Robbins Essential Pathology Heart PDF

8 Heart O U T L I N E Congenital Heart Disease, 118 Rheumatic Valvular Disease, 128 Malformations Associated with Left-to-...

8 Heart O U T L I N E Congenital Heart Disease, 118 Rheumatic Valvular Disease, 128 Malformations Associated with Left-to-Right Shunts, 119 Infective Endocarditis, 130 Malformations Associated with Right-to-Left Shunts, 120 Nonbacterial Thrombotic Endocarditis, 131 Malformations Leading to Obstruction, 120 Cardiomyopathies and Myocarditis, 131 Ischemic Heart Disease, 121 Dilated Cardiomyopathy, 131 Angina Pectoris, 121 Hypertrophic Cardiomyopathy, 132 Myocardial Infarction, 121 Restrictive Cardiomyopathy, 133 Chronic Ischemic Heart Disease, 125 Myocarditis, 133 Arrhythmia, 125 Other Causes of Myocardial Disease, 134 Hypertensive Heart Disease, 126 Congestive Heart Failure, 135 Systemic (Left-Sided) Hypertensive Heart Disease, 126 Left-Sided Heart Failure, 135 Right-Sided (Pulmonary) Hypertensive Heart Disease, 126 Right-Sided Heart Failure, 136 Valvular Heart Disease, 126 Cardiac Tumors, 136 Degenerative Valve Disease, 127 he ear s a remarkaby resen organ, beang more an 40 m- Mos snge-gene deecs a dsrup cardac deveopmen are auo- on mes per year and pumpng more an 7500 L o bood a day. soma domnan muaons, some o wc afec ranscrpon acors Gven s workoad and e mporance o e crcuaor y sysem or a ser ve as “maser reguaors” o cardac deveopmen. he uncon e uncon o ever y organ n e body,  s no surprsng a e o e same genes may aso be mpared by ransen envronmena consequences o ear dsease may be severe: Cardovascuar dsease s sresses a crca eary sages o cardac deveopmen, gvng rse o e eadng cause o moray wordwde and accouns or one n our esons smar o ose caused by genec acors. deas n e Uned Saes. In s caper, we ocus on e mos common orms o ear ds- Clncal Features. Srucura anomaes n congena ear dsease can ease, ncudng congena and acqured orms, and en ns our ds- be caegorzed as (1) maormaons causng a e-o-rg sun; (2) cusson w ear aure. maormaons causng a rg-o-le sun (cyanoc congena ear dseases); and (3) maormaons causng obsrucon. CONGENITAL HEART DISEASE A shunt is an abnorma communicaion beween cambers or bood vesses a permis bood o low from e et o e rig side of Both genetic and environmental factors contribute to congenital e ear (or vice versa) abnormalities of the heart or great vessels, which account for 20%    Le-o-rg suns ncrease bood low no e pumonar y cr- to 30% of all birth defects. cuaon and expose e ow-pressure, ow-ressance pumonar y Congena ear dsease afecs neary 1% o brs (rougy 40,000 crcuaon o ncreased pressures and voumes, resung n rg nans per year), w a ger ncdence n premaure nans and s- venrcuar yperropy and, evenuay, rg-sded ear aure borns. Deecs compabe w ve br usuay nvove ony snge (cor pulmonale). W me, ncreased pumonar y ressance may cambers or regons o e ear. Tweve enes accoun or 85% o cases ead o sun reversa (rg o et) and ae-onse cyanoss (Esen- o congena ear dsease; er requences are sown n Tabe 8.1 menger syndrome).    