Hostname: page-component-586b7cd67f-t7fkt Total loading time: 0 Render date: 2024-11-28T05:04:07.777Z Has data issue: false hasContentIssue false
  • English
  • Français

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

Published online by Cambridge University Press:  19 August 2008

Toyoki Fukuda ,
Takaaki Suzuki  and
Tsutomu Ito
Toyoki Fukuda*
Affiliation:
Department of Cardiovascular Surgery, Tokyo Metropolitan Kiyose Children's Hospital, Tokyo, Japan
Takaaki Suzuki
Affiliation:
Department of Cardiovascular Surgery, Tokyo Metropolitan Kiyose Children's Hospital, Tokyo, Japan
Tsutomu Ito
Affiliation:
Department of Cardiovascular Surgery, Tokyo Metropolitan Kiyose Children's Hospital, Tokyo, Japan
*
Toyoki Fukuda, MD, Director of Cardiovascular Surgery, Tokyo Metropolitan Kiyose Children's Hospital, 1–3–1 Umezono, Kiyose-shi, Tokyo 204–8567, Japan. Tel: +81–424–91–0011; Fax: +81–424–92–6291; E-mail: [email protected]
Get access Rights & Permissions [Opens in a new window]

Abstract

The aim of our study was to elucidate the clinical and morphologic features of those perimembranous ventricular septal defects which extend between the ventricular outlets, particularly when found in association with anterior deviation of the muscular outlet septum and overriding of the aorta – the so called Eisenmenger ventricular septal defect. From 1990 through 1998, we have undertaken surgical correction in 203 patients with perimembranous ventricular septal defect. Of these, 15 patients had the Eisenmenger ventricular septal defect. We conducted retrospective analyses of the clinical records, catheterization data, and angiocardiographic and echocardiographic finding of these patients. Comparative studies were then made with the patients having tetralogy of Fallot, and those with simple perimembranous ventricular septal defects without overriding of the aorta. In the patients with the Eisenmenger ventricular septal defect, the extent of anterior deviation of the outlet septum was comparable with that seen in tetralogy of Fallot, but there was less rightward displacement of the aortic valvar orifice. In contrast to earlier investigators, however, we found evidence of progressive narrowing of the subpulmonary infundibulum in those with the Eisenmenger defect. These morphological features were reflected in the clinical features, since all patients showed evidence of increased pulmonary flow and congestive heart failure in early infancy, but with twothirds of them subsequently developing right-to-left shunting. We conclude, therefore, that the Eisenmenger ventricular septal defect is a discrete cardiac abnormality in which the morphologic substrate of anterior deviation of the outlet septum gives rise to a potential for progressive narrowing of the subpulmonary infundibulum. Surgical management, therefore, needs to take account of such narrowing as an additional cardinal morphologic feature.

Keywords

perimembranous ventricular septal defectoverriding of the aorta
Type
Original Articles
Information
Cardiology in the Young , Volume 10 , Issue 4 , July 2000 , pp. 343 - 352
Copyright
Copyright © Cambridge University Press 2000

