Background:High-frequency ultrasound has facilitated in vivo measurement of murine ascending aorta, allowing aortic strain to be determined from 2-D imaging. Thoracic aortic aneurysms associated with mutations infibrillin-1(FBN1) display elastin fragmentation, which may affect aortic strain. In this study, we determined the relationship between elastin fragmentation and aortic circumferential strain in wild-type (WT) and fibrillin-1 hypomorphic (FBN1^mgR/mgR) mice.

Methods and Results:Luminal diameter of the ascending aorta from WT andFBN1^mgR/mgRmice was measured in systole and diastole. Expansion of the ascending aorta during systole in male and female WT mice was 0.21±0.02 mm (16.3%) and 0.21±0.01 mm (17.0%), respectively, while expansion in male and femaleFBN1^mgR/mgRmice was 0.11±0.04 mm (4.9%) and 0.07±0.02 mm (4.5%), respectively. Reduced circumferential strain was observed inFBN1^mgR/mgRmice compared with WT littermates. Elastin fragmentation was inversely correlated to circumferential strain (R²=0.628, P=0.004) and significantly correlated with aortic diameter (systole, R²=0.397, P=0.038; diastole, R²=0.515, P=0.013).

Conclusions:FBN1^mgR/mgRmice had increased aortic diameter, reduced circumferential strain, and increased elastin fragmentation. Elastin fragmentation inFBN1^mgR/mgRand their WT littermates was correlated with reduced circumferential strain.

Background:In persistent AF, the effect of adjunctive ablation in addition to PV isolation (PVI) is controversial. We considered a new modified PVI including complex fractionated atrial electrogram (CFAE) area.

Methods and Results:In 57 patients with persistent AF undergoing first ablation, CFAE were mapped before ablation and CFAE-guided extensive encircling PVI (CFAE-guided EEPVI) was performed. The PVI line was designed to include the CFAE area near PV or to cross the minimum cycle length points of the CFAE area near PV (CFAE-guided EEPVI group). The outcome was compared with conventional PVI in 34 patients with persistent AF (conventional PVI group). During a mean follow-up of 365±230 days after the first procedure, AF in 13 and atrial tachycardia (AT) in 9 patients recurred in the CFAE-guided EEPVI group, while only AF in 17 patients recurred in the conventional PVI group. Eight of 9 AT in the CFAE-guided EEPVI group were successfully ablated at second procedure. After first and second procedures, the recurrence of atrial tachyarrhythmia in the CFAE-guided EEPVI group was significantly reduced compared with the conventional PVI group (8 patients, 14% vs. 11 patients, 32%, respectively; P<0.01, log-rank test).

Conclusions:CFAE-guided EEPVI was more effective for persistent AF compared with conventional PVI after first and second procedures, because recurring AT as well as re-conduction of PV was successfully ablated.

Background:It is commonly believed that a full moon affects human behavior or the occurrence and outcome of various diseases; thus, the occurrence of out-of-hospital cardiac arrest (OHCA) might increase during full moon nights.

Methods and Results:This nationwide, population-based observational study consecutively enrolled OHCA patients in Japan with attempted resuscitation between 2005 and 2016. The primary outcome measure was the occurrence of OHCA. Based on the double-control method, assuming Poisson sampling, we evaluated the average number of OHCA events that occurred on full moon nights compared with that which occurred on control nights, which included events that occurred on the same calendar days 1 week before and after the full moon nights. A total of 29,552 OHCA that occurred on 148 full moon nights and 58,707 OHCA that occurred on 296 control nights were eligible for analysis. The occurrence of OHCA did not differ between full moon and control nights (199.7 vs. 198.3 per night; relative risk [RR], 1.007; 95% CI: 0.993–1.021). On subgroup analysis, compared with control nights, the RR of OHCA occurrence were 1.013 (95% CI: 0.994–1.032, P=0.166) and 0.998 (95% CI: 0.977–1.020, P=0.866) for cardiac and non-cardiac origins, respectively.

Conclusions:In this population, there was no significant difference in OHCA occurrence between full moon and control nights.

Background:Evolocumab is the first monoclonal antibody against proprotein convertase subtilisin/kexin type 9 (PCSK9) approved in Japan for the treatment of patients with familial hypercholesterolemia (FH) and hypercholesterolemia (HC). This study assessed the 12-week effectiveness and safety of low-density lipoprotein cholesterol (LDL-C)-lowering therapy by PCSK9 inhibition in patients with FH (homozygous [HoFH] or heterozygous [HeFH]) and HC by analyzing evolocumab data collected in the real-world setting in Japan.

Methods and Results:Overall, 427 patients (mean±SD age, 61.6±13.8 years; female, 38.4%; 28 HoFH, 320 HeFH, 79 HC), enrolled from 299 clinical sites, were included in the safety analysis set. The major cardiovascular risk factors were coronary artery disease (77.3%), diabetes mellitus/impaired glucose tolerance (38.6%), and hypertension (65.1%). Median follow-up duration was 85.0 days. After 12 weeks of evolocumab treatment, the mean±SD percent change from baseline in LDL-C was −45.5%±27.0% (n=23) in HoFH (P<0.001 vs. baseline; t-test), −54.2%±29.0% (n=280) in HeFH (P<0.001), and −64.6%±22.4% (n=72) in HC (P<0.001) patients. The incidence of adverse drug reactions was 5.4% (23/427).

Conclusions:Results suggest that patients receiving evolocumab treatment in the real-world setting were predominantly those with FH and HC in the secondary prevention group. LDL-C-lowering effectiveness with evolocumab was observed in FH (both HoFH and HeFH) and HC patients.

Background:While hemodynamics and exercise capacity in patients with chronic thromboembolic pulmonary hypertension (CTEPH) can be improved by invasive therapy such as pulmonary endarterectomy (PEA) and balloon pulmonary angioplasty (BPA), there has been little data on the health-related quality of life (HRQOL) in such patients.

Methods and Results:This single-center and observational study compared the impact of invasive therapy on HRQOL. We utilized the Medical Outcome Study 36-Item Short Health Survey (SF-36) to measure HRQOL and compared HRQOL changes after PEA and BPA. A total of 48 patients were diagnosed with CTEPH. Of these, 39 patients completed questionnaires before and after invasive therapy. The PEA group (n=15) and the BPA group (n=24) had similar improvements in clinical parameters. With regard to HRQOL score, both groups had fairly low scores in physical functioning (PF), role physical (RP), general health (GH), social functioning (SF), role emotional (RE), and physical component summary (PCS) at baseline. PF, GH, vitality (VT), mental health (MH), and PCS had significant improvements in the PEA group while PCS and all subscales except for bodily pain (BP) had significant improvements in the BPA group. Furthermore, changes between baseline and follow-up were not significantly different between the 2 groups.

Conclusions:BPA for patients who are ineligible for PEA can recover HRQOL to a similar level to that achieved by PEA.

Background:The prevalence, patient profile, and outcomes of sleep-disordered breathing (SDB) in aortic stenosis (AS) remain unknown, especially in East Asia.

Methods and Results:One hundred and eighty-one AS patients undergoing transcatheter aortic valve implantation (TAVI) were enrolled. Sixty-one patients (33.7%) had SDB, and lower stroke volume index was an independent determinant of SDB. Incidence of in-hospital stroke after TAVI was higher in the SDB group.

Conclusions:SDB is associated with left ventricular systolic dysfunction in Japanese AS patients referred for TAVI. SDB was highly associated with the incidence of stroke as a procedural complication.