Willixhofer Robin, Salvioni Elisabetta, Capra Nicolò, Contini Mauro, Campodonico Jeness, Agostoni Piergiuseppe
Centro Cardiologico Monzino, IRCCS, Milan, Italy.
Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy.
Physiol Rep. 2025 May;13(9):e70308. doi: 10.14814/phy2.70308.
In cardiac amyloidosis (CA) cardiopulmonary exercise testing (CPET) is underexplored. This study evaluated exercise limitations in CA using CPET, focusing on the ventilation-to-carbon dioxide production (VE/VCO) slope and peak oxygen uptake (VO). Seventeen studies involving 1505 patients were analyzed and systematically reviewed according to PRISMA reporting guidelines. Subgroup analyses assessed differences by diagnosis (ATTR vs. AL), CPET modality, and age. The cohort included 12% with AL, 80% with ATTR (23% hereditary [ATTRv], 70% wild-type [ATTRwt], 7% unspecified), and 8% unidentified subtypes. VE/VCO slope was elevated across ATTR subgroups: 38.4 (95% CI: 36.9-40.0, I = 57%) in ATTRwt and 37.9 (95% CI: 35.1-40.7, I = 70%) in ATTRv. ATTR patients were older than AL patients by 9.0 years (95% CI: 0.4-17.6, I = 88%) and had a higher VE/VCO slope: 2.5 (95% CI: 0.2-4.8, I = 0%). CPET modality influenced peak VO, which was lowest for treadmill exercise (13.7, 95% CI: 12.7-14.8, I = 0%, mL/min/kg) compared to upright cycle ergometry (14.7, 95% CI: 14.3-15.1, I = 33%) and semi-recumbent cycle ergometry (14.5, 95% CI: 14.1-14.9, I = 28%). A high VE/VCO slope characterizes both ATTRwt and ATTRv, while AL patients are younger with lower VE/VCO slope levels. Peak VO in ATTR patients may depend on exercise modality.
在心脏淀粉样变性(CA)中,心肺运动试验(CPET)尚未得到充分研究。本研究使用CPET评估CA患者的运动受限情况,重点关注通气与二氧化碳产生量(VE/VCO)斜率和峰值摄氧量(VO)。根据PRISMA报告指南,对17项涉及1505例患者的研究进行了分析和系统评价。亚组分析评估了诊断(ATTR与AL)、CPET方式和年龄的差异。该队列包括12%的AL患者、80%的ATTR患者(23%为遗传性[ATTRv]、70%为野生型[ATTRwt]、7%未明确)以及8%未明确亚型的患者。ATTR各亚组的VE/VCO斜率均升高:ATTRwt为38.4(95%CI:36.9 - 40.0,I = 57%),ATTRv为37.9(95%CI:35.1 - 40.7,I = 70%)。ATTR患者比AL患者年龄大9.0岁(95%CI:0.4 - 17.6,I = 88%),且VE/VCO斜率更高:2.5(95%CI:0.2 - 4.8,I = 0%)。CPET方式影响峰值VO,与直立式自行车测力计运动(14.7,95%CI:14.3 - 15.1,I = 33%)和半卧位自行车测力计运动(14.5,95%CI:14.1 - 14.9,I = 28%)相比,跑步机运动的峰值VO最低(13.7,95%CI:12.7 - 14.8,I = 0%,mL/min/kg)。高VE/VCO斜率是ATTRwt和ATTRv的特征,而AL患者年龄较小,VE/VCO斜率水平较低。ATTR患者的峰值VO可能取决于运动方式。