Atrial Septal Defect in Adults: Thirty-eight-year Follow-up of a Surgically and a Conservatively Managed Group

This report describes the long-term (up to 38 years) outcome, in terms of mortality and cardiovascular morbidity, in a non-randomized clinical observation study of a surgically and a conservatively managed group of adult patients with shunt lesions at atrial level. Haemodynamic status was assessed at baseline and at repeat examination. Twelve patients underwent surgical repair of the lesion between these two heart catheterizations, resulting in a marked reduction in heart size and right ventricular systolic pressure. These changes were associated with improvement in functional class and a trend towards less risk of cerebrovascular incidents, but not atrial fibrillation, during follow-up. Among 12 patients not operated on between catheterizations, symptomatic deterioration was common, often necessitating later surgical repair. The results support the assumption that early surgery should be recommended for adults with a haemodynamically significant lesion, to reduce the risk of mortality and prevent symptomatic deterioration.     

Growth hormone deficiency predicts cardiovascular risk in young adults treated for acute lymphoblastic leukemia in childhood

Acute lymphoblastic leukemia (ALL) is the most common childhood malignancy, and until recently prophylactic cranial radiotherapy (CRT) was important for achieving long-term survival. Hypothalamic-pituitary hormone insufficiency is a well-recognized consequence of CRT for childhood cancer. Another problem is increased cardiovascular risk, which has been shown in long-term survivors of other childhood cancers. In the only previously reported study on cardiovascular risk after childhood ALL, obesity and dyslipidemia were recorded in a small subgroup treated with CRT, compared with patients treated with chemotherapy. The mechanisms behind the increase in cardiovascular risk in survivors of childhood cancer are not clarified.The aim of the present study was to elucidate mechanisms of increased cardiovascular risk in former childhood ALL patients. A group of 44 ALL survivors (23 males, median age 25 yr, range 19-32 yr at the time of study) treated with CRT (median 24 Gy, 18-30 Gy) at a median age of 5 yr (1-18 yr) and chemotherapy were investigated for prevalence of GH deficiency and cardiovascular risk factors. Comparison was made with controls randomly selected from the general population and individually matched for sex, age, smoking habits, and residence. All patients and controls underwent a GHRH-arginine test, and patients with a peak GH 3.9 microg/liter or greater were further investigated with an additional insulin tolerance test.Significantly higher plasma levels of insulin (P = 0.002), blood glucose (P = 0.01), and serum levels of low-density lipoprotein cholesterol, apolipoprotein (Apo) B, triglycerides, fibrinogen, and leptin (all P 相似文献   

Mitroflow pericardial bioprosthesis in the aortic position. Low incidence of structural valve deterioration in elderly patients during an 11-year follow-up

Aortic valve replacement with Mitroflow pericardial bioprosthesis, with or without concomitant bypass surgery was performed on 403 consecutive patients between March 1984 and December 1994. The mean age was 74 (range 10-92) years, with male/female ratio 183/220. Early mortality was 3.5% (14/403) and late mortality 25% (99/389). Actuarial survival was 35.4%+/-9.4% at 10 years and freedom from valve-related mortality 86.2%+/-9.6% at 8 years (total follow-up 1 270 years). The thromboembolic rate was 6.5% (fatal 0.7%)/patient year. The incidence of prosthetic valve endocarditis was 0.6% (fatal 0.3%)/patient year. Structural valve deterioration was found in 0.8%/patient year (no fatality). Actuarial freedom from all reoperations at 8 years was 92.4+/-8%. The Mitroflow pericardial heart valve is proposed as a good choice for aortic valve replacement in elderly patients, in view of the low rates of reoperation, anticoagulant treatment and valve-related thromboembolism.     

Atrial septal defect in adults. Thirty-eight-year follow-up of a surgically and a conservatively managed group

This report describes the long-term (up to 38 years) outcome, in terms of mortality and cardiovascular morbidity, in a non-randomized clinical observation study of a surgically and a conservatively managed group of adult patients with shunt lesions at atrial level. Haemodynamic status was assessed at baseline and at repeat examination. Twelve patients underwent surgical repair of the lesion between these two heart catheterizations, resulting in a marked reduction in heart size and right ventricular systolic pressure. These changes were associated with improvement in functional class and a trend towards less risk of cerebrovascular incidents, but not atrial fibrillation, during follow-up. Among 12 patients not operated on between catheterizations, symptomatic deterioration was common, often necessitating later surgical repair. The results support the assumption that early surgery should be recommended for adults with a haemodynamically significant lesion, to reduce the risk of mortality and prevent symptomatic deterioration.     

Cuff electrode implantation around the sciatic nerve is associated with an upregulation of TNF-alpha and TGF-beta 1

Epineurial fibrosis, fiber loss, limited reproducibility of recordings and variability of stimulation conditions have been documented after extraneural cuff electrode implantation. These morphological and electrophysiological modifications could be due to the local release of cytokines. We report the expression of two cytokines, tumor necrosis factor-alpha (TNF-alpha) and transforming growth factor-beta1 (TGF-beta1) in the rat sciatic nerve after 'cuff' implantation for 18 h, 7 days and 1 month. Immunohistochemical and Western blot analyses showed a transient upregulation of TNF-alpha, during the first week, and a prolonged increase of TGF-beta1, over the 1-month period duration of this study. Considering the known pro-inflammatory roles of TNF-alpha and the pro-fibrotic action of TGF-beta, our results strongly suggest that these cytokines may contribute to nerve alterations occurring within the acute and sub-acute phases after cuff electrode implantation.     