W rg-o-le suns, a dusky bueness o e skn (cyanoss) Pathogeness. Congena ear dsease usuay arses rom auy resus because e pumonar y crcuaon s bypassed and poory embryogeness durng gesaona weeks 3 roug 8, wen major car- oxygenaed bood eners e sysemc crcuaon. dovascuar srucures deveop. he cause s unknown n amos 90%    S ome congenal anomales obsruc vascuar low, by narrowng e o cases. Known eoogc acors ncude acqured condons suc as cambers, vaves, or major bood vesses. A maormaon carac- congena rubea necon, eraogen exposure, maerna dabees, and erzed by compee obsrucon s caed an aresa. genec acors suc as rsomes 13, 15, 18, and 21 and Turner syndrome. 118  CHAPTER 8 Heart 119 Table 8.1 Frequency of Congenital Cardiac a Malformations Ao LA Incidence per 1 Million Malformation Live Births Percentage PT Ventricular septal defect 42 4482 Atrial septal defect 10 1043 LA Pulmonary stenosis 8 836 RA Patent ductus arteriosus 7 781 LV RV Tetralogy of Fallot 5 577 Coarctation of aorta 5 492 Atrioventricular septal 4 A 396 defect Aortic stenosis 4 ASD 388 Transposition of great 4 388 Ao arteries Ao Truncus arteriosus 1 136 PT Total anomalous pulmo- 1 120 PT nary venous connec- LA LA tion RA RA Tricuspid atresia 1 118 LV TOTAL 9757 LV RV RV Malformations Associated with Left-to-Right Shunts B C Let-o-rg suns are assocaed w ara sepa deecs, venrc- VSD PDA uar sepa deecs, and paen ducus arerosus and are e mos Fig. 8.1 Common congenital causes of left-to-right shunts ( arrows indi- common ype o congena cardac maormaon (Fg. 8.1). Ara cate direction of blood flow). (A) Atrial septal defect (ASD). (B) Ventric- sepa deecs ypcay ncrease ony rg venrcuar and pumo- ular septal defect (VSD). (C) Patent ductus arteriosus (PDA). Ao, aorta; nar y oulow voumes, wereas venrcuar sepa deecs and paen LA, left atrium; LV, left ventricle; PT, pulmonary trunk; RA, right atrium; ducus arerosus cause ncreases n pumonar y bood low and bood RV, right ventricle. pressure. Cyanoss s no an eary eaure o ese deecs, bu reversa o e sun due o pumonar y yperenson can cause c yanoss and rreparabe cardac dysuncon hus, et-o-rg suns mus be repared beore sun reversa occurs, usuay w surger y. Mos are assocaed w oer cardac maormaons. he ven- rcuar sepum s ormed by e uson o a muscuar rdge a Atrial Septal Defects and Patent Foramen Ovale grows upward rom e ear apex and a nner membranous par- Aria sepa defecs and paen foramen ovae are e mos common on a grows downward o mee  (Suppemena eFg. 8.1). he congenia cardiac anomaies diagnosed in adus. membranous poron s e se o approxmaey 70% o venrcuar Durng cardac deveopmen, paency s mananed beween e rg sepa deecs. and et ara by e foramen ovae, wc s aer cosed by ssue laps. In Sma deecs may be asympomac, and deecs n e muscuar 80% o peope, ese sepa evenuay use. In e remanng cases, a patent wa o e sepum may cose sponaneousy durng nancy or eary foramen ovae may aow transent rg-o-et bood low, as may occur cdood. Larger deecs, owever, resu n cronc et-o-rg w sneezng or sranng durng bowe movemens. Suc rg-o-et sunng compcaed by pumonar y yperenson and congesve low o bood pus paens a rsk or a paradoxca embosm: a venous ear aure. Eary surgca correcon s mperave or suc esons. embous (e.g., rom deep eg vens) a eners e arera crcuaon va Patent Ductus Arteriosus an ara deec due o ncreased rg-sded ara pressures. hs may gve rse o sroke due o odgng o paradoxca embo n vesses o e cenra he ducus areriosus is a connecing ink bewe en pumonar y nervous sysem. In conras, an ara sepa deec s a xed openng n e arer y and aora a normay coses ater bir ara sepum a aows unresrced bood low rom et o rg arum. I arses rom e et pumonar y arer y and jons e aora jus ds- Ara sepa deecs