Argirò Alessia, Silverii Maria Vittoria, Burgisser Costanza, Fattirolli Francesco, Baldasseroni Samuele, di Mario Carlo, Zampieri Mattia, Biagioni Giulia, Mazzoni Carlotta, Chiti Chiara, Allinovi Marco, Ungar Andrea, Perfetto Federico, Cappelli Francesco
Tuscan Regional Amyloidosis Centre, Careggi University Hospital, Florence, Italy.
Cardiac Rehabilitation Unit, Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy.
Can J Cardiol. 2024 Mar;40(3):364-369. doi: 10.1016/j.cjca.2023.09.028. Epub 2023 Oct 2.
Transthyretin amyloid cardiomyopathy (ATTR-CM) is associated with a progressive reduction of functional capacity. The progression of cardiopulmonary exercise testing (CPET) parameters over time is still unknown.
In this study, 55 patients with ATTR-CM underwent 2 serial cardiologic evaluations and CPETs in a national referral center for cardiac amyloidosis (Careggi University Hospital, Florence).
Forty-three patients (78%) had wild-type ATTR. Median age was 80 years (interquartile range [IQR] 76-83 years), and 50 of the patients (91%) were men. At baseline, median peak oxygen consumption (pVO) was 15 mL/kg/min (IQR 12-18 mL/kg/min), percentage of predicted pVO (%ppVO) was 71% (IQR 60%-83%) and VE/VCO slope was 31 (IQR 26-34). After a median follow-up of 14 months (IQR 13-16 months), pVO, %ppVO and VE/VCO slope were significantly worsened (-1.29 mL/kg/min [95% confidence interval (CI): -1.85 to -0.74; P < 0.01], -4.5% [95% CI: -6.9 to -2.02; P < 0.01], and 8.6 [95% CI 6-11; P < 0.01], respectively). Furthermore, exercise time (-39 s, 95% CI: -59 to -19; P < 0.01), exercise tolerance (-0.47 metabolic equivalents, 95% CI: -0.69 to -0.2; P < 0.01), and peak systolic pressure (-10.8 mm Hg, 95% CI: -16.2 to -5.4; P < 0.01) were significantly reduced. The worsening in CPET variables did not correspond with a significant change in echocardiographic parameters.
Cardiorespiratory response to exercise significantly worsened over a short period of time in patients with ATTR-CM. Serial CPET may be useful to identify early disease progression.
转甲状腺素蛋白淀粉样变心肌病（ATTR-CM）与功能能力的逐渐下降有关。心肺运动试验（CPET）参数随时间的变化情况仍不清楚。
在本研究中，55例ATTR-CM患者在一家全国性心脏淀粉样变性转诊中心（佛罗伦萨卡雷吉大学医院）接受了2次连续的心脏评估和CPET检查。
43例患者（78%）为野生型ATTR。中位年龄为80岁（四分位间距[IQR]76 - 83岁），50例患者（91%）为男性。基线时，中位峰值耗氧量（pVO）为15 mL/kg/min（IQR 12 - 18 mL/kg/min），预测pVO百分比（%ppVO）为71%（IQR 60% - 83%），VE/VCO斜率为31（IQR 26 - 34）。中位随访14个月（IQR 13 - 16个月）后，pVO、%ppVO和VE/VCO斜率显著恶化（分别为-1.29 mL/kg/min [95%置信区间（CI）：-1.85至-0.74；P < 0.01]，-4.5% [95% CI：-6.9至-
