regurgitation treated by conservative surgery. fibromuscular subaortic ...

Quadricuspid aortic valve associated with fibromuscular subaortic stenosis and aortic regurgitation treated by conservative surgery. A Iglesias, J Oliver, J E Muñoz and L Nuñez Chest 1981;80;327-328 DOI 10.1378/chest.80.3.327 The online version of this article, along with updated information and services can be found online on the World Wide Web at: http://chestjournal.chestpubs.org/content/80/3/327

Chest is the official journal of the American College of Chest Physicians. It has been published monthly since 1935. Copyright1981by the American College of Chest Physicians, 3300 Dundee Road, Northbrook, IL 60062. All rights reserved. No part of this article or PDF may be reproduced or distributed without the prior written permission of the copyright holder. (http://chestjournal.chestpubs.org/site/misc/reprints.xhtml) ISSN:0012-3692

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We have not noted loud diastdic murmurs across this prosthesis in the mitral position in any of o w other patients with the prosthesis, and, given the very favorable hexnodynamic features of tbe valve reported elsewhere and in our own srperience, a diastolic rumble would not be expected. Thrombus formation presumably occurred in the immediate patoperative period before adequate control of prothrombin index was obtained. We suggest that a significant diastolic murmur across a St.Jude prosthesis in the m i t d position is an early sign of valve malfunction and an indication for f t u o ~ p i examination c to ensure wnnal l d e t motion. ACKNOWDCMENTS: The authors wish to thank the Medical Superintendent of Groote Schuur Hospital for permission to kblish.

Fp;wa 1. Quadricaspid aartk valve noticed dnrfng oper-

ation. 1 Emery RW, Palmquist WE, Mettler E, et al. A new cardiac valve prosthesis: in vitro & Am Soc Arti6c Intern Org 1978; 24550 2 Status repvrt No. 7. Cardiac valve, clinical evaluation programme. S t Jude Medical Inc. 3 Emery RW, Mettler E, Nicold DM. A new cardiac prosthesis: the St. Jude medical cardiac valve in vivo resalts. Circulation 1970; (supp1)80:1-48,154

Quadricuspid Aortic Valve Associated with Fibromuxular Subaortic Stenosis and Aortic Regurgitation Treated by Conservative Surgery* A l f m I g h M.D.; lase O h m M D . ; J.E. MuAo;t M.D.; crnd Lub Nt&z M.D.

A 42year~ldman was admitted to th6 hospital because of progressive dyspnea and substernal pain of two ywa' duration. On physical examination, he was in no distra9s. His blood pressure was W)/Smm Hg, and his pulse was 87 beats per minute. On d t a t i o n , there was a systolic ejectb murmur, grade 3/4 and a diastolic munnur, grade 3/4 along the left s t e d border. Chest X-ray 6bn showed moderate cardiomegaly. The ECC s h m d left ventricular h m y . Heart catheaElization sbowed a subaortic vahdm dent of 50 mm Hg. Aortic angiogmpby l o w e d moderate mmbtiL sCIWCULUwPhy

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upon. When the aortic valve was e x p a d at operation, it showed a quadricuspid valve with three cusps of equal size and a small fourth cusp situated between the right and the noncoronary cusp (Fin 1). The attachment of tbe valve was d. The Gasps did not meet in the midhe and hod an obvious regurgitation in the central area. Tbe four ' wm wen developed and the cusps were mildly thickened although pliable. In the suboortic area, there was a l a 5 b stello& ~ ~ stemsis,

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uadricuspid aortic valve not associated to truncal anomalies is a very rare cardiac malformatioa.'~' Most cases reported have been autopsy h d i n g in patients without cardiac symptoms. Other cases reported, however, had severe aortic valve i n d a e n c y of such a degree to need aortic valve replacement1*' We report a case of quadricuspid aortic valve with severe regurgitation, associated with fibromuscular subaortic stenosis. Surgical correction was performed by resection of the subaortic stenosis and plastic repair of the quadricuspid aortic valve. -

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*From the Depariment of Cardiac Snrgery, Ciudad Sanitaria La Paz, Autonomous University of Madrid, Madrid Spain. ReprbJ tsq.uests: DT. zgwa9, cfudcrd sonitrrrb La Paz, Madrid 34, Spain

CHEST, 80: 3, SEPTEMBER, 1981

QUADRICUSPI0 AORTIC VALVE 327


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t l K J i n ~ ~ s e p t a m . T b e ~ u a t i c V a l v 0cIPodrlarspid aortic valve with displac%mmt of tb6 wasconvertedinathree-vahmaupbys\rtarhngtheri&t left ummuy o&e. Am J Cardiol 1989; 23:288 5 Simonds JP.Congenital malformati011~ of the qmtic a d coronarv and d m (Rg 8 ) . Afber-byp.ss,v-hk pnbnollPry valves. Am J Med S d 1933; 168:584 8 Pore& DL, C%a&oot GH, Gowlayr H. Four gradient was fd betwean the left ventride and the aorta. During the postopGIPthe peaiod, no signs d amtic .ortlcMhrewEthsimrifir?nnt~~. insufficiency were found. Six months tidy, the Am J Cardiol 1989; %3:291 patient is asyrnptomatiq and tbeae are no signs of aor&k insuf6ciew.

