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Mitral Atresia with a Large Left Ventricle and an Underdeveloped Or Absent Right Ventricular Sinus: Clinical Profile, Anatomic Data and Surgical Considerations

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Mitral Atresia with a Large Left Ventricle and an Underdeveloped Or Absent Right Ventricular Sinus: Clinical Profile, Anatomic Data and Surgical Considerations JACC Vol . 19, No. 7 1561 June 1992 :1561-76 PEDIATRIC CARDIOLOGY Mitral Atresia With a Large Left Ventricle and an Underdeveloped or Absent Right Ventricular Sinus: Clinical Profile, Anatomic Data and Surgical Considerations HIDETO SHINPO, MD, STELLA VAN PRAAGH, MD, IRA PARNESS, MD, STEPHEN SANDERS, MD, MARIAN MOLTHAN, MD, FACC, ALDO CASTANEDA, MD, PHD, FACC Boston, Massachusetts In mitral atresia with a large left ventricle, the tricuspid valve is evidence, or both, of tricuspid regurgitation was present in 14 either straddling and biveutricular or entirely left ventricular . T o cases (56%). learn how to assess the identity of the tricuspid voice in such cases It is concluded that 1) the Identity of the atrioventricular valves 15 heart sv oimms were examined as well as the echocardiograms is reflected in their chordal attachments ante aenurately than in of 10 living patients . When the right ventricular sinus was their leaflet morphology and dey : s;s primarily an the type of underdeveloped (11 cases), a straddling Iekuspid valve was ventricular loop present; 2) as a role, the tlitsrspid valve is present ; when it was absent (14 cases), the tricuspid valve was right-sided in D-looped and tuft-sided to Ldonped ventricles ; entirely left ventricular. 3) valve identity expressed as the number and position of the Regardless of biventricular or exclusively kit ventricular at- papillary muscle attachments is generally recognizable echocar- ta hments, the tricuspid valve was tricommissnral (at postmortem diographically and can be used to diagnose the type of ventricular examloadon or on echocardiography) in 22 cases (88%). Its loop that is present; and 4) the presence and degree of tricusald chordal attachments showed considerable variations but were regurgitation deserve attention when choosing oplimad initiative usually par'aseplal or on the ventricular septa) crest a conal surgery. septum . When biventricular, the tricuspid valve straddled (J Am Col Cordial 1992;19:1561-76) through an Intel ventricular septal defect. Clinical or anatomic As a rule, atresia of the mitral or tricuspid valve is associated Methods with extreme underdevelopment of the corresponding ven- Study cases and petlents. Among the 3,052 heart speci. tricle. Yet atresia of the mitral valve has been reported (1-7) mens from the Cardiac Registry of the Children's Hospital in to occur with a normal-sized or even a larger than normal left Boston, we identified 15 cases of mitral valve atresia asso- ventricle . In such cases, the single patent atrioventricular ciated with a well developed or enlarged left ventricle and an (AV) valve entering the morphologically left ventricle repre- underdeveloped or absent right ventricular sinus . In addi- sents the tricuspid valve. tion, we compared the morphology of what