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Progressive myxomatous degeneration of the cardiac valves

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Abstract

Pure valvular insufficiency with no history of rheumatic fever may be caused by myxomatous degeneration. The involved valve has a characteristic gross and histologic appearance. The “floppy valve syndrome” as described by Read et al1 has been observed in stillborn infants, those with ventricular septal defect with prolapse of aortic cusps,2 fibroelastosis, Hurler’s syndrome,3 Ehler-Danlos syndrome,4 atrial septal defect,5 and Marfan’s syndrome and its formes frustes6. This pathologic change has also been described in the mitral valve prolapse-click syndrome,7 which occurs in approximately 6.3% of the healthy female population.8 This entity is probably a milder expression of the myxomatous process, since few of these patients require valve replacement.9, 10

We report a case of myxomatous degeneration involving the mitral and aortic valves that resulted in mitral valve replacement followed by aortic valve replacement within 15 months.

Case report

A 63-year-old welder was in good health until he was hospitalized with acute pulmonary edema in August 1976. During hospitalization he responded to appropriate therapy, but continued to experience dyspnea on exertion and orthopnea after discharge despite adequate Digoxin and diuretic therapy. He was referred to the Cleveland Clinic for further evaluation.

On physical examination, he weighed 75.2 kg (167 pounds) and was 172.5 cm (5 feet 9 inches) tall. The blood pressure was 140/80 mm Hg and the pulse rate was 75 beats per minute. The lungs revealed fine crackling rales at the bases. The point of maximum impulse was at the sixth left intercostal space 2 cm left of the midclavicular . . .


 

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