Inhibition of Programmable Demand

+- Pacemaker by Pacemaker-Induced

Supernumerary amtic cusp is a nue cardiac mation. To the best of our knowledge, only 16 cases have been published2 The first case was raported by Balington in 1882.4 According to Simond~,~the^ incidence of quadricuspid aortic valve is less than 0.008 percent. Cinical diagnosis of quactricuspid aortic valve has been reported very rarely and most of the 16 cases reported bave been through autopsy bdings.l.6 Quadricuspid aortic valves usually function m d y although aortic regurgitation has been described. Aortic valve replacement has been the aperation performed in aIl cases treated su~gicalIy.s An patients treated surgidy, including our case, were adults. The aortic and puhnonary valves are formed after the formation of the main bulbar ridges which fuse to give the aorticopulmonary septum. In both the aortic and pulmonary trunks, three subendothehl swellings appear and grow to form the trhgbshaped vaIves. Alterations in the early embryogenic state division may cause a dyssymetry resulting in four valve cusps m the aortic trunk and possibly two in the pulmonary trunk6 On the other hand, the puhnonary valve may also have four valve cusps. This anomaly, although rare, is much more common than quadricuspid aortic valves. All previously published cases of quadricuspid aortic valves were not associated with other d e a Tbe association with subaortic stenosis has not hem previously reported. HUTWitz and Roberts1 have studied the anatomic cusp variations found in quadricuspid valves. They described seven different types.The most common one is that formed by three cusps of normal d m and one small supernumerary cusp This was the variation found in our case. All previous cases of quadrkuqid aortic valves operated upon in the past had aortic valve m&amemt In our case, we used a plastic repair of the d m with -Il~nt d. Possibly, this t d d q t ~ d d be nsed in some 0 t h cases ~ m the future.

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Worra, D. Widmum, M.D., F.C.C.P.;t

JohnK. Edoga, M.D.;$. Lucille Thomas, RJV.;g Peter Pcdmer;f and Stephen M u c c h e #

myopotckLWtiOI a# demamdprctarrterintw0~in~ibtOai.. pohu~eacrrpwlntedpacem9lrcrtllppcdoverfn ig pocket and directly stimulmted tBe muscles ef tbe c b e d v m n A k i s p 8 C e ~ m i g c 9 t r ~ tioa,pc#BEMg-----==@=

T-wave~~~(orboth),~tberrrfeaftacdb nrrrrd pacenudter to fall when tbe output af tbc pro~pDRm&blepsccmrler rrPrr stt to &KVm high e n e r ~ y chga!i.By~tbermittode&erabrra

cbarge(~widthofpulse),tbcrateroec~ prhtdy to the pgmmmd rate. Lkewise, manual rot a t i o n o f t h e ~ ~ i n t o p r o p u o r i s n ~ tion with tbe active dmmhted suttace dErectcd t

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inhibition of demand cardiac paceInappropriate makers has been reported m a wide variety of clinical situations. This report describes inhibition of a

pacemaker by a cause that has not previously been described. The pacemaker itself, by malposition ("flipped over"), stimulated the muscles of the chest wall and caused significant myopotentials, which, when sensed, d t e d m slowing of the set rate of the p9cemaker.

This 80-year-old woman with cwnpbte heart bbdt and syncup underwent implantation of a unipolar cardiac

'From tbe t of Snrgery, the Cardimiratmy section, M1 H n r w i t z L E , R o b e r t s W C . Q ~ m b h @ d ~ ~ S e C t h , d r w Entown Memorial Hos&al, M NJ. Am J Cardi011973; 31:633 tASag- d CktRoEsom of 2 NaIbuntgil I, -tan G. Qtddcwpid aortic valve. Thoracic S w , New Jersey College af Medicine and Chest 1975; 67:633 Dentistry, R u m Medical School. 3 l - t o ~ i dF, Sangbt pw, $hmc&e Attending Surgeon. HK, Uinical Nurse. CC. Congenital qw&h@d mxtk d v e with qTedrnicalSupervirar,~ira~~a~. ment of the left corarnry Am J Cardid 1988; #chi8f, Biomedical Engineerin8 section. 23 :288 RqnW requsstr: Dr. Widmom* 64 Mop& Aomw* M e 4 Bahgton, quoted by Robicsek F, et aL CcmgmM town, New Jmwy tl79m

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CHEST, 80: 3, SEPTEMBER, 1981


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Quadricuspid aortic valve associated with fibromuscular subaortic stenosis and aortic regurgitation treated by conservative surgery. A Iglesias, J Oliver, J E Muñoz and L Nuñez Chest 1981;80; 327-328 DOI 10.1378/chest.80.3.327 This information is current as of July 13, 2011 Updated Information & Services Updated Information and services can be found at: http://chestjournal.chestpubs.org/content/80/3/327 Cited Bys This article has been cited by 3 HighWire-hosted articles: http://chestjournal.chestpubs.org/content/80/3/327#related-urls Permissions & Licensing Information about reproducing this article in parts (figures, tables) or in its entirety can be found online at: http://www.chestpubs.org/site/misc/reprints.xhtml Reprints Information about ordering reprints can be found online: http://www.chestpubs.org/site/misc/reprints.xhtml Citation Alerts Receive free e-mail alerts when new articles cite this article. To sign up, select the "Services" link to the right of the online article. Images in PowerPoint format Figures that appear in CHEST articles can be downloaded for teaching purposes in PowerPoint slide format. See any online figure for directions.
