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The Ventricular Septal Defect: Developmental Significance in Conotruncal and Aortic Arch Anomalies

The Ventricular Septal Defect: Developmental Significance in Conotruncal and Aortic Arch Anomalies Abstract A study of ventricular septal defects (VSD) is made in relation to anomalies of the conotruncus and aortic arch. One hundred-nineteen autopsy cases were examined. These included 66 of VSD and 53 of aortic arch anomalies. Within the group of aortic arch anomalies, 14 cases of interrupted aortic arch are tabulated. Five cases bearing on the question of transformation of VSD to tetralogy of Fallot are presented. A further case is presented, demonstrating the heretofore undescribed combination of tetralogy of Fallot with interrupted aortic arch. The VSD is discussed in terms of the developmental concept of a ventricular flow pathway. It is emphasized that the defect is not the primary cause of the conotruncal or aortic arch malformation. The study suggests that the natural history of the VSD may include transformation to the tetralogy of Fallot via Eisenmenger's syndrome. References 1. Grant RP: The embryology of ventricular flow pathways in man . Circulation 25:756-779, 1962.Crossref 2. Gasul BM, Dillon RF, Vrla V, et al: Ventricular septal defects: Their natural transformation into those with infundibular stenosis or into the cyanotic or non-cyanotic type of tetralogy of Fallot . JAMA 164:847-853, 1957.Crossref 3. Van Praagh R, Van Praagh, S: The anatomy of common aorticopulmonary trunk (truncus arteriosus communis) and its embryologic implications: A study of 57 necropsy cases . Amer J Cardiol 16:406-425, 1965.Crossref 4. McCord MC, Van Elk J, Blount SG: Tetralogy of Fallot: Clinical and hemodynamic spectrum of combined pulmonary stenosis and ventricular septal defect . Circulation 16:736-749, 1957.Crossref 5. Wood P: The Eisenmenger syndrome . Brit Med J 2:755-762, 1958.Crossref 6. Lev M: Pathologic anatomy and interrelationship of hypoplasia of the aortic tract complexes . Lab Invest 1:61-70, 1952. 7. Noonan JA, Nadas AS: The hypoplastic left heart syndrome . Pediat Clin N Amer 5:1029-1056, 1958. 8. Heath D, Edwards JE: The pathology of hypertensive pulmonary vascular disease: A description of six grades of structural changes in the pulmonary arteries with special reference to congenital cardiac septal defects . Circulation 18:533-547, 1958.Crossref 9. Rich AR: A hitherto unrecognized tendency to the development of widespread pulmonary vascular obstruction in patients with congenital pulmonary stenosis (tetralogy of Fallot) . Bull Hopkins Hosp 82:389-403, 1948. 10. Kaplan S, Daoud GI, Benzing G III, et al: Natural history of ventricular septal defect . Amer J Dis Child 105:581-587, 1963. 11. Lucas RV Jr, Adams P Jr, Anderson RC, et al: The natural history of isolated ventricular septal defect: A serial physiologic study . Circulation 24:1372-1387, 1961.Crossref 12. Nadas AS, Fyler DC: Ventricular septal defect: A review of current thoughts . Arch Dis Child 43:268-282, 1968.Crossref 13. Selzer A, Laquer GL: The Eisenmenger complex and its relation to the uncomplicated defect of the ventricular septum . Arch Intern Med 87:218-241, 1951.Crossref 14. Cartmill TB, DuShane JW, McGoon DC, et al: Results of repair of Ventricular septal defect . J Thorac Cardiovasc Surg 52: 486-501, 1966. 15. Naeye RL: The pulmonary arterial bed in ventricular septal defect: Anatomic features in childhood . Circulation 34:962-970, 1966.Crossref 16. Celoria GC, Patton RB: Congenital absence of the aortic arch . Amer Heart J 58:407-413, 1959.Crossref 17. Kleinerman J, Wen-Min Yang, Hackel DB, et al: Absence of the transverse aortic arch . Arch Path 65:490-498, 1958. 18. Steiner P, Finegold MJ: Truncus arteriosus with atresia of the aortic arch . Arch Path 79:518-523, 1965. 19. Lie JT: The malformation complex of the absence of the arch of the aorta: Steidele's complex . Amer Heart J 73:615-625, 1967.Crossref 20. McNamara DG, Rosenberg HS: Coarctation of the aorta , in Watson H (ed): Pediatric Cardiology , ed 1. St. Louis, CV Mosby Co, 1968. 21. Schulz DM, Giordano DA: Hearts of infants and children . Arch Path 74:464-471, 1962. 22. Edwards JE: Pathology of the Heart and Blood Vessels , ed 3. SE Gould (ed), Springfield, Ill, Charles C Thomas Publisher, 1968. 23. Skoda, cited by Wielenga G, Dankmeyer J: Coarctation of the aorta . J Path Bact 95:265-274, 1968.Crossref 24. Wilson JG: Embryology of the heart and major vessels , in Luisada, AA (ed): Development and Structure of the Cardiovascular System . New York, McGraw-Hill Book Co Inc, 1961, chap 1. 25. Jaffee OC: Hemodynamic factors in the development of the chick embryo heart . Anat Rec 151:69-75, 1965.Crossref 26. Jaffee OC: Hemodynamic analysis of experimentally produced cardiac malformations . Anat Rec 154:509, 1965. 27. Jaffee OC: The development of the arterial outflow tract in the chick embryo heart . Anat Rec 158:35-42, 1967.Crossref 28. Jaffee OC: Rheological aspects of the development of blood flow patterns in the chick embryo heart . Biorheology 3:59-62, 1966. 29. Rychter Z: Experimental morphology of the aortic arches and the heart loop in chick embryos , in Abercrombie B, Brachet J (eds): Advances in Morphogenesis . New York, Academic Press Inc, 1962, vol 2, pp 333-371. 30. DeHaan RL: Development of form in the embryonic heart: An experimental approach . Circulation 35:821-833, 1967.Crossref 31. Van Praagh R, Vlad P, Keith JD: Complete transposition of the great arteries , Heart Disease in Infancy and Childhood , ed 2. New York, Macmillan Co, 1967, pp 682-744. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png American Journal of Diseases of Children American Medical Association

