Advertisement

What Determines Whether the Great Arteries Are Normally or Abnormally Related?

      The situs, or pattern of anatomic organization, of the subarterial infundibulum and of the great arteries and the degree of development of the subarterial infundibulum largely determine whether the great arteries are normally or abnormally related. There are 2 types of situs: solitus (normal) and inversus (a mirror image of solitus). Situs ambiguus means that the pattern of anatomic organization is uncertain or unknown. Infundibular development varies from absent, to atretic, to severely stenotic, to mildly or moderately stenotic; great arteries are solitus normally related or inversus normally related, respectively. When the situs of the subarterial infundibulum and the situs of the great arteries are discordant (different), then the great arteries are abnormally related. Equations indicating the situs of the infundibulum and the situs of the great arteries show whether infundibuloarterial (IA) situs concordance or discordance is present. Many types of IA anomalies typically have IA situs discordance, including transposition of the great arteries, double-outlet right ventricle, double-outlet left ventricle, and anatomically corrected malposition of the great arteries. However, tetralogy of Fallot and truncus arteriosus typically have IA situs concordance, with hypoplasia or atresia of the subpulmonary infundibulum. The relation between the great arteries in tetralogy of Fallot and in truncus arteriosus is almost normal. The IA equations demonstrate the infundibular situs, the great arterial situs, the IA situs concordance or discordance, and the degree of development of the infundibulum. The infundibular situs and the great arterial situs are the formulas or “recipes” for each of the abnormal types of conotruncal malformation.
      To read this article in full you will need to make a payment

      References

      1. Quain R, 1844, cited by Peacock TB. On Malformations etc of the Human Heart, with Original Cases, London, J Churchill 1858, 115, 116.

