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Truncal Inversion with Biventricular Pulmonary Trunk and Aorta from Right Ventricle (Variant of Taussig-Bing Complex) By AARON B. SHAFFER, M.D., JosE' F. LOPEZ, M.D., AND IRWIN K. KLINE, M.D., MAURICE LEV, M.D. SUMMARY Downloaded from http://circ.ahajournals.org/ by guest on April 29, 2017 A heart is described pathologically in which the aorta emerged from the right ventricle and was not related to the ventricular septal defect, while the pulmonary trunk emerged from both ventricles, but mostly the right, and was related to the ventricular septal defect. This Taussig-Bing arrangement of vessels was coupled with the presence of the aortic orifice to the left and the pulmonic orifice to the right, which is an inverted position. The anatomic concept of inversion is an abnormality in position from the standpoint of laterality. A careful study of the conal regions of the left and right ventricles showed that those regions were not inverted. Therefore, this represents a case of Taussig-Bing complex with exclusively truncal inversion, which is unique. This may be explained on the basis of opposite metameric contribution to the development of the truncus. Additional Indexing Words: Transposition of the great vessels Congenital heart disease Double-outlet right ventricle Truncus tion. Thus, there was isolated inversion of the aorta and pulmonary trunk, which is unique; hence, the present report. W E HAVE RECENTLY studied a heart with biventricular origin of the pulmonary trunk, in which the aorta arose from the left side of the right ventricle anterior to the pulmonary trunk, which arose from the right side of the right ventricle posteriorly. The heart was in levocardia (normal position) with all organs in situs solitus. The atria and inlets of the ventricles and coni were in normal posi- Clinical Review The subject was a 3-month-old white female infant, with the following diagnoses, based on clinical, hemodynamic, and angiographic findings: nearly complete transposition of the great vessels with ventricular septal defect, bi-directional but predominantly left-to-right shunt, pulmonary hypertension, "infantile" or "transitional" coarctation of the aorta, and patent foramen ovale. The aortic root was found to be anterior to the root of the pulmonary trunk; however, the usual laterality of the ascending aorta was not noted during life. It could have been recognized by an unusual course of the catheter in the ascending aorta in the frontal plane. An aortogram in this projection was not obtained. In view of the precarious condition of the patient, surgical correction of the coarctation was recommended. At surgery (November 1961), a large patent ductus arteriosus was found to join the left pulmonary artery to the aorta distal to the coarctation. The coarctated segment of the aorta was re- From the Congenital Heart Disease Research and Training Center, Hektoen Institute for Medical Research; the Division of Cardiovascular Disease, Department of Medicine, and the Otto Saphir Pathology Institute, Michael Reese Hospital and Medical Center; the Departments of Pathology of Northwestern University Medical School, University of Chicago School of Medicine, and University of Illinois College of Medicine, Chicago, Illinois. This investigation was supported in full by Grant 5 PO HE-07605-05 from the National Institutes of Health, National Heart Institute, Bethesda, Maryland. Dr. Lev is Career Investigator and Educator of the Chicago Heart Association. Circulation, Volume XXXVI, November 1967 783 SHAFFER ET AL. 784 ectasis (bilateral). The viscera were all in normal position. Heart Figure 1 Downloaded from http://circ.ahajournals.org/ by guest on April 29, 2017 Diagramimatic sketch of the anterior viewl of the iniopened heart made fromti the opened heart. SVC supjerior venia ecav; P ==ptlmonary tr-uttk; and A= aorta. moved with an end-to-end anastomosis, and the patent ductus arteriosus was also resected. The patient did well initially, btut succumbed on the second postoperative day. Pathological Exainination Aside from the findings in the heart, the pathological diagnosis was marked pulmonary atel- Estimation of chamber size