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Isolated Free-Wall Hypoplasia of the Right Ventricle

Isolated Free-Wall Hypoplasia of the Right Ventricle Abstract IN 1952, Henry S. M. Uhl published observations of a case of focal fibrosis or muscular hypoplasia of the right ventricle in a 7-month-old child.30 Possibly related to this, if only grossly, are: Osler's case of "parchment heart,"27 reports of coronary insufficiency with or without healed myocardial infarct,7 congenital ventricular aneurysms, healed or healing myocarditis,8 and atypical extreme degrees of congenital endocardial fibrosis. Since Uhl's report, only three similar observations have been published.2,20,23,28 In these case reports, we offer morphologically proved cases of partial fibrous replacement of the right ventricular myocardium. In one instance, two brothers of the patient had died in infancy with morphological abnormalities of the heart. These are the fourth and fifth cases of "Uhl's anomaly" with autopsy and the second with a "positive" family history. The second case is an elaboration of a previously recorded but briefly noted example.28 Two references2,20 record the same case References 1. Anderson, R. C.: Causative Factors Underlying Congenital Heart Malformation , Pediatrics 14:143, 1954. 2. Arcilla, R. A., and Gasul, B. M.: Congenital Aplasia or Marked Hypoplasia of Myocardium of Right Ventricle (Uhl's Anomaly) , J Pediat 58:381, 1961.Crossref 3. Black-Schaffer, B., and Turner, M. E.: Hyperplastic Infantile Cardiomegaly , Amer J Path 34:745, 1958. 4. Brent, L. B., et al: Familial Muscular Subaortic Stenosis , Circulation 21:167, 1960.Crossref 5. Chelius, C. J.; Rowe, G. G., and Crumpton, C. W.: Familial Aspects of Congenital Heart Disease , Amer J Cardiol 9:508, 1962.Crossref 6. Connor, A. C., et al: Familial Congenital Heart Block , Amer J Obstet Gynec 78:75, 1959. 7. Dalla-Volta, S.; Battglia, G.; and Zerbini, E.: "Auricularization" of Right Ventricular Pressure Curve , Amer Heart J 61:25, 1961.Crossref 8. Froboese, C.: Fibrosis myocardii congenita (angeborenes Schwielenherz) oder Säuglings: Myokarditis , Arch Path Anat 284:761, 1932. 9. Garrett, G.; Hay, W. J.; and Rickards, A. G.: Familial Cardiomegaly , J Clin Path 12:355, 1959.Crossref 10. Gross, P.: Concept of Fetal Endocarditis , Arch Path 31:163, 1941. 11. Hollman, A., et al: Family With Obstructive Cardiomyopathy (Asymmetrical Hypertrophy) , Brit Heart J 22:449, 1960.Crossref 12. Holt, M., and Oram, S.: Familial Heart Disease With Skeletal Malformations , Brit Heart J 22:236, 1960.Crossref 13. Kelly, J., and Andersen, D. H.: Congenital Endocardial Fibroelastosis: II. Clinical and Pathologic Investigation of Those Cases Without Associated Cardiac Malformations Including Report of Two Familial Instances , Pediatrics 18:539, 1956. 14. Keith, J. D.; Rowe, R. D.; and Vlad, P.: Heart Disease in Infancy and Childhood , New York: The Macmillan Company Publishers, 1958, chap 9 , 129; chap 18, pp 314-315. 15. Landing, B. H., et al: Some Genetic and Biochemical Implications of Congenital Heart Disease , Quart Rev Pediat 16:160, 1961. 16. Cardiomyopathy , Lancet 1:1214 ( (June 3) ) 1961. 17. Lev, M., and Simkins, C. S.: Architecture of Human Ventricular Myocardium , Lab Invest 5:396, 1956. 18. Mayer, F. E.; Nadas, A. S.; and Ongley, P. A.: Ebstein's Anomaly , Circulation 16:1057, 1957.Crossref 19. Medd, W. E., et al: Isolated Hypoplasia of Right Ventricle and Tricuspid Valve in Siblings , Brit Heart J 23:25, 1961.Crossref 20. Novak, G., et al: Congenital Aplasia of the Myocardium of the Right Ventricle, Chicago Pathological Society , Proc Inst Med Chicago 21:334, 1957. 21. Pare, J. A. P., et al: Hereditary Cardiovascular Dysplasia , Amer J Med 31:37, 1961.Crossref 22. Paulley, J. W., et al: Myocardial Toxoplasmosis , Lancet 2:624, 1954.Crossref 23. Reeve, R., and MacDonald, D.: Partial Absence of Right Ventricular Musculature: Partial Parchment Heart , Amer J Cardiol 14:415, 1964.Crossref 24. Sackner, M. A., et al: Isolated Right Ventricular Hypoplasia With Atrial Septal Defect or Patent Foramen Ovale , Circulation 24:1388, 1961.Crossref 25. Schiebler, G. L., et al: Clinical Study of Ebstein's Anomaly of Tricuspid Valve , Circulation 19: 165, 1959.Crossref 26. Schiebler, G. L.; Adams, P.; and Anderson, R. C.: Familial Cardiomegaly in Association With Wolff-Parkinson-White Syndrome , Amer Heart J 58: 113, 1959.Crossref 27. Segall, H. N.: Parchment Heart (Osler) , Amer Heart J 40:948, 1950.Crossref 28. Taussig, H.: Congenital Malformation of Heart: II. Specific Malformations , Cambridge, Mass: Commonwealth Fund, Harvard University Press, 1960, pp 138-145. 29. Whitfield, A. G. W.: Familial Cardiomyopathy , Quart J Med 30:119, 1961. 30. Uhl, H. S. M.: Previously Undescribed Congenital Malformation of Heart: Almost Total Absence of Myocardium of Right Ventricle , Bull Hopkins Hosp 91:197, 1952. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png American Journal of Diseases of Children American Medical Association

