Abstract
Four hundred and ninety-nine surgically excised valves were examined for pathological evidence of myxomatous degeneration. Thirty-six valves (7%) had myxomatous degeneration as a major pathological finding. Fourteen valves (3%) had significant myxomatous degeneration of the pars fibrosa, a finding which we define as "primary myxomatous degeneration". Echocardiographic findings and catheterisation results were correlated with the clinical course, surgical results, and follow-up in these 14 patients. Echocardiograms in 10 of the 11 patients who had them (91%) showed abnormalities suggesting the presence of primary myxomatous degeneration. Echocardiography was more helpful than angiography in diagnosis. The histological pattern of primary mitral myxomatous degeneration appears to be identical to that seen in patients with mitral valve prolapse and five of six patients with mitral lesions had echocardiographic evidence of prolapse. None of the patients with primary myxomatous degeneration of the aortic valve had syphilis of Marfan's syndrome. While the aetiology of primary myxomatous degeneration of cardiac valves is not known, a link to a more generalised disorder is suggested.
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- Bittar N., Sosa J. A. The billowing mitral valve leaflet. Report on fourteen patients. Circulation. 1968 Oct;38(4):763–770. doi: 10.1161/01.cir.38.4.763. [DOI] [PubMed] [Google Scholar]
- Corrigall D., Bolen J., Hancock E. W., Popp R. L. Mitral valve prolapse and infective endocarditis. Am J Med. 1977 Aug;63(2):215–222. doi: 10.1016/0002-9343(77)90235-2. [DOI] [PubMed] [Google Scholar]
- Davis R. H., Schuster B., Knoebel S. B., Fisch C. Myxomatous degeneration of the mitral valve. Am J Cardiol. 1971 Oct;28(4):449–455. doi: 10.1016/0002-9149(71)90009-9. [DOI] [PubMed] [Google Scholar]
- DeMaria A. N., Amsterdam E. A., Vismara L. A., Neumann A., Mason D. T. Arrhythmias in the mitral valve prolapse syndrome. Prevalence, nature, and frequency. Ann Intern Med. 1976 Jun;84(6):656–660. doi: 10.7326/0003-4819-84-6-656. [DOI] [PubMed] [Google Scholar]
- DeMaria A. N., King J. F., Bogren H. G., Lies J. E., Mason D. T. The variable spectrum of echocardiographic manifestations of the mitral valve prolapse syndrome. Circulation. 1974 Jul;50(1):33–41. doi: 10.1161/01.cir.50.1.33. [DOI] [PubMed] [Google Scholar]
- DeMaria A. N., Neumann A., Lee G., Mason D. T. Echocardiographic identification of the mitral valve prolapse syndrome. Am J Med. 1977 Jun;62(6):819–829. doi: 10.1016/0002-9343(77)90648-9. [DOI] [PubMed] [Google Scholar]
- Devereux R. B., Perloff J. K., Reichek N., Josephson M. E. Mitral valve prolapse. Circulation. 1976 Jul;54(1):3–14. doi: 10.1161/01.cir.54.1.3. [DOI] [PubMed] [Google Scholar]
- Dillon J. C., Haine C. L., Chang S., Feigenbaum H. Use of echocardiography in patients with prolapsed mitral valve. Circulation. 1971 Apr;43(4):503–507. doi: 10.1161/01.cir.43.4.503. [DOI] [PubMed] [Google Scholar]
- Frable W. J. Mucinous degeneration of the cardiac valves: the "floppy valve" syndrome. J Thorac Cardiovasc Surg. 1969 Jul;58(1):62–70. [PubMed] [Google Scholar]
- Gooch A. S., Vicencio F., Maranhao V., Goldberg H. Arrhythmias and left ventricular asynergy in the prolapsing mitral leaflet syndrome. Am J Cardiol. 1972 May;29(5):611–620. doi: 10.1016/0002-9149(72)90161-0. [DOI] [PubMed] [Google Scholar]
- Goodman D., Kimbiris D., Linhart J. W. Chordae tendineae rupture complicating the systolic click-late systolic murmur syndrome. Am J Cardiol. 1974 May 6;33(5):681–684. doi: 10.1016/0002-9149(74)90263-x. [DOI] [PubMed] [Google Scholar]
- Humphries W. C., Jr, Hammer W. J., McDonough M. T., Lemole G., McCurdy R. R., Spann J. F., Jr Echocardiographic equivalents of a flail mitral leaflet. Am J Cardiol. 1977 Nov;40(5):802–807. doi: 10.1016/0002-9149(77)90200-4. [DOI] [PubMed] [Google Scholar]
- Jamshidi A., Klein-Robbenhaar J. Myxomatous transformation of the aortic and mitral valve with subaortic "sail-like" membrane. Am J Med. 1970 Jul;49(1):114–117. doi: 10.1016/s0002-9343(70)80120-6. [DOI] [PubMed] [Google Scholar]
- Jeresaty R. M. Mitral valve prolapse--Click syndrome. Prog Cardiovasc Dis. 1973 May-Jun;15(6):623–652. doi: 10.1016/s0033-0620(73)80026-x. [DOI] [PubMed] [Google Scholar]
