Heart and Vessels

, Volume 2, Issue 2, pp 117–126 | Cite as

Pathological anatomy of ventricular septal defect associated with aortic valve prolapse and regurgitation

  • Masahiko Ando
  • Atsuyoshi Takao
Originals

Summary

In an attempt to clarify the pathogenetic morphology of aortic regurgitation (AR) due to prolapse of the aortic valve (prolapsing AR) associated with ventricular septal defect (VSD), 201 specimens from Japanese autopsy series with isolated VSD were examined. Among these hearts, there were 128 cases (64%) of infundibular VSD (IVSD); 29 of them (14%) showed AR due to prolapsed cusp, of which nine cases developed a large aneurysm of the sinus of Valsalva. Another 32 cases (16%) had varying degrees of prolapse but without AR and were considered to show the prodrome of prolapsing AR. These 61 cases (30%) were examined with special reference to the type of septal alignment, location of the defect, relation of the defect to the aortic valve, and anomalies of the aortic valve and sinus of Valsalva. There were two principal forms in this syndrome: The common form, i.e., simple punched-hole IVSD with normal septal alignment in 82% (50/61) of cases, and a rare form, i.e., malalignment IVSD in 18% (11/61) of cases. The latter included Eisenmenger-type IVSD due to anteriorly deviated outlet septum (10/11 cases) and coarctation-type IVSD due to posteriorly deviated septum (1/11). Both forms had several subtypes according to the location of the defect, i.e., subpulmonic, muscular, perimembranous, and total IVSD.

The relevant anatomical findings of the common form of the syndrome were: (1) There was no septal malalignment with a normal aortic valve position. (2) The VSD was a simple muscular defect in any part of the infundibular septum between the pulmonary valve above and the membranous septum below, the majority of cases (80%), however, showed subpulmonic IVSD. (3) The annulus and sinus of Valsalva wall of the right coronary cusp, which is normally supported firmly by this septum, became exposed in the muscle defect and were poorly supported. (4) The majority of cases showed a normally formed aortic valve but with poor support. (5) The muscular defect was relatively large, but the functioning VSD was usually less than moderate in size with a half-moon shape below the denuded sinus of Valsalva wall and annulus. (6) The functioning VSD appeared to become narrower depending on the degree of prolapse into the defect, resulting in a crescent-moon or slit-like shape, and it may close in rare cases.

The major anatomical findings of the rare form were: (1) There was mild to moderate dextroposition (or levoposition) of the aortic valve due to a septal malalignment. (2) The size of the VSD was moderate or less with a location in any part of the infundibular septum, but most commonly involving the perimembranous infundibular portion (70%). (3) It was always associated with anomalies of the aortic valve, such as bicuspid valve or uneven division of the cusps, and a deformed wall of the sinus of Valsalva, which appeared to be related to a less than moderate septal malalignment. (4) The deformed aortic valve was exposed in the defect.

The development of the prolapsing AR in the two forms of the syndrome could be attributed to the interaction between the above-mentioned anatomical defects and hemodynamic factors, among which the most important was the “Venturi effect” caused by a rapid blood flow through the functioning VSD according to Bernoulli's theorem. With progress of the disease, this syndrome is clinically subdivided into three stages: The prolapsing stage which is characterized by the Venturi effect, the reversible AR stage which has AR and the Venturi effect, and the irreversible AR stage in which the deterioration of the aortic valve is complete. Early surgical intervention to stop the Venturi effect is recommended, at least at or preferably before the reversible AR stage, even in those patients with malalignment-type VSD who have inherent anomalies of the aortic valve and its apparatus.

