Author + information
- Received April 18, 2013
- Revision received May 1, 2013
- Accepted May 6, 2013
- Published online December 17, 2013.
- Pablo Jorge, MD∗,
- Martín J. García González, MD, PhD∗,
- Sagrario G. Rebollo, MD, PhD†,
- Sonia García, MD, PhD‡,
- Francisco Bosa, MD, PhD∗,
- Ignacio Laynez, MD, PhD∗ and
- Alejandro De la Rosa, MD, PhD∗
A 53-year-old woman had a cardiac deposit of oxalate due to primary hyperoxaluria causing progressive dilated and restrictive cardiomyopathy, with some admissions for heart failure. Primary hyperoxaluria is a very uncommon liver disease, and myocardial involvement is rare and characterized by progressive diastolic dysfunction, which precedes systolic dysfunction that may occur over time. These patients usually have a poor prognosis, and many require a kidney and liver transplantation. Chest x-rays before diagnosis (A and B) and those obtained 1 year after diagnosis (C and D) show significant cardiomegaly. Electrocardiography shows a progressive increase in ventricular chambers (E to H, Online Videos 1, 2, and 3). Echocardiographic evolution shows a restrictive pattern of diastolic function and myocardial shiny appearance. Hemodynamic study of the right atrium (RA) venous pulse curve showed a restrictive pattern with deep descent of x and y and the right ventricle (RV) with early diastolic dip plateau phase (I). An endomyocardial biopsy was performed, revealing the presence of calcium oxalate crystals (arrow in J). Nephrocalcinosis was seen (red arrow in K), and a diagnosis, based on criteria, of primary hyperoxaluria was made.
- Received April 18, 2013.
- Revision received May 1, 2013.
- Accepted May 6, 2013.
- American College of Cardiology Foundation