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Atrial fibrillation (AF) is a frequent and costly health care problem representing the most common arrhythmia resulting in hospital admission. Total mortality and cardiovascular mortality are significantly increased in patients with AF compared to controls. In addition to symptoms of palpitations patients with AF have an increased risk of stroke and may also develop decreased exercise tolerance and left ventricular dysfunction. All of these problems may be reversed with restoration and maintenance of sinus rhythm. External electrical cardioversion has been a remarkably effective and safe method for termination of this arrhythmia. Originally described by Lown et al. in 1963, it has been a well accepted mode of acute therapy. However, this technique requires general anesthesia or heavy sedation. Internal atrial defibrillation has been evaluated as an alternative approach to the external technique for over 2 decades. Recent studies have shown that low-energy internal atrial defibrillation using biphasic shocks is an effective and safe means in restoring sinus rhythm in patients with AF and should be considered especially in patients in whom external cardioversion attempts have failed. IMPLANTABLE ATRIAL DEFIBRILLATOR: Recently, a stand alone IAD, the Metrix System (model 3000 and 3020), has entered clinical investigation. Atrial defibrillation is accomplished by a shock delivered between electrodes in the right atrium and the coronary sinus. The right atrium lead has an active fixation in the right atrium. The coronary sinus lead has a natural spiral configuration for retention in the coronary sinus, and can be straightened with a stylet. Both leads are 7 French in diameter and the defibrillation coils are each 6 cm in length. The electrodes may be placed using separate leads, or very soon by using a single bipolar lead. A separate bipolar right ventricular lead is used for R wave synchronization and post shock pacing. The Metrix defibrillator can be used to induce AF by using R wave synchronous shocks and can store intracardiac electrograms (EGMs) for up to 2 minutes from the most recent 6 AF episodes. The device can be programmed into one of the following operating modes: fully automatic, patient activated, monitor mode, bradycardia pacing only, and off. As AF is not life-threatening, in the automatic mode the device is only intermittently active in detecting and treating AF, and this "sleep wake-up" cycle interval is programmable. The device employs extensive processing both for detection and R wave synchronization. In April 1996, the phase I Metrix multicenter clinical trial was started. As of May 1997, a total of 51 Metrix systems had been implanted as part of the phase I multicenter clinical trial. Preliminary data suggest that both defibrillation thresholds and electrograms are stable over time (implant to 3 months). Detection accuracy has been excellent (100% specificity, 92.3% sensitivity) and there have been no errors of R wave selection for synchronization. No proarrhythmias have resulted from over 3700 shocks delivered. The device is effective in electrically converting 96% of the spontaneous episodes of AF. In 27% of episodes several shocks were required because of early recurrence of AF. In 5 patients, the atrial defibrillator was removed: 2 infections, 1 cardiac tamponade, 1 permanent loss of telemetry, 1 patient required His-Bundle ablation because of frequent episodes of drug refractory AF with rapid ventricular response. Initial clinical experience under controlled conditions with the Metrix system suggests that the implantable atrial defibrillator may offer a therapeutic alternative for a subgroup of patients with drug refractory, symptomatic, long lasting, and infrequent episodes of AF. Further efforts must be undertaken to reduce the patient discomfort associated with internal atrial defibrillation in an attempt to make this new therapy acceptable to a larger patient population with AF. (ABSTRACT TRUNCATED)
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PMID:[Atrial defibrillator]. 969 Jan 12

