Hostname: page-component-cd9895bd7-mkpzs Total loading time: 0 Render date: 2024-12-27T11:08:42.307Z Has data issue: false hasContentIssue false
  • English
  • Français

Correlating the morphological features of tetralogy of Fallot and the Eisenmenger malformation

Published online by Cambridge University Press:  08 April 2016

Angelo Restivo ,
Robert H. Anderson ,
Raffaella Carletti  and
Cira R. T. di Gioia
Angelo Restivo
Affiliation:
Departmentof Radiology, Oncology, and Pathological Anatomy, Museum of Pathological Anatomy, Sapienza University of Rome, Rome, Italy
Robert H. Anderson
Affiliation:
Institute of Genetic Medicine, Newcastle University, Newcastle-upon-Tyne, United Kingdom
Raffaella Carletti
Affiliation:
Departmentof Radiology, Oncology, and Pathological Anatomy, Museum of Pathological Anatomy, Sapienza University of Rome, Rome, Italy
Cira R. T. di Gioia*
Affiliation:
Departmentof Radiology, Oncology, and Pathological Anatomy, Museum of Pathological Anatomy, Sapienza University of Rome, Rome, Italy
*
Correspondence to: Prof. C. R. T. di Gioia, Dipartimento di, Scienze Radiologiche, Oncologiche e Anatomopatologiche, Sezione di, Anatomia Patologica, Sapienza Università di Roma, Policlinico Umberto I, Viale Regina Elena, 324, 00161 Roma, Italy. Tel/Fax: +39 0649 977 3329; E-mail: cira.digioia@uniroma1.it
Get access Rights & Permissions [Opens in a new window]

Abstract

We studied a series of 43 autopsied cases of tetralogy of Fallot, assessing the mode of insertion of the outlet septum relative to the limbs of the septomarginal trabeculation, and compared the findings in retrospective fashion with our previous observations of a group of hearts with the so-called Eisenmenger malformation. In the majority of hearts with tetralogy of Fallot, the outlet septum inserted frontally relative to the septomarginal trabeculation, but in a minority of cases the outlet septum inserted in lateral fashion, as had been observed in all our hearts studied with the Eisenmenger malformation. The different modes of insertion were found to correlate, first, with the axis of anatomical aortic rightward rotation, coincident with the commissure between the right coronary and the left coronary leaflets of the aortic valve. The different modes of insertion of the outlet septum also correlated with the level of attachment of the arterial valvar leaflets on its subpulmonary and subaortic surfaces; concomitantly, correlation was found between the length of the subpulmonary infundibulum and the length of the muscular outlet septum itself. In the majority of hearts showing tetralogy of Fallot, the elongated infundibulum was also uniformly narrow, but in a minority the infundibulum was well expanded, obstructed only at its mouth, but widening at the valvar level. In all the hearts with the Eisenmenger malformation, in contrast, the unobstructed infundibulum was well expanded. The morphological findings of the present study show unequivocally that tetralogy of Fallot and Eisenmenger malformation are two phenotypically different congenital cardiac anomalies.

Keywords

Tetralogy of FallotEisenmenger malformationaortic rightward positionoutlet septumseptomarginal trabeculation
Type
Original Articles
Information
Cardiology in the Young , Volume 27 , Issue 1 , January 2017 , pp. 161 - 172
Check for updates
Copyright
© Cambridge University Press 2016 

