Attr Is Underdiagnosed In Aortic Stenosis Despite High Prevalence Of Associated Clinical Conditions

Abstract

Introduction Cardiac transthyretin amyloidosis (CA-ATTR) is a treatable condition that may co-exist in patients with paradoxical low-flow, low-gradient aortic stenosis (LFLG-AS) with normal ejection fraction (EF) undergoing transcatheter aortic valve replacement (TAVR). CA-ATTR is associated with non-cardiac clinical conditions such as carpel tunnel syndrome, lumbar spinal stenosis, biceps tendon rupture, peripheral neuropathy, and autonomic dysfunction. Hypothesis: We hypothesized that the prevalence of these associated conditions in paradoxical LFLG-AS patients undergoing TAVR is higher than the reported diagnosis of CA-ATTR in this population. Methods The electronic medical record was used to identify patients with severe LFLG-AS (mean aortic valve gradient <40mmHg, aortic valve area <1cm2) with normal EF(≥50%). Patients were excluded for bicuspid aortic valve or valve-in-valve procedure. Charts were reviewed retrospectively to determine if CA-ATTR was diagnosed and if CA-ATTR associated clinical conditions were present; these were summarized with descriptive statistics. Results A total of 278 patients met inclusion/exclusion criteria. Table 1 shows the prevalence of CA-ATTR associated clinical conditions. There were no patients identified with a diagnosis of CA-ATTR. Having a CA-ATTR associated clinic condition did not predict 1 year-post TAVR NYHA class or all-cause mortality using a multivariable regression model. Conclusion No patients had been diagnosed with CA-ATTR in our cohort of paradoxical LFLG-AS, despite a high prevalence of associated clinical conditions. Our results support the need for standardized screening measures for CA-ATTR in the paradoxical LFLG-AS population. Cardiac transthyretin amyloidosis (CA-ATTR) is a treatable condition that may co-exist in patients with paradoxical low-flow, low-gradient aortic stenosis (LFLG-AS) with normal ejection fraction (EF) undergoing transcatheter aortic valve replacement (TAVR). CA-ATTR is associated with non-cardiac clinical conditions such as carpel tunnel syndrome, lumbar spinal stenosis, biceps tendon rupture, peripheral neuropathy, and autonomic dysfunction. Hypothesis: We hypothesized that the prevalence of these associated conditions in paradoxical LFLG-AS patients undergoing TAVR is higher than the reported diagnosis of CA-ATTR in this population. The electronic medical record was used to identify patients with severe LFLG-AS (mean aortic valve gradient <40mmHg, aortic valve area <1cm2) with normal EF(≥50%). Patients were excluded for bicuspid aortic valve or valve-in-valve procedure. Charts were reviewed retrospectively to determine if CA-ATTR was diagnosed and if CA-ATTR associated clinical conditions were present; these were summarized with descriptive statistics. A total of 278 patients met inclusion/exclusion criteria. Table 1 shows the prevalence of CA-ATTR associated clinical conditions. There were no patients identified with a diagnosis of CA-ATTR. Having a CA-ATTR associated clinic condition did not predict 1 year-post TAVR NYHA class or all-cause mortality using a multivariable regression model. No patients had been diagnosed with CA-ATTR in our cohort of paradoxical LFLG-AS, despite a high prevalence of associated clinical conditions. Our results support the need for standardized screening measures for CA-ATTR in the paradoxical LFLG-AS population.