Possner Mathias, Alsaied Tarek, Siddiqui Saira, Morales David, Trout Andrew T, Veldtman Gruschen
Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.
Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA.
CJC Open. 2020 Jan 20;2(2):55-61. doi: 10.1016/j.cjco.2019.12.004. eCollection 2020 Mar.
Fontan palliation results in a chronic multisystem disorder with diminished exercise capacity and increased risk of muscle wasting. The aims of this study were to assess the feasibility of skeletal muscle mass measurements in Fontan patients undergoing magnetic resonance imaging liver surveillance to compare muscle mass with a historic control and to assess its correlation with cardiorespiratory fitness.
Skeletal muscle area (SMA) and skeletal muscle index (SMI) were measured at T12 and L3. A young, healthy historic cohort was used as a comparison group.
Forty patients with a Fontan circulation (mean age, 25.5 ± 7.9 years; 50% were men) were included. Measurements of SMA and SMI were feasible and highly reproducible. Mean SMA and SMI were significantly lower in women compared with men at both T12 (SMA: 25.1 ± 4.9 cm vs 33.5 ± 8.4 cm, 0.001; SMI: 9.7 ± 2.1 cm/m vs 11.3 ± 2.7 cm/m, 0.045) and L3 (SMA: 121 ± 12 cm vs 162 ± 24 cm, 0.001; SMI: 46.9 ± 7.0 cm/m vs 54.5 ± 7.4 cm/m, 0.002). Mean SMI at L3 was significantly lower in the male Fontan population compared with the healthy historic cohort (54.5 ± 7.4 cm/m vs 60.9 ± 7.8 cm/m, 0.001), but was similar for women (46.9 ± 7.0 cm/m vs 47.5 ± 6.6 cm/m, 0.692). SMI at L3, but not at T12, was positively correlated with peak oxygen consumption, oxygen pulse, and workload. Four patients (10%) met criteria for muscle wasting in the sarcopenic range based on L3 measurements.
Abdominal skeletal muscle mass can be reproducibly determined on surveillance liver magnetic resonance imaging scans. Muscle wasting appears to occur commonly in Fontan patients. Further research is needed to better define the value of SMI as a biomarker in the Fontan population.
Fontan姑息术会导致慢性多系统疾病,运动能力下降,肌肉萎缩风险增加。本研究的目的是评估在接受肝脏磁共振成像监测的Fontan患者中测量骨骼肌质量的可行性,将肌肉质量与历史对照组进行比较,并评估其与心肺适能的相关性。
在T12和L3水平测量骨骼肌面积(SMA)和骨骼肌指数(SMI)。将一个年轻、健康的历史队列用作比较组。
纳入了40例Fontan循环患者(平均年龄25.5±7.9岁;50%为男性)。SMA和SMI的测量是可行的且具有高度可重复性。在T12和L3水平,女性的平均SMA和SMI均显著低于男性(T12:SMA:25.1±4.9cm对33.5±8.4cm,P<0.001;SMI:9.7±2.1cm/m对11.3±2.7cm/m,P=0.045;L3:SMA:121±12cm对162±24cm,P<0.001;SMI:46.9±7.0cm/m对54.5±7.4cm/m,P=0.002)。与健康历史队列相比,Fontan男性人群L3水平的平均SMI显著降低(54.5±7.4cm/m对60.9±7.8cm/m,P=0.001),但女性相似(46.9±7.0cm/m对47.5±6.6cm/m,P= 0.692)。L3水平而非T12水平的SMI与峰值耗氧量、氧脉搏和工作量呈正相关。根据L3测量结果,4例患者(10%)符合肌肉减少症范围内的肌肉萎缩标准。
腹部骨骼肌质量可在肝脏监测磁共振成像扫描中重复测定。肌肉萎缩似乎在Fontan患者中普遍存在。需要进一步研究以更好地确定SMI作为Fontan人群生物标志物的价值。