Although not all newborns with Ebstein's anomaly present with severe cardiomegaly in utero, some of them cannot live after birth because of the lung hypoplasia. To clarify the relationship between the intrauterine cardiomegaly and the outcome of the patients with Ebstein's anomaly or tricuspid valve dysplasia, we calculated the ratio of the area of the heart against the thorax (CTAR) in the transverse view of the thorax at the level of the cardiac four‐chamber view in the fetal echocardiogram and compared it to the outcome. The study population consisted of four patients with Ebstein's anomaly and one patient with tricuspid valve dysplasia who were diagnosed in utero. The ranges obtained from 53 normal fetal cases were 20%± 8% (mean ± 2 SD) at below 20 week's gestation, 25%± 10% at the gestational age of 21 to 30 weeks, and 29%± 6.4% at the gestational age of 31 to 40 weeks. The CTARs of these five cases measured 81.6%, 51%, 55.2%, 47.5%, and 75.6%, respectively, and were abnormally higher than the normal value. Two fetuses died in utero with severe hydrops fetalis. Two fetuses whose cardiothoracic ratios by chest X ray were 100% died at twelve hours of life. One patient died at 110 days. The cross‐sectional area of the thorax was smaller than the normal range in 3 (cases 1, 4, and 5) out of these 5 cases. Thus, we conclude that fetal Ebstein's anomaly and tricuspid valve dysplasia associated with massive tricuspid regurgitation with a large CTAR ratio (higher than 50%) and small thoracic cross‐sectional area has a very poor prognosis bothprena‐tally and neonatally. (ECHOCARDIOGRAPHY, Volume 11, May 1994)
The preventive effect of estrogen on Alzheimer's disease (AD) has become clearer with many epidemiological reports. However, the therapeutic effects of estrogen have been controversial until now. In our trials, estrogen treatment showed a beneficial therapeutic effect for women with mild to moderate AD. Improvement of cognitive function was recognized during the third week from the beginning of administration and maintained as long as estrogen treatment continued. The longer the duration of HRT, the more HRT is useful for the prevention and therapy of AD. However, in most cases, administration of estrogen is discontinued because of the adverse effects on the uterus and breast. J 861 is a derivative of estradiol-17alpha, which has little effect on the sexual organs. The effects of estradiol-17beta (E2) and J 861 on neuronal function and vascular factors were investigated. J 861 was suggested to prevent both the intracellular calcium increase and peroxidation induced by amyloid beta (Abeta), more effectively than E2. The effect of J 861 may be related with both the direct non-genomic and the ER-mediated systems. J 861 showed neurotrophic effects like E2. J 861 inhibited the adhesion of monocytes to vascular endothelium, more effectively than E2. Also, J 861 suppressed the expression of adhesive factors, such as E-selectin and intercellular cell adhesion molecule-1 (ICAM-1), more effectively than E2.
It has been reported that most patients with untreated tetralogy of Fallot (TOF) die by the time they reach adulthood. We report the case of a 72-year-old female diagnosed by echocardiography and cardiac cathetherization as having TOF and diagnosed at birth with a ventricular septal defect (VSD). During childhood, she was very thin and lacking in physical strength. On first consultation at our hospital, she was suffering from mild dyspnea, classified as NYHA functional class III, and her fingers were clubbed and cyanotic. Her PaO2 was 48.0 mmHg under room air, and hypoxia was recognized. An echocardiography and cardiac cathetherization showed a VSD, hypertrophy of the right ventricle, over-riding of the aorta and stenosis of the right ventricular outflow tract with a pressure gradient of 84 mmHg. There was a bidirectional shunt with 24% flow from the left to right and 43% from the right to left ventricle. Her Qp/Qs was 0.75. Surgical treatment was recommended. However, the patient refused, because her symptoms were alleviated with home oxygen therapy. This report shows the prolonged survival of this 72-year-old female with untreated TOF.
There are regional differences in the patient characteristics, management, and outcomes of hospitalized patients with heart failure (HF). The aim of this study was to evaluate the clinical characteristics and outcomes of Japanese patients who are hospitalized with HF on the basis of the left ventricular ejection fraction (LVEF) stratum.We retrospectively conducted a multicentre cohort study of 1245 hospitalized patients with decompensated HF between 2013 and 2014. Of these patients, 36% had an LVEF < 40% [HF with reduced ejection fraction (HFrEF), median age 72 years, 71% male], 21% had an LVEF 40-49% [HF with mid-range EF (HFmrEF), 77 years, 56% male], and 43% had an LVEF ≥ 50% [HF with preserved EF (HFpEF), 81 years, 44% male]. The primary outcome was death from any cause, and the secondary outcomes were cardiac death and re-hospitalization due to worsened HF after hospital discharge. There were high proportions of non-ischaemic cardiomyopathy (32%) in HFrEF patients, coronary artery disease (44%) in HFmrEF patients, and valvular disease (39%) in HFpEF patients. The frequencies of intravenous diuretic and natriuretic peptide administration during hospitalization were 66% and 30%, respectively. The median hospital stay for the overall population was 19 days, and the length of stay was >7 days for >90% of patients. In-hospital mortality was 7%, but was not different among the LVEF groups (HFrEF 7%, HFmrEF 6%, and HFpEF 8%). After a median follow-up of 19 months (range, 3-26 months), 192 (17%) of the 1156 patients who were discharged alive died, and 534 (46%) were re-hospitalized after hospital discharge. There were no significant differences in mortality after hospital discharge among the three LVEF groups (HFrEF 18%, HFmrEF 16%, and HFpEF 16%). There were no differences in cardiac death or re-hospitalization due to worsened HF after hospital discharge among the LVEF groups (cardiac death: HFrEF 8%, HFmrEF 7%, and HFpEF 7%; re-hospitalization due to worsened HF: HFrEF 19%, HFmrEF 16%, and HFpEF 17%). Multivariable-adjusted analyses showed that the HFmrEF and HFrEF groups, compared with the HFpEF group, were not associated with an increased risk for in-hospital death or death after hospital discharge. Non-cardiac causes of death and re-hospitalization after hospital discharge accounted for 35% and 38%, respectively.Our results revealed different clinical characteristics but similar mortality rates in the HFrEF, HFmrEF, and HFpEF groups. The most common cause of death and re-hospitalization after hospital discharge was HF, but non-cardiac causes also contributed to their prognosis. Integrated management approaches will be required for HF patients.
A simulation approach by "Package Flow Model (PFM)" was previously proposed, which enables us to intuitively understand the dynamic behavior of various systems. The model does not directly consider the physical process of given actual system, but will replace it a simple visual mechanism (PFM) which is equivalent only in a view point of "time delay" of the system-response. A total system such as a nuclear reactor fluid system is modeled by a combination of several PFMs. Using the PFMs network, we can instantly calculate many transient phenomena of the system even by a notebook-type personal computer. Experts' intuition or experiences can be enhanced by using it with effective representation methods. In addition, PFM method has a capability to develop into an more effective simulation method of total system by including Lattice Gas Methods applied to its subsystems because the calculation processes are in common, i.e., neural networks.