Carbohydrate-deficient glycoprotein syndrome type 1 (CDGS-1) is an autosomal recessive hereditary metabolic disorder, the gene locus of which is chromosome 16p13. The disorder is characterised by genetic heterogeneity, and by decrease in the gene product, phosphomannomutase 2, though the heterogeneity is far less manifest in affected Swedish families. Its incidence is 1/80,000 live births, and the under-5 mortality rate over 30 per cent. The causes of death are liver failure, cardiac tamponade, haemorrhaging, and severe infection. The characteristic biochemical aberration is the occurrence of deficient carbohydrate chains in many but not all circulating glycoproteins, and the serum and blood concentrations of some glycoproteins may be above or below normal. These changes may improve over time, but never normalise. The clinical picture is generally more problematic during the first years of life when psychomotor retardation is complicated by failure to thrive, liver dysfunction, pericardial effusions, and stroke-like episodes. In addition, strabismus, lipocutaneous anomalies, and gluteal fat pads are always present, and muscular hypotonia and restricted joint mobility are common. Failure to thrive is common, with vomiting and diarrhoea and subsequent slow growth. Inflammation is a constant finding in the liver, and very common in the small bowel. Pancreatic function is also affected. Pericardial effusion has been reported in 50 per cent of the youngest children, requiring pericardectomy in 30 per cent of cases. Haemorrhaging and thromboembolic complications may occur, and the serum concentrations of several factors and inhibitors are low, particularly those of factors V and XI, protein C and antithrombin. Stroke-like episodes occur in about 30 per cent of cases, often following an infection, with coma lasting for hours to several days. Such sequelae as hemiplegia, blindness, and other focal neurological pathology have been observed transiently. Diagnosis is based on the serum carbohydrate-deficient transferrin level, verified by isoelectric focusing. Molecular genetic procedures enable point mutations to be identified and prenatal diagnosis to be performed in many families.
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PMID:[CDGS-1--a recently discovered hereditary metabolic disease. Multiple organ manifestations, incidence 1/80,000, difficult to treat]. 988 93

Atrial fibrillation (AF) is a frequent and costly health care problem representing the most common arrhythmia resulting in hospital admission. Total mortality and cardiovascular mortality are significantly increased in patients with AF compared to controls. In addition to symptoms of palpitations, patients with AF have an increased risk of stroke and may also develop decreased exercise tolerance and left ventricular dysfunction. All of these problems may be reversed with restoration and maintenance of sinus rhythm. External electrical cardioversion has been a remarkably effective and safe method for termination of this arrhythmia. Originally described by Lown et al. in 1963, it has been a well accepted mode of acute therapy. However, this technique requires general anesthesia or heavy sedation. Internal atrial defibrillation has been evaluated as an alternative approach to the external technique for over 2 decades. Recent studies have shown that low-energy internal atrial defibrillation using biphasic shocks is an effective and safe means in restoring sinus rhythm in patients with AF and should be considered especially in patients in whom external cardioversion attempts have failed. Implantable Atrial Defibrillator: Recently, a stand alone IAD, the Metrix System (models 3000 and 3020), has entered clinical investigation. Atrial defibrillation is accomplished by a shock delivered between electrodes in the right atrium and the coronary sinus. The right atrium lead has an active fixation in the right atrium. The coronary sinus lead has a natural spiral configuration for retention in the coronary sinus, and can be straightened with a stylet. Both leads are 7 French in diameter and the defibrillation coils are each 6 cm in length. The electrodes may be placed using separate leads, or very soon by using a single bipolar lead. A separate bipolar right ventricular lead is used for R wave synchronization and post shock pacing. The Metrix defibrillator can be used to induce AF by using R wave synchronous shocks and can store intracardiac electrograms (EGMs) for up to 2 minutes from the most recent 6 AF episodes. The device can be programmed into one of the following operating modes: fully automatic, patient activated, monitor mode, bradycardia pacing only, and off. As AF is not life-threatening, in the automatic mode the device is only intermittently active in detecting and treating AF, and this "sleep wake-up" cycle interval is programmable. The device employs extensive processing both for detection and R wave synchronization. In April 1996, the phase I Metrix multicenter clinical trial was started. As of May 1997, a total of 51 Metrix systems had been implanted as part of the phase I multicenter clinical trial. Preliminary data suggest that both defibrillation thresholds and electrograms are stable over time (implant to 3 months). Detection accuracy has been excellent (100% specificity, 92.3% sensitivity) and there have been no errors of R wave selection for synchronization. No proarrhythmias have resulted from over 3700 shocks delivered. The device is effective in electrically converting 96% of the spontaneous episodes of AF. In 27% of episodes several shocks were required because of early recurrence of AF. In 5 patients, the atrial defibrillator was removed: 2 infections, 1 cardiac tamponade, 1 permanent loss of telemetry, 1 patient required His-Bundle ablation because of frequent episodes of drug refractory AF with rapid ventricular response. Initial clinical experience under controlled conditions with the Metrix system suggests that the implantable atrial defibrillator may offer a therapeutic alternative for a subgroup of patients with drug refractory, symptomatic, long lasting, and infrequent episodes of AF. Further efforts must be undertaken to reduce the patient discomfort associated with internal atrial defibrillation in an attempt to make this new therapy acceptable to a larger patient population with AF. (ABSTRACT TRUNCATED)
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PMID:[Atrial defibrillator]. 1081 Aug 4