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

1Goor, DA, Lillehei, CW, Edwards, JE. Ventricular septal defects and pulmonic stenosis with and without dextroposition. Anatomic features and embryologic implications. Chest 60; 1971: 117–128Google ScholarPubMed
2Moulaert, AJ, Bruins, CC, Oppenheimer-Dekker A. Anomalies of the aortic arch and ventricular septal defects. Circulation 1976; 53: 1011–1015CrossRefGoogle ScholarPubMed
3Juaneda, E, Gittenberger-de Groot, AC, Oppenheimer-Dekker, A, Haworth, SG. Pulmonary arterial development in infants with large perimembranous ventricular septal defects associated with overriding of aortic valve. Int J Cardiol 1985; 7. 223–230CrossRefGoogle ScholarPubMed
4Ueda, M, Becker, AE. Classification of hearts with overriding of aortic and pulmonary valves. Int J Cardiol 1985; 9: 357–369CrossRefGoogle ScholarPubMed
5Moene, RJ, Oppenheimer-Dekker, A, Bartelings, MM, Wenink, ACG, Gittenberger-de Groot, AC. Ventricular septal defect with normally connected and with transposed great arteries. Am J Cardiol 1986; 58: 627–632CrossRefGoogle ScholarPubMed
6Oppenheimer-Dekker, A, Gittenberger-de Groot, AC, Bartelings, MM, Wenink, ACG, Moene, RJ, van der Harden, JJ. Anomalous architecture of the ventricles in hearts with overriding of aortic valve and a perimembranous ventricular septal defect (“Eisenmenger VSD”). Int J Cardiol 1989; 9: 341–355CrossRefGoogle Scholar
7Anderson, RH, Wilcox, BR. The surgical anatomy of ventricular septal defect. J Card Surg 1992; 7: 17–35CrossRefGoogle ScholarPubMed
8Anderson, RH, Wilcox, BR. The surgical anatomy of ventricular septal defects associated with overriding valvar orifices. J Card Surg 1993; 8: 130–142CrossRefGoogle ScholarPubMed
9Gatzoulis, MA, Soukias, N, Ho, SY, Josen, M, Anderson, RH. Echocardiographic and morphological correlation in tetralogy of Fallot. Eur Heart J 1999; 20: 221–231CrossRefGoogle ScholarPubMed
10Isaaz, K, Cloez, JL, Maròbon, F, Worms, AM, Pernot, C. Is the aorta truly dextroposed in tetralogy of Fallot? Circulation 1986; 73: 892–899CrossRefGoogle ScholarPubMed
11Howell, CE, Yen Ho, S, Anderson, RH, Elliott, MJ. Variations within the fibrous skeleton and ventricular outflow tracts in tetralogy of Fallot. Ann Thorac Surg 1990; 50: 450–457CrossRefGoogle ScholarPubMed
12Gasul, BM, Dillon, RF, Vrla, V, Hait, G. Ventricular septal defects. Their natural transformation into those with infundibular stenosis or into the cyanotic or noncyanotic type of tetralogy of Fallot. JAMA 1957; 164: 847–853Google ScholarPubMed
13Bartelings, MM, Wenink, ACG, Gittenberger-de Groot, AC, Oppenheimer-Dekker, A.Contribution of the aortopulmonary septum to the muscular outlet septum in the human heart. Acta Morphol Neerl-Scand 1986; 24: 181–192Google Scholar
14Bartelings, MM, Gittenberger-de Groot, AC. The outflow tract of the heart — embryologic and morphologic correlations. Int J Cardiol 1989; 22: 289–300CrossRefGoogle ScholarPubMed
15Anderson, RH, Tynan, M. Tetralogy of Fallot - a centennial review. Int J Cardiol 1988; 21: 219–232CrossRefGoogle ScholarPubMed
16Anderson, RH, Wilcox, BR. Understanding of Cardiac Anatomy: The Prerequisite for Optimal Cardiac Surgery Ann Thorac Surg 1995; 59: 1366–1375CrossRefGoogle ScholarPubMed
17Becker, AE, Connor, M, Anderson, RH. Tetralogy of Fallot: A morphometric and geometric study.Am J Cardiol 1975; 35: 402–412CrossRefGoogle ScholarPubMed
18Pacifico, AD, Sand, ME, Bargeron, LM, Colvin, EC. Transatrialtranspulmonary repair of tetralogy of Fallot. J Thorac Cardiovasc Surg 1987; 93: 919–924CrossRefGoogle ScholarPubMed
19Karl, TR, Sano, S, Pornvilivan, S, Mee, RBB. Tetralogy of Fallot Favorable outcome of non-neonatal transatrial-transpulmonary repair. Ann Thorac Surg 1992; 54: 903–907Google Scholar