and muscle mass of chambers was made by the method of Lev and associates.' The heart was enlarged and weighed 59 g (normal, about 28 g). The baseapex axis pointed toward the left and down. The apex was formed by both ventricles. Txvo arterial trunks emerged from the base: a large ptulmonary trunk was situated posteriorly and to the right, and a smaller aorta anteriorly and to the left (fig. 1). The relationiships of the atria, inlets of the ventricles, and coni were normal. The right atrium was niormal in size and its wall was of normal thickness. This chamber received the superior and inferior venae cavae and coronary sinus in a normal manner; the eustachian and thebesian valves were normal. The limbus was well formed, although small in extent, and the foramen ovale was obliquely patent, measuring 0.1 to 0.2 cm in greatest dimension. The endocardium of this chamber cu t enrAd ,)F P, Figure 2 (Left) Right ventricular view showing emergence of pulmonary trunk. (Right) Diagrammatic sketch of left panel. PT = pulmonary trunk; A aorta; D - ventricular septal defect; TVtricuspid valve; P, first parietal band; and P, second parietal band. Arrowt voints to the outflow tract into the aorta. Circulazion, Volume XXXVI, November 1967 ,-85 TRUNCAL INVERSION Downloaded from http://circ.ahajournals.org/ by guest on April 29, 2017 Figure 3 (Left) Right veiitriciular view shiowing emergence of the aorta. (Righit) Diagratmmatic sketch of left panel. A = aorta; S septal band group; an'd P, = first prarital band. Area enclosedc in irregular black line is cutt end of first parietal hatnd. dliffusely wvhitened. The tricuspid orifice xvas enlarged; the tricuspid valve was normally formed but presented increased hemodynamic vas change. 2 The right ventricle was enlarged and its wall tlhickened. The inflow tract had the normal architecture of the riglht ventricle. The outflow tract howvever Wxas abnormal (figs. 2 and 3). A septal band consisting of several components proceeded from the left lateral wall to the base of the aorta. Here it fornmed a wide muscular parietal structure. This structure gave off band (first parietal band) xvhich extended over the anterior wall of the right xventricle. Part of the latter anchored on the septum again in the lower part of the outflow tract. A second parietal band extended fromr- the base of the pulmonary trunk over the region adjacent to the ainterior leaflet of the tricuspid valve. Thus the outflow tract of the right veiltricle was divided into tvo parts leading into the aorta and pulmonary trunk xvith a more distinct conus-like structure beneath the aorta. This outflow tract, although abnormal, was clearly was a Clt-cuZ{ation,! Voliiie XXXVI, Norlember 7967 that of a right venitricle. The endocardiumn of the outflow tract xvas thickened. The aortic orifice was somewhat larger than normal. The aortic valve xvas normally formed, with mnarked hemyiodynamic change and prominent hillock formation at the commissures. Its annulus was niot related to the tricuspid or mitral annulus. The coronary ostia were situated in the posterior and right anterior sinuses of Valsalva. The right coronary ostium gave rise to the anterior descending coronary artery and to the right circumflex. The latter gave off its usual l)ranches to the right ventricle and terminated in the posterior descending coronary artery. The posterior coronary ostinin gave rise to the left circumflex coronlary artery, wxhich in tuirn gave off the ramus anterior ventriculi sinistri and the ramilus obtusus ventriculi sinistri. The coronary veins wvere not dissected. The transverse aorta vas smaller than- normal andI presented groups of sutures. The pulmonary truniik emerged mostly from the right ventricle but straddled the ventricular septum over a defect to be (lescribed later. 786j SHAFFER ET AL. Taussig-Bing lheart with truncal imxversion-i 1. Hypertrophy and enlargemenit of the heart a. Right xcentricular lhypertr-oplhv and enlargement I. Left atrial] hypertroplhy and enlargement 2. 3. 