The Ventricular Septal Defect: Developmental Significance in Conotruncal and Aortic Arch Anomalies

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Publisher
American Medical Association
Copyright
Copyright © 1970 American Medical Association. All Rights Reserved.
ISSN
0002-922X
DOI
10.1001/archpedi.1970.02100050409005
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Abstract

Abstract A study of ventricular septal defects (VSD) is made in relation to anomalies of the conotruncus and aortic arch. One hundred-nineteen autopsy cases were examined. These included 66 of VSD and 53 of aortic arch anomalies. Within the group of aortic arch anomalies, 14 cases of interrupted aortic arch are tabulated. Five cases bearing on the question of transformation of VSD to tetralogy of Fallot are presented. A further case is presented, demonstrating the heretofore undescribed combination of tetralogy of Fallot with interrupted aortic arch. The VSD is discussed in terms of the developmental concept of a ventricular flow pathway. It is emphasized that the defect is not the primary cause of the conotruncal or aortic arch malformation. The study suggests that the natural history of the VSD may include transformation to the tetralogy of Fallot via Eisenmenger's syndrome. References 1. Grant RP: The embryology of ventricular flow pathways in man . Circulation 25:756-779, 1962.Crossref 2. Gasul BM, Dillon RF, Vrla V, et al: Ventricular septal defects: Their natural transformation into those with infundibular stenosis or into the cyanotic or non-cyanotic type of tetralogy of Fallot . JAMA 164:847-853, 1957.Crossref 3. Van Praagh R, Van Praagh, S: The anatomy of common aorticopulmonary trunk (truncus arteriosus communis) and its embryologic implications: A study of 57 necropsy cases . Amer J Cardiol 16:406-425, 1965.Crossref 4. McCord MC, Van Elk J, Blount SG: Tetralogy of Fallot: Clinical and hemodynamic spectrum of combined pulmonary stenosis and ventricular septal defect . Circulation 16:736-749, 1957.Crossref 5. Wood P: The Eisenmenger syndrome . Brit Med J 2:755-762, 1958.Crossref 6. Lev M: Pathologic anatomy and interrelationship of hypoplasia of the aortic tract complexes . Lab Invest 1:61-70, 1952. 7. Noonan JA, Nadas AS: The hypoplastic left heart syndrome . Pediat Clin N Amer 5:1029-1056, 1958. 8. Heath D, Edwards JE: The pathology of hypertensive pulmonary vascular disease: A description of six grades of structural changes in the pulmonary arteries with special reference to congenital cardiac septal defects . Circulation 18:533-547, 1958.Crossref 9. Rich AR: A hitherto unrecognized tendency to the development of widespread pulmonary vascular obstruction in patients with congenital pulmonary stenosis (tetralogy of Fallot) . Bull Hopkins Hosp 82:389-403, 1948. 10. Kaplan S, Daoud GI, Benzing G III, et al: Natural history of ventricular septal defect . Amer J Dis Child 105:581-587, 1963. 