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European Heart Journal 2006 27(21):2496; doi:10.1093/eurheartj/ehi871
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© The European Society of Cardiology 2006. All rights reserved. For Permissions, please e-mail: journals.permissions@oxfordjournals.org

Cardiac oxalosis: a rare cause of diastolic dysfunction

Sonia Vélez-Roa1, Michel Depierreux2, Joëlle Nortier3 and Philippe Unger1,*

1 Department of Cardiology, Erasme Hospital, Université Libre de Bruxelles, 808 route de Lennik, Brussels, Belgium
2 Department of Pathology, Erasme Hospital, Université Libre de Bruxelles, Brussels, Belgium
3 Department of Nephrology, Erasme Hospital, Université Libre de Bruxelles, Brussels, Belgium

* Corresponding author. Tel: +32 255 53907; fax: +32 255 54609; E-mail address: punger{at}ulb.ac.be

A 26-year-old woman was admitted because of atypical chest pain and exertional dyspnoea. Type 1 primary oxaluria was diagnosed in 1991. Since 2002, end-stage renal failure required iterative high-flux haemodialysis (HD). Blood pressure was 120/75 mmHg and heart rate 86 bpm. There was no heart murmur, abnormal heart sounds, pulmonary crackles, or signs of cardiac failure. Serum oxalate level was 104 µm/L (n: 11–27). ECG showed criteria of left ventricular (LV) hypertrophy and strain. Chest X-ray showed an increased cardiothoracic ratio. Transthoracic echocardiography revealed hyperechogenic myocardium with a speckled pattern, concentric hypertrophy (Panels A and B), and a 55% LV ejection fraction. Doppler mitral inflow and tissue Doppler imaging at septal annulus demonstrated an E/A and an E/E' ratio of 2.1 and 18, respectively, consistent with increased LV filling pressure (Panels C and D). Coronary artery angiography was normal and left heart catheterization revealed an end-diastolic LV pressure of 17 mmHg. Right ventricular endomyocardial biopsy demonstrated extensive deposition of calcium oxalate crystals (haematoxylin and eosin, x400; Panel E). Despite the initiation of long daily HD and a combined liver–kidney transplantation 5 months later, no negative oxalate balance was achieved, as suggested by the persistence of echocardiographic abnormalities 1 year after transplantation.

Primary oxalosis is a rare metabolic disorder leading to deposition of calcium oxalate in several organs. Oxalate deposition in the heart may lead to congestive heart failure, cardio-embolism, and cause conduction disturbances. The present case is consistent with primary oxalosis and cardiac involvement leading to diastolic dysfunction.

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This Article
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