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VENTRICULAR SEPTAL DEFECTS: Title and subTitle BreakTHEIR NATURAL TRANSFORMATION INTO THOSE WITH INFUNDIBULAR STENOSIS OR INTO THE CYANOTIC OR NONCYANOTIC TYPE OF TETRALOGY OF FALLOT FREE

Benjamin M. Gasul, M.D.; Robert F. Dillon, M.D.; Vlastimil Vrla, M.D.; Gershon Hait, M.D.
[+] Author Affiliations

Read before the Society for Pediatric Research, Carmel, Calif., June 17,1957.


Chicago

From the Hektoen Institute for Medical Research, the Cardiophysiology Department of Cook County Children's Hospital, and the departments of pediatrics of the University of Illinois College of Medicine and Presbyterian Hospital.


JAMA. 1957;164(8):847-853. doi:10.1001/jama.1957.02980080017003
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• The natural course of patients with ventricular septal defects is not known. During the past 10 years, 27 autopsies were performed on the authors' 336 patients with this defect. Twenty-six of these patients were under 6 months of age. It has been observed repeatedly that some infants with large ventricular septal defects manifest marked improvement in their symptomatology after their first year of life. In an attempt to explain these observations, clinical follow-up examinations were combined with cardiac recatheterization and angiocardiography, especially on those patients who had their initial studies during their first year of life. So far, 10 patients have been restudied and recatheterized. The time interval between their first cardiac catheterization and the recent ones varied from three to five years. Two of these patients now present typical clinical and hemodynamic data of ventricular septal defect with infundibular stenosis with left-to-right shunts. The third patient shows typical findings of tetralogy of with marked cyanosis and predominant right-to-left shunt. The frequency with which this natural transformation of ventricular septal defects into apparently different entities occurs is at present not known. The concept of the unity of origin of the three entities has been demonstrated in these patients, and this concept should lead to a re-evaluation of our knowledge concerning the diagnosis and medical and surgical treatment of congenital cardiac defects.

REFERENCES

Gasul, B. M., and Fell, E. H.:  Salient Points in Clinical Diagnosis of Congenital Heart Disease , J. A. M. A. 161:39-44 ( (May 5) ) 1956;.
Selzer, A., and Laqueur, G. L.:  Eisenmenger Complex and Its Relation to Uncomplicated Defect of Ventricular Septum: Review of 35 Autopsied Cases of Eisenmenger's Complex, Including Two New Cases , A. M. A. Arch. Int. Med. 87:218-241 ( (Feb.) ) 1951;.
Eisenmenger, V.:  Ursprung der Aorta aus beiden Ventrikeln beim Defect des Septum Ventriculorum , Wien. klin. Wchnschr. 11:25, 1898;.
Cournand, A.; Baldwin, J. S.; and Himmelstein, A.: Cardiac Catheterization in Congenital Heart Disease: Clinical and Physiological Study in Infants and Children , New York, Commonwealth Fund, 1949;.
Abrahams, D. G., and Wood, P. H.:  Pulmonary Stenosis with Normal Aortic Root , Brit. Heart J. 13:519-548 ( (Oct.) ) 1951;.
Broadbent, J. C.; Wood, E. H.; and Burchell, H. B.:  Symposium on Cardiac Catheterization: Left to Right Intercardiac Shunts in Presence of Pulmonary Stenosis , Proc. Staff Meet. Mayo Clin. 28:101-106 ( (Feb. 25) ) 1953;.
Moffitt, G. R., Jr., and others:  Pulmonary Stenosis with Left to Right Intracardiac Shunts , Am. J. Med. 16:521-527 ( (April) ) 1954;.
Rudolph, A. M.; Nadas, A. S.; and Goodale, W. T.:  Intracardiac Left-to-Right Shunt with Pulmonic Stenosis , Am. Heart J. 48:808-816 ( (Dec.) ) 1954;.
Magidson, O., and others:  Pulmonary Stenosis with Left to Right Shunt , Am. J. Med. 17:311-321 ( (Sept.) ) 1954;.
Sell, G. G. R., and others:  Physiological Studies in Cases of "Tetralogy of Fallot" with Little or No Overriding of Aorta , read before the second World Congress of Cardiology, Washington, D. C., Sept. 16,1954 .
Castellanos y Gonzalez, A.: Cardiopatias congénitas de la infancia , Havana, M. V. Fresneda, 1948;.
Rowe, R. D.; Vlad, P.; and Keith, J. D.:  Atypical Tetralogy of Fallot: Noncyanotic Form with Increased Lung Vascularity: Report of Four Cases , Circulation 12:230-238 ( (Aug.) ) 1955;.
Becu, L. M., and others:  Anatomic and Pathologic Studies in Ventricular Septal Defect , Circulation 14:349-364 ( (Sept.) ) 1956;.
McCord, M. C., and Blount, S. G., Jr.:  Complications Following Infundibular Resection in Fallot's Tetralogy , Circulation 11:754-760 ( (May) ) 1955;.
Gasul, B. M.: Dillon, R. F.; Vrla, V.; and Hait, G.: Unpublished data.
Kjellberg, S. R.; Mannheimer, E.; Rudhe, U.; and Jonsson, B.: Diagnosis of Congenital Heart Disease: Clinical and Technical Study by Cardiologic Team of Pediatric Clinic, Karolinska Sjukhuset , Stockholm, Chicago, Year Book Publishers, Inc., 1955;.
Edwards, J. E.:  Structural Changes of Pulmonary Vascular Bed and Their Functional Significance in Congenital Heart Disease, 26th Ludvig Hektoen Lecture , Proc. Inst. Med. Chicago , 18:134-146 ( (June 15) ) 1950;.
Dammann, J. F., Jr., and Muller, W. H., Jr.:  Role of Pulmonary Vascular Bed in Congenital Heart Disease , Pediatrics 12:307-325 (Sept.,pt. (1) ) 1953;.

