C65 TRANSTYRETIN CARDIAC AMYLOIDOSIS IN THE ELDERLY PATIENT: ALWAYS A WILD TYPE?
نویسندگان
چکیده
Abstract Background Transthyretin amyloidosis (ATTR) exists in two forms: a genetic form transmitted by autosomal dominant inheritance (ATTRv) and wild type (ATTRwt). Clinically, the hereditary tends to have an early onset adulthood it is characterized particular tropism for nervous as well cardiac tissue. In contrast, "wild type" has later with major involvement neurological manifestations usually limited carpal tunnel syndrome. Methods results our centre, from January October 2022, 20 patients older than 75 years received diagnosis of ATTR (73% male, 27% female, mean age 83±5). all patients, blood tests showed significant stable increase values troponin Ths (0.070± 0.074 ng/mL) NT–proBNP (1533±1204 pg/ml), absence monoclonal component urine, myocardial bisphosphonate scintigraphy positive grade 2/3. All peripheral neuropathy presence syndrome 9 cases. Before starting therapy Tafamidis underwent testing amplification exons 2,3 4 TTR gene subsequent sequencing. Unexpectedly, 3 elderly male (15%) test resulted different mutations pathogenetic significance (Val50Met; Ile88Leu; Val142Ile). First–degree relatives (10 consanguineous, average 49±12 years) agreed be subjected screening; these (1 females, 47±8 were corresponding mutation echocardiogram, MRI scintigraphy. normal they included annual follow–up protocol at identify manifestation disease. Conclusions experience shows that 15% diagnosed may affected form. Making this context particularly important screening family members: current therapies are fact much more effective if started before organ damage can occur.
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Results Comparison of clinical findings between males and females affected by wt-ATTR amyloidosis are shown in table 1. Female patients with wt-ATTR did not differ from male patients regarding demographic or clinical parameters except for modified body mass index (1140±184 vs. 1029±154, p<0.05), glomerular filtration rate (66±23 vs. 85±31 ml/min*m*1.73; p<0.05), NYHA class (2.4±0.7 vs. 2.9±0.3;...
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ژورنال
عنوان ژورنال: European Heart Journal Supplements
سال: 2023
ISSN: ['1520-765X', '1554-2815']
DOI: https://doi.org/10.1093/eurheartjsupp/suad111.064