usuay are asympomac un aduood, a o e orgn o e et subcavan arer y. Durng nrauerne e,  wen ong-sandng cronc rg-sded voume and pressure over- perms bood low rom e pumonar y arer y o e aora, bypassng oads may evenuay produce pumonar y yperenson and sun e unoxygenaed ungs. Wn 1 o 2 days o br, e ducus nor- reversa. hese compcaons do no occur w paen oramen ovae. may consrcs and coses n response o ncreased arera oxygen- aon, decreased pumonar y vascuar ressance, and decnng eves Ventricular Septal Defects o prosagandn E derved rom e pacena. Known causes o per- 2 Defecs in e venricuar sepum aow et-o-rig suning and ssen paen ducus arerosus ncude ypoxa (e.g., due o rg o are e mos common congenia cardiac anomaies a bir, bu et suns) and ceran auosoma domnan gene deecs, bu 90% o mos cose sponaneousy and do no come o cinica aenion cases are o unceran paogeness.  CHAPTER 8 Heart 119.e1 Supplemental eFig. 8.1 A ventricular septal defect (membranous type), denoted by the arrow. (Courtesy William D. Edwards, MD, Mayo Clinic, Rochester, Minnesota.) 120 CHAPTER 8 Heart Sma duca suns generay cause no sympoms, bu arger Morphology. Teraogy o Fao resus rom anerosuperor deecs evenuay ead o sun reversa, cyanoss, and ear aure. dspacemen o e muscuar sepum a separaes e pumonar y Paen ducus arerosus creaes a g-pressure et-o-rg sun a runk and e aorc roo (Fg. 8.2A). he ear s enarged and boo- produces a ars, “macner y-ke” murmur. Isoaed paen ducus saped due o rg venrcuar yperropy ; e proxma aora s requres surgca ner venon as eary n e as possbe o preven ese daed; and e pumonar y runk s ypopasc. he venrcuar compcaons. sepa deec usuay s arge and s overrdden by e aorc vave, wc receves mos o e oupu rom bo venrces. Obsrucon Malformations Associated with Right-to-Left Shunts o rg venrcuar oulow may be due o narrowng jus beow e Cardac maormaons resung n rg-o-et suns gve rse o cya- pumonar y vave (mos common) or pumonar y vave senoss or noss due o admxure o venous bood w e arera crcuaon. aresa. In suc cases, a paen ducus arerosus or daed bronca he mos common condons assocaed w cyanoc congena areres provde e ony roue or bood o reac e ungs. ear dsease are eraog y o Fao and ransposon o e grea ves- ses (Fg. 8.2). C ln cal Feature s. Te cnc a  s e ver  y d ep e nds on  e d e g re e Tetralogy of Fallot o pu monar y ou  ow ob s r u c   on. In mos c as es , pu  mon  c Teraog y of Fao is e mos common cause of c yanoic congenia obs r uc  on s s e vere enoug  o c aus e r  g  -o - e  sun  ng ear disease (⁓5% of congenia cardiac maformaions) and c yanoss, w c wors e ns w     me b e c aus e  e ma or me d he eraog y consss o e oowng our abnormaes: pu monc or  ce do es no e xp and as  e re s o  e e ar  g rows.    Venrcuar sepa deec I  e pu monc obs r uc  on s m d,  e cond   on re s embes an    O verrdng o e venrcuar sepa deec by e aora s o ae d ven r c u  ar s ep a  d ee c  , b e c aus e sun ng o c c urs  rom    Rg venrcuar oulow rac obsrucon (subpumonc senoss) e  o r g . Te pu mon  c senoss prov  de s proe c   on  rom    Rg venrcuar yperropy pu monar y yp er ens  on , bu o e r s e qu e ae o c y ano c e ar  ds e as e are s e en, suc as p oyc y  em  a ( due o y p ox  a). R  g  - o - e  sun ng a s o  ncre as e s  e r sk or  ne c  ve e nd o c ard  s and p aradoxc a  emb o za  on. C omp ee surg  c a  re p a r s p oss be bu s more compc ae d  pu  monar y a re s  