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158 Racialdifferences inval122Ile associatedtransthyretin cardiacamyloidosis Alessia ArgiròMattia ZampieriJaya Batra2021 год

158 Racial differences in val122Ile associated transthyretin cardiac amyloidosis
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Аннотация: Abstract Aims The valine-to-isoleucine substitution (Val122Ile) is the most common variant of transthyretin (TTR) amyloidosis in the USA, primarily affecting individuals of African descent and leading to a restrictive cardiomyopathy. This variant has recently been identified in a cluster of White individuals in Italy. In this study we aimed to investigate differences in the clinical phenotype of Val122Ile associated TTR cardiac amyloidosis (ATTR-CA) between Black and White individuals. Methods and results In this retrospective study of 70 patients (mean age 72 years) with Val122Ile associated TTR ATTR-CA, cardiac chamber performance was compared using noninvasive pressure-volume analysis. Compared to White patients (n = 17), Black individuals (n = 53) had lower systolic blood pressures (110 vs. 131 mmHg, P < 0.001), reduced pulse pressures (41 vs. 58 mmHg, P < 0.001), and impaired renal function (eGFR 46 vs. 67 mL/min/1.73 m2, P < 0.001) at presentation. Systolic properties and arterial elastance were similar. Black patients had an end-diastolic pressure-volume relationship shifted upward and leftward relative to White patients, indicating reduced left ventricular chamber capacitance. Pressure-volume area at a left ventricular end-diastolic pressure of 30 mmHg was lower in Black compared to White individuals (8055 mmHg*ml vs. 11 538 mmHg*ml, P = 0.008). Conclusions Despite presenting at a similar age to White patients, Black individuals with Val122Ile associated ATTR-CA have a greater degree of cardiac chamber dysfunction at the time of diagnosis due to impaired ventricular capacitance. Whether these differences are attributable to amyloidosis or other cardiovascular disease requires further study.
Год издания: 2021
Авторы: Alessia Argirò, Mattia Zampieri, Jaya Batra, Hannah Rosenblum, Daniel Burkhoff, Mathew S. Maurer, Iacopo Olivotto, Francesco Cappelli
Издательство: Oxford University Press
Источник: European Heart Journal Supplements
Ключевые слова: Amyloidosis: Diagnosis, Treatment, Outcomes, Parathyroid Disorders and Treatments
Другие ссылки: European Heart Journal Supplements (PDF)
European Heart Journal Supplements (HTML)
Florence Research (University of Florence) (PDF)
Florence Research (University of Florence) (HTML)