Key words

Infundibular VSD Prolapsed aortic valve Aortic regurgitation VSD Venturi effect Racial difference 

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References

  1. 1.
    Tatsuno K, Ando M, Takao A, Hatsune K, Konno S (1975) Diagnostic importance of aortography in conal ventricular septal defect. Am Heart J 89: 171–177PubMedGoogle Scholar
  2. 2.
    Ando M, Takao A (1978–1979) Racial differences in the morphology of common cardiac anomalies. Bulletin of the Heart Institute Japan: 47–66Google Scholar
  3. 3.
    Van Praagh R, McNamara JJ (1968) Anatomic types of ventricular septal defect with aortic insufficiency. Am Heart J 75: 604–619PubMedGoogle Scholar
  4. 4.
    Nadas A, Thilenius OG, LaFarge CG, Hauck AJ (1964) Ventricular septal defect with aortic regurgitation; Medical and pathologic aspects. Circulation 29: 862–873PubMedGoogle Scholar
  5. 5.
    Tatsuno K, Konno S, Ando M, Sakakibara S (1973) Pathogenetic mechanisms of prolapsing aortic valve and aortic regurgitation associated with ventricular septal defect. Circulation 48: 1028–1037PubMedGoogle Scholar
  6. 6.
    Tatsuno K, Konno S, Sakakibara S (1973) Ventricular septal defect with aortic insufficiency; Angiographic aspects and a new classification. Am Heart J 85: 13–21PubMedGoogle Scholar
  7. 7.
    Sakakibara S, Konno S (1968) Congenital aneurysm of the sinus of Valsalva associated with ventricular septal defect. Am Heart J 75: 593–603Google Scholar
  8. 8.
    Gonzalez-Lavin L, Barratt-Boyes (1969) Surgical consideration in the treatment of ventricular septal defect associated with aortic valve incompetence. J Cardiovasc Surg 57: 422–430Google Scholar
  9. 9.
    Someville J, Brandao A, Ross DH (1970) Aortic regurgitation with ventricular septal defect, sugical management and clinical features. Circulation 41: 317–330PubMedGoogle Scholar
  10. 10.
    Keane JF, Plauth WH, Nadas AS (1977) Ventricular septal defect with aortic regurgitation. Circulation 56: I-72-77Google Scholar
  11. 11.
    Soto B, Becker AE, Mouleart AJ, Lie JT, Anderson RH (1980) Classification of ventricular septal defect. Br Heart J 43: 332–343PubMedGoogle Scholar
  12. 12.
    Goor DA, Edwards JE, Lillehei CW (1970) The development of the interventricular septum of human heart; correlative morphogenetic study. Chest 58: 453–467PubMedGoogle Scholar
  13. 13.
    Selzer A, Laqueun GL (1951) The Eisenmenger complex and its relation to the uncomplicated defect of the ventricular septum; Review of thirty-five autopsied cases of Eisenmenger complex, including two new cases. Arch Intern Med 87: 218Google Scholar
  14. 14.
    Goor DA, Lillehei CW, Rees R, Edwards JE (1970) Isolated ventricular septal defect. Developmental bases for various types and presentations of classification. Chest 58: 468–482PubMedGoogle Scholar
  15. 15.
    Momma K, Toyama K, Takao A, Nakazawa M, Hirosawa K, Imai Y (1984) Natural history of subarterial infundibular VSD. Am Heart J 108: 1312–1317PubMedGoogle Scholar
  16. 16.
    Tatsuno K, Ando M, Takao A, Hatsune K, Konno S (1975) Diagnostic importance of aortography in conal ventricular septal defect. Am Heart J 89:-171–177PubMedGoogle Scholar
  17. 17.
    de la Cruz MV, Campillo-Sainz C, Munoz-Armas S (1966) Congenital heart defect in chick embryos subjected to temperature variations. Circ Res 18:257–262PubMedGoogle Scholar

Copyright information

© Springer-Verlag 1986

Authors and Affiliations

  • Masahiko Ando
    • 1
  • Atsuyoshi Takao
    • 1
  1. 1.Department of Pediatric CardiologyThe Heart Institute of Japan, Tokyo Women's Medical CollegeTokyoJapan

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