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November 1965, Vol 110, No. 5 >
Article | November 1965

Aorta Surgery in a Child With Marfan's Syndrome FREE

ROBERT E. DURNIN, MD; GEORGE LINDESMITH, MD; BERTRAND MEYER, MD; DONALD C. FYLER, MD
[+] Author Affiliations

Received for publication March 22, 1965.

Reprint requests to 4614 Sunset Blvd, Los Angeles, Calif 90027 (Dr. Durnin).


Am J Dis Child. 1965;110(5):547-550. doi:10.1001/archpedi.1965.02090030571010.
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References
Comments

THE PURPOSE of this report is to present the clinical course of an 8-year-old girl with Marfan's syndrome who developed massive hemopericardium secondary to an aortic aneurysm, and who ultimately came to successful replacement of the ascending aorta with a prosthetic graft. Review of the literature indicates this is the youngest case in which surgical intervention has been successfully attempted.

Report of a Case  An 8-year-old Negro female was admitted on Oct 3, 1963, with shortness of breath for one week and orthopnea of three days duration. Past history revealed she had been followed for heart disease and a convulsive disorder in another center. She had undergone cardiac catheterization at age 4 years and no shunts or pressure abnormalities were demonstrated. Angiocardiography had shown aneurysmal enlargement of the ascending aorta. The girl's father was also examined and found to have some findings of Marfan's syndrome with an associated aortic insufficiency

REFERENCES

1 +
Leeming, J.T., and McKusick, V.A.:  Serum Seromucoid Levels in the Marfan's Syndrome , Bull Hopkins Hosp 110:38-48, 1962;.
2 +
Bacchus, H.:  Serum Seromucoid and Acid Mucopolysaccharide in the Marfan's Syndrome , J Lab Clin Med 55:221-228, 1960;.
3 +
Bacchus, H.:  Serum Seromucoid and Acid Mucopolysaccharide in the Marfan's Syndrome , Amer J Med 25:744, 1958;.
4 +
Wagenvoort, C.A.; Neufield, H.N.; and Edwards, J.E.:  Cardiovascular System in Marfan's Syndrome and in Idiopathic Dilatation of the Ascending Aorta , Amer J Cardiol 9:496-507, 1962;.
5 +
McKusick, V.A.: Heritable Disorders of Connective Tissue , ed 2, St. Louis: C. V. Mosby Co., 1960;.
6 +
McKusick, V.A.:  The Cardiovascular Aspects of Marfan's Syndrome: A Heritable Disorder of Connective Tissue , Circulation 11:321-342, 1955;.
7 +
Piper, R.K., and Irvine-Jones, E.:  Arachnodactyly and Its Association With Congenital Heart Disease , Amer J Dis Child 31:832, 1926;.
8 +
Papaioannou, A.C.; Agustsson, M.H.; and Gasul, B.M.:  Early Manifestations of Cardiovascular Disorders in Marfan's Syndrome , Pediatrics 27:255, 1961;.
9 +
Bronson, E., and Sutherland, G.A.:  Ruptured Aortic Aneurysms in Childhood With the Report of a Case , Brit J Child Dis 15:241, 1918;.
10 +
Traisman, H.S., and Johnson, F.R.:  Arachnodactyly Associated With Aneurysm of the Aorta , Amer J Dis Child 87:156, 1954;.
11 +
Wong, F.L.; Friedman, S.; and Yakovac, W.:  Cardiac Complications of Marfan's Syndrome in a Child , Amer J Dis Child 107:404, 1964;.
12 +
Bolande, R.P., and Tucker, A.S.:  Pulmonary Emphysema and Other Cardio-Respiratory Lesions as Part of the Marfan Abiotrophy , Pediatrics 33:356-366, 1964;.
13 +
Baer, R.W.; Taussig, H.S.; and Oppenheimer, E.H.:  Congenital Aneurysmal Dilatation of the Aorta Associated With Arachnodactyly , Bull Hopkins Hosp 72:309, 1943;.
14 +
Killip, T., and Holmquist, N.D.:  Aortic Surgery in Marfan's Syndrome , Ann Intern Med 54:431, 1961;.
15 +
Conklin, W.S.; Grismer, J.T.; and Aalpol, J.A.:  Hufnagel Valve Surgery for Aortic Insufficiency , J Thorac Surg 36:238, 1958;.
16 +
Dimond, E.G., et al:  Post Traumatic Aortic Insufficiency Occurring in Marfan's Syndrome With Attempted Repair With a Plastic Valve , New Eng J Med 256:8, 1957;.
17 +
Castleman, B.:  Case Records of the Massachusetts General Hospital , New Eng J Med 256:31, 1957;.
18 +
Muller, W.; Dammann, J.F.; and Warren, W.D.:  Surgical Correction of Cardiovascular Deformities in Marfan's Syndrome , Ann Surg 152:506, 1960;.
19 +
Dillard, D.H., et al:  Correction of Aneurysm of the Ascending Aorta and of Aortic Insufficiency in Marfan's Syndrome , Amer J Surg 104:337, 1962;.
20 +
Blanchard, R.J.W., et al:  Cardiovascular Surgery in Marfan's Syndrome , Ann Surg 158:990, 1963;.
21 +
Spencer, F.C., and Blake, H.:  A Report of the Successful Surgical Treatment of Aortic Regurgitation From a Dissecting Aneurysm in a Patient With Marfan's Syndrome , J Thorac Cardiov Surg 44:238, 1962;.
22 +
DeBakey, M.E., et al:  Surgical Treatment of Dissecting Aneurysms of the Aorta, Analysis of 72 Cases , Circulation 24:290, 1961;.
23 +
Tuna, N., and Thal, A.:  Some Unusual Features of Marfan's Syndrome , Circulation 24:1154, 1961;.