Every acute dissection involving the ascending aorta (Stanford type A) must undergo emergency surgical repair. However, the surgical techniques must vary according to the clinical presentation of the patients or the anatomical patterns observed. Furthermore, surgery is generally difficult because of the poor condition of the aortic tissues. To reduce those difficulties many technical artifacts have been described. In 1977, we proposed the use of gelatin-resorcin-formalin (GRF) biological glue to reinforce the suture areas. From January 1977 to July 1999, 212 patients (pts) (152 males and 60 females) aged from 15 to 80 years (mean age: 54 +/- 11 years) underwent an emergency operation for type A aortic dissection. One-hundred-seventy-eight pts (84%) were operated on within 4 hours after being referred to the hospital. Twenty-eight pts (13.2%) had Marfan's syndrome. In 44 patients (20.7%), the aortic valve was replaced either independently (6 cases--2.8%) or by means of a composite graft (38 cases--17.9%). Because of the location of the intimal tear, the aortic replacement was extended to the transverse arch in 61 pts (28.7%). Hospital mortality amounts to 21.6% (46 pts), 25% in pts with arch replacement and 19.4% in pts without arch replacement (n.s). Analysis of hospital mortality demonstrates that the main causes of death were cardiac tamponade, neurologic disorders and visceral malperfusion. One-hundred-sixty-six pts were discharged and surveyed from 5 months to 22 years postoperatively (mean follow-up: 85 +/- 66 months). During this period of time, 25 pts (15%) had to be reoperated for a total of 33 reoperations. Seven pts (28%) died at reoperation. Using univariate analysis, the presence of Marfan's syndrome (p < 0.05) and absence of arch replacement (p < 0.02) were determinant risk factors for reoperation. Emergency (p < 0.01) and thoraco-abdominal replacement (p < 0.04) were determinant riskfactors for death at reoperation. The freedom from reoperation (Kaplan-Meier, CI: 95%) is 96% (90-98), 87% (79-92), 80% (70-88), 66% (51-78) at 1, 5, 10 and 15 years respectively. A total of 39 pts (24.3%) died during follow-up. The presence of Marfan's syndrome (p < 0.01), reoperation (p < 0.02), stroke (p < 0.05), and cardiac failure (p < 0.05) were determinant risk factors of late mortality. The late survival rate (K-M. C.I.: 95%), including hospital mortality, is 71% (64-77), 66% (58-73), 56% (47-64), 46% (36-56), 37% (28-44) at 1, 10, 15 and 20 years, respectively. From our experience extending over more than 23 years, GRF glue has proved to be extremely useful, making the procedure much easier and safer. Nevertheless, many factors are of importance in the pre-, intra- and postoperative management of the patients. Cardiac tamponade and visceral malperfusion must be properly diagnosed and treated. During aortic repair, the main intimal tear must be resected. The transverse arch must be checked and replaced whenever necessary. The aortic valve should be preserved whenever possible. During CPB, perfusing the aorta in the regular antegrade manner seems to dramatically reduce the rate of malperfusion. The quality of the first emergency operation seems to have a major influence on the late results, especially concerning the rate of late reoperations and aortic ruptures. However, those late results depend also on the patient's basic condition, particularly in Marfan patients.
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PMID:Surgery of acute type A dissection: what have we learned during the past 25 years? 1109 59