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Gasul, B. M., and Fell, E. H.:  Salient Points in Clinical Diagnosis of Congenital Heart Disease , J. A. M. A. 161:39-44 ( (May 5) ) 1956;.
Selzer, A., and Laqueur, G. L.:  Eisenmenger Complex and Its Relation to Uncomplicated Defect of Ventricular Septum: Review of 35 Autopsied Cases of Eisenmenger's Complex, Including Two New Cases , A. M. A. Arch. Int. Med. 87:218-241 ( (Feb.) ) 1951;.
Eisenmenger, V.:  Ursprung der Aorta aus beiden Ventrikeln beim Defect des Septum Ventriculorum , Wien. klin. Wchnschr. 11:25, 1898;.
Cournand, A.; Baldwin, J. S.; and Himmelstein, A.: Cardiac Catheterization in Congenital Heart Disease: Clinical and Physiological Study in Infants and Children , New York, Commonwealth Fund, 1949;.
Abrahams, D. G., and Wood, P. H.:  Pulmonary Stenosis with Normal Aortic Root , Brit. Heart J. 13:519-548 ( (Oct.) ) 1951;.
Broadbent, J. C.; Wood, E. H.; and Burchell, H. B.:  Symposium on Cardiac Catheterization: Left to Right Intercardiac Shunts in Presence of Pulmonary Stenosis , Proc. Staff Meet. Mayo Clin. 28:101-106 ( (Feb. 25) ) 1953;.
Moffitt, G. R., Jr., and others:  Pulmonary Stenosis with Left to Right Intracardiac Shunts , Am. J. Med. 16:521-527 ( (April) ) 1954;.
Rudolph, A. M.; Nadas, A. S.; and Goodale, W. T.:  Intracardiac Left-to-Right Shunt with Pulmonic Stenosis , Am. Heart J. 48:808-816 ( (Dec.) ) 1954;.
Magidson, O., and others:  Pulmonary Stenosis with Left to Right Shunt , Am. J. Med. 17:311-321 ( (Sept.) ) 1954;.
Sell, G. G. R., and others:  Physiological Studies in Cases of "Tetralogy of Fallot" with Little or No Overriding of Aorta , read before the second World Congress of Cardiology, Washington, D. C., Sept. 16,1954 .
Castellanos y Gonzalez, A.: Cardiopatias congénitas de la infancia , Havana, M. V. Fresneda, 1948;.
Rowe, R. D.; Vlad, P.; and Keith, J. D.:  Atypical Tetralogy of Fallot: Noncyanotic Form with Increased Lung Vascularity: Report of Four Cases , Circulation 12:230-238 ( (Aug.) ) 1955;.
Becu, L. M., and others:  Anatomic and Pathologic Studies in Ventricular Septal Defect , Circulation 14:349-364 ( (Sept.) ) 1956;.
McCord, M. C., and Blount, S. G., Jr.:  Complications Following Infundibular Resection in Fallot's Tetralogy , Circulation 11:754-760 ( (May) ) 1955;.
Gasul, B. M.: Dillon, R. F.; Vrla, V.; and Hait, G.: Unpublished data.
Kjellberg, S. R.; Mannheimer, E.; Rudhe, U.; and Jonsson, B.: Diagnosis of Congenital Heart Disease: Clinical and Technical Study by Cardiologic Team of Pediatric Clinic, Karolinska Sjukhuset , Stockholm, Chicago, Year Book Publishers, Inc., 1955;.
Edwards, J. E.:  Structural Changes of Pulmonary Vascular Bed and Their Functional Significance in Congenital Heart Disease, 26th Ludvig Hektoen Lecture , Proc. Inst. Med. Chicago , 18:134-146 ( (June 15) ) 1950;.
Dammann, J. F., Jr., and Muller, W. H., Jr.:  Role of Pulmonary Vascular Bed in Congenital Heart Disease , Pediatrics 12:307-325 (Sept.,pt. (1) ) 1953;.
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