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References

1 +
Leeming, J.T., and McKusick, V.A.:  Serum Seromucoid Levels in the Marfan's Syndrome , Bull Hopkins Hosp 110:38-48, 1962;.
2 +
Bacchus, H.:  Serum Seromucoid and Acid Mucopolysaccharide in the Marfan's Syndrome , J Lab Clin Med 55:221-228, 1960;.
3 +
Bacchus, H.:  Serum Seromucoid and Acid Mucopolysaccharide in the Marfan's Syndrome , Amer J Med 25:744, 1958;.
4 +
Wagenvoort, C.A.; Neufield, H.N.; and Edwards, J.E.:  Cardiovascular System in Marfan's Syndrome and in Idiopathic Dilatation of the Ascending Aorta , Amer J Cardiol 9:496-507, 1962;.
5 +
McKusick, V.A.: Heritable Disorders of Connective Tissue , ed 2, St. Louis: C. V. Mosby Co., 1960;.
6 +
McKusick, V.A.:  The Cardiovascular Aspects of Marfan's Syndrome: A Heritable Disorder of Connective Tissue , Circulation 11:321-342, 1955;.
7 +
Piper, R.K., and Irvine-Jones, E.:  Arachnodactyly and Its Association With Congenital Heart Disease , Amer J Dis Child 31:832, 1926;.
8 +
Papaioannou, A.C.; Agustsson, M.H.; and Gasul, B.M.:  Early Manifestations of Cardiovascular Disorders in Marfan's Syndrome , Pediatrics 27:255, 1961;.
9 +
Bronson, E., and Sutherland, G.A.:  Ruptured Aortic Aneurysms in Childhood With the Report of a Case , Brit J Child Dis 15:241, 1918;.
10 +
Traisman, H.S., and Johnson, F.R.:  Arachnodactyly Associated With Aneurysm of the Aorta , Amer J Dis Child 87:156, 1954;.
11 +
Wong, F.L.; Friedman, S.; and Yakovac, W.:  Cardiac Complications of Marfan's Syndrome in a Child , Amer J Dis Child 107:404, 1964;.
12 +
Bolande, R.P., and Tucker, A.S.:  Pulmonary Emphysema and Other Cardio-Respiratory Lesions as Part of the Marfan Abiotrophy , Pediatrics 33:356-366, 1964;.
13 +
Baer, R.W.; Taussig, H.S.; and Oppenheimer, E.H.:  Congenital Aneurysmal Dilatation of the Aorta Associated With Arachnodactyly , Bull Hopkins Hosp 72:309, 1943;.
14 +
Killip, T., and Holmquist, N.D.:  Aortic Surgery in Marfan's Syndrome , Ann Intern Med 54:431, 1961;.
15 +
Conklin, W.S.; Grismer, J.T.; and Aalpol, J.A.:  Hufnagel Valve Surgery for Aortic Insufficiency , J Thorac Surg 36:238, 1958;.
16 +
Dimond, E.G., et al:  Post Traumatic Aortic Insufficiency Occurring in Marfan's Syndrome With Attempted Repair With a Plastic Valve , New Eng J Med 256:8, 1957;.
17 +
Castleman, B.:  Case Records of the Massachusetts General Hospital , New Eng J Med 256:31, 1957;.
18 +
Muller, W.; Dammann, J.F.; and Warren, W.D.:  Surgical Correction of Cardiovascular Deformities in Marfan's Syndrome , Ann Surg 152:506, 1960;.
19 +
Dillard, D.H., et al:  Correction of Aneurysm of the Ascending Aorta and of Aortic Insufficiency in Marfan's Syndrome , Amer J Surg 104:337, 1962;.
20 +
Blanchard, R.J.W., et al:  Cardiovascular Surgery in Marfan's Syndrome , Ann Surg 158:990, 1963;.
21 +
Spencer, F.C., and Blake, H.:  A Report of the Successful Surgical Treatment of Aortic Regurgitation From a Dissecting Aneurysm in a Patient With Marfan's Syndrome , J Thorac Cardiov Surg 44:238, 1962;.
22 +
DeBakey, M.E., et al:  Surgical Treatment of Dissecting Aneurysms of the Aorta, Analysis of 72 Cases , Circulation 24:290, 1961;.
23 +
Tuna, N., and Thal, A.:  Some Unusual Features of Marfan's Syndrome , Circulation 24:1154, 1961;.

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