Chest
Volume 78, Issue 1, July 1980, Pages 113-115
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Restrictive Cardiomyopathy in Pseudoxanthoma Elasticum

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A unique case of severe and diffuse endocardial fibroelastosis covering the entire left ventricle and associated with pseudoxanthoma elasticum is presented. The clinical picture was that of an apparently primary restrictive cardiomyopathy. Specific histologic lesions could be recognized in the endocardium.

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CASE REPORT

A 39-year-old woman was admitted to the hospital because of congestive heart failure and atypical pain of two years’ duration. Her mother died of heart disease at the age of 63. On examination, the apical impulse and the first heart sound were normal. There was a grade 2 apical systolic murmur and a soft opening snap followed by a short middiastolic rumble. Blood pressure was 120/80 mm Hg. The chest roentgenogram showed a typical mitral silhouette with a normal left ventricle. The ECG showed

DISCUSSION

Our case provides anatomic and functional evidence that the heart failure in PXE may be due to a genuine obliterative or restrictive disease, analogous to the more severe cases of Loeffler's endocarditis or Davies’ endomyocardial fibrosis.4 The marked elevation of the end diastolic pressure in the presence of a small left ventricular cavity reflects the restriction of the filling imposed by the endocardial lining.4 The mitral regurgitation has been considered a distinctive feature of the

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