        • Keith A.
        The Hunterian lectures on malformations of the heart.
        Lancet. 1909; 2: 359-363
        • Lev M.
        • Saphir O.
        Transpositions of the large vessels.
        J Tech Methods Bull Internat A Med Museums. 1937; 17: 126-162
        • De la Cruz M.V.
        • Anselmi G.
        • Cisneros F.
        • Reinhold M.
        • Portillo B.
        • Espino-Vela J.
        An embryologic explanation for the corrected transposition of the great vessels: additional description of the main anatomic features of this malformation and its varieties.
        Am Heart J. 1959; 57: 104-117
        • Grant R.P.
        The morphogenesis of transposition of the great vessels.
        Circulation. 1962; 26: 819-840
        • Van Praagh R.
        • Vlad P.
        Complete transposition of the great arteries.
        in: Keith J.D. Rowe R.D. Vlad P. Heart Disease in Infancy and Childhood. second ed. Macmillan, New York1967: 682-744
        • Simpson D.P.
        Cassell's Latin Dictionary, Latin-English, English-Latin. Situs–ūs, m.
        Macmillan Publishing, New York1968: 558
        • Van Praagh R.
        • Van Praagh S.
        Isolated ventricular inversion, a consideration of the morphogenesis, definition, and diagnosis of nontransposed and transposed great arteries.
        Am J Cardiol. 1966; 17: 395-406
        • Van Praagh R.
        The cardiovascular keys to air-breathing and permanent land-living in vertebrates: the normal human embryonic aortic switch procedure produced by complete right-left asymmetry in the development of the subarterial conal free walls, and the evolution of the right ventricular sinus.
        Kardiochirurgia i Torakochirurgia Polska. 2011; 8: 1-22
        • Taussig H.B.
        • Bing R.J.
        Complete transposition of the aorta and a levoposition of the pulmonary artery, clinical, physiological, and pathological findings.
        Am Heart J. 1949; 37: 551-559
        • Van Praagh R.
        What is the Taussig-Bing malformation?.
        Circulation. 1968; 38: 445-449
        • Paul M.H.
        • Muster A.J.
        • Sinha S.N.
        • Cole R.B.
        • Van Praagh R.
        Double-outlet left ventricle with an intact ventricular septum: clinical and autopsy diagnosis and developmental implications.
        Circulation. 1970; 41: 129-139
        • Van Praagh R.
        • Durnin R.E.
        • Jockin H.
        • Wagner H.R.
        • Korns M.
        • Garbedian H.
        • Ando M.
        • Calder A.L.
        Anatomically corrected malposition of the great arteries {S, D,L}.
        Circulation. 1975; 51: 20-31
        • Van Praagh R.
        The first Stella Van Praagh memorial lecture: the history and anatomy of tetralogy of Fallot.
        Semin Thorac Cardiovasc Surg Pediatr Card Surg Ann. 2009; 12: 19-38
        • Van Praagh R.
        • Van Praagh S.
        The anatomy of common aorticopulmonary trunk (truncus arteriosus communis) and its embryologic implications, a study of 57 necropsied cases.
        Am J Cardiol. 1965; 16: 406-425
        • Calder L.
        • Van Praagh R.
        • Van Praagh S.
        • Sears W.P.
        • Corwin R.
        • Levy A.
        • Keith J.D.
        • Paul M.H.
        Truncus arteriosus communis: clinical, angiocardiographic, and pathologic findings in 100 patients.
        Am Heart J. 1976; 92: 23-38
        • Vizcaino A.
        • Campbell J.
        • Litovsky S.
        • Van Praagh R.
        Single origin of right and left pulmonary artery branches from ascending aorta with nonbranching main pulmonary artery: relevance to a new understanding of truncus arteriosus.
        Pediatr Cardiol. 2002; 23: 230-234
        • Spitzer A.
        Über den Bauplan des normalen und missbildeten Herzens. Versuch einer Phylogenetischen Theorie.
        Virchows Arch F Path Anat. 1923; 243: 81-201
        • Spitzer A.
        The Architecture of Normal and Malformed Hearts, a Phylogenetic Theory of Their Development, with Summary and Analysis by M Lev and A Vass.
        Charles C. Thomas, Springfield, IL1951: 1-145
        • Bremer J.L.
        Transposition of the aorta and the pulmonary artery. An embryologic study of its cause.
        Arch Path. 1942; 34: 1016-1030
        • Goettler K.
        Hamodynamische Untersuchungen über die Entstehung der Missbildugen Des arteriellen Herzendes.
        Virchows Arch Path Anat. 1956; 328: 391-420
        • DeVries P.A.
        • Saunders JB. de CM.
        Development of the ventricles and spiral outflow tract in the human heart, a contribution to the development of the human heart from age group 1X to age group XV.
        Carnegie Inst Washinton, Contrib Embryol. 1962; 37: 89-114
        • Jaffee O.C.
        Comparative and experimental studies of the development of blood flow patterns in embryonic hearts. Cardiac Development with Special Reference to Congenital Heart Disease.
        University of Dayton Press, Dayton, Ohio1970: 45-56
        • Van Mierop L.H.S.
        • Wiglesworth F.W.
        Pathogenesis of transposition complexes. III. True transposition of the great vessels.
        Am J Cardiol. 1963; 12: 233-239
        • Van Praagh R.
        • Layton W.M.
        • Van Praagh S.
        The morphogenesis of normal and abnormal relationships between the great arteries and the ventricles: pathologic and experimental data.
        in: Van Praagh R. Takao A. Etiology and Morphogenesis of Congenital Heart Disease. Futura Publishing, Mt. Kisco, New York1980: 271-316
        • Foran R.B.
        • Becourt C.
        • Nanton M.A.
        • Murphy D.A.
        • Weinberg A.G.
        • Liebman J.
        • Castañeda A.R.
        • Van Praagh R.
        Isolated infundibuloarterial inversion {S, D,I}: a newly recognized form of congenital heart disease.
        Am Heart J. 1988; 116: 1337-1350
        • Van Praagh R.
        • Santini F.
        • Geva T.
        Segmental situs in congenital heart disease: a fundamental concept.
        G Ital Cardiol. 1990; 20: 246-253
        • Van Praagh R.
        • Geva T.
        • Kreutzer J.
        Ventricular septal defects: how shall we describe, name, and classify them?.
        J Am Coll Cardiol. 1989; 14: 1298-1299
        • Van Praagh R.
        Approccio segmentario alla diagnosi delle cardiopatie congenite.
        in: Squarcia U. Progressi in Cardiologia Pediatrica. Casa Editrice Ambrosiana, Milano1978: 7-45