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1. Fallot, ELA. Contribution à l’anatomie pathologique de la maladie bleu (cyanose cardiaque). Marseille Med 1888; 25: 77–93.Google Scholar
2. Abbott, ME. Atlas of Congenital Heart Disease. American Heart Association, New York, 1936, 46–47.Google Scholar
3. Van Praagh, R, Van Praagh, S, Nebesar, RA, Muster, AJ, Sinha, SN, Paul, MH. Tetralogy of Fallot: underdevelopment of the pulmonary infundibulum and its sequelae. Am J Cardiol 1970; 26: 25–33.Google Scholar
4. Anderson, RH, Tynan, M. Tetralogy of Fallot – a centennial review. Int J Cardiol 1988; 21: 219–232.Google Scholar
5. Anderson, RH, Weinberg, PM. The clinical anatomy of tetralogy of Fallot. Cardiol Young 2005; 15 (Suppl 1): 38–47.Google Scholar
6. Anderson, RH, Spicer, DE, Giroud, JM, Mohun, TJ. Tetralogy of Fallot: morphological and morphogenetic considerations. Cardiol Young 2013; 23: 855–886.Google Scholar
7 Fukuda, T, Suzuki, T, Ito, T. Clinical and morphologic features of perimembranous ventricular septal defect with overriding of the aorta – the so called Eisenmenger ventricular septal defect. A study making comparisons with tetralogy of Fallot and perimembranous ventricular defect without aortic overriding. Cardiol Young 2000; 103: 343–352.Google Scholar
8. Restivo, A, di Gioia, CRT, Anderson, RH, Carletti, R, Gallo, P. The Eisenmenger malformation: a morphologic study. Cardiol Young 2016; 26: 269–279.Google Scholar
9. Goor, DA, Lillehey, WC, Edwards, JE. Ventricular septal defects and pulmonic stenosis with and without dextroposition. Anatomic and embryologic implications. Chest 1971; 60: 117–128.Google Scholar
10. Pernkopf, E, Wirtinger, W. Die transposition der herzostien fin versuch der erklarung dieser erscheinung. Z Anat Entwiccklungsgesch 1933; 10: 563.Google Scholar
11. Goor, Da, Dische, R, Lillhei, CW. The conotruncus. I. Its normal inversion and conus absorption. Circulation 1972; 46: 375–378.Google Scholar
12. Anderson, RH, Wilkinson, JL, Arnold, R, Becker, AE, Lubkiewicz, K. Morphogenesis of bulboventricular malformations. II. Observations on malformed hearts. Br Heart J 1974; 36: 948–970.CrossRefGoogle ScholarPubMed
13. Bajolle, F, Zaffran, S, Kelly, RG, et al. Rotation of the myocardial wall of the outflow tract is implicated in the normal positioning of the great arteries. Circ Res 2006; 98: 421–428.Google Scholar
14. Eisenmenger, V. Die angeborenen defecte der kammersheidewand des herzens. Ztschr F Klin Med 1898; 32 (Suppl): 1–56.Google Scholar
15. Altshuler, G. The ventricular septal defect. Am J Dis Child 1970; 119: 407–415.Google Scholar
16. Gasul, BM, Dillon, RF, Vrla, V, Hait, G. Ventricular septal defects. Their natural transformation into those with infundibular stenosis or into the cyanotic or non cyanotic type of tetralogy of Fallot. JAMA 1957; 164: 847–853.Google Scholar
17. Isaaz, K, Cloez, JL, Marcon, F, Worms, AM, Pernot, C. Is the aorta truly dextroposed in tetralogy of Fallot? A two dimensional ecocardiographic answer. Circulation 1986; 73: 892–899.Google Scholar
18. Anderson, RH, Becker, AE. Tetralogy of Fallot. In Anderson RH and Becker AE (eds). Controversies in the description of congenitally malformed hearts. Imperial College Press, London, United Kingdom, 1997: 113–148.Google Scholar
19. Griffin, ML, Sullivan, ID, Anderson, RH, Macartney, FJ. Doubly committed subarterial ventricular septal defect: new morphological criteria with echocardiographic and angiocardiographic correlation. Br Heart J 1988; 59: 474–479.Google Scholar
20. Becker, AE, Connor, M, Anderson, RH. Tetralogy of Fallot: a morphometric and geometric study. Am J Cardiol 1975; 35: 402–412.Google Scholar
21. Howell, CE, Ho, SY, Anderson, RH, Elliott, MJ. Variations within the fibrous skeleton and ventricular outflow tracts in tetralogy of Fallot. Ann Thorac Surg 1990; 50: 450–457.Google Scholar
22. Dickinson, DF, Wilkinson, JL, Smith, A, Hamilton, DI, Anderson, RH. Variations in the morphology of the ventricular septal defect and disposition of the atrioventricualr conduction tissues in tetralogy of Fallot. Thorac Cardiovasc Surg 1982; 30: 243–249.Google Scholar
23. Van Praagh, S, Davidoff, A, Chin, A, Shiel, FS, Reynolds, J, Van Praagh, R. Double outlet right ventricle: anatomic types and developmental implications based on a study of 101 autopsied cases. Coeur 1982; 13: 389–439.Google Scholar
24. Van Mierop, LHS, Wiglesworth, FW. Anomalies due to faulty transfer of the posterior great artery. Am J Cardiol 1963; 12: 226–232.Google Scholar
25. Suzuki, A, Yo, SY, Anderson, RH, Deanfield, JE. Further morphologic studies on tetralogy of Fallot, with particular emphasis on the prevalence and structure of the membranous flap. J Thorac Cardiovasc Surg 1990; 99: 528–535.Google Scholar
26. Kurosawa, H, Morita, K, Yamagishi, M, Shimizu, S, Becker, AE, Anderson, RH. Conotruncal repair for tetralogy of Fallot: midterm results. J Thorac Cardiovasc Surg 1998; 115: 351--360.Google Scholar