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    13 views47 pages

    MOSS Transposición de Grandes Vasos PDF

    Uploaded by

    MairaAlejandraRoseroRosero

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    © All Rights Reserved
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    of 47
    Abnormalities of the Origin of the Great Arteries ‘Wernovsky ‘ane porition ofthe great arteries (TGA) ica lethal and rela ‘ively frequent malformation, accounting for 5% to 7% of all congerutal cardiac malformations. Without treatment, shout 30% of there infants die in the fst wreek of life, 50% within the frst month, 70% within 6 months, and 90% rithin the fist year (Fig. 49.1). Today, aggressive medical and surgi- cal interventions in the neonate can provide >90% early and midterm suvival and, for many patients, the prospect of a vigorous adolescent and adult life ‘The incidenceis reported tovange from 20.1 1030 5/100,000 live bathe with a sting (60% to 70%) male preponderance “Although eazlier epidemiologic and genetic survey: suggested some other a:zociation,, for example, increased prevalence in infants of diabetic mothess or prenatal expomure to sex- homone therapy, these have not been confirmed. Extacar diac anomalies are less frequent in infants with TGA (<10%) compared with other fom: of congerital heart disease, such az tmuncus axterionas (45%), venbicular septal defect (VSD) (G47), or tevalogy of Fallot 31%) ANATOMY AND PATHOLOGY Nomenclature Azatomists, sugeor, and cardiologists have chosen a vari- ety of temas to descube the basic anatomic abnomality in ‘rareposition: the aorta ansing fiom a morphologically ight ventncle and the pulmonary autery ansing froma morphologi- cally left ventricle Fig. 49 2). The great arteries maybe related sonmally or abnommally to each other to the ventacles, to the ventncular septum, and to the atrioventicular (AV) valves Nomally and abromually related great arteries customanly. sue designated in tere of their venbiculoastenal comectors or alignments, that is, normal, anspozed, double-outlet right or left ventricle, and anatomically comected malposition ‘The common clinical type, that i, with situs solits of the atria, concordant AV (night atrium to right ventricle and left atmumtoleftventicle) and discordant venticuloasterialaligr- ment, is widely termed complete TGA. The modifying tem complete by present classification and nomenclature is vedur- dant, however it has, by commonusage, come to indicate that the tramposed great arteries are physiologically unconected, that i, that systemic venous blood flows predominantly to the aorta and pulmonary venous blood to the pulmonary artery In ths usage, the term complete is contrasted to the teum.cor rected, a: inconected TGA (ie, physiologically conected), in C8) Transposition of the Great Arteries which systemic venous blood ovr: to the palmonaay asteny ‘and pulmonary venous blood to the aorta, tzhould be 1ecog- nized, however that both the physiologically uncoected anti ‘physiologically conected transposition hearts are morphologi- cally complete trar:position:; that i, both great arteries are ‘completely (or predominantly) misplaced across the ventricu- Jarseptim and arise fiom morphologically inappropriate ver tricles and thts have dizcondant ventacelonsterial connections ‘Using the segmental (atria, ventricles, arteries) approach ‘and the situs-independent dexbo-(b) and levo-(t) denotation: favored by Van Praagh, the clinical material in this chapter concen: almart exchusively TGA (5,D,D), that is, TGA with, ‘ates soliter (S) of the atia and viscera, wival (D) looping of the ventricles, and an anterior and rightward (0) aosta. If the tran-pored acita at the valve anmmius level i positioned to the Jeftof the transposed pulmonary artery, the malformation may bbe denoted a7 TGA (S,D,L}. A zare type of trarcposition has ‘been desenibed with the aorta posteriorly positioned but nev- ertheless aligned with and connected to the antenor morpho- logically ight ventricle. An additional modifying texm, simple, das been used by some mozphologists to exchide hearts with, frarposition that have additional essociated malformations suchas AV valve atresia or straddling, common AV onifce, or ‘Single-ventucle heaste wath small outlet chambess. This chap- tris concemed primazily with the category of simple tranepo- ‘Aton and inchder TCA with intact ventricular septam (IVS), ‘VSD, patent ducts arteriomus (PDA), and left ventnicular out. flow tract obstruction (LVOTO), in all, a group comprising about 80% of patients with TGA In addition, double-cutlet ght venticle (DORV) vith aubpulmonary VSD (considered within the spectum of Taussig-Bing anomaly) is alzo discussed Jhere because it has some of the morphologic features, simular physiology, and all the surgical option: that pertain to TGA with lange subpalmonary VSD. It is important to note that ‘many nugical and clinical reports use this same term, sine ple TGA, fo denignate the large important subgroup of TGA patients with IVS (or exhemely small VSD) but without any. other significant associated lesion (Hs exchading TGA with, Inge VSD, lage PDA, and zignuficant LVOTO) Morphogenesis and Etiology ‘The detailed developmental axpects of abmomal ventricu- Jostenial relationship remain largely unknown; howeres, abnommal development, growth, and absorption of the distal infundibulum (coms) are comidered by some major factor Recent work with more modem genome-wide anay testing 1087 1098 SECTION © Congenital Cardiovascular Maformations 90 90 80 80 50 50 40 4 10 10 ‘SURVIVAL (X) (x) Wwainuns te 88 288 AGE IN DAYS (log:x scale) igure 49.1. Survival of patient: with TGA. Lall and Livs epre- sent “satura history” survival data on 742 patient fiom 1957 Duough 1964 antedating both Mustard zepair and BAS. (Data om Lisbman J, Callum L, Belloc NB. Natural stony of tare ‘poritionof the great artenc:- anatomy are bisth and death char- ‘etensties. Ciroulation 1969:40,237-262,) Lall represent all ‘TGA subtypes, and Livs represents only patient with TGAMIVS. ‘Tl represents 20-yearactuazial auvivalof 106 infants with TGA/ IVS following Mustard repair performed fiom 1963 thnough 1973; 72 represents 12-year actuarial survival of 223 childxen {following Mustard zepair performed fiom 1974 thacugh 1985. (Data fiom Tusler GA, Castaneda AR, Rosenthal A, etal ;Com- ‘genital Heast Suigeome Society Canent reults of management tn bansposition of the great arteries, with special emphasis on. ‘patient with associated ventricular septal defect J.4m Coll Car- ‘dio! 1987,10.1061-1071.) K represents sleadjusted probability (of survival after an artesial switch epairina neonate with TGA/ IVS or TGAIVSD az cbzerved ina study conducted by the Com- {genital Heast Suageons Society om 1985 though 1989 on 513 patents undergomg the arfenal switch operation (Data fiom, ‘Kbklin JW, Blackstone EH, Teherwenkor Cl, et al.; Congeni- tal Heart Suigeore Society. Clinical cutcomes after the arterial switch operation for hansposition: patent, support, procedural ‘an itittional nsk factors. Ciroularion 1992,86:1501-1515) Rirkeadjustment condition: inchided neonate operated on in, ‘an itvttution of proven competence (“low-=isk" ititutions, n= 7), “usual” TGA comonazy artery pattem, single VSD, ‘absence of coexisting noncardiac anomalies, and average birth weight igure 492. Complete TGA. A: Angiocardiogram (left anterior cblique view). B: Two-dimercional echocardiogram (subcostal view) showing discordant venticuloarte- nial alignments. Right-sded (anterior) ‘aorta (AO) connected to right-sided (anterior) morphologically night ver- fhicle (RV) and leftsided (porterios) pulmonary artery (PA) comected to left-sided (po:tenor) morphologically eft ventricle (LV) shows eanly promice in detecting a genetic etiology in some, ‘puticularly mutation: in the laterality genes an the

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