4. Downloaded from http://circ.ahajournals.org/ by guest on April 29, 2017 Figure 4 Left ilcnticidar liic. ) = reentiicldai septatl dcfce. The pulimonary lvorifice wxas larger than normiial, its xalve was niorml-ally formned, and it presented imiarkedly incIreased lhemodynamiiic chanlge. Its aInniulus wxxas in part related to the tricuspid valve. It xvas separated from the mlitral annulus by mnuscular ridge. The two pulmonoary arteries were given off normallv. The aL duictus arteriosus had been patent but xvas tied off at sutrgery. The left atritm avas niarkedly enlarged and its xvall xvas thickened. Its endocardium xvas diffusely wlxitenied. The mitral orifice xwas enlarged, its valxe xas n-iormlnally formlUed, and it presented miarked hemiiodynainiic change. The left ventricle (fig. 4) vas enlarged, but its wall normal iin thickness. The architecture of the inflow and outflow tracts reniormal left ventricle. Its sembled that of endocardium was diffusely whitenied. The ventricular septum at its base presented defect imieasuring about 1 cm in greatest dim-ension. This defect was situiated in the anterior part of the ventricular septum and was confluent with the left side of the mouth of the pulmonary trunk. From this defect, a triangular muscle l)and extended obliquely downward to the base of the posterior papillary muscle and sent a prong to the region of the anterior papillary muscle. The anatomic diaggnoses vere as follows: xvas a a 5. c. Left ventricular hypertrophy land enlargemenit Veintricular septal defect Fetal coarctation with an adult component with patenit ductus and surgical intervention (removal of adult coarctation ain-d closure of ductus) Abnormal architecture of the xentrieular septumn Patent foramyieni ovale. DIiseussion Hearts with l)iventricular orijin of the pulmonoary trunk wvith origin of the aorta fromii the right ventricle, with or vithout pulmonaomry steniosis, have recently been studied by one of us.4' A concept of xvhat we call the Taussigl3ing spectrum of hearts (or douille-outlet right ventricle of the Taussig-Bing type) vas thereby developed. It xvas suggested that the Tanssig-Bing heart be considered anianatoml-ic entity, in which the aorta arises from the right ventricle and is not related to the dlefect. while the puliimonary trunk arises fromii the right or 1)oth ventricles and is related to the defect. In addition1, there is a distinct Imluscenlature of the conal region of the right ventricle, in which a septal band group proceeds to the l)ase of the pulmonary trunk and connects to the first parietal band which separates the aorta from the pulmonary trunk. A second parietal l)and proceeds downward from the aorta and is related to the anterolateral leaflet of the tricuspid. Accordingly Taussig-Bing lhearts xvere cl assified as (1) right ventricular without overriding pulmonary trunk, (2) right ventricular with overriding pulmonary trunk, (3) intermnediate, and (4) left ventricular. In our case the pulmonary trunk overrides the defect but emerges mostly from the right ventricle xvhile the aorta emerges exclusively from the right ventricle away from the defect. Circulaion, Volume XXXVI, November 1967 TRUNCAL INVERSION Downloaded from http://circ.ahajournals.org/ by guest on April 29, 2017 The aorta and pulmonary trunk arise from positions in the right ventricle opposite in laterality to those in the usual Taussig-Bing heart as described. This is accompanied by a change in the conus of the right ventricle, where the septal band is related to the aorta and the second parietal band is related to the pulmonary trunk. The first parietal band however continues to separate the aorta and pulmonary trunk. Despite the alteration in muscular architecture of the conus of the right ventricle, we consider this to be related to other hearts with biventricular origin of the pulmonary trunk, with the aorta emerging from the right ventricle. We consider the alteration in musculature as being related to the alteration in laterality of the aorta and the pulmonary trunk. This alteration in laterality of the vessels, together with the anterior descending coronary artery coming off the right aortic sinus of Valsalva, is the anatomic hallmark of inversion of the truncal, trunco-conal or bulboventricular regions.6-22 Inversion, anatomically, may be considered to be a disturbance in laterality pursuant to a certain axis. This axis is the longitudinal