11. Lucas RV Jr, Adams P Jr, Anderson RC, et al: The natural history of isolated ventricular septal defect: A serial physiologic study . Circulation 24:1372-1387, 1961.Crossref 12. Nadas AS, Fyler DC: Ventricular septal defect: A review of current thoughts . Arch Dis Child 43:268-282, 1968.Crossref 13. Selzer A, Laquer GL: The Eisenmenger complex and its relation to the uncomplicated defect of the ventricular septum . Arch Intern Med 87:218-241, 1951.Crossref 14. Cartmill TB, DuShane JW, McGoon DC, et al: Results of repair of Ventricular septal defect . J Thorac Cardiovasc Surg 52: 486-501, 1966. 15. Naeye RL: The pulmonary arterial bed in ventricular septal defect: Anatomic features in childhood . Circulation 34:962-970, 1966.Crossref 16. Celoria GC, Patton RB: Congenital absence of the aortic arch . Amer Heart J 58:407-413, 1959.Crossref 17. Kleinerman J, Wen-Min Yang, Hackel DB, et al: Absence of the transverse aortic arch . Arch Path 65:490-498, 1958. 18. Steiner P, Finegold MJ: Truncus arteriosus with atresia of the aortic arch . Arch Path 79:518-523, 1965. 19. Lie JT: The malformation complex of the absence of the arch of the aorta: Steidele's complex . Amer Heart J 73:615-625, 1967.Crossref 20. McNamara DG, Rosenberg HS: Coarctation of the aorta , in Watson H (ed): Pediatric Cardiology , ed 1. St. Louis, CV Mosby Co, 1968. 21. Schulz DM, Giordano DA: Hearts of infants and children . Arch Path 74:464-471, 1962. 22. Edwards JE: Pathology of the Heart and Blood Vessels , ed 3. SE Gould (ed), Springfield, Ill, Charles C Thomas Publisher, 1968. 23. Skoda, cited by Wielenga G, Dankmeyer J: Coarctation of the aorta . J Path Bact 95:265-274, 1968.Crossref 24. Wilson JG: Embryology of the heart and major vessels , in Luisada, AA (ed): Development and Structure of the Cardiovascular System . New York, McGraw-Hill Book Co Inc, 1961, chap 1. 25. Jaffee OC: Hemodynamic factors in the development of the chick embryo heart . Anat Rec 151:69-75, 1965.Crossref 26. Jaffee OC: Hemodynamic analysis of experimentally produced cardiac malformations . Anat Rec 154:509, 1965. 27. Jaffee OC: The development of the arterial outflow tract in the chick embryo heart . Anat Rec 158:35-42, 1967.Crossref 28. Jaffee OC: Rheological aspects of the development of blood flow patterns in the chick embryo heart . Biorheology 3:59-62, 1966. 29. Rychter Z: Experimental morphology of the aortic arches and the heart loop in chick embryos , in Abercrombie B, Brachet J (eds): Advances in Morphogenesis . New York, Academic Press Inc, 1962, vol 2, pp 333-371. 30. DeHaan RL: Development of form in the embryonic heart: An experimental approach . Circulation 35:821-833, 1967.Crossref 31. Van Praagh R, Vlad P, Keith JD: Complete transposition of the great arteries , Heart Disease in Infancy and Childhood , ed 2. New York, Macmillan Co, 1967, pp 682-744.

Journal

American Journal of Diseases of ChildrenAmerican Medical Association

Published: May 1, 1970

References