Prominent Papillary Muscles in Fabry Disease: A Diagnostic Marker?
| Autor: | Jörg Strotmann, Kai Hu, Wolfram Voelker, Sebastian Herrmann, Stefan Störk, Christoph Wanner, Markus Niemann, Frank Weidemann, Frank Breunig, Dan Liu, Georg Ertl |
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| Rok vydání: | 2011 |
| Předmět: |
Male
medicine.medical_specialty Acoustics and Ultrasonics Biophysics Cardiomyopathy Muscle hypertrophy Internal medicine medicine Humans Radiology Nuclear Medicine and imaging Prospective Studies Prospective cohort study Papillary muscle Aged Analysis of Variance Radiological and Ultrasound Technology business.industry Amyloidosis Case-control study Diagnostic marker Middle Aged Papillary Muscles medicine.disease Fabry disease medicine.anatomical_structure Echocardiography Case-Control Studies Cardiology Fabry Disease Female business |
| Zdroj: | Ultrasound in Medicine & Biology. 37:37-43 |
| ISSN: | 0301-5629 |
| DOI: | 10.1016/j.ultrasmedbio.2010.10.017 |
| Popis: | Fabry disease is often linked with a prominent papillary muscle. It remains unknown whether this sign could be used as a diagnostic marker to screen for Fabry patients. Standard echo was performed in 101 consecutive patients with concentric left ventricular (LV) hypertrophy (28 Fabry, 30 Friedreich, 34 isolated arterial hypertension, 9 amyloidosis) and 50 healthy controls. In addition, the areas of both papillary muscles, as well as the LV endocardial circumference, were manually traced in short axis views. A ratio of papillary muscle size to LV circumference was calculated (PM_LV_ratio). The papillary muscle area was positively correlated to LV wall thickness in this cohort (p < 0.0001; r = 0.58). In all patient subgroups, the absolute papillary muscle area was significantly enlarged and the PM_LV_ratio was significantly higher when compared with controls. However, Fabry patients showed a significantly larger absolute papillary muscle area than Friedreich and amyloidosis patients and a higher PM_LV_ratio than hypertensive and amyloidosis patients. Enlarged absolute papillary muscle area was evidenced in 21 (75%), and increased PM_LV_ratio was found in 22 (78%) of 28 Fabry patients. Combining these two parameters yields a sensitivity of 75% and specificity of 86% for diagnosing Fabry disease with LV hypertrophy. Only 10 of 73 non-Fabry patients (14%) (4 Friedreich, 1 amyloidosis, 5 hypertensive) showed an increased absolute papillary muscle area and PM_LV_ratio. In conclusion, this study confirmed the assumption that the prominent papillary muscle could be an echocardiographic marker for detection of Fabry patients with concentric LV hypertrophy. |
| Databáze: | OpenAIRE |
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