Ventricular interdependence is that property of the normal heart such that distension of one ventricle alters the distensibility and filling pressure of the other. This phenomenon coupled with reciprocal changes in right and left heart venous return during normal quiet respiration results in minor decreases in left ventricular stroke volume, systolic blood pressure, pulse pressure, total electromechanical systole (Q-A2), left ventricular ejection time and mitral e-wave velocity during inspiration and minor increases in these parameters during expiration. Opposite changes in these parameters occur in the right heart with increases occurring during inspiration and decreases during expiration. Exaggerated ventricular interdependence occurs in cardiac tamponade when the pericardial constraint limits the total contents in the pericardial sac. This, together with the decreased effective left ventricular filling pressure which occurs during inspiration, is responsible for the exaggerated decrease in stroke volume, blood pressure, pulse pressure, left ventricular ejection time and mitral e-wave velocity in this condition. These observations, together with the echocardiographic findings of right atrial collapse, right ventricular collapse, and inferior vena cava plethora constitute the noninvasive diagnosis of pericardial tamponade. The utility of these noninvasive tests in detecting both the presence and degree of increased pericardial pressure was evaluated in 33 invasively studied patients with pericardial effusion. In Group 1 (n = 13) intrapericardial pressure was elevated but less than both right atrial pressure and pulmonary wedge pressure, in Group 2 (n = 10) intrapericardial pressure equaled right atrial pressure but was less than pulmonary wedge pressure, and in Group 3 (n = 10) intrapericardial pressure equaled right atrial pressure and pulmonary wedge pressure. From these data it is concluded that right atrial and right ventricular collapse are highly sensitive techniques for predicting increased intrapericardial pressure in all three groups, but fail to predict the level of intrapericardial pressure and the severity of hemodynamic compromise. However, the absence of inferior vena cava plethora helped separate Group 1 patients from Groups 2 and 3 patients, thereby aiding in distinguishing a group of patients with severe hemodynamic derangement requiring urgent intervention. Exaggerated reciprocal changes in mitral and tricuspid e-wave velocity is a very sensitive finding for increased intrapericardial pressure. However, its presence correlates poorly with the severity of tamponade. Acute pericardial tamponade is a clinical diagnosis determined by the integration of the history, physical exam and appropriate noninvasive physiologic and imaging techniques.
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PMID:Noninvasive/invasive correlates of exaggerated ventricular interdependence in cardiac tamponade. 1143 31

The aim of this study was to identify the most important variables associated with early and late mortality in patients operated on for type A aortic dissection over a 15-year period. From January 1984 to March 1999, 110 patients underwent surgery for type A aortic dissection. The 88.1% of patients had an acute type A dissection (AD) and 11.8% had a chronic dissection (CD). Cardiac tamponade and shock occurred in 21.8% and 14.5% of the patients, respectively. The location of the primary intimal tear was in the ascending aorta in 70.9% of cases, in the arch in 17.2%, and in the descending aorta in 7.2%. Univariate and multivariate analyses were conducted to identify non-embolic variables independently correlated to in-hospital death. Kaplan-Meier and Cox regression analyses and hazard function for death risk were used to analyze factors influencing overall and surgical survival. The overall in-hospital mortality rate was 20.9% (23/110 patients), being 9% for CD and 21.6% for AD. Emergent procedures had an in-hospital mortality rate of 47.6%, whereas nonemergent operations had an in-hospital mortality rate of 13.7% (p < 0.01). Univariate analysis revealed 41 preoperative and operative variables, including age (years), age >70 years, remote myocardial infarction, cerebrovascular dysfunction, diabetes, preoperative renal failure, shock, cardiopulmonary bypass time (minutes), emergency operation, as factors associated to in-hospital death (p < 0.05). Stepwise logistic regression analysis for in-hospital death selected as independent predicting variables (p < 0.05) remote myocardial infarction [p = 0.006, odds ratio (OR) = 1.9], preoperative renal failure (p = 0.031; OR = 0.8), shock (p = 0.001; OR = 3.1), and age >70 years (p = 0.007; OR = 1.7). Follow-up ranged from 9 to 172 months (median 78 months), with Kaplan-Meier survivals for all the patients and hospital survivors of 42% and 54% at 10 years, respectively. Cox regression analysis has identified postoperative stroke [relative risk (RR) = 3.7; p = 0.012), intimal tear in the aortic arch (RR = 2.3; p = 0.036), and postoperative renal failure (RR = 4.5; p = 0.007) as independent predictors of reduced survival at follow-up. When this kind of analysis was performed on hospital survivors only, preoperative renal dysfunction (RR = 1; p = 0.013), reoperation (RR = 1.7;p = 0.004) and intimal tear in the aortic arch (RR = 1.2; p = 0.002) emerged as risk factors. The actuarial freedom from reoperation was 85.4% at 5 years. Multiple factors still influence early and late survival after surgery for type A aortic dissection. Preoperative renal impairment both affects early and late outcome. Early postoperative course affects late outcome in hospital survivors. The presence of the intimal tear in the aortic arch has a negative impact on late survival.
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PMID:Determinants of early and late outcome after surgery for type A aortic dissection. 1177 81

