关键词: Amiloidosis cardiaca Amiloidosis cardiaca por transtirretina Cardiac amyloidosis Fracción de eyección conservada Heart failure Hipertrofia del ventrículo izquierdo Insuficiencia cardiaca Left ventricular hypertrophy Preserved ejection fraction Prevalence Prevalencia Transthyretin Transthyretin cardiac amyloidosis Transtirretina

来  源:   DOI:10.1016/j.rec.2024.07.005

Abstract:
OBJECTIVE: Transthyretin cardiac amyloidosis (ATTR-CA) is a frequent cause of heart failure with preserved ejection fraction (HFpEF). This study sought to determine the prevalence of ATTR-CA among HFpEF patients in a multicenter nationwide study.
METHODS: Consecutive ambulatory or hospitalized patients aged ≥ 50 years with HFpEF and left ventricle hypertrophy ≥ 12mm were studied at 20 Spanish hospitals. Screening for cardiac amyloidosis was initiated according to the usual clinical practice of each center. Positive scintigraphs were centrally analyzed.
RESULTS: 422 patients were included, of whom 387 underwent further screening for cardiac amyloidosis. A total of 65 patients (16.8%) were diagnosed with ATTR-CA, none below 75 years. There was an increase of prevalence with age. Of them, 60% were male, with a mean age of 85.3±5.2 years, mean left ventricle ejection fraction of 60.3±7.6% and a mean maximum left ventricle wall thickness of 17.2 [12-25] mm. Most of the patients were New York Heart Association class II (48.4%) or III (46.8%). Besides being older than non-ATTR-CA patients, ATTR-CA patients had higher median NT-proBNP levels (3801 [2266-7132] vs 2391 [1141-4796] pg/mL; P=.003). There was no statistical difference in the prevalence of ATTR-CA by sex (19.7% for men and 13.8% for women, P=.085). A ∼7% (4/56) of the patients exhibited a genetic variant (ATTRv).
CONCLUSIONS: This multicenter nationwide study found a prevalence of 16.8%, confirming that ATTR-CA is a significant contributor to HFpEF in male and female patients with left ventricle hypertrophy and more than 75 years.
摘要:
目的:转甲状腺素蛋白心脏淀粉样变性(ATTR-CA)是射血分数保留的心力衰竭(HFpEF)的常见原因。本研究旨在在一项多中心的全国性研究中确定HFpEF患者中ATTR-CA的患病率。
方法:在西班牙20家医院研究了年龄≥50岁的HFpEF和左心室肥厚≥12mm的连续门诊或住院患者。根据每个中心的常规临床实践开始CA筛查。对阳性闪烁显像进行集中分析。
结果:共纳入422例患者,其中387人接受了进一步的CA筛查。65例患者(16.8%)被诊断为ATTR-CA,没有一个小于75岁。患病率随年龄增长而增加。在这些患者中,60%是男性,平均年龄85.3±5.2岁,平均左心室射血分数为60.3±7.6%,和平均最大左心室壁厚17.2(范围,12-25)mm。大多数患者为纽约心脏协会II级(48.4%)或III级(46.8%)。除了比没有ATTR-CA的患者年龄大,ATTR-CA患者的NT-proBNP中位数水平较高(3801[2266-7132]vs2391[1141-4796]pg/mL;P=.003).ATTR-CA的患病率按性别分类差异无统计学意义(男性为19.7%,女性为13.8%,P=.085)。在大约7%(4/56)的患者中发现了遗传变异(ATTRv)。
结论:这项全国性的多中心研究发现,ATTR-CA的患病率为16.8%,确认它是75岁以上男女左心室肥厚患者HFpEF的重要原因。
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