we considered to What is the underlying morphogenelic process of this be the left ventricular or straddling tricuspid valve in the apparent paradox? How can one differentiate a left ventric- cases from this series with the morphology of the left ular tricuspid valve from a left ventricular mitral valve? Does ventricular mitral valve in 72 postmortem cases of tricuspid a left ventricular tricuspid valve function normally? If it does valve atresia and with the morphology of the AV valves in 55 not function normally, what are the surgical implications and autopsy cases with a double-inlet left ventricle . therapeutic options in patients with this condition? This We also searched our computerized echocardiography study, which includes the largest series of patients with this data base for the diagnosis of AV or ndtral valve atresia rare cardiac malformation to date, attempted to answerthese during the years 1991 to 1990. We then reviewed the records questions. of those patients whose left ventricle was reported as normal or enlarged in size . On the basis of the apparent ventricular looping, as well as the morphology of the patent AV valve, we identified nine patients whose atretic valve was consid- From the Departments of Cardiology, Cardiovascular Surgery and Pathol- ogy, C.iidrcos Hospital, Boston, Massuhusetls. ered to be the mitral valve . All nine patients had a large left Menuw6l t remind July 29, 1991 ; revised maause,ipt received Novem- ventricle and an underdeveloped or absent right ventricular ber 14, 1991, accepted December 5, 1991. sinus; all nine are alive . One additional patient (Case 25, Adtaovrfor rcpdu5 : Stalls Van Praagh, Mn, children's Hospital, 309 Lonawood Avenue, Boston, Massachusetts 02115. Table I) was diagnosed angiocardiographically as having a 01992 by the American College of Cardiology 0735-1997192,55.01) Table 1. Mitral Atresia With a Large Left Ventricle : Anatomic Data, Surgical Procedures and Clinical Course in 25 Cases Csse PM No ., ClardseSegments PFO or ASD 06500 Cardlte Agree Opera=, Staideal Pmcedam and N No. Age,'Gendcr and Chambers (rose) Tricuspid Valve VSD (nor,) Grear Aredea Fhtdieg, Clmlml (rare, A. Let -Sided Mint A.-in I ASS-163, RAE . PFO snaddlingantIf,tMmeunissurel, AVC type and Sm ell PA, N apemtionn,Cyareo d severe dyspne, 2 days, M .all RV attechtS On RVFW, VS real ant CS mat, bP5 omteapan m Medical treatment . D and PMPM TV I(x 2~ Dy C72-107 (S,D,S), RAH+E, PFO, sepe 1° Soadmlng ant If, oicommissurat, AVCC,peobso Natmd Small FDA, coemheoo Noopemtion.Cyarmsisandseveredyspota, D 2.5 mo, M all RV. bulges , RA attaches an RVLW. VS crest TV BC tmnk SVTdurlngcandmcntbetenratioe,livedIn LAH, LVE and PMPM x 5) an Oa tent for 3 ma A73-78, (5,0,5), RAE. Sergtaol A5D Straddling an II, oicottMifsaral, AVC type oboe Mild ca arcs Car Iriabiatunr, eingda 2 mot PAR, (HF de-ad. 6 yr. A511 and 6.51eF em ill RV, (15 x IS) edandanr, attaches on RVSep, by TV LOrtst(um. repeGOfunespectedmrtrientatunr,anemp, LAH. LVE LVSepi. ALPM and PMPM, (19 . 