axis of the body when the arterial trunks are considered, and the longitudinal axis of the heart when the atria and ventricles are considered. It is well known that the atria, ventricles, or the trunco-conal areas may be selectively inverted. It is not known whether the coni or arterial trunks may each be inverted alone, separate from the other. In our case the atria and sinuses of the ventricles were not inverted. On the other hand, the bases of the two arterial trunks, derived from the truncus, were clearly inverted. A problem arose in the interpretation of the conus. The normal conus has a distinct right side with septal and parietal bands, and an abbreviated left side fused with the remainder of the left ventricle. Traditionally we consider a conus inverted when its distinct portion with septal and parietal bands lies in the left-sided chamber rather than in the right. This was not true in our case, in which the distinct portion, bearing facsimiles of the septal and parietal bands, lay in the Circulation, Volume XXXVI, November 1967 787 right-sided chamber. Therefore we judge the conus not to be inverted. A Taussig-Bing heart, as above defined, with trunco-conal inversion should have the pulmonary trunk straddling the interventricular septum, while the aorta emerges completely from the left-sided ventricle over a septal and parietal band; hence it would be similar to a corrected transposition. The aortic annulus, of course, would be anterior and to the left of the pulmonary annulus. This anatomic arrangement would not be physiologically the same as Taussig-Bing heart without inversion, or in our interpretation of the Taussig-Bing heart with truncal inversion, in both of which the aorta is in the line of systemic venous blood. This anatomic concept of inversion may be expanded into a pathogenetic one, as Spitzer7 has done. We may be dealing with a reverse contribution of metameres as may occur in any one segment during the formation of the original single heart tube from its two-sided anlage.2 This inverse contribution of metameres in our case is assumed to occur only in the aortic bulb (truncal) area. The sino-atrial and ventriculobulbar contributions are presumed to be normal in this view, and the bulboventricular loop is normally formed. In this respect it may be recalled that the "aortic bulb" (truncus) is a distinct structure separate from the bulbus cordis.28 Together with the reverse metameric formation of the truncus is the unknown cause of transposition leading to biventricular origin of the pulmonary trunk, with the aorta emerging from the right ventricle. Conclusion A case is presented with biventricular origin of the pulmonary trunk, and origin of the aorta from the right ventricle, with truncal inversion. The aorta emerged from the right ventricle unrelated to a ventricular septal defect while the pulmonary trunk overrode the septum over a defect, but emerged mostly from the right ventricle. Altered septal and parietal bands were found in the right-sided ventricle. The aorta and pulmonary trunk SHAFFER ET AL 788 were inverted, while the atria and the sinuses of the ventricles were not inverted. Evidence is presented toward the concept that the conus was not inverted. 11. References 1. LEV, M., ROWLATT, U. 2. Downloaded from http://circ.ahajournals.org/ by guest on April 29, 2017 3. 4. 5. 6. 7. 8. 9. 10. F., AND RIMOLDI, H. J. A.: Pathologic methods for study of congenitally malformed heart: Methods for electrocardiographic and physiologic correlation. Arch Path (Chicago) 72: 493, 1961. LEV, M., AND ECKNER, F. A. O.: Embryologic, pathogenetic and pathologic considerations. In GASUL, B. M., ARCILLA, R. A., AND LEV, M.: Heart Disease in Children: Diagnosis and Treatment. Philadelphia, J. B. Lippincott Co., 1966, p. 11. LEV, M.: Some newer concepts of the pathology of congenital heart disease. Med Clin N Amer 50: 3, 1966. LEV, M., RIMOLDI, H. J. A., EcKNER, F. A. O., MELHUISH, B. P., MENG, C. C. L., AND PAUL, M. H.: Taussig-Bing heart: Qualitative and quantitative anatomy. Arch Path (Chicago) 81: 24, 1966. NAVARRO LOPEZ, F., ET AL.: Taussig-Bing complex with pulmonary stenosis. Dis Chest 50: 1, 1966. LEV, M., AND ROWLATT, U. F.: Pathologic