A 9-year-old boy was first noted to have a heart murmur on the 7th postnatal day. Cardiac catheterization at the age of 4 months showed combined valvular and supravalvular aortic stenosis, bicuspid aortic valve and hypoplastic aortic annulus. Emergency open aortic valvotomy was performed. At the age of 6 years, he had infectious endocarditis which was treated medically. Echo-cardiography at this time showed a 90mmHg pressure gradient across the aortic valve. In August 1992 and in March 1993, ballon valvuloplasties were done but without a significant reduction in the pressure gradient. In July 1993, when the patient was 7 years old, repeated surgical valvotomy and Doty's aortoplasty were performed. Two years later the boy had exertional dyspnea, and a cardiac catheterization showed pulmonary artery pressure of 60/27mmHg, and a 110mmHg pressure gradient across the aortic value. In July 1995, he underwent aortic valve replacement with Konno's aortoventriculoplasty, and a SJM 19mm AHP valve was implanted. One year after surgery, he is without any symptoms. Although the end result was acceptable, earlier AVR with Konno procedure may have spared the child from one extra surgical procedure. Strategic options in the surgical therapy of this condition are discussed.

The patient was a 73-year-old man who was referred to our hospital due to an abnormal thoracic shadow. CT scans revealed Kommerell's diverticulum and saccular aortic arch aneurysm accompanied by abnormal origins of the right aortic arch and the left subclavian artery. Although there were no subjective symptoms, a surgical operation was planned considering the risk of a rupture of the saccular aneurysm. For the surgery, a median sternotomy approach was employed. Under cardiopulmonary bypass, the aortic arch was detached using the open distal method. Further, an open stent graft was inserted, and the aortic arch was replaced with a four-branched artificial blood vessel. After weaning off the cardiopulmonary bypass, coil embolization was performed on the left subclavian artery, and the site was checked to ensure that there was no endoleak. Although hoarseness was noted postoperatively due to paralysis of the right vocal cord, the patient progressed without any other major complications and was discharged 30 days after the operation.

The case patient was a 61-year-old man who fell while working on the back of a truck and bruised his abdomen. Immediately thereafter, the patient started experiencing lumbar pain and weakness in both lower limbs. He was then transported to our hospital by ambulance. Based on the abdominal CT findings, he was diagnosed with acute lower limb ischemia due to a ruptured abdominal aortic aneurysm. The patient underwent graft replacement surgery within 3.5 hours after the onset of the rupture. The patient had no adverse findings, such as ischemia-reperfusion injury and compartment syndrome, after resumption of blood flow (6.5 hours after the onset) and both lower limbs were well perfused. Although there was mild muscle weakness and numbness in the distal left lower limb, the patient was discharged 9 days after surgery.

BACKGROUND: The burden of dementia is growing rapidly and has become a medical and social problem in Japan. Prospective cohort studies have been considered an effective methodology to clarify the risk factors and the etiology of dementia. We aimed to perform a large-scale dementia cohort study to elucidate environmental and genetic risk factors for dementia, as well as their interaction. METHODS: The Japan Prospective Studies Collaboration for Aging and Dementia (JPSC-AD) is a multisite, population-based prospective cohort study of dementia, which was designed to enroll approximately 10,000 community-dwelling residents aged 65 years or older from 8 sites in Japan and to follow them up prospectively for at least 5 years. Baseline exposure data, including lifestyles, medical information, diets, physical activities, blood pressure, cognitive function, blood test, brain magnetic resonance imaging (MRI), and DNA samples, were collected with a pre-specified protocol and standardized measurement methods. The primary outcome was the development of dementia and its subtypes. The diagnosis of dementia was adjudicated by an endpoint adjudication committee using standard criteria and clinical information according to the Diagnostic and Statistical Manual of Mental Disorders, 3rd Revised Edition. For brain MRI, three-dimensional acquisition of T1-weighted images was performed. Individual participant data were pooled for data analyses. RESULTS: The baseline survey was conducted from 2016 to 2018. The follow-up surveys are ongoing. A total of 11,410 individuals aged 65 years or older participated in the study. The mean age was 74.4 years, and 41.9% were male. The prevalence of dementia at baseline was 8.5% in overall participants. However, it was 16.4% among three sites where additional home visit and/or nursing home visit surveys were performed. Approximately two-thirds of dementia cases at baseline were Alzheimer's disease. CONCLUSIONS The prospective cohort data from the JPSC-AD will provide valuable insights regarding the risk factors and etiology of dementia as well as for the development of predictive models and diagnostic markers for the future onset of dementia. The findings of this study will improve our understanding of dementia and provide helpful information to establish effective preventive strategies for dementia in Japan.
Aged ; Alzheimer Disease/genetics* ; Dementia/genetics* ; Environment ; Female ; Humans ; Incidence ; Japan/epidemiology* ; Male ; Middle Aged ; Prevalence ; Prospective Studies ; Risk Factors