Slower Skeletal Muscle Oxygenation Kinetics in Adults With Complex Congenital Heart Disease

BackgroundAdults with complex congenital heart disease (CHD) show reduced aerobic exercise capacity and impaired skeletal muscle function compared with healthy peers. Peripheral muscle factors are presumed to be important contributors to the aerobic capacity, but the mechanisms are poorly understood. The aim of the present study was to investigate differences between adults with CHD and controls in muscle oxygenation kinetics at rest, and during and after exercise.MethodsSeventy-four patients with complex CHD (mean age 35.6 ± 14.3 years, female n = 22) were recruited. Seventy-four age- and sex-matched subjects were recruited as controls. Muscle oxygenation was successfully determined on the anterior portion of the deltoid muscle using near-infrared spectroscopy in 65 patients and 71 controls. Measurements were made at rest, during isotonic shoulder flexions (0-90°) to exhaustion, and during recovery.ResultsThe patients with CHD performed fewer shoulder flexions (40 ± 17 vs 69 ± 40; P < 0.001), had lower muscle oxygen saturation (StO₂) at rest (58 ± 18% vs 69 ± 18%; P < 0.001), slower desaturation rate at exercise onset (?9.7 ± 5.9 vs ?15.1 ± 6.5% StO₂ × 3.5 s^?1, P <0.001), and slower resaturation rate post exercise (4.0 ± 2.7 vs 5.4 ± 3.6% StO₂ × 3.5 s^?1; P = 0.009) compared with the controls.ConclusionsIn comparison with age- and sex-matched controls, adults with complex CHD had slower oxygenation kinetics. This altered skeletal muscle metabolism might contribute to the impaired skeletal muscle endurance capacity shown and thereby also to the reduced aerobic capacity in this population.     

Two-way communication for programming and measurement in a miniature implantable stimulator

Implantable stimulators are needed for chronic electrical stimulation of nerves and muscles in experimental studies. The device described exploits the versatility of current microcontrollers for stimulation and communication in a miniature implant. Their standard outputs can provide the required selectable constantcurrent sources. In this device, pre-programmed stimulation paradigms were selected by transcutaneous light pulses. The potential of a programmable integrated circuit (PIC) was thus exploited. Implantable devices must be biocompatible. A novel encapsulation method that require no specialised equipment and that used two classical encapsulants, silicone and Teflon was developed. It was tested for implantation periods of up to four weeks. A novel way to estimate electrode impedance in awake animals is also presented. It was thus possible to follow the evolution of the nerve-electrode interface and, if necessary, to adjust the stimulation parameters. In practice, the electrode voltage at the end of a known constant-current pulse was measured by the PIC. The binary coded value was then indicated to the user as a series of muscle twitches that represented the binary value of the impedance measurement. This neurostimulator has been successfully tested in vitro and in vivo. Thresholds and impedance values were chronically monitored following implantation of a self-sizing spiral cuff electrode. Impedance variations in the first weeks could reflect morphological changes usually observed after the implantation of such electrodes.     

Longitudinal course of lung function in myotonic dystrophy type 1

Introduction: Quality of life and prognosis among patients with myotonic dystrophy type 1 (DM1) depend on the degree of respiratory impairment. However, the changes over time in pulmonary function in DM1 have not been clearly described. Methods: We retrospectively reviewed pulmonary function tests of 80 DM1 patients followed for at least 5 years. Results: During 9.02 ± 3.4 years of follow‐up, the average annual changes were: forced vital capacity (FVC) –0.034 ± 0.06 L (–0.72 ± 1.7% predicted); forced expiratory volume in 1 second (FEV₁) –0.043 ± 0.05 L (–1.07 ± 1.7% predicted); and total lung capacity (TLC) –0.047 ± 0.1 L (–1.15 ± 1.7% predicted). Conclusions: These results suggest that, compared with other neuromuscular disorders, DM1 is, overall, associated with slowly progressive impairment of lung function. Muscle Nerve 56 : 816–818, 2017     

Long-term outcome after right ventricle to pulmonary artery conduit surgery and reintervention

Background. Reconstruction of the right ventricular outflow tract with a conduit is an established surgical procedure in congenital heart disease and reinterventions are common. Objective. An increasing number of patients have a conduit, but there are few population-based studies of long-term outcomes after conduit surgery, reoperations, and transcatheter pulmonary valve replacement. Methods. In April 2015, all adult patients with a conduit were identified in the Swedish National Registry for Congenital Heart Disease (SWEDCON). Data on patients who died before age of 16 years are not included in the registry and thus not included in the study. Results. We found 574 patients with a mean age 36.1 years. The largest proportion had tetralogy of Fallot (45%). In total there were 762 operations and 50 transcatheter pulmonary valve replacements. Mean age at first conduit operation was 20.2 years. Long-term survival up to 48 years including perioperative mortality (<1%) was 93% at 20 years. The most common cause of death was cardiac-related. Higher age at first conduit operation was associated with increased mortality risk. Reintervention-free survival was 77% and 54% at 10 and 20 years, respectively. Conduit reinterventions were common. Ten-year reintervention-free survival after first conduit reintervention (n?=?176) was significantly lower than after first conduit operation (70% vs 77% p?=?.04). Higher age at first conduit operation was associated with a reduced risk of reintervention, whereas male sex and complex malformations were associated with increased risk of reintervention. Conclusions. The mortality of repeated conduit reinterventions is low. The need for reintervention of conduits is considerable, and reintervention-free survival after the first conduit reintervention is poorer than after first conduit implantation. The findings in this study only applies for patients reaching 16 years of age.