The relation of aortic dimensions and obesity in adults with Marfan or Loeys-Dietz syndrome

Abstract

Background: Aortic aneurysm and aortic dissection can have a major impact on the life expectancy of Marfan syndrome (MFS) or Loeys-Dietz syndrome (LDS) patients. Although obesity can influence the development of aortic complications, evidence on whether obesity influences the development of aortic aneurysm or dissection in MFS and LDS is limited. The aim of the present study was to elucidate the relationship between aortic size and body composition, assessed by modern bioelectrical impedance analysis (BIA) in MFS/LDS-patients. Methods: In this exploratory cross-sectional study in MFS or LDS patients, enrolled between June 2020 and May 2022, 34 patients received modern BIA and magnetic resonance imaging (MRI) (n=32) or computed tomography (CT) imaging (n=2) of the entire aorta. A P value of <0.05 was considered significant.Results: Fifty-one patients (66% female; mean age: 37.7±11.7; range, 17-68 years) with MFS or LDS were enrolled; 34 patients, 27 with MFS and 7 with LDS, underwent aortic MRI or CT scanning. The mean aortic length was 503.7±58.7 mm, and the mean thoracic aortic length and abdominal aortic length were 351.5±52.4 and 152.2±27.4 mm, respectively. The aortic bulb and the ascending aorta were measured only in the non-surgically repaired patients. Fifteen MFS (88.2%) and two LDS (40.0%) patients had an aortic aneurysm. In these, the aortic bulb tended to be larger in MFS than in LDS patients [42.6×41.9×41.2 vs. 37.8×37.4×36.8 mm; P=0.07 (−1.1; 9.1); P=0.07 (−1.2; 8.4); P=0.07 (−1.5; 7.9)]. BIA revealed mean body fat levels of 31.6%±8.7% (range, 9.5-53.5%), indicating that 18 patients (52.9%) were obese. There was a significant correlation between body fat content and thoracic aortic length (R=−0.377; P=0.02), muscle mass and total aortic length (R=0.359; P=0.03), thoracic aortic length (R=0.399; P=0.02), extracellular mass (ECM),

Editorial: Aortopathy in congenital heart disease

“…Among CoA patients, e.g., the risk for acquired cardiovascular risk factors appears particularly high ( 21 , 22 ). Moreover, in MFS the body mass index may not be a good predictor of overweight and obesity as the relative muscle mass is decreased and the proportion of fat is increased ( 23 ). Additionally, overweight or obese MFS patients may be at increased risk for adverse aortic events ( 24 ).…”

Continuous Long-Term Assessment of Heart Rate Variability in Adults with Cyanotic Congenital Heart Disease after Surgical Repair

“…A wide range of different examination methods is required to record and assess the above-mentioned residua and comorbidities, including imaging techniques such as echocardiography, X-ray, magnetic resonance imaging (MRI), and computed tomography (CT). Electrocardiogram (ECG), long-term ECG, telemetry, and cardiopulmonary exercise tests are also of particular importance [9,10]. Another technique is continuous heart rate variability (HRV) measurement [11,12].…”