Acute aortic dissection is one of the most common catastrophes affecting the aorta. Because dissection can involve any aortic segment, the disease can manifest itself through a variety of clinical presentations. The most spectacular manifestation is frank rupture, usually into the pericardial or pleural cavity. Dissections of the ascending aorta are associated with rapidly fatal complications such as cardiac tamponade, major stroke, or massive myocardial infarction, justifying emergent operation. Dissections of the descending aorta are managed medically with surgery reserved for those patients with aortic rupture, aneurysmal dilatation, or ischemic symptoms. Aortic branch occlusion occurs in up to one third of patients with aortic dissection and is associated with increased risk of early death and serious complications. The therapeutic armamentarium of the vascular surgeon has evolved during the last 20 years to include endovascular (balloon fenestration or branch stenting) and surgical options. This article will focus on the open surgical management of patients with acute aortic dissection complicated by side branch occlusion and organ or limb malperfusion.
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PMID:Acute aortic dissection with side branch vessel occlusion: open surgical options. 1206 Aug 98

Most patients with ventricular tachycardia (VT) associated with structural heart disease should receive an implantable cardioverter-defibrillator as initial therapy. Patients with symptomatic recurrences of tachycardia, including those with multiple defibrillator shocks, are considered for ablation. The vigor with which antiarrhythmic drug therapy is pursued as antecedent therapy to ablation depends on patient factors (eg, medical comorbidity, type of heart disease, number and hemodynamic tolerance of tachycardias) and the previous history of antiarrhythmic drug exposure (eg, side effects, inefficacy). In patients with mild left ventricular dysfunction and well-tolerated tachycardia, ablation may be offered as primary definitive therapy in selected individuals. In patients without structural heart disease, ablation is usually offered as primary definitive therapy to highly symptomatic patients, and is strongly recommended for patients with recurrent tachycardia following initial attempts at drug suppression. Optimal outcome of VT ablation depends on the availability of an experienced team and sophisticated facilities to accommodate the technical challenges associated with the broad spectrum of clinical presentations and arrhythmia mechanisms. Historically, major complications have been reported in up to 10% of patients, including death, stroke, cardiac tamponade, complete heart block, and myocardial infarction. In our own experience with VT ablation over the past 10 years, major complications occurred in three (1.8%) of 168 patients with structural heart disease and one (0.7%) of 142 patients without structural heart disease.
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PMID:Catheter Ablation of Ventricular Tachycardia. 1294 Dec 6

Aortic intramural hematoma (IMH) is an acute, potentially lethal disorder that is similar to but pathologically distinct from acute aortic dissection. Although hemorrhage into the aortic media occurs in both disorders, an intimal tear with resultant false lumen is not present in IMH. Instead, hemorrhage occurs within the aortic wall either due to rupture of the vasa vasorum or, less commonly, because of an atherosclerotic penetrating aortic ulcer. The most common risk factors associated with IMH are hypertension, atherosclerosis, and advanced age. IMH is life-threatening because the hematoma may extend along or rupture through the aorta, leading to hemothorax, cardiac tamponade, stroke, mesenteric ischemia, or renal insufficiency. Optimal treatment is still somewhat controversial; however, there is no question that hypertension must be treated effectively and immediately. This is usually best accomplished by intravenous infusion of beta-blocking agents, with or without the addition of sodium nitroprusside. Recent studies support surgical treatment (ie, aortic root replacement) for IMH involving the ascending aorta, although some subsets of this population may be at lower risk and may benefit from medical therapy alone. In patients with IMH involving only the descending aorta, medical therapy alone is recommended (unless impending rupture, aortic aneurysm, or end-organ ischemia occurs). Patients who survive the acute event require intensive treatment of hypertension and frequent follow-up examinations. Because this population (especially the subset with penetrating aortic ulceration) is at high risk for aortic aneurysm and rupture, serial imaging studies of the aorta are essential.
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PMID:Aortic Intramural Hematoma: Current Diagnostic and Therapeutic Recommendations. 1506 38

For select individuals with chronic, drug-resistant, paroxysmal atrial fibrillation, who have failed pharmacologic suppression or mechanical cardioversion, radiofreqency ablation of ectopic foci in and around the ostia of the pulmonary veins can restore normal sinus rhythm, prevent recurrence of the arrhythmia, and improve the quality of life. Radiofrequency ablation of these sites is an evolving process as improvements are made in the catheters and procedure. Major complications include pulmonary vein stenosis, stroke, and cardiac tamponade.
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PMID:Radiofrequency ablation in the pulmonary veins for paroxysmal, drug-resistant atrial fibrillation. 1558 38


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