Isolated Free-Wall Hypoplasia of the Right Ventricle

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Publisher
American Medical Association
Copyright
Copyright © 1965 American Medical Association. All Rights Reserved.
ISSN
0002-922X
DOI
10.1001/archpedi.1965.02090020560011
Publisher site
See Article on Publisher Site

Abstract

Abstract IN 1952, Henry S. M. Uhl published observations of a case of focal fibrosis or muscular hypoplasia of the right ventricle in a 7-month-old child.30 Possibly related to this, if only grossly, are: Osler's case of "parchment heart,"27 reports of coronary insufficiency with or without healed myocardial infarct,7 congenital ventricular aneurysms, healed or healing myocarditis,8 and atypical extreme degrees of congenital endocardial fibrosis. Since Uhl's report, only three similar observations have been published.2,20,23,28 In these case reports, we offer morphologically proved cases of partial fibrous replacement of the right ventricular myocardium. In one instance, two brothers of the patient had died in infancy with morphological abnormalities of the heart. These are the fourth and fifth cases of "Uhl's anomaly" with autopsy and the second with a "positive" family history. The second case is an elaboration of a previously recorded but briefly noted example.28 Two references2,20 record the same case References 1. Anderson, R. C.: Causative Factors Underlying Congenital Heart Malformation , Pediatrics 14:143, 1954. 2. Arcilla, R. A., and Gasul, B. M.: Congenital Aplasia or Marked Hypoplasia of Myocardium of Right Ventricle (Uhl's Anomaly) , J Pediat 58:381, 1961.Crossref 3. Black-Schaffer, B., and Turner, M. E.: Hyperplastic Infantile Cardiomegaly , Amer J Path 34:745, 1958. 4. Brent, L. B., et al: Familial Muscular Subaortic Stenosis , Circulation 21:167, 1960.Crossref 5. Chelius, C. J.; Rowe, G. G., and Crumpton, C. W.: Familial Aspects of Congenital Heart Disease , Amer J Cardiol 9:508, 1962.Crossref 6. Connor, A. C., et al: Familial Congenital Heart Block , Amer J Obstet Gynec 78:75, 1959. 7. Dalla-Volta, S.; Battglia, G.; and Zerbini, E.: "Auricularization" of Right Ventricular Pressure Curve , Amer Heart J 61:25, 1961.Crossref 8. Froboese, C.: Fibrosis myocardii congenita (angeborenes Schwielenherz) oder Säuglings: Myokarditis , Arch Path Anat 284:761, 1932. 9. Garrett, G.; Hay, W. J.; and Rickards, A. G.: Familial Cardiomegaly , J Clin Path 12:355, 1959.Crossref 10. Gross, P.: Concept of Fetal Endocarditis , Arch Path 31:163, 1941. 