From the Guidelines
Sparkling myocardium is a distinctive echocardiographic finding characterized by increased echogenicity of the heart muscle, typically associated with amyloidosis, and should prompt further diagnostic evaluation to confirm the underlying condition. This phenomenon is most commonly associated with amyloidosis, a condition where abnormal protein deposits called amyloid infiltrate the heart tissue, as noted in a study published in Circulation 1. The sparkling or granular appearance occurs because amyloid fibrils reflect ultrasound waves differently than normal heart tissue, creating this characteristic pattern.
Some key points to consider when evaluating sparkling myocardium include:
- The condition is often associated with thickened ventricular walls, diastolic dysfunction, and atrial enlargement, as seen in cardiac amyloidosis 1.
- Other conditions causing myocardial infiltration or hypertrophy can sometimes produce similar appearances, including infiltrative diseases such as Anderson-Fabry disease, acute sarcoidosis, and mucopolysaccharidoses 1.
- Diagnosis is based on a combination of clinical evaluation, echocardiography, and other imaging tests, with endomyocardial biopsy sometimes required for definitive diagnosis 1.
- Early diagnosis is crucial for appropriate treatment of the underlying condition, as sparkling myocardium can be a sign of a serious and potentially life-threatening disease.
In terms of diagnostic evaluation, tissue biopsy, nuclear imaging, or cardiac MRI should be considered to confirm amyloidosis or other underlying conditions. A study published in the European Heart Journal notes that clues suggesting myocardial storage disease or infiltration include sparkling or granular myocardial texture, small pericardial effusion, and thickening of the interatrial septum 1. Another study published in the Journal of the American College of Radiology highlights the importance of imaging tests in evaluating infiltrative disease, including cardiac amyloidosis 1.
Overall, sparkling myocardium is a significant finding that requires prompt and thorough evaluation to determine the underlying cause and guide appropriate treatment. Further diagnostic evaluation is essential to confirm the diagnosis and initiate appropriate management, as noted in a study published in Circulation 1.
From the Research
Definition of Sparkling Myocardium
- A sparkling myocardium refers to a granular and sparkling appearance of the myocardium, which is a characteristic feature of cardiac amyloidosis 2.
- This appearance is due to the deposition of amyloid fibrils in the myocardium, which can be detected by echocardiography.
Association with Cardiac Amyloidosis
- The presence of a granular and sparkling myocardial appearance is a strong predictor of cardiac amyloidosis, along with thickened heart valves 2.
- Cardiac amyloidosis is a progressive and life-threatening disease characterized by the aggregation and deposition of amyloidogenic misfolded transthyretin (TTR) in the myocardium 3.
Diagnosis and Treatment
- Noninvasive methods, such as electrocardiography and echocardiography, can be useful for detecting the myocardial infiltrative process and diagnosing cardiac amyloidosis 2.
- Tafamidis, a TTR stabilizer, has been approved for the treatment of adults with transthyretin amyloid cardiomyopathy (ATTR-CM) and has been shown to reduce all-cause mortality and frequency of cardiovascular-related hospitalizations 3, 4.