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Magnetic Resonance is more sensitive than Echocardiography to detect Cardiomyopathy in Friedreich’s Ataxia. (P2.121)

2015; Lippincott Williams & Wilkins; Volume: 84; Issue: 14_supplement Linguagem: Inglês

10.1212/wnl.84.14_supplement.p2.121

1526-632X

Alberto Martínez, Ingrid Faber, Thiago Junqueira Ribeiro de Rezende, Cynthia Bonilha Da Silva, Thiago Venâncio, Anelyssa D’Abreu, Íscia Lopes‐Cendes, Otávio R. Coelho‐Filho, Marcondes C. França,

Mitochondrial Function and Pathology

OBJECTIVE: to evaluate the usefulness of cardiac magnetic resonance imaging (cMRI) to investigate heart damage in Friedreich’s ataxia (FDRA). BACKGROUND: FDRA is the most common inherited ataxia and is caused by homozygous GAA expansions in the FXN gene. Besides its overt neurological features, FDRA is a multisystem disorder. Cardiomyopathy is often progressive and the main cause of death in the disease, so that sensitive diagnostic methods would be helpful to improve long-term prognosis. DESIGN/METHODS: General demographics, GAA expansion size, age at onset and clinical characteristics were evaluated. For each patient, echocardiography and cMRI were performed within one year interval. We used a 3T scanner to obtain cMRI, and the acquisition protocol included: cine CMR images for LV volumes, function and mass assessment; late gadoliniun enhancement for scar assessment and T2*-images for iron overload assessment. Results are detailed with descriptive statistics. We used Fisher exact test to compare the sensitivity of both methods. P values ≤0.05 were considered significant. RESULTS: We enrolled 14 FDRA patients (7 men). Mean age and age at onset were 22.2±6 and 11.3±3.27 years, respectively. GAA-expansion lengths were: GAA1-1135.3±262.9 and GAA2-1002.2 ±221.84. Mean FARS-III score was 64.1±19.3. All patients had scoliosis, 8/14(57[percnt]) pes cavus and 7/14(50[percnt]) had diabetes/impaired glucose tolerance. Echocardiography findings were normal in 10/14(71[percnt]) and showed concentric left ventricle hypertrophy in 4/14(29[percnt]) patients. In contrast, cMRI proved more sensitive to detect abnormalities (cMRI: 85.7[percnt] vs echo: 29[percnt], p=0.006). The most frequent finding was left ventricular hypertrophy with preserved ejection fraction. Evaluation of T2*-weighted images were performed in 8 patients with normal echocardiograms and demonstrated signs of iron accumulation in 62.5[percnt]. CONCLUSIONS: cMRI is a robust tool to identify cardiomyopathy in FDRA and more sensitive than standard echocardiography. Another relevant advantage is that it enables in vivo demonstration of iron accumulation in the heart which is a major feature of FDRA. Disclosure: Dr. Martinez has nothing to disclose. Dr. De Vasconcellos has nothing to disclose. Dr. Rezende has nothing to disclose. Dr. Silva has nothing to disclose. Dr. Venâncio has nothing to disclose. Dr. D9Abreu has nothing to disclose. Dr. Lopes-Cendes has nothing to disclose. Dr. Coelho-Filho has nothing to disclose. Dr. Franca, Jr. has nothing to disclose.