Myocardial Fatty Foci in Tuberous Sclerosis Complex: Imaging Findings
| Autor: | Jamil Armaly, Edward Derrick, Jeremy R. Burt, Baiywo Rop, Usman Siddiqui |
|---|---|
| Jazyk: | angličtina |
| Rok vydání: | 2016 |
| Předmět: |
cardiac lipoma
Pathology medicine.medical_specialty congenital hereditary and neonatal diseases and abnormalities Cardiology Disease tuberous sclerosis complex 030204 cardiovascular system & hematology Rhabdomyoma Intracardiac injection cardiac imaging 030218 nuclear medicine & medical imaging Benign tumor 03 medical and health sciences Tuberous sclerosis 0302 clinical medicine myocardial fatty foci hemic and lymphatic diseases medicine Genetics rhabdomyoma Cardiac imaging business.industry General Engineering Soft tissue medicine.disease cardiovascular system cardiac mri Cardiac Lipoma business Radiology |
| Zdroj: | Cureus |
| ISSN: | 2168-8184 |
| Popis: | Tuberous sclerosis complex (TSC) is a rare autosomal dominant genetic syndrome. The hallmark of the disease is multiple hamartomatous lesions in multiple organ systems. Common cardiac manifestations of TSC are rhabdomyomas, which are a benign tumor of striated muscle. In some patients with TSC, myocardial fatty foci (MFF) deposition has been described with or without the presence of rhabdomyomas. We present the case of a 24-year-old female with TSC and refractory seizures, who was evaluated with cardiac magnetic resonance (CMR) for an intracardiac right ventricular mass thought to be rhabdomyoma on echocardiography and for multiple areas of myocardial fatty deposition. Myocardial fatty deposition is a common finding in patients at cardiac imaging. In patients with TSC, it is critical that fatty deposits and lipomas are clearly distinguished from rhabdomyoma. CMR is an integral part of characterizing cardiac masses as it has superior soft tissue characterization and a wider field of view compared to echocardiography. A positive correlation has been shown between the number of MFF and the degree of extracardiac tuberous sclerosis (TS) manifestations suggesting that MFF may indicate more severe multiorgan disease in patients with TSC. Cardiac MR is superior to echocardiogram in evaluating and distinguishing intracardiac lipomas and fatty deposits from rhabdomyomas. Published studies have indicated that in patients with TSC, the presence of MFF correlates with the severity of multiorgan disease as was seen in our case. |
| Databáze: | OpenAIRE |
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