Assessment of body composition, metabolism, and pulmonary function in patients with myotonic dystrophy type 1.

Abnormal body composition in myotonic dystrophy type 1 (DM1) are affected by energy intake above resting energy expenditure (REE). We aim to investigate the characteristics and relationship between body composition, REE, and pulmonary function in patients with DM1, and to examine their changes in 1 year. The study design was a single-center, cross-sectional, and longitudinal study of body composition, REE characteristics, and pulmonary function. Twenty-one male patients with DM1 and 16 healthy volunteers were registered in the study. Body composition was measured using dual-energy X-ray absorptiometry (DEXA). Fat mass (FM) index (kg/m2), fat-FM index (kg/m2), and skeletal mass index (kg/m2) were calculated. The measurements were taken breath by breath with a portable indirect calorimeter. The REE was calculated using the oxygen intake (VO2) and carbon dioxide output (VCO2) in the Weir equation. Basal energy expenditure (BEE) was calculated by substituting height, weight, and age into the Harris-Benedict equation. The study enrolled male patients with DM1 (n = 12) and healthy male volunteers (n = 16). Patients with DM1 (n = 7) and healthy volunteers (n = 14) could be followed in 1 year. The body composition of patients with DM1 was significantly higher in the FM index and significantly lower in the fat-FM index and skeletal mass index. The REE of patients with DM1 was significantly lower and was not associated with body composition. Patients with DM1 had poor metabolism that was not related to body composition. FM was high and lean body mass was low.

Public assistance in patients with acute heart failure: a report from the KCHF registry.

AIMS: There is a scarcity of data on the post-discharge prognosis in acute heart failure (AHF) patients with a low-income but receiving public assistance. The study sought to evaluate the differences in the clinical characteristics and outcomes between AHF patients receiving public assistance and those not receiving public assistance. METHODS AND RESULTS: The Kyoto Congestive Heart Failure registry was a physician-initiated, prospective, observational, multicentre cohort study enrolling 4056 consecutive patients who were hospitalized due to AHF for the first time between October 2014 and March 2016. The present study population consisted of 3728 patients who were discharged alive from the index AHF hospitalization. We divided the patients into two groups, those receiving public assistance and those not receiving public assistance. After assessing the proportional hazard assumption of public assistance as a variable, we constructed multivariable Cox proportional hazard models to estimate the risk of the public assistance group relative to the no public assistance group. There were 218 patients (5.8%) receiving public assistance and 3510 (94%) not receiving public assistance. Patients in the public assistance group were younger, more frequently had chronic coronary artery disease, previous heart failure hospitalizations, current smoking, poor medical adherence, living alone, no occupation, and a lower left ventricular ejection fraction than those in the no public assistance group. During a median follow-up of 470 days, the cumulative 1 year incidences of all-cause death and heart failure hospitalizations after discharge did not differ between the public assistance group and no public assistance group (13.3% vs. 17.4%, P = 0.10, and 28.3% vs. 23.8%, P = 0.25, respectively). After adjusting for the confounders, the risk of the public assistance group relative to the no public assistance group remained insignificant for all-cause death [hazard ratio (HR), 0.97; 95% confidence interval (CI), 0.69-1.32; P = 0.84]. Even after taking into account the competing risk of all-cause death, the adjusted risk within 180 days in the public assistance group relative to the no public assistance group remained insignificant for heart failure hospitalizations (HR, 0.93; 95% CI, 0.64-1.34; P = 0.69), while the adjusted risk beyond 180 days was significant (HR, 1.56; 95% CI, 1.07-2.29; P = 0.02). CONCLUSIONS: The AHF patients receiving public assistance as compared with those not receiving public assistance had no significant excess risk for all-cause death at 1 year after discharge or a heart failure hospitalization within 180 days after discharge, while they did have a significant excess risk for heart failure hospitalizations beyond 180 days after discharge. CLINICAL TRIAL REGISTRATION: https://clinicaltrials.gov/ct2/show/NCT02334891 (NCT02334891) and https://upload.umin.ac.jp/cgi-open-bin/ctr_e/ctr_view.cgi?recptno=R000017241 (UMIN000015238).

Clinical characteristics and outcomes of Japanese women undergoing coronary revascularization therapy.

BACKGROUND: Limited data are available for gender-based differences in patients undergoing coronary revascularization. This study aimed to identify gender-based differences in risk factor profiles and outcomes among Japanese patients undergoing coronary revascularization. METHODS AND RESULTS: The subjects consisted of 2,845 women and 6,843 men who underwent first percutaneous coronary intervention or coronary artery bypass grafting in 2000-2002. The outcome measures were all-cause death, major adverse cardiovascular events (MACE) as the composite of cardiovascular death, myocardial infarction and stroke, and any coronary revascularization. The females were older than the males and more frequently had histories of heart failure, diabetes, hypertension, chronic kidney disease, anemia, and dyslipidemia. Unadjusted survival analysis revealed a significantly lower incidence of any revascularization in women (at 3 years: 28.2% vs. 31.2%, P = 0.0037), although no significant gender-based differences were shown in the incidence of all-cause death (at 3 years: 8.8% vs. 8.5%, P = 0.37) or MACE (at 3 years: 12.0% vs. 11.5%, P = 0.61). Multivariate analysis revealed that female gender was associated with significantly lower risks of any revascularization (relative risk = 0.93, 95% confidence interval [CI] = 0.88-0.99, P = 0.014) and all-cause death (relative risk = 0.86, 95%CI = 0.77-0.96, P = 0.005). CONCLUSIONS: In Japanese patients undergoing first coronary revascularization, the coronary risk factor burden appeared greater in women than in men. Despite the greater modifiable risk factor accumulation, female gender was associated with a lower incidence of repeated revascularization relative to male gender.

Inter- and intra-observer variability for assessment of the synergy between percutaneous coronary intervention with TAXUS and cardiac surgery (SYNTAX) score and association of the SYNTAX score with clinical outcome in patients undergoing unprotected left main stenting in the real world.

BACKGROUND: The Synergy Between Percutaneous Coronary Intervention With TAXUS and Cardiac Surgery (SYNTAX) score was proposed as a method to evaluate the complexity of coronary anatomy. However, the reproducibility of assessment for the SYNTAX score in unprotected left main coronary artery (ULMCA) disease has not yet been adequately evaluated. The purpose of this study is to assess inter- and intra-observer variability for the assessment of the SYNTAX score in patients undergoing ULMCA stenting in daily clinical practice. METHODS AND RESULTS: The SYNTAX score of 101 consecutive patients who underwent ULMCA stenting with sirolimus-eluting stent was independently assessed by 2 experienced interventional cardiologists. One of the 2 cardiologists evaluated all the cases again 6 months after the initial assessment. The κ value for inter-observer variability in estimating the SYNTAX score was 0.62 according to the dichotomized analysis (≥ 33, < 33) and 0.58 according to the tertile analysis (< 23, 23 ≤ - < 33, ≥ 33), while the intra-observer variability was 0.78 and 0.69, respectively. Patients with a high SYNTAX score (≥ 33, n = 55) compared with those with low or intermediate score (< 33, n = 46) had a significantly higher rate of target-lesion revascularization (TLR) of the ULMCA lesion at 2 years (24% vs. 4.4%, P = 0.01). CONCLUSIONS: Both inter- and intra-observer variability for estimating the SYNTAX score were within an acceptable range and a high SYNTAX score showed a higher rate of TLR in patients undergoing ULMCA stenting in daily clinical practice.