To provide additional information on clinical and echocardiographic outcomes after reimplantation of the aortic valve (RAV) in patients with aortic root aneurysm.
Methods
All 333 patients who underwent RAV at our hospital between 1989 and 2012 were followed prospectively with periodical clinical assessment and echocardiography. The mean duration of clinical follow-up was 10.3 ± 6.8 years, and follow-up was completed within 2 years before this report.
Results
The study cohort had a mean patient age was 46 ± 5 years and was 78% male. The aortic root aneurysm was associated with Marfan syndrome in 124 patients, with bicuspid aortic valve in 45, with type A aortic dissection in 28, and with moderate to severe aortic regurgitation (AR) in 144. In addition to the RAV, 113 patients underwent another cardiac procedure owing to associated pathology. There were 4 early deaths (<90 days) and 35 late deaths. Survival at 15 and 20 years was 77.9 ± 2.9% and 72.4 ± 3.8%, respectively. Eleven patients developed moderate or severe AR during the follow-up; using interval censoring, 96.2 ± 1.0% were free from this event at 15 to 20 years. Six patients underwent reoperation on the aortic valve at 2 days to 23 years after RAV, including 1 patient for endocarditis and 5 patients for AR; freedom from reoperation at 15 to 20 years was 96.9 ± 1.3%. Seventeen patients sustained stroke or transient ischemic attacks; 92.5 ± 2.8% were free from thromboembolism at 15 and 20 years. Three patients developed infective endocarditis: 1 in the aortic valve and 2 in the mitral valve.
Conclusions
RAV continues to provide excellent clinical results and stable aortic valve function during the second decade of observation. 相似文献
Introduction: Erythropoiesis-stimulating agents (ESAs) are not perfect, since they have potential side effects. Iron therapy is also receiving growing attention in recent years.
Areas covered: We performed a literature search on PubMed using the following key words: anemia, chronic kidney disease, HIF stabilisers, sotatercept, actin traps, iron, iron-containing phosphate binders, iron dialysate. We reviewed new drugs that are under clinical development to obtain better safety and activity and/or easier and cheaper manufacturing processes in comparison to available ESAs. We also considered new strategies to increase iron stores.
Several phase 1 and 2 studies support the beneficial role of increasing Hypoxia Inducible factor (HIF) activity for stimulating endogenous erythropoiesis. Sotatercept and luspatercept, two activin traps, are undergoing clinical development mainly for indications other than CKD. They have the additional effect of improving osteoporosis. Iron-containing phosphate binders have become available recently.
Expert opinion: Several medical needs are unmet with ESA. HIF stabilisers are the most appealing drugs undergoing clinical development. They expose patients to lower levels of EPO than ESA, possibly reducing unintended effects. Their long-term safety is still to be demonstrated. One new iron-containing phosphate binders has the potential of combining two indications: hyperphosphoremia and iron deficiency, possibly improving compliance. 相似文献
Objective BAG3-related myopathy is a rare condition so far reported in twenty patients worldwide.The purpose of this study was to draw attention to this rare disease and to the fact that BAG3-related myopathy should be considered as a rare differential diagnosis of hypercapnia.Methods We report a sporadic case of a 14-year-old Chinese girl with a de novo p.Pro209Leu mutation in BAG3 and reviewed the literatures for reported cases related to this mutation.Results We described a 14-year-old Chinese girl who presented with gradually appearing symptoms of hypercapnia that required assisted ventilation.The muscle biopsy and the blood whole-exome sequencing results confirmed the diagnosis of myofibrillar myopathy with a de novo p.Pro209Leu mutation in BAG3.Totally twenty-one patients from twenty families with a confirmed diagnosis of BAG3-related myopathy were reported to date,including this patient and literature review.The male to female ratio was 11 :10 and most showed initial symptoms in the first decade of life.Most patients presented toe/clumsy walking or running as the onset symptom,followed by muscle weakness or atrophy.Creatine kinase levels were elevated in fourteen patients and were normal in three.Eighteen patients developed respiratory insufficiency during the disease course and thirteen (one could not tolerate non-invasive assisted ventilation) required non-invasive assisted ventilation for treatment.Except for one not reported,heart involvement was found in seventeen patients during the disease course and seven underwent heart transplantation.Z-disk streaming and aggregation could be observed in most of the patients' muscle histology.In the long-term follow-up,five patients died of cardiac or respiratory failure.Conclusion BAG3-associated myopathy is a rare type of myofibrillar myopathy.It should be considered as a rare differential diagnosis of hypercapnia. 相似文献
Background Specific sub-groups of the population, such as older people, are at increased risk of poor vitamin D status. There is growing
evidence, however, that vitamin D insufficiency may be a more widespread problem.
Aim To determine the prevalence of vitamin D insufficiency in a group of healthy Irish adults.
Methods Serum 25-hydroxyvitamin D was measured by radioimmunoassay in 70 healthy Irish adults working in South Dublin.
Results Overall, half of the group (51%) had vitamin D insufficiency; this was more pronounced in wintertime, with prevalence rising
to 58%. Winter was a significant negative predictor of serum 25-OH D levels. Sixteen percent of the participants reported
taking a vitamin D-containing supplement (multivitamin), but this did not significantly influence the serum 25-OH D levels.
Conclusion Vitamin D insufficiency is not confined to “at-risk groups” and is likely to be prevalent in the general public in Ireland,
especially in wintertime. 相似文献