anatomy of mixed levocardia: Review of thirteen cases of atrial or ventricular inversion with or without corrected transposition. Amer J Cardiol 8: 216, 1961. LEY, M., AND VASS, A.: Spitzer's Architecture of the Normal and Malformed Hearts. (Translation of Spitzer, A.: tber den Bauplan des normalen und missbildeten Herzens: Versuch einer phylogenetischen Theorie. Virchow Arch Path Anat 243: 81, 1923.) Springfield, Illinois, Charles C Thomas, Publisher, 1951. CARDELL, B. S.: Corrected transposition of the great vessels. Brit Heart J 18: 186, 1956. ANDEHRSON, R. C., LILLEHEI, C. W., AND LESTER, R. G.: Corrected transposition of the great vessels of the heart: Review of 17 cases. Pediatrics 20: 626, 1957. DE LA CRUZ, M. V., ANSELMI, G., CISNEROs, F., R EINHOLD, M., PORTILLO, B., AND ESPINOVELA, J.: Embryologic explanation for the corrected transposition of the great vessels: Additional description of the main anatomic 12. 13. 14. features of the malformation and its varieties. Amer Heart J 57: 104, 1959. SCHIEBLER, G. L., EDWARDS, J. E., BURCHELL, H. B., DUSHANE, J. W., ONGLEY, P. A., AND WOOD, E. H.: Congenital corrected transposition of the great vessels: Study of 33 cases. Pediatrics 27: 851, 1961. HONEY, M.: Anatomical and physiological features of corrected transposition of the great vessels. Guy Hosp Rep 111: 250, 1962. SHAHER, R. M.: Syndromes of corrected transposition of the great vessels. Brit Heart J 25: 431, 1963. VAN MIEROP, L. H. S., AND WIGLESWORTH, F. W.: Pathogenesis of transposition complexes: III. True transposition of the great vessels. Amer J Cardiol 12: 233, 1963. 15. GRANT, R. P.: Morphogenesis of corrected transposition and other anomalies of cardiac polarity. Circulation 29: 71, 1964. 16. ROSENBAUM, H. D.: Simplified basic classification of spatial alignments of the heart, its chambers, and the great vessels. Circulation 30: 194, 1964. 17. BERRY, W. B., ROBERTS, W. C., MoRmow, A. G., AND BRAUNWALD, E.: Corrected transposition of the aorta and pulmonary trunk. Amer J Med 36: 35, 1964. 18. SHAHER, R. M.: Complete and inverted transposition of the great vessels. Brit Heart J 26: 51, 1964. 19. VAN PRAAGH, R., ONGLEY, P. A., AND SWAN, H. J. C.: Anatomic types of single or common ventricle in man: Morphologic and geometric aspects of 60 necropsied cases. Amer J Cardiol 13: 367, 1964. 20. VAN PRAAGH, R., VAN PRAAGH, S., VLAD, P., AND KErrH, J. D.: Anatomic types of congenital dextrocardia: Diagnostic and embryologic implications. Amer J Cardiol 13: 510, 1964. 21. VAN PRAAGH, R., AND VAN PRAAGH, S.: Isolated ventricular inversion: Consideration of the morphogenesis, definition and diagnosis of nontransposed and transposed great arteries. Amer J Cardiol 17: 395, 1966. 22. RAGHIB, G., ANDERSON, R. C., AND EDWARDS, J. E.: Isolated bulbar inversion in corrected transposition. Amer J Cardiol 17: 407, 1966. 23. DAVIS, C. L.: Development of the human heart from its first appearance to the stage found in embryos of twenty paired somites. Contrib Embryol 19: 245, 1927. Circulation, Volutme XXXVI, Novemnbet 1967 Truncal Inversion with Biventricular Pulmonary Trunk and Aorta from Right Ventricle (Variant of Taussig-Bing Complex) AARON B. SHAFFER, JOSÉ F. LOPEZ, IRWIN K. KLINE and MAURICE LEV Downloaded from http://circ.ahajournals.org/ by guest on April 29, 2017 Circulation. 1967;36:783-788 doi: 10.1161/01.CIR.36.5.783 Circulation is published by the American Heart Association, 7272 Greenville Avenue, Dallas, TX 75231 Copyright © 1967 American Heart Association, Inc. All rights reserved. Print ISSN: 0009-7322. Online ISSN: 1524-4539 The online version of this article, along with updated information and services, is located on the World Wide Web at: http://circ.ahajournals.org/content/36/5/783 Permissions: Requests for permissions to reproduce figures, tables, or portions of articles originally published in Circulation can be obtained via RightsLink, a service of the Copyright Clearance Center, not the Editorial Office. Once the online version of the published article for which permission is being requested is located, click Request Permissions in the middle column of the Web page under Services. 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