Author + information
- Received July 22, 2011
- Revision received August 9, 2011
- Accepted August 15, 2011
- Published online May 15, 2012.
- Kian Keong Poh, MBBChir, MA, MMed⁎,
- Ping Chai, MBBS, MMed⁎,
- Raymond C.C. Wong, MBBS⁎ and
- Kong Bing Tan, MBBS†
A 37-year-old man was admitted for a 1-week history of increasing lethargy, chest discomfort, and dyspnea. Examination revealed biventricular failure. Chest x-ray confirmed cardiomegaly and bat's wing pulmonary edema (A). Electrocardiography showed Goldberger's triad (prominent precordial and low limb lead QRS voltages and poor R-wave progression) (B).
Cardiac magnetic resonance imaging demonstrated severe biventricular systolic dysfunction (C and D, Online Videos 1, 2, and 3). There were severe atrioventricular valvular regurgitations from malcoaptation and moderate pericardial effusion, but no myocardial late gadolinium enhancement.
The coronary angiogram was normal. Myocardial biopsy was performed. The histopathology featured hypertrophic cardiomyocytes with enlarged and hyperchromatic nuclei. There were prominent intracytoplasmic perinuclear vacuoles (E, arrows). There was mild interstitial fibrosis, but no significant inflammatory infiltrate, glycogen accumulation, or amyloid deposition. Electron microscopy showed presence of membrane-bound vacuoles (F). Unlike lysosomal disorders and glycogenoses, the vacuoles appeared empty, reflecting an idiopathic form of vacuolar cardiomyopathy (1). Mitochondria were focally increased, but did not show abnormal cristae patterns or crystalline inclusions. Despite optimal medical therapy, the patient expired soon after discharge.
- Received July 22, 2011.
- Revision received August 9, 2011.
- Accepted August 15, 2011.
- American College of Cardiology Foundation