Conduction System Studies
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JAM COll CARDIOl 879 19R3.1(3) R79-R6 PATHOLOGIC STUDIES Sudden Death in Three Teenagers: Conduction System Studies SAROJA BHARATI, MD, FACC, ROBERT BAUERNFEIND, MD, FACC. LYNN B. MILLER, MD, FACC, BORIS STRASBERG, MD, FACC, MAURICE LEV, MD, FACC Chicago. Illinois The pathologicsubstratefor sudden death in the middle•tient 3 (age 19, feinale) died suddenly at home. Autopsy aged or elderlyad!11tis usually ischemicheartdisease. revealedmitralvalve prolapse, thrombosis of the sino• In contrast,few dataare availableregardingthe pa• atrial(SA) nodeartery,andprematureaging, sclerosis thology of sudden death in teenagers.report This de• of the left side of thecardiacskeleton, with involvement scribes three teenagers without clinically suspectedof heart theventricularseptum more on the rightventricular disease dying suddenly. Patient 1 (age 15, male)side was and involvement ofatrioventricular the bundle and known to have rightventricularprematureventricular trifascicularconduction system. beats.Postmortemexamination revealed marked pre• In conclusion, unexpected deaths in three teenagers matureaging, sclerosis of thecardiacskeleton extending occurred withdemonstrablepathologic findings in the to the right side of the summit with fibrosis of theheart. left Two of the three patients had mitral valve pro• and right bundlebranches.Patient 2 (age 17, male) waslapse, one of whom also had thrombosis or embolism of a trainedathlete who died during football scrimmage.thesinoatrialnodeartery.All three had sclerosis of not Autopsy revealed moderate mitral valve prolapseonly and the left side but alsothe right side ofthe ventricular markedprematureaging, sclerosis of the left side of theseptum with involvement of the conduction system. The cardiacskeleton, which extended to theventricular right anatomicsubstratedemonstratedin these three patients side, and secondary involvement oftrifascicular the con• could relate to lethalbradyarrhythmiaor tachyarrhyth• duction system with mononuclear cell infiltration.mia, Pa- or both. The conductionsystemhas beeninfrequentlyexaminedin cerns threeapparentlyhealthyteenagerswho diedsuddenly. casesof suddendeath inapparentlyhealthyteenagersor The conductionsystem in all threesubjectswas studied young people.The reportsthus far show somepathdlogic extensivelyand the findings arediscussedin relationto changespertinentto theconductionsystemin some cases sudden death. (1-24) and none in others(25,26). Our presentstudy con- CaseReports Case I From the Congenital Heart DIsease Research and Training Center,The patientwas a 15 yearold boy who was known to have Hektoen Institute for Medical Research, Chicago; the Departments ofright Pa•ventricularprematureventricularcomplexesfor anumberof thology ofNorthwesternUniversity Medical School, Chicago;Pntzker years,butwasapparentlyin good health.He suddenlycollapsed School ofMedicine,University of Chicago, Chicago; The Chicago Medical School,University of Health Sciences; Loyola University, Stritch Schoolwhile playingsoccerwith his friends.Therewas no historyof of Medicine,Chicago; The SectionCardiology, of Department of Medicine trauma.He waspronounceddeadon arrivalat theCanton-Potsdam and theDepartmentof Pathology,AbrahamLincoln School ofMedicine, Hospital,Potsdam,New York about35 minuteslater.Family University of Illinois, Chicago; and the Departments of Pediatrics, Med• historyindicatesthatonebrotherhas aorticstenosis. icine andPathology,Rush Medical College, Chicago. Present address for Drs. Bharati and Lev:Departmentof Pathology,Deborah Heart and Lung Postmortem examination.Acute congestionof the viscera Center,Browns Mills, New Jersey. This study was aided by Grant HLwas noted. 07605 from the National Heart, Lung, and Blood Institute, Bethesda,Heart. The heartweighed300 g Diffusefibrosisof the myo• Maryland,and the National Institutes of Health, Bethesda, Marylandcardiumwas seen at thegross level by the coroner'sphysician. ManuscriptreceivedSeptember13, 1982; revised manuscript received Left ventricularwall thickness em. All the valves October26, 1982, acceptedOctober29, 1982 measuredI 3 Address forrepnnts.Saroja Bharau, MD, Department of Pathology.andthe extramuralcoronaryarterieswerenormal. Deborah Heart and Lung Center, Browns Mills, New Jersey 08015 Conduction system, The heartwassentto (SB andML) in six © 1983 bytheAmerican Collegeof Cardiology 0735-1097/83/030879-8$0300 880 J AM COLL CARDIOL BHARATI ET AL 1983.1(3) 879- 86 pieces. One fragment that wasapparentl y the main partof the heart was sent from the emb almer. The other pieces of the heart were sent by the pathol ogist. The superior vena cava region , the sinoatrial (SA) noderegion,the atrioventricular (AV) node region, the bundle of His region and the