- Kern W. H., Tucker B. L. Myxoid changes in cardiac valves: pathologic, clinical, and ultrastructural studies. Am Heart J. 1972 Sep;84(3):294–301. doi: 10.1016/0002-8703(72)90361-4. [DOI] [PubMed] [Google Scholar]
- Leichtman D., Nelson R., Gobel F. L., Alexander C. S., Cohn J. N. Bradycardia with mitral valve prolapse: a potential mechanism of sudden death. Ann Intern Med. 1976 Oct;85(4):453–457. doi: 10.7326/0003-4819-85-4-453. [DOI] [PubMed] [Google Scholar]
- Marshall C. E., Shappell S. D. Sudden death and the ballooning posterior leaflet syndrome. Detailed anatomic and histochemical investigation. Arch Pathol. 1974 Aug;98(2):134–138. [PubMed] [Google Scholar]
- McCarthy L. J., Wolf P. L. Mucoid degeneration of heart valves: "blue valve syndrome". Am J Clin Pathol. 1970 Dec;54(6):852–856. doi: 10.1093/ajcp/54.6.852. [DOI] [PubMed] [Google Scholar]
- Pomerance A. Ageing changes in human heart valves. Br Heart J. 1967 Mar;29(2):222–231. doi: 10.1136/hrt.29.2.222. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Pomerance A. Ballooning deformity (mucoid degeneration) of atrioventricular valves. Br Heart J. 1969 May;31(3):343–351. doi: 10.1136/hrt.31.3.343. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Popp R. L., Brown O. R., Silverman J. F., Harrison D. C. Echocardiographic abnormalities in the mitral valve prolapse syndrome. Circulation. 1974 Mar;49(3):428–433. doi: 10.1161/01.cir.49.3.428. [DOI] [PubMed] [Google Scholar]
- Procacci P. M., Savran S. V., Schreiter S. L., Bryson A. L. Prevalence of clinical mitral-valve prolapse in 1169 young women. N Engl J Med. 1976 May 13;294(20):1086–1088. doi: 10.1056/NEJM197605132942004. [DOI] [PubMed] [Google Scholar]
- Read R. C., Thal A. P., Wendt V. E. Symptomatic valvular myxomatous transformation (the floppy valve syndrome). A possible forme fruste of the Marfan syndrome. Circulation. 1965 Dec;32(6):897–910. doi: 10.1161/01.cir.32.6.897. [DOI] [PubMed] [Google Scholar]
- Rippe J. M., Angoff G., Sloss L. J., Wynne J., Alpert J. S. Multiple floppy valves: an echocardiographic syndrome. Am J Med. 1979 May;66(5):817–824. doi: 10.1016/0002-9343(79)91122-7. [DOI] [PubMed] [Google Scholar]
- Roberts W. C., Perloff J. K. Mitral valvular disease. A clinicopathologic survey of the conditions causing the mitral valve to function abnormally. Ann Intern Med. 1972 Dec;77(6):939–975. doi: 10.7326/0003-4819-77-6-939. [DOI] [PubMed] [Google Scholar]
- Roberts W. C., Virmani R. Aschoff bodies at necropsy in valvular heart disease. Evidence from an analysis of 543 patients over 14 years of age that rheumatic heart disease, at least anatomically, is a disease of the mitral valve. Circulation. 1978 Apr;57(4):803–807. doi: 10.1161/01.cir.57.4.803. [DOI] [PubMed] [Google Scholar]
- Salazar A. E., Edwards J. E. Friction lesions of ventricular endocardium. Relation to chordae tendineae of mitral valve. Arch Pathol. 1970 Oct;90(4):364–376. [PubMed] [Google Scholar]
- Salomon N. W., Stinson E. B., Griepp R. B., Shumway N. E. Surgical treatment of degenerative mitral regurgitation. Am J Cardiol. 1976 Oct;38(4):463–468. doi: 10.1016/0002-9149(76)90464-1. [DOI] [PubMed] [Google Scholar]
- Sherman E. B., Char F., Dungan W. T., Campbell G. S. Myxomatous transformation of the mitral valve producing mitral insufficiency. Floppy valve syndrome. Am J Dis Child. 1970 Feb;119(2):171–175. doi: 10.1001/archpedi.1970.02100050173019. [DOI] [PubMed] [Google Scholar]
- Shrivastava S., Guthrie R. B., Edwards J. E. Prolapse of the mitral valve. Mod Concepts Cardiovasc Dis. 1977 Dec;46(12):57–61. [PubMed] [Google Scholar]
- Smith E. R., Fraser D. B., Purdy J. W., Anderson R. N. Angiographic diagnosis of mitral valve prolapse: correlation with echocardiography. Am J Cardiol. 1977 Aug;40(2):165–170. doi: 10.1016/0002-9149(77)90003-0. [DOI] [PubMed] [Google Scholar]
- Swartz M. H., Teichholz L. E., Donoso E. Mitral valve prolapse: a review of associated arrhythmias. Am J Med. 1977 Mar;62(3):377–389. doi: 10.1016/0002-9343(77)90835-x. [DOI] [PubMed] [Google Scholar]
- Torii S., Bashey R. I., Nakao K. Acid mucopolysaccharide composition of human-heart valve. Biochim Biophys Acta. 1965 Nov 1;101(3):285–291. doi: 10.1016/0926-6534(65)90007-2. [DOI] [PubMed] [Google Scholar]
- Trent J. K., Adelman A. G., Wigle E. D., Silver M. D. Morphology of a prolapsed posterior mitral valve leaflet. Am Heart J. 1970 Apr;79(4):539–543. doi: 10.1016/0002-8703(70)90260-7. [DOI] [PubMed] [Google Scholar]