We evaluated the 80 cases of porcine valve replacement, accomplished between April 1975 and December 1980. There were 48 males and 32 females aged between 16 and 60 years old with a mean of 37.4 +/- 11.4. Ten patients underwent aortic valve replacement (AVR), 63 mitral valve (MVR), and 7 AVR plus MVR. Twenty-seven Hancock valves (H) were implanted in aortic (3) and mitral (24) position and 61 Carpentier-Edwards valves (CE) were used in aortic (14), mitral (46), and tricuspid (1) position. The mean duration of follow-up was 9.3 years (0.2-19.2 years) and cumulative follow-up was 747.2 patient years (py). The late mortality was 3.3%/py (25 patients). Actuarial survival rates for all patients at 10 and 15 years were 67%, and 49% respectively. The linearized rates (LR) of structural valve deterioration (SVD) was 7.0%/py. The freedom from SVD for all patients at 10, and 15 years were 47.1%, and 12.4% respectively. There were no differences of free rates of SVD between H and CE. between valve sites implated, and among age at operation. The incidence of occurrence of SVD was gradually increased 6 years after operation. The LR of thromboembolism (TE), anticoagulant-related hemorrhage (ACH), prosthetic valve endocarditis (PVE), nonfunctional valve dysfunction (NVD), and reoperation (RO) were 0.7%/py, 0.1%/py, 0.3%/py, 0.1%/py and 7.4%/py, respectively. The freedom from TE, ACH, PVE, NVD, and RO for all patients at 15 years were 91.3%, 98.1%, 95.6%, 80%, and 6.5%, respectively. The surgical results of glutaraldehyde-preserved porcine bioprostheses was excellent during the first 5 years after operation. A high incidence of SVD has been occurred thereafter. According the indication for valve replacement with bioprotheses is now strictly limited for elderly, child bearing female case, contraindition for therapy of anticoagulants, and some social condition.
Variants in the type IV collagen gene (COL4A1/2) cause early-onset cerebrovascular diseases. Most individuals are diagnosed postnatally, and the prenatal features of individuals with COL4A1/2 variants remain unclear.We examined COL4A1/2 in 218 individuals with suspected COL4A1/2-related brain defects. Among those arising from COL4A1/2 variants, we focused on individuals showing prenatal abnormal ultrasound findings and validated their prenatal and postnatal clinical features in detail.Pathogenic COL4A1/2 variants were detected in 56 individuals (n=56/218, 25.7%) showing porencephaly (n=29), schizencephaly (n=12) and others (n=15). Thirty-four variants occurred de novo (n=34/56, 60.7%). Foetal information was available in 47 of 56 individuals, 32 of whom (n=32/47, 68.1%) had one or more foetal abnormalities. The median gestational age at the detection of initial prenatal abnormal features was 31 weeks of gestation. Only 14 individuals had specific prenatal findings that were strongly suggestive of features associated with COL4A1/2 variants. Foetal ventriculomegaly was the most common initial feature (n=20/32, 62.5%). Posterior fossa abnormalities, including Dandy-Walker malformation, were observed prenatally in four individuals. Regarding extrabrain features, foetal growth restriction was present in 16 individuals, including eight individuals with comorbid ventriculomegaly.Prenatal observation of ventriculomegaly with comorbid foetal growth restriction should prompt a thorough ultrasound examination and COL4A1/2 gene testing should be considered when pathogenic variants are strongly suspected.
The Japanese government recommended in 2000 that women planning pregnancy should take 400 μg of folic acid daily to decrease the risk of having an infant with spina bifida. We aimed to identify risk factors for the occurrence of spina bifida and to evaluate how the prevalence rate has altered over the past 3 decades.Subjects comprised 360 women who gave birth to spina bifida-affected offspring and 2333 women who gave birth to offspring without spina bifida between 2001 and 2012. A self-administered questionnaire was used to collect data, which were analyzed by multiple logistic regression models. The prevalence rate of spina bifida was obtained through data provided by international and domestic organizations.Four variables were significantly associated with the increased risk of having newborns afflicted with spina bifida: not taking folic acid supplements (odds ratios [OR], 2.50; 95% confidence interval [CI], 1.72-3.64), presence of spina bifida patients within third-degree relatives (OR, 4.26; 95% CI, 1.12-16.19), taking anti-epileptic drugs without folic acid (OR, 20·20; 95% CI, 2.06-198.17), and low birth weight in the newborns ≤ 2500 g (OR, 4.21; 95% CI, 3.18-5.59). The prevalence rate of spina bifida has remained 5 to 6 per 10,000 total births and has not shown any decreasing trend over the past 11 years.Four risk factors were identified among Japanese women. Because recommendations and information have not decreased the occurrence of spina bifida, the Japanese government should implement mandatory food fortification.
Acute aortic dissection is a catastrophic event requiring immediate diagnosis and definitive treatment, while the clinical characteristics of closing aortic dissection are not well known. From Jan. 1991 through Dec. 1994 a total of 24 patients with closing aortic dissection (CAD) was managed at our institution. There were 19 men and 5 women, ranging in age from 49 to 74 years with a mean of 65 years. All patients presented with a sudden onset of severe chest and back pain. Twenty patients had a history of hypertension with a wide mediastinal silhouette on chest X-ray. The diagnosis of CAD was established by contrast computed tomographic (CT) scans in all patients with no evidence of patent false lumen. The descending thoracic aorta was involved in 17 patients and the ascending in 5. Conservative medical management was initially attempted for all. Seven patients (Stanford type A/B: 2/5) ultimately required surgical intervention, and 2 patients died. The follow-up was 100% complete with a mean term of 14.6 months. The overall survival rate was 74% at 3 years after the initial presentation. Based on our clinical experience, all patients with CAD need careful antihypertensive therapy and frequent follow-up studies to assess the aorta.
Study Design. A retrospective cohort study. Objective. Scoliosis and hydromyelia have frequently been observed in patients with spinal dysraphism. We investigated the applicability of curved planar reformation (CPR) for evaluating hydromyelia in patients with scoliosis associated with spinal dysraphism. Summary of Background Data. It is quite difficult to evaluate scoliosis and hydromyelia in patients with spinal dysraphism. Methods. We identified 11 patients with scoliosis and a Cobb angle of >20° among 107 spinal dysraphism patients. In addition to routine T1- and T2-weighted axial and sagittal MRI, we obtained three-dimensional constructive interference in steady-state magnetic resonance imaging (MRI) as sagittal cross-section volume images. The spinal cord and hydromyelia were rendered into a single-plane, two-dimensional image using the straightened CPR technique. In cases of scoliosis with hydromyelia, the sagittal length and maximal diameters of hydromyelia and the spinal cord were measured by three examiners. Measurement errors among examiners were evaluated using standard deviation (SD) and coefficient of variation (CV). Results. Each series of image sets provided a straightened CPR image that clearly delineated the entire length of the linearized spinal cord in a single plane. The straightened CPR image also demonstrated the accurate lengths and estimated volume of hydromyelia. Given that three of the 11 patients underwent serial MRI during this period, we were able to accurately compare volume changes. In the sagittal length of the hydromyelia, SD and CV were smaller with the straightened CPR technique than with the conventional T2 WI sagittal section in all cases, showing a statistically significant difference between both techniques (SD: P = 0.014, CV: P = 0.013). Even in the measurements, the difference in CV between both techniques was close to statistical significance. Conclusion. The straightened CPR technique is useful for accurately identifying volume changes in hydromyelia, even in patients with severe scoliosis. Level of Evidence: 1