16) inttamural LCA to repair a$ if it Were CAVC. Died 7 b Sy TR poatop. D Lmmg, (s,D,S), RAE, Large argical Entire)y into LV, triwmmlorord, Smell BVF, mid. MPA band FDA, COS mifira 25 mot ASR and PAR . CHF partly con5Olled; 8 yr, F ..la absent RV ASD mi1d7R )stake Isege a reengeede PAD migmtivn, te1Wtog is RPA 550001 a and 0 as, IAE, VSDCTV fbw .LSVC LPAhypcteeerdos.6'.leoroImage, PD.A LYE attathaxnts -etna- .RSVC-.RA OIdedwith etamvtSdnvimLPA0000 Out its loner pee_ foB from 701. 33. Mild TR. 7 yr: run RSVC to RPA anastoetoeis (Gkrm). Doing web 10 dq poelop. Living, (S,D,S), RAE, Lvg orpral EnurelymlaLVlravmmssuml, Small reotriatve Nuance LSVC to CoS, RPV5 8 mo to 17 yr. cyenalr dmrczad sranona, an 291St yr. M absent RV ASD re4erdanl,noTR BVF eorR )with dd~.17 yr. reeunem PAPA , quiteidier A LVH+E 22, LV 9S5Ao - S3%. ASR Lang biopsy = normal pd--y vessels 23 yr: pomp NVA preame - 10. 26 yr. attempted ~'oWlmmemy amslomosis. Imposeibk d perfonR blouse of nYSO«b on of 9PV5 d 28Rx yr. letetmitreot aotal Sseer W-Works.,k- .-as a prvxtr. MaKed, one cbad. b A6a-268, 7GA, (S,D,D), ASDN(6x3 .5) Srtaddlirgantlf,bOannannssurd, AYC bye abso An isthmus LSVC to ImgeBC =GS, No ration . Rapid deterioration dspgt 10 days, M RAE, 55100 RV. thick mdunamt Se, attaches oa by TV (S o 5) hypop os eiwogrsdve manes,. Ded 3 days aner LVH+E CS, VS erect and PMPM PDA cardiac ntheteNZataunt. 7 A71-111, TC2A,(S,D,D), Surgirnl ASD Sbaddlhg ant and eepret bs, AVCtyptand Puanonaey 2011 : ASR,Wete0055ehusLtmprovedfore 23 day, m RAE, Small RV. (73 x 16) tricanasssorel, shed, on posstte for C oleeela Rw days, died 22 days poetop . LAH,LVH RVFW, VS erestand PMPM of LV (9 x 7) 8 A81-50, DORV, (&DiD), sr Wca1 AS Straddling am std repeal bs, AVE type SOW'S SupemirMenar B em: ASR and PAR d imprtmmeoe of CHF 951, yr, M RAE, large, i if Rimmmsraml.ad, rote datee a (29 x 21) acquired v+atrirdes ",yr. modified Foneee proeemne RV ltd, Ifs, a on APM and PPM I rrostsoitidive =IPA diachargod 13 days pmmp, remand m 7 LAH+E RV, VS crest and Pk1PM of days dy_pneie, acitlak, b large pleural LV,TR Rus)orsand died 0 the ER. 9 Lsnng, TGA, (S,D,D). Large -pea! font pwvLS of Large AVC type MPA band LSVC to COS. RAVE 25 sent ASR and PAR . 5 yr. and syaorYs. 7'Vnyr,F RAE, amdtRV, ASD mst; vrodemte to severe "d-sad- mbdydetreaxdc,ceraaelok0Mry LAE, LVH TR superoinfreia SStayr..OASUSSestedaodovetsewn, venlddes LSVCeaeelamancdIOLPA .644,yrsubfo mu5maereeeoosisdpaasutegeadlene7R-a0. krrtyr, sobAO sbaosis remeeon W TV pGwy.Osrtop t5aeedtedd Clubbing persist. cy ..ah PAR' 10 Ltwng, TGA,15,D,D7, Saeeiod ASD Straddling, eeimremioseeral AVC type d ant BOA band 5 w«lestan. bounce acrid septost«ty. 7 mo, M RAE, Small RV, ex omens snows RA o 2, [A= tl . Blede abut -at rA, ledge erpeoeomyrtwdminRA=6,fA=9atd ,. C LVH+E kssedogo[(7IP.7mo:nthstmwsbfPA band gaediest - 70. RA= 7, LA = 12, PA - 25. ASR. Poaop PA - 18. ^o Table 1 . Conthwed C C- 17111~1~1 T6 ... p,d V,l- vsv( . .) G-1 A,..k, otlFindings CImICUICuvrx A. LAIRdvIMiwlAlrctir " TGA., ,, ,V~i 1, 1-fla-. L sL 1,1~W I. IL'S . PDA N. 7%cek.,: 1,11WA auiJl A, RA ml free IsubPO) count mud :nays mwr, wall MPA LSVC 1, C CoS n: 5511 and PAR; rapid do:eriurad,n RAE, uhsc^1 111 a 11 x hl 81',,IAO1 bA R"F, -1 in RA, small PDA poll MPANO -A, AIR -d fur 2 'F - ill, 1 mv.2", n then CW ccvncdr . AI I of .%T t Ginahem IIV' 11 tAL111 P,M'M.Tp, P,d,&1- S . LEE SLIl,,,.IASf) I-!tydALP, I .
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