Skip to main content
NCBI home page
The American Journal of Pathology logoLink to The American Journal of Pathology
. 1973 Oct;73(1):157–172.

The Pathogenesis of Tetralogy of Fallot

Kevin J Winn, Grover M Hutchins
PMCID: PMC1904044  PMID: 4270678

Abstract

A pathogenetic mechanism for the formation of tetralogy of Fallot is postulated. We propose that the right ventricular ejection stream is divided into a transseptal aortic stream and an infundibular pulmonary stream during embryonic life before the ventricular septum is closed. The surgically unaltered hearts from 77 cases of tetralogy of Fallot were examined, and measurements made of certain morphologic features. A malformed, stenotic pulmonary valve was found in most all cases and accounts for a divided ejection stream. Infundibular stenosis is shown to be a postnatally acquired, progressive lesion. The detailed anatomic features of the heart and great vessels in tetralogy of Fallot are explained as a response of the developing heart and great vessels to the abnormal blood flow pattern of a divided right ventricular ejection stream. This pathogenetic mechanism, compared to previously proposed hypotheses, appears better able to account for the malformation complex of tetralogy of Fallot.

Full text

PDF
157

Images in this article

171Fig 1
on p.171
171Fig 2
on p.171
171Fig 3
on p.171
171Fig 4
on p.171
171Fig 5
on p.171

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. BECU L., IKKOS D., LJUNGQVIST A., RUDHE U. Evolution of ventricular septal defect and pulmonary stenosis with left to right shunt into classic tetralogy of fallot. A case report with clinical, angiocardiographic and anatomic correlations. Am J Cardiol. 1961 Apr;7:598–607. doi: 10.1016/0002-9149(61)90519-7. [DOI] [PubMed] [Google Scholar]
  2. FABRICIUS J., HANSEN P. F., LINDENEG O. Pulmonary atresia developing after a shunt operation for Fallot's tetralogy. Br Heart J. 1961 Sep;23:556–560. doi: 10.1136/hrt.23.5.556. [DOI] [PMC free article] [PubMed] [Google Scholar]
  3. FYLER D. C., RUDOLPH A. M., WITTENBORG M. H., NADAS A. S. Ventricular septal defect in infants and children; a correlation of clinical, physiologic, and autopsy data. Circulation. 1958 Nov;18(5):833–851. doi: 10.1161/01.cir.18.5.833. [DOI] [PubMed] [Google Scholar]
  4. Frater R. W., Rudolph A. M., Hoffman J. I. Acquired pulmonary atresia in tetralogy of Fallot with a functioning Blalock-Taussig shunt. Thorax. 1966 Sep;21(5):457–458. doi: 10.1136/thx.21.5.457. [DOI] [PMC free article] [PubMed] [Google Scholar]
  5. Hutchins G. M. Coarctation of the aorta explained as a branch-point of the ductus arteriosus. Am J Pathol. 1971 May;63(2):203–214. [PMC free article] [PubMed] [Google Scholar]
  6. Hutchins G. M., Maron B. J. Development of endocardial valvuloids with valvular insufficiency. Arch Pathol. 1972 May;93(5):401–407. [PubMed] [Google Scholar]
  7. LEV M., ECKNER F. A. THE PATHOLOGIC ANATOMY OF TETRALOGY OF FALLOT AND ITS VARIATIONS. Dis Chest. 1964 Mar;45:251–261. doi: 10.1378/chest.45.3.251. [DOI] [PubMed] [Google Scholar]
  8. LEV M. The pathologic anatomy of ventricular septal defects. Dis Chest. 1959 May;35(5):533–545. doi: 10.1378/chest.35.5.533. [DOI] [PubMed] [Google Scholar]
  9. Levin A. R., Boineau J. P., Spach M. S., Canent R. V., Jr, Capp M. P., Anderson P. A. Ventricular pressure-flow dynamics in tetralogy of Fallot. Circulation. 1966 Jul;34(1):4–13. doi: 10.1161/01.cir.34.1.4. [DOI] [PubMed] [Google Scholar]
  10. Maron B. J., Hutchins G. M. Truncus arteriosus malformation in a human embryo. Am J Anat. 1972 Jun;134(2):167–173. doi: 10.1002/aja.1001340204. [DOI] [PubMed] [Google Scholar]
  11. Mizuno A., Sato F., Hasegawa T., Tsuzuki M., Furuse A. Acquired obstruction of the right ventricular outflow tract in tetralogy of Fallot after Blalock-Taussig anastomosis. Report of two cases successfully treated with total correction. Jpn Heart J. 1970 Jan;11(1):113–119. doi: 10.1536/ihj.11.113. [DOI] [PubMed] [Google Scholar]
  12. ROCKOFF S. D., GILBERT J. FUNCTIONAL PULMONARY ATRESIA: A CAUSE OF ANGIOCARDIOGRAPHIC MISINTERPRETATION IN TETRALOGY OF FALLOT. Am J Roentgenol Radium Ther Nucl Med. 1965 May;94:85–91. [PubMed] [Google Scholar]
  13. Rao B. N., Anderson R. C., Edwards J. E. Anatomic variations in the tetralogy of Fallot. Am Heart J. 1971 Mar;81(3):361–371. doi: 10.1016/0002-8703(71)90106-2. [DOI] [PubMed] [Google Scholar]
  14. SABISTON D. C., Jr, CORNELL W. P., CRILEY J. M., NEILL C. A., ROSS R. S., BAHNSON H. T. THE DIAGNOSIS AND SURGICAL CORRECTION OF TOTAL OBSTRUCTION OF THE RIGHT VENTRICLE. AN ACQUIRED CONDITION DEVELOPING AFTER SYSTEMIC ARTERY--PULMONARY ARTERY ASASTOMOSIS FOR TETRALOGY OF FALLOT. J Thorac Cardiovasc Surg. 1964 Oct;48:577–587. [PubMed] [Google Scholar]
  15. Van Praagh R., Van Praagh S., Nebesar R. A., Muster A. J., Sinha S. N., Paul M. H. Tetralogy of Fallot: underdevelopment of the pulmonary infundibulum and its sequelae. Am J Cardiol. 1970 Jul;26(1):25–33. doi: 10.1016/0002-9149(70)90754-x. [DOI] [PubMed] [Google Scholar]
  16. WATSON H., LOWE K. G. FUNCTIONAL ADAPTATIONS OF THE RIGHT VENTRICULAR OUTFLOW TRACT IN CONGENITAL HEART DISEASE. Br Heart J. 1965 May;27:408–412. doi: 10.1136/hrt.27.3.408. [DOI] [PMC free article] [PubMed] [Google Scholar]
  17. Zaret B. L., Conti C. R. Infundibular pulmonic stenosis with intact ventricular septum in the adult. Johns Hopkins Med J. 1973 Jan;132(1):50–60. [PubMed] [Google Scholar]

Articles from The American Journal of Pathology are provided here courtesy of American Society for Investigative Pathology

RESOURCES