a s pres en. Ao Transposition of the Great Vessels PT In ransposiion of e grea vesses, e aora arises from e rig venrice and e pumonary arery emanaes from e et venrice. LA I s ncompabe w posnaa e uness a sun exss a dev- RA ers oxygenaed bood o e aora suc as a venrcuar sepa deec (one rd o cases) (Fg. 8.2B; Suppemena eFg. 8.2). In ese cases, e rg LV venrce s yperroped because  s e pump or e g-pressure RV sysemc crcuaon, and e et venrce s ypopasc because  pro- vdes bood o e ow-pressure pumonary crcuaon. In oer nsances, suns are provded by a paen oramen ovae or ducus arerosus; ese end o cose soon ater br, and suc nans requre emergen surgca nervenon. he domnan eaure s cyanoss. Improved surgca ec- Classic tetralogy of Fallot A nques now perm repar, and paens oten survve no aduood. Malformations Leading to Obstruction Ao Ao Congena obsrucons o bood low can occur proxma o e ear vaves, a e eve o e vaves, or dsay wn a grea vesse. he PT aer mos commony nvove e aora and mer a bre dscusson. PT LA LA Aortic Coarctation RA RA Coarcaon (narrowng, or consrcon) o e aora s a common orm o obsrucve congena ear dsease. Maes are afeced wce as oten LV LV as emaes. Coarcaon may be a soary deec or may be assocaed RV RV w oer deecs, o wc e mos common s a bcuspd aorc vave. here are wo orms o coarcaon (Fg. 8.3):    An “nfante” form eaurng crcumerena narrowng o e aor- c segmen beween e et subcavan arer y and a paen duc- us arerosus (reerred o as preduca). In s crcumsance, e With VSD Without VSD ducus s e man source o (unoxygenaed) bood devered o e B Complete transposition dsa aora. he pumonar y runk s daed due o ncreased bood Fig. 8.2 Common congenital right-to-left shunts (cyanotic congenital low, and because e rg sde o e ear peruses e body dsa heart disease). (A) Tetralogy of Fallot (arrow indicates direction of blood o e narrowed segmen, e rg venrce s yperroped. flow). (B) Transposition of the great vessels with and without ventricular    An “adut” form conssng o rdge-ke nodng o e aora septal defect. Ao, aorta; LA, left atrium; LV, left ventricle; PT, pulmonary adjacen o e gamenum arerosum, a remnan o e ducus trunk; RA, right atrium; RV, right ventricle; VSD, ventricular septal defect. arerosus (reerred o as posduca) (Fg. 8.4). Proxma o e  CHAPTER 8 Heart 120.e1 Supplemental eFig. 8.2 Transposition of the great vessels. (Courtesy William D. Edwards, MD, Mayo Clinic, Rochester, Minnesota.)  CHAPTER 8 Heart 121 ISCHEMIC HEART DISEASE Ao Ao Ischemic heart disease encompasses several related syndromes caused by insufcient delivery of oxygen and nutrients to meet myo PT cardial demand. PT I s mos oten due o coronar y arer y dsease, e ce cause o LA LA moray n e Uned Saes and oer g ncome naons. Encour- RA RA agngy, moray reaed o scemc ear dsease n e Uned Saes as decned by 50% snce 1963. hs mprovemen s argey due o LV LV RV ner venons a ave reduced coronar y arer y aerosceross, RV ncudng smokng cessaon programs, beer reamens or yperen- son and dabees, and use o coesero-owerng drugs. herapeuc advances suc as more efecve arryma conro, mprovemens n coronar y care uns, angopasy, and endovascuar senng ave aso conrbued. With PDA Without PDA Coarctation of aorta Pathogeness. In a arge majory o cases, scemc ear dsease sems Fig. 8.3 Coarctation of the aorta with (“infantile” or preductal form) and rom reduced bood low caused by aerosceross o e