Chest
Restrictive Cardiomyopathy in Pseudoxanthoma Elasticum
Section snippets
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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Vascular calcification in pseudoxanthoma elasticum in children
2017, JMV-Journal de Medecine VasculaireCardiac manifestations of cutaneous disorders
2013, Journal of the American Academy of DermatologyCitation Excerpt :Redundant lax skin resembles “plucked chicken skin.”41 Cardiovascular findings include mitral valve prolapse and restrictive cardiomyopathy,42,43 but premature atherosclerotic vascular disease is the most serious complication. Accelerated atherosclerosis develops as a result of calcification of the internal elastic lamina of small and medium arteries.
Cutaneous manifestations of cardiovascular diseases
2008, Clinics in DermatologyCitation Excerpt :The areas most commonly involved are flexural areas including the neck, axillae, inguinal regions, antecubital and popliteal fossae, and the inner lower lip (Fig. 6). Cardiovascular manifestations in pseudoxanthoma elasticum are common and include mitral valve prolapse and restrictive cardiomyopathy.70,71 The most serious cardiac complication is accelerated atherosclerosis, which develops as a consequence of calcification of the internal elastic laminae of small and medium-sized arteries, including the coronary arteries.
Restrictive Cardiomyopathy: Diagnosis and Prognostic Implications
2008, Practice of Clinical Echocardiography, Thrid EditionLeft Ventricular Systolic and Diastolic Function by Echocardiogram in Pseudoxanthoma Elasticum
2006, American Journal of Cardiology