Abstract 16333: Racial Differences in Pressure-volume Relationships in Val122Ile Associated Cardiac Amyloidosis

• Published in: Circulation (New York, N.Y.) N.Y.), 2020-11, Vol.142 (Suppl_3 Suppl 3), p.A16333-A16333
• Main Authors: Batra, Jaya, olivotto, iacopo, Maurer, Mathew S
• Format: Article
• Language: English
• ISSN: 0009-7322; 1524-4539
• DOI: 10.1161/circ.142.suppl_3.16333

BackgroundTransthyretin cardiac amyloidosis (ATTR-CA) is the leading cause of restrictive cardiomyopathy in older adults. The valine-to-isoleucine substitution (Val122Ile) is the most common inherited variant in the U.S., primarily affecting patients of Afro-Caribbean descent. This variant has also been identified in white individuals in Northern Italy who present with a similar disease phenotype. It is unknown whether there are between-race differences in cardiac chamber function at diagnosis of Val122Ile associated ATTR-CA. MethodsIn this retrospective study of 70 patients from two amyloid centers with Val122Ile associated ATTR-CA diagnosed over two decades, clinical and echocardiographic features at diagnosis were compared between races. Cardiac chamber performance was compared using noninvasive, single beat pressure-volume analysis. ResultsAverage age at diagnosis was 72 years. Compared to white patients (n=17), black individuals (n=53) had lower systolic blood pressure (110 vs. 131 mmHg , p