right and left bundle branch regions were recognized in the part sent byembalmer. the The block containing the SA node and its approaches was serially sectioned and every )Oth section was retained and con• secutively stained withhematoxylin-eosin , Weigert-Van Gieson and Gomori trichromestains . The block conta iningapproach the es to the SA node , the AV node , the AV bundle and the bundle branches was seriallsectioned y and every 10th section was re• tained . Theapproachesto the AV node and the peripheral portions of the bundlebrancheswerealternatelystained withhematoxylin• eosin andWeigert-VanGiesonstains. Theremainderwas stained with Gomori trichromein addition.The remainderof the myo• cardiumwas cut into blocks and two sections were taken from each block andstainedwith hematoxylin-eosin and Weigert -Van Gieson stains. In thismanner566sections were examined . These were matched withsections of hearts from a similar age group (controls). Microscopic examination.SA node. Fatty infiltration was present at the headof the node . A slightmononuclearcell infil• tration was also seen. Approaches to SA node. There was moderate fatty infiltration. Approaches to AV node. A slight infiltrationmononucle of ar cells was seen. The re was also fatty infiltration. AV node. A slightmononuclear cell infiltration was present. AV bundle. Therewas slight fibrosis in the penetrating portion . A large thin-walledvascularchannel coursed through the pene• tratingortion p . Left bundle branch. There was moderate fibrosis in the beginning. Figure1. Case I. Right bundle branch showingfibros is(arrows). Weig• Right bundle branch . Slight fibrosis was present in the first crt-Van Gieso n slam X45, reduced by 25%.= Vventricular septum part , moderatein the second and considerable in the third (Fig. I). Summit of the ventricular septum . There werenumerousfocal areas of fibrosisthroughoutthe septum, especiallymarked ante• riorly on the right side. Some of the arteriolesthickened were (Fig. 2). Postmortem examination.On gross examination, no ana• Left ventricular myocardium . Large areas of fibrosis were pres•tomic abnormalities were noted in other organs. ent beneath theepicardiumin all sections. Heart. The heart weighed 355 g. There werehypertrophy and dilation of the right ventricle. The whole region of the pars membranacea and the adjacent aorticof leafletthe mitral valve Case 2 were thickened for this subject's age andembled res those of a The patient was a 17 year old boy, an honor student andperson a aged 50 years. The mitral orifice was somewhat enlarged. trained athlete, who died durin g football scrimmage. HeThe was posterior leaflet was enlarged and divided intocompo three• I.9 m tall and weighed 77.) kg. The mother related that thenents. boy There was a mild to moderate amountedundan of rcy in the had a temperatureof40.5C 6 month s before death and a physicianentire leaflet. All other valves were normal.coronar They arteries made a diagnosis of viralinfection. Threedays later the boy went were normal. to footballpracticebut sat on the sidelines. Subsequentl y he had Conduction system. The SA and AV nodes and their ap• no complaints andengagedin full athletics . A day or two beforeproaches, the AV bundle and bund le branches up to the periph ery his death, he was noted to be moving his shoulder and claimedwere serially sectioned . Every 20th section was taken from the SA he had a little musclescomfort di . He also had a head cold. The node region. every 10th section from the AV node10 the up day before and on the morn ing of his death he did not eat well.beginning of the bundle branches and every 20th section from the He had beenworkingout for about I hour and 10 minutes whenperiphery of the bundle branches. The remainder of the heart was he collapsed. cut into block s and two sections were taken from each block . All The parents said that the boy had receivedthorough a physic al sections were alternately stained with hematoxylin -eosin and Weigert• checkupapproximately3 weeks before and was reported to be inVan Gieson stains. In this manner , a total of 732 sections were excellentcondition. studied. CONDUCTION SYSTEM IN SUDDEN DEATH JAM cou.CARDIOl 881 1983.1(3)879-86 : , J•, • r. I '/ ,I ,I, , I "I,. I ' , ~ , ," { , I' II I ; •. r Figure3. Case2. Chromeinflammatorycells(arrow)in thepenetrating Figure2. Case I. Summitof theventricularseptumshowingfibrosison partof thebundleof His Hematoxylin-eosinstainX150,reducedby27o/c. therightventricularside(arrows).Weigert-VanGiesonstainXIO, reduced B = penetratingpartof thebundleofHis; CFB = centralfibrousbody. by27%. B = bundleofHis; LBB = leftbundlebranch;N = AV node; RA = rightatrium;V = ventricularseptum. Microscopicexamination.SA node. There were no changes. right ventricle. The latter was alsoIn seenthe left ventncle, lower