Freilinger Sebastian, Suleiman Mathieu N, Bischoff Gert, Ewert Peter, Freiberger Annika, Huntgeburth Michael, Kaemmerer Ann-Sophie, Schopen Judith, Meierhofer Christian, Nagdyman Nicole, Kaemmerer Harald, Weyand Michael, Harig Frank
Clinic for Congenital Heart Disease and Pediatric Cardiology, German Heart Centre Munich, Technical University Munich, 80636 Munich, Germany.
Department of Sport and Health Sciences, Technical University Munich, Munich, 80992 Munich, Germany.
Rev Cardiovasc Med. 2022 Jun 15;23(6):215. doi: 10.31083/j.rcm2306215. eCollection 2022 Jun.
It is clinically widely overlooked that many patients with Marfan- (MFS) or Loeys-Dietz-Syndrome (LDS) are obese. While anthropometric routine parameters are not very suitable, the modern Bioelectrical Impedance Analysis (BIA) seems superior for the acquisition of reliable noninvasive assessment of body composition of patients. The aim of the study was to assess the body composition of patients with MFS/LDS by BIA in order to detect occult obesity, which may be a risk marker for aortic or vascular complications.
In this exploratory cross-sectional study, 50 patients (66% female; mean age: 37.7 11.7 [range: 17-64] years) with a molecular genetic (n = 45; 90%) or clinical (n = 5; 10%) proven diagnosis of MFS or LDS were enrolled between June 2020 and February 2022. All BIA-measurements were performed with the Multifrequence-Impedance-Analyzer Nutriguard-MS (Data Input, Poecking, Germany).
The MFS/LDS collective was significantly different from an age-, sex-, and BMI-adjusted control in terms of body fat, percent cellularity, body cell mass, extra cellular mass/body cell mass index, and phase angle (all 0.05). The mean BIA-measured bodyfat was 31.7 8.7% [range: 9.5-53.5%], while the mean calculated BMI of the included patients was 23.0 4.8 kg/ [range: 15.2-41.9 kg/ ]. Therefore, using the obesity cut-off values for the body fat percentage of 25% in men and 35% in women, the BIA classifies as many as 28 patients (56.0%) as obese. In contrast only 12 patients (24.0%) were pre-obese, respectively 3 (6.0%) obese by BMI. The significant difference ( 0.001) had an accordance of 42.7%. Overall, 15 patients (13 MFS; 2 LDS) had previous aortic surgery (n = 14) and/or interventional treatment (n = 2) for aortic complications (aneurysm, aortic dissection). 11 out of these 15 (73.3%) were currently classified as obese by BIA.
The fact that many patients with MFS or LDS are obese is widely unknown, although obesity may be associated with impaired vascular endothelial function and an increased risk of cardiovascular complications. Also, in patients with MFS/LDS, BIA allows a reliable assessment of the body composition beyond the normal anthropometric parameters, such as BMI. In the future, BIA-data possibly may be of particular importance for the assessment of the vascular risk of MFS/LDS patients, besides the aortic diameters.
临床上普遍忽视的是,许多患有马凡综合征(MFS)或洛伊茨-迪茨综合征(LDS)的患者存在肥胖问题。虽然人体测量常规参数不太适用,但现代生物电阻抗分析(BIA)似乎更适合用于可靠地无创评估患者的身体成分。本研究的目的是通过BIA评估MFS/LDS患者的身体成分,以检测隐匿性肥胖,这可能是主动脉或血管并发症的风险标志物。
在这项探索性横断面研究中,2020年6月至2022年2月期间纳入了50例经分子遗传学(n = 45;90%)或临床(n = 5;10%)确诊的MFS或LDS患者(66%为女性;平均年龄:37.7 ± 11.7 [范围:17 - 64]岁)。所有BIA测量均使用多频阻抗分析仪Nutriguard-MS(德国波京的数据输入公司)进行。
在体脂、细胞百分比、身体细胞质量、细胞外质量/身体细胞质量指数和相位角方面,MFS/LDS患者群体与年龄、性别和BMI调整后的对照组有显著差异(均P < 0.05)。BIA测量的平均体脂为31.7 ± 8.7% [范围:9.5 - 53.5%],而纳入患者的平均计算BMI为23.0 ± 4.8 kg/m² [范围:15.2 - 41.9 kg/m²]。因此,使用男性体脂百分比25%和女性35%的肥胖临界值,BIA将多达28例患者(56.0%)分类为肥胖。相比之下,仅12例患者(24.0%)为超重前期,分别有3例(6.0%)根据BMI为肥胖。显著差异(P < 0.001)的一致性为42.7%。总体而言,15例患者(13例MFS;2例LDS)曾因主动脉并发症(动脉瘤、主动脉夹层)接受过主动脉手术(n = 14)和/或介入治疗(n = 2)。这15例患者中有11例(73.3%)目前经BIA分类为肥胖。
许多MFS或LDS患者存在肥胖这一事实广为人知,尽管肥胖可能与血管内皮功能受损和心血管并发症风险增加有关。此外,对于MFS/LDS患者,BIA能够在超出正常人体测量参数(如BMI)的情况下可靠地评估身体成分。未来,除了主动脉直径外,BIA数据可能对评估MFS/LDS患者的血管风险特别重要。