P983 ATTR cardiac amyloidosis and aortic stenosis: the same physiopathology, different prognosis?

I Almeida, M Victor,I Cruz,A Marques, C Gomes,H Pereira

European Journal of Echocardiography(2020)

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摘要
Abstract Introduction Degenerative aortic stenosis and age-related amyloidosis share common demographic and clinical characteristics. Both surgical and transcatheter aortic valve replacement provide excellent outcomes in elderly patients with isolated aortic stenosis, however previous reports have suggested the potential negative impact of concomitant transthyretin amyloidosis. Purpose Evaluation of the association of ATTR amyloidosis and degenerative aortic stenosis. Methods Single center retrospective analysis of patients’ data referred to perform a 99mTc-DPD between September 2014- July 2018 due to an abnormal echocardiogram or family history of ATTR. Data was collected regarding clinical, echocardiographic and 99mTc-DPD parameters to evaluate prognostic impact of concomitant cardiac ATTR amyloidosis and aortic stenosis. Results 35 patients were reviewed. 12 (34.3%) had a positive 99mTc-DPD, performing the diagnosis of an ATTR amyloidosis. 33.3% had a cardiac uptake level 2 and 66.6% level 3. 16.7% of patients with ATTR amyloidosis had at the same time of amyloidosis diagnosis, a diagnosis of at least moderate degenerative aortic stenosis (mean maximal velocity 3.73 m/sec and mean gradient 22mmHg). These patients were older (mean age 80.5 ± 14.1 versus 77.9 ± 6.4 years, p 0.527) and all male gender in both groups. There was no evidence of higher level of cardiac uptake in patients with aortic stenosis. Cardiac function was not significantly different between groups: mean ejection fraction 45.5 ± 13.4% in patients with concomitant aortic stenosis versus 45.5 ±10.1% in patients without (p 0.672); mean global longitudinal strain -8.7 ± 2.4% versus -7.3 ± 3.0% (p 0.402). Most patients in both groups were at NYHA class II at the diagnosis. There was no difference regarding mean value of NTproBNP (7381.5 ± 32.6 versus 43332.1 ± 23.7 pg/ml, p 0.267). During follow up, 30% of patients without aortic stenosis was hospitalized due to acute heart failure and 50% died. In the group of aortic stenosis, the hospitalization and all-cause mortality rate was 100%. Conclusions In this study, although there were no differences regarding demographic neither clinical parameters between groups, patients with aortic stenosis presented more cardiac events and higher mortality, dying yet before the aortic stenosis became symptomatic. More studies are needed to evaluate the prognosis of cardiac amyloidosis on the postoperative.
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关键词
cardiac amyloidosis,aortic stenosis,different prognosis,same physiopathology
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