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转甲状腺素蛋白心脏淀粉样变患者右心室与肺动脉解偶联与运动能力受损有关。

Right Ventricular to Pulmonary Artery Uncoupling Is Associated With Impaired Exercise Capacity in Patients With Transthyretin Cardiac Amyloidosis.

作者信息

Hashimoto Toru, Ikuta Kei, Yamamoto Shoei, Yoshitake Tomoaki, Suenaga Tomoyasu, Nakashima Shunsuke, Kai Takashi, Misumi Kayo, Fujino Takeo, Shinohara Keisuke, Matsushima Shouji, Atsumi Rina, Isoda Takuro, Kinugawa Shintaro, Abe Kohtaro

机构信息

Department of Cardiovascular Medicine, Faculty of Medical Sciences, Kyushu University.

Department of Advanced Cardiopulmonary Failure, Faculty of Medical Sciences, Kyushu University.

出版信息

Circ J. 2024 Dec 25;89(1):31-40. doi: 10.1253/circj.CJ-24-0402. Epub 2024 Sep 28.

DOI:10.1253/circj.CJ-24-0402
PMID:39343601
Abstract

BACKGROUND

Exercise capacity is related to mortality and morbidity in heart failure (HF) patients. Determinants of exercise capacity in transthyretin cardiac amyloidosis (ATTR-CA) have not been established.

METHODS AND RESULTS

This single-center study retrospectively evaluated ATTR-CA patients and patients with non-amyloidosis HF with preserved/mildly reduced ejection fraction (HFpEF/HFmrEF) (n=32 and n=51, respectively). In the ATTR-CA group, the median age was 75.5 years (interquartile range [IQR] 71.3-78.8 years), 90.6% were male, and the median left ventricular (LV) ejection fraction was 53.5% (IQR 41.4-65.6%). Cardiopulmonary exercise tests revealed a median peak oxygen consumption and anaerobic threshold of 15.9 (IQR 11.6-17.4) and 10.6 (IQR 8.5-12.0] mL/min/kg, respectively, and ventilatory efficiency (minute ventilation/carbon dioxide production [V̇/V̇] slope) of 35.5 (IQR 32.0-42.5). Among exercise variables, V̇/V̇slope has the greatest prognostic value. Univariate analysis revealed a significant correlation between V̇/V̇slope and age, LV global longitudinal strain, tricuspid annular plain systolic excursion/pulmonary arterial systolic pressure (TAPSE/PASP) ratio, and mixed venous oxygen saturation. In multivariate analyses, the TAPSE/PASP ratio was an independent predictor of V̇/V̇slope (95% confidence interval -44.5, -10.8; P=0.0067). In non-amyloidosis HFpEF/HFmrEF patients, the TAPSE/PASP ratio was not independently correlated with V̇/V̇slope.

CONCLUSIONS

Right ventricular-pulmonary artery coupling estimated by the TAPSE/PASP ratio determines exercise capacity in ATTR-CA patients. This highlights the importance of early therapeutic intervention against underappreciated right ventricular dysfunction associated with ATTR-CA.

摘要

背景

运动能力与心力衰竭(HF)患者的死亡率和发病率相关。转甲状腺素蛋白心脏淀粉样变性(ATTR-CA)患者运动能力的决定因素尚未明确。

方法和结果

这项单中心研究回顾性评估了ATTR-CA患者以及射血分数保留/轻度降低的非淀粉样变性HF患者(HFpEF/HFmrEF)(分别为32例和51例)。在ATTR-CA组中,中位年龄为75.5岁(四分位间距[IQR]71.3 - 78.8岁),90.6%为男性,左心室(LV)射血分数中位数为53.5%(IQR 41.4 - 65.6%)。心肺运动试验显示,峰值摄氧量和无氧阈值中位数分别为15.9(IQR 11.6 - 17.4)和10.6(IQR 8.5 - 12.0)mL/min/kg,通气效率(分钟通气量/二氧化碳产生量[V̇/V̇]斜率)为35.5(IQR 32.0 - 42.5)。在运动变量中,V̇/V̇斜率具有最大的预后价值。单因素分析显示,V̇/V̇斜率与年龄、左心室整体纵向应变、三尖瓣环平面收缩期位移/肺动脉收缩压(TAPSE/PASP)比值以及混合静脉血氧饱和度之间存在显著相关性。在多因素分析中,TAPSE/PASP比值是V̇/V̇斜率的独立预测因素(95%置信区间 -44.5,-10.8;P = 0.0067)。在非淀粉样变性HFpEF/HFmrEF患者中,TAPSE/PASP比值与V̇/V̇斜率无独立相关性。

结论

通过TAPSE/PASP比值估算的右心室 - 肺动脉耦合决定了ATTR-CA患者的运动能力。这凸显了针对与ATTR-CA相关的未被充分认识的右心室功能障碍进行早期治疗干预的重要性。

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