2014
DOI: 10.1186/1471-2261-14-86
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Myocardial fibrosis as the first sign of cardiac involvement in a male patient with Fabry disease: report of a clinical case and discussion on the utility of the magnetic resonance in Fabry pathology

Abstract: BackgroundCardiovascular magnetic resonance (CMR) with late gadolinium enhancement (LGE) imaging is increasingly used to assess myocardial involvement in patients with Fabry disease, an X linked lipid storage disorder. However, it is often proposed as an optional tool. A different cardiomyopathic disease progression between male and female patients was hypothesised in previous studies, as in female myocardial fibrosis was found without left ventricular (LV) hypertrophy, while myocardial fibrosis was always det… Show more

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Cited by 4 publications
(4 citation statements)
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“…However, our observation of LGE in two males who did not have increased LVM index highlights that this is not entirely a female phenomenon. The only other support of this concept of early fibrosis prior to hypertrophy in males is a recent case report documenting it in a 19-year-old male 16. It is likely that this important observation has not been frequently observed because of the paucity of CMR studies in Fabry disease at early stages.…”
Section: Discussionmentioning
confidence: 99%
“…However, our observation of LGE in two males who did not have increased LVM index highlights that this is not entirely a female phenomenon. The only other support of this concept of early fibrosis prior to hypertrophy in males is a recent case report documenting it in a 19-year-old male 16. It is likely that this important observation has not been frequently observed because of the paucity of CMR studies in Fabry disease at early stages.…”
Section: Discussionmentioning
confidence: 99%
“…The typical LGE pattern of cardiac amyloidosis is diffuse subendocardial or transmural, which provides not only high sensitivity and specificity for diagnosis but also a strong prognostic value for all-cause mortality ( 107 109 ). Myocardial fibrosis can be the first sign of organ involvement in patients with Anderson-Fabry disease ( 110 ) and it typically presents LGE in the basal inferolateral myocardium ( 111 ).…”
Section: Detection: Laboratory Biomarkers and Imaging Techniquesmentioning
confidence: 99%
“…70 Women may develop fibrosis without LVH, and recently, this behavior in young male patients with FD has also been observed. 8,71 Typically, cardiologists tend to diagnose FD late, and unfortunately, cardiac involvement is the leading cause for the substantially increased morbidity and death in these patients. 32,72,73 In advanced stages of the disease, patients may develop conduction disorders, arrhythmias, progressive deterioration of ventricular function, and so on, which determine increased risk of stroke due to atrial fibrillation (AF), death from heart failure, and SCD.…”
Section: Prognosis and Treatmentmentioning
confidence: 99%