Background: 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. Methods: 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). Results: 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 p 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 ( p 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. Conclusions: 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.
Background: Pulmonary arterial hypertension (PAH), a common complication in adults with congenital heart disease (CHD), leads to significant morbidity and mortality. Targeted PAH medication is available, but PAH-CHD patient data are limited. Several questions regarding indication, treatment escalation, and combination therapy remain unanswered. The aim of this study was therefore to evaluate PAH-specific treatment in adults with PAH-CHD to better understand PAH-specific therapy management. Methods:In this cross-sectional study we retrospectively examined clinical, demographic, and cardiaccatheterization data and medical management for PAH-CHD, and analyzed clinical course and midterm outcome.Results: Over up to 14 years (median, 6.2 years), 103 PAH-CHD patients (66% female) receiving targeted PAH-therapy for pre-tricuspid-shunt (15.5%), post-tricuspid-shunt (32.0%), and complex CHD (52.4%) were followed. Based on modified clinical European Society of Cardiology (ESC) classification, patients were assigned to the following subgroups: Eisenmenger syndrome (ES) (45.6%), severe pulmonary vascular disease (PVD) in complex CHD (20.4%), post-repair patients (19.4%), prevalent systemic-to-pulmonary shunt (3.9%), coincidental/small defects (0%), and Fontan circulation (10.7%). Changes in targeted PAH therapy were observed 249 times, with up to 6 (median, 2) therapy changes over a median period of 1.3 years. Over the study course, the medical treatment strategy changed towards combination therapy (baseline, 13.6%; study-end, 41%), resulting mostly in stabilized functional class or even improvement in cases of prevalent systemic-to-pulmonary shunt, ES, and patients with repaired CHD. Functional class deterioration, however, was seen in patients with severe PVD due to complex CHD, and Fontan patients.Of the 103 patients in the study, 25 died (24.3%). Patients with repaired CHD and patients with systemicto-pulmonary shunt or ES showed the best survival rates. Mortality was remarkably higher in patients with severe PVD in complex CHD and Fontan patients.Conclusions: Many patients with PAH-CHD benefited from targeted PAH therapy over a median period of 6.2 years. Treatment decisions after targeted PAH-medication initiation were based mainly on clinical assessment. To counteract disease progression, an escalation towards combination therapy was observed during the study course. We consider survival rates under targeted PAH medication to be favorable, particularly in the ES subgroup. Nevertheless, further research is needed to optimize the use of PAH medication, especially in patients with complex CHD.
Gestational diabetes mellitus (GDM) is a common complication in pregnancy. The effect of GDM on the cardiovascular system after birth is still unclear. Between August 2015 and December 2018, 205 pregnant women were included in the prospective controlled observational study. Patients with GDM were assigned to the study group (n = 99), whereas (n = 107) healthy women served as controls. Postnatal follow up of their offspring was performed at 12 months of age. All included children (n = 125) underwent a specific standardized protocol including anthropometric data, such as weight, height, body mass index (BMI), blood pressure (BP) recordings and ultrasound measurements of the abdominal aortic intima-media-thickness (IMT). Furthermore, at least 10 min 3-channel electrocardiogram recording was done to evaluate the autonomic nervous system (ANS) by phase rectified signal averaging. There were no significant differences in anthropometric data between the groups, neither in the blood pressure nor in the intima-media-thickness of the aorta abdominals. However, in the study group, significantly lower average acceleration capacity (AAC) (study group −20.10 ± 3.04 ms, control group −18.87 ± 4.00 ms, p = 0.02) was found, indicating ANS activation at one year of age. Further studies are required to determine if these results are persistent and if these findings have long-term effects.
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