coronar y without a patent ductus arteriosus (PDA) (“adult” or postductal form); areres, oten reerred o smpy as coronar y arer y dsease. Aero- arrow indicates direction of blood flow. Ao, aorta; LA, left atrium; LV, sceross (see Caper 7) can afec any o e man coronar y areres, left ventricle; PT, pulmonary trunk; RA, right atrium; RV, right ventricle. sngy or n any combnaon. Angina Pectoris Angina pectoris is intermittent chest pain caused by reversible myocardial ischemia. hree varans are recognzed.    Stabe angna s predcabe epsodc ces pan assocaed w par- cuar eves o exeron or ncreased demand (e.g., acycarda). I s usuay assocaed w sabe aerosceroc paques a narrow e umen o a coronary arery by 70% or more (crtca stenoss). he pan s a crusng or squeezng subserna sensaon a may radae o e et arm or e et jaw I s reeved by res or by nrogycern, a vasodaor a ncreases coronary peruson.    Prnzmeta or varant angna occurs a res and s caused by cor- onar y arer y spasm, ypcay near aerosceroc paques. I aso responds o vasodaors suc as nrogycern and cacum canne bockers. Fig. 8.4 Coarctation of the aorta, postductal type. The coarctation is a    Unstabe angna (crescendo angna) s caracerzed by ncreasngy segmental narrowing of the aorta (arrow). Such lesions typically mani- requen pan a s precpaed by progressvey ess exeron or fest later in life than preductal coarctations. The dilated ascending aorta even occurs a res. I may be assocaed w severe narrowng o and major branch vessels are to the left of the coarctation. The lower a coronar y arer y (obsrucon o > 90% o e vesse umen) or by extremities are perfused predominantly by way of dilated, tortuous col- paque rupure and supermposed romboss. Unsabe angna can lateral channels. (Courtesy of Sid Murphree, MD, Department of Pathol- ogy, University of Texas Southwestern Medical School, Dallas.) be a arbnger o myocarda narcon. Myocardial Infarction coarcaon, e aorc arc and s branc vesses are daed and e et venrce s yperroped. Myocardial infarction is necrosis of the heart muscle resulting from ischemia. Clncal Features. hese depend on e poson and severy o e he ncdence o myocarda narcon rses progressvey w age narrowng and e paency o e ducus arerosus: and e presence o ncreasng numbers o rsk acors or aerosce-    Preducta coarctaton wt a patent ductus usuay presens eary n ross. Men are afeced sgncany more oten an women, bu s e w cyanoss n e ower a o e body. Wou ner ven- gender gap narrows w age. on, mos afeced nans do no sur vve e neonaa perod.    Postducta coarctaton wtout a patent ductus usuay s asympomac Pathogeness. Mos myocardia infarcions are caused by acue eary n e and may reman unrecognzed no aduood. here oten romboic obsrucion of a coronar y arer y due o rupure of an s upper-exremy yperenson and reave ypoenson n e ower aerosceroic paque (Fig. 8.5). exremes assocaed w caudcaon. Coaera crcuaon oten he nang paque dsrupon o myocarda narcon (MI) s deveops roug e nercosa and nerna mammary areres, and ypcay sudden. Paques a conan arge pd-rc cores or ave ncreased bood low and daon o ese vesses can produce vsbe n overyng brous caps are parcuary vunerabe o rupure Hem- “nocng” o e rbs on radoogc sudes. Treamen w baoon orrage no e paque can cause rapd paque expanson and rupure, daon or surgca resecon generay yeds exceen oucomes. or an occusve rombus can orm because o exposure o coagen,