Clinical vignette
Cardiac oxalosis: a rare cause of diastolic dysfunction
Sonia Vélez-Roa1, Michel Depierreux2, Joëlle Nortier3 and Philippe Unger1*
1Department of Cardiology, Erasme Hospital, Université Libre de Bruxelles, 808 route de Lennik, Brussels, Belgium; 2Department of Pathology, Erasme Hospital, Université Libre de Bruxelles, Brussels, Belgium; 3Department of Nephrology, Erasme Hospital, Université Libre de Bruxelles, Brussels, Belgium
*Corresponding author. Tel: +32 255 53907; fax: +32 255 54609; E-mail address: punger@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, ×400; 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.
[Table of Contents]