorticvalve LA let! atrium R fight o dorsal

/listology

Macroscopically the excised mass was spongy in cons istency and encapsulated in a part ially transparent membra ne showing variegated colors ranging from white to grey, Light microscopy showed a tumor consisting of ca pillary vessels partially with large vascular spaces. In the interstitial tissue chronically inflammatory cellular infiltration was demonst rable . In summary, the histological examination revealed a mixed capillary and cavernous polypoid hemangioma (Fig. 4), An essential inflam mation characteristic of a gran ulation tissue rich in vessels could not be dem onstrated. A rnyxoid matrix or so-called lepidic cells typical for a cardiac myxoma could not be foun d. Discussion Primary hema ngioma of the heart is an extremely rare conditlon. In the largest series of primary tumors of the heart, reporte d by ,'l--f cA flist er and Fenog lio (3), 15 out of a total of 3 19 tumor s were primary hemangiomas. These lesions may involve every layer of the hea rt and have been described in all heart cha mbers an d the pericardium (61. ln general, only

Fig. 3 Naclear-megretic-resonence imaging Ao aortic valve RV rightventricle LA left atrium o dorsal PA pulmonaryartery

235

Downloaded by: National University of Singapore. Copyrighted material.

Right- Vent rirular /I t'lI/ungioma CUlisilig Serioll .~ OIltj701l'-Tract Obstruction

N. ReiP. P. Theissen. and W. Feaux de Lacroix

Thore r , cerdiQI'esr, SurgeO/l 39 ( 1991)

lead to the correct diagnosis of a cardiac tumor. Ctneangtog raph y possibly demonst rating filling defects or abnormalities of wall motion is not required. so that in the majority of cases invasive exa mina tions can be omitted.

References 1

l

J

• Fia. 4

HIStologic !UITllOal IOllf!V!aled a 1TlI~!d capiIaI'y and cavernous ~

""" """"""'" 40 % of the cases cause varying clinical symptoms, which depend on the size an d location of the tumor (5). Hernan gioma of the right ventricle causing pulmonary infundibular obstruction is an extremely rare en tity. Remarkab le is that despite a nearly total obstruction of the RVOT in systole. hemangiomas in this location may appear nearl y asymptomatic. Our patient revealed only nonexert iona l dyspnea and a systolic murmu r at the left upper sternal borde r. The same symptom constellation was found in two othe r patients with evidence for right-ventricular outflow-tract obstru ction due to an hemangioma (2, 4). A thorough noninvasive evaluation including the difTere nt modes of echocardiograph y and magnetic resonance imaging ....:i11

s • 7

I

Baroldi. G.• F. Colombo. and W. C Motion: Benign pri mary he mangioma of the right at rium of the heart, Med. Ann. OC 36 11 967) 287-289 Ihla!l~, J., P. Crossord. et C A. S~n~ur: Lestumeurs primi tivesd u venmdes droi l. A propos d'u n cas d'hemangio-end otheliome ben in open! avec succes.J . Med . Lyon 53( 1972) 483-494 McAlliJter. JI. A__ and J. J. Fenoglio: Tumors of the cerdtovascular syste m. Atlas of tumor pathology. Fasicle IS , seco nd se ries . Was hington OC, Armed Forres lnst inn e ofPatholog}' Nicl:s. R.: lIamano ma of the right vl'ntride. J. Thorac. Canliovasc. Surg. 47 fl%41762-768 ""orit:l:y. D., A. G. Ros~, J . A..\lorgan, and C N. Barntmi· Prima ry ca nliac hemangiomas. A repo rt of two cases. S. Afr. Med. J . 66 119841267-270 SUrerman. N. .4.: Primary ca nliac tu rners . Ann . Surg. t 9 1 ( t 980) 127-1 38 S~rman, "'- 5., IV. H. Plou th. K. J . Winn, G. C For~st. C. R. Jlotch~r, and J. D. Sinh: Hemangioma ofthe right ventnde cau sfng outIlow-tract obs truction. J. Thorac. Canl iovasc. Surg. 96 (19881 307-309 Sulayman. R., and D. E. Casstls: Myoca rd ial coronary hemangiomatous tumors in children. Chl'S1 68 119751113- 115

Dr. med. N. Rt iP

Oinic for Heart Surgl'r)' Un tversny ofCo lognl' Jose ph-Stelzman n-Strage 9 W-5OOO Koln 4 1 Germa ny

Downloaded by: National University of Singapore. Copyrighted material.

236

Right-ventricular hemangioma causing serious outflow-tract obstruction.

A 28-year-old woman had a partially nonexertional dyspnea and a murmur of pulmonary stenosis. Echocardiography and magnetic resonance imaging revealed...
488KB Sizes 0 Downloads 0 Views