11. Hollman, A., et al: Family With Obstructive Cardiomyopathy (Asymmetrical Hypertrophy) , Brit Heart J 22:449, 1960.Crossref 12. Holt, M., and Oram, S.: Familial Heart Disease With Skeletal Malformations , Brit Heart J 22:236, 1960.Crossref 13. Kelly, J., and Andersen, D. H.: Congenital Endocardial Fibroelastosis: II. Clinical and Pathologic Investigation of Those Cases Without Associated Cardiac Malformations Including Report of Two Familial Instances , Pediatrics 18:539, 1956. 14. Keith, J. D.; Rowe, R. D.; and Vlad, P.: Heart Disease in Infancy and Childhood , New York: The Macmillan Company Publishers, 1958, chap 9 , 129; chap 18, pp 314-315. 15. Landing, B. H., et al: Some Genetic and Biochemical Implications of Congenital Heart Disease , Quart Rev Pediat 16:160, 1961. 16. Cardiomyopathy , Lancet 1:1214 ( (June 3) ) 1961. 17. Lev, M., and Simkins, C. S.: Architecture of Human Ventricular Myocardium , Lab Invest 5:396, 1956. 18. Mayer, F. E.; Nadas, A. S.; and Ongley, P. A.: Ebstein's Anomaly , Circulation 16:1057, 1957.Crossref 19. Medd, W. E., et al: Isolated Hypoplasia of Right Ventricle and Tricuspid Valve in Siblings , Brit Heart J 23:25, 1961.Crossref 20. Novak, G., et al: Congenital Aplasia of the Myocardium of the Right Ventricle, Chicago Pathological Society , Proc Inst Med Chicago 21:334, 1957. 21. Pare, J. A. P., et al: Hereditary Cardiovascular Dysplasia , Amer J Med 31:37, 1961.Crossref 22. Paulley, J. W., et al: Myocardial Toxoplasmosis , Lancet 2:624, 1954.Crossref 23. Reeve, R., and MacDonald, D.: Partial Absence of Right Ventricular Musculature: Partial Parchment Heart , Amer J Cardiol 14:415, 1964.Crossref 24. Sackner, M. A., et al: Isolated Right Ventricular Hypoplasia With Atrial Septal Defect or Patent Foramen Ovale , Circulation 24:1388, 1961.Crossref 25. Schiebler, G. L., et al: Clinical Study of Ebstein's Anomaly of Tricuspid Valve , Circulation 19: 165, 1959.Crossref 26. Schiebler, G. L.; Adams, P.; and Anderson, R. C.: Familial Cardiomegaly in Association With Wolff-Parkinson-White Syndrome , Amer Heart J 58: 113, 1959.Crossref 27. Segall, H. N.: Parchment Heart (Osler) , Amer Heart J 40:948, 1950.Crossref 28. Taussig, H.: Congenital Malformation of Heart: II. Specific Malformations , Cambridge, Mass: Commonwealth Fund, Harvard University Press, 1960, pp 138-145. 29. Whitfield, A. G. W.: Familial Cardiomyopathy , Quart J Med 30:119, 1961. 30. Uhl, H. S. M.: Previously Undescribed Congenital Malformation of Heart: Almost Total Absence of Myocardium of Right Ventricle , Bull Hopkins Hosp 91:197, 1952.

Journal

American Journal of Diseases of ChildrenAmerican Medical Association

Published: Jun 1, 1965

References