Congenital long QT syndrome

The long QT syndrome (LQTS) combines a prolonged QT interval with an enhanced risk of polymorphous ventricular arrhythmias that may lead to syncope and sudden cardiac death. It may be congenital or acquired (the latter sometimes caused by drugs). Congenital LQTS is a rare disease, usually discovered during the clinical evaluation of understood syncopes or at cardiological examination after an unexpected sudden cardiac death of a close relative. The syncope frequently occurs during physical exercise, fear or sudden loud noises. In patients with symptomatic LQTS, the mortality 10 years after the first syncope amounts to approximately 50%. A prolonged QT interval indicates abnormal repolarization or deceleration of the depolarization. An increase of the sympathetic tone, e.g. during physical exercise and emotions, causes prolongation of the QT interval. Congenital LQTS has been associated with genetic mutations, for instance on chromosomes 3 and 7. Treatment consists af administration of beta-blockers, sympathectomy and, if necessary, implantation of an automatic cardioverter/defibrillator.     

Implantable cardioverter-defibrillator therapy for prevention of sudden cardiac death

Patients with known symptomatic VT or VF are at high risk for sudden cardiac death. Various therapeutic choices can be used to reduce the incidence of arrhythmic sudden cardiac death. These include beta-blockers, class I and III antiarrhythmic agents, VT focal ablations, and ICD therapy. The overall incidence of sudden cardiac death in ICD recipients is less than 2% per year, a rate of survival not achieved with any of the available antiarrhythmic agents. VT surgical therapy can produce comparable survival results, but the minimal operative mortality is higher than that with ICD therapy. In patients with noninducible VT/VF or inducible polymorphic VT, and in those refractory to or intolerant of antiarrhythmic agents and poor left ventricular function, ICD therapy may be the only realistic option.     

Breathing measurement reduces false-negative classification of tachypneic preextubation trial failures

A method using a parameter from the field of nonlinear dynamics to quantify the variability of ventricular premature complexes (VPCs) is presented. One hundred patients with coronary artery disease and > or = 10 VPCs/hour were included in the study. The RR intervals were plotted in a three-dimensional artificial phase space, and the structures in phase space were quantified by the local scaling indices, alpha. In the frequency distribution histogram, n(alpha), for each patient, the maximum of the ventricular ectopies alpha VPC, adjusted to the VPC frequency, was assessed; alpha VPC was used as the risk indicator. Endpoints were total mortality and sudden cardiac death. During follow-up (mean 3.1 years), 28 out of 100 patients died, 16 suddenly; alpha VPC had a significant prognostic impact and was independent from other risk indicators, such as left ventricular ejection fraction (LVEF). Patients who died during follow-up were characterized by a high alpha VPC. The optimal discrimination of high risk patients and low risk patients occurred at alpha VPC = 3.0. After 4 years, the survival rate of patients with a alpha VPC > 3.0 was 59%, in contrast to 97% in patients with alpha VPC < or = 0.3. As to the sudden death mortality, the survival rates were 74% and 97%, respectively. The difference between the groups were significant for both endpoints. Patients with an increased VPC variability (i.e., alpha VPC > 3.0) were at enhanced risk of sudden death and total mortality risk; alpha VPC was independent from other risk indicators such as the LVEF or heart rate variability parameters.     

The implantable cardioverter/defibrillator

Despite all advances in the diagnosis and therapy of cardiovascular diseases, the mortality from malignant ventricular tachyarrhythmias is still a major health problem. In addition to established therapeutic strategies in the prevention of sudden cardiac death such as antiarrhythmic drug treatment, catheter ablation or antiarrhythmic drug treatment, cardioverter/defibrillator was introduced to clinical practice in 1980. The number of 50,000 overall implants reflects the current clinical status of the therapy with implantable cardioverter/defibrillators. Significant technical improvements in the defibrillator therapy may contribute to an increase in therapy acceptance. These advances include the introduction of nonthoracotomy lead systems, enhanced defibrillation efficacy, full programmable devices providing tiered electrical therapy, improved diagnostic Holter functions and enhanced arrhythmia detection algorithms. The major present goals of defibrillator therapy are detection and termination of malignant ventricular tachyarrhythmias, prevention of sudden cardiac death, reduction in patient's mortality and improvement in quality of life. The efficacy and safety of defibrillator therapy to prevent sudden arrhythmic death has been proven in several large clinical investigations In patients with this device the annual sudden cardiac death mortality is < 2% even in high-risk patient populations. Compared to sudden cardiac death rate there is a much higher rate of overall cardiac mortality because a defibrillator is not able to prevent nonarrhythmic cardiovascular deaths. There is a clinical impression that cardiovascular mortality is lower in patients treated with an implantable cardioverter/defibrillator compared to patients treated with other therapies. However, there are no results from controlled studies providing scientific evidence that defribillator therapy can decrease overall cardiovascular mortality.(ABSTRACT TRUNCATED AT 250 WORDS)     

Prevalence of sudden cardiac death during competitive sports activities in Minnesota high school athletes

OBJECTIVES: Reliable prevalence data would be useful in assessing the impact of sudden cardiac death in young competitive athletes on the community and designing effective preparticipation screening strategies. BACKGROUND: The frequency with which these catastrophes occur is largely unknown. METHODS: We utilized a circumstance unique to Minnesota in which the precise number of participants and deaths due to cardiovascular disease could be ascertained over a substantial period of time based on a long-standing insurance program for catastrophic injury or death, mandatory for all student athletes engaged in interscholastic sports. RESULTS: Over the 12-year period, 1985/1986 to 1996/1997, inclusive, three sudden deaths due to cardiovascular disease occurred in competitive high school athletes (grades 10-12) during competition or practice. At autopsy, 1 each proved to be due to anomalous origin of the left main coronary artery from the right sinus of Valsalva, congenital aortic valve stenosis (with bicuspid valve) and myocarditis. All three athletes were white and male, 16 or 17 years of age; two competed in cross-country/track and one in basketball. During the study period there were 1,453,280 overall sports participations and 651,695 student athlete participants among the 27 high school sports. The calculated risk for sudden death was 1:500,000 participations and 1:217,400 participants per academic year (or 0.46/100,000, annually). Over a 3-year high school career for a student athlete the estimated risk was 1:72,500. CONCLUSIONS: The risk of sudden cardiac death in a population of high school student athletes was small, in the range of one in 200,000 per year, and was higher in male athletes. The rare occurrence of sudden cardiac death in competitive sports underlines the limitations implicit in structuring productive and cost-effective broad-based preparticipation screening strategies for high school athletes.     

Gender differences in the outcomes of participants in home programs compared to those in structured cardiac rehabilitation programs

This study compared gender differences in outcomes of patients who had had coronary bypass surgery and either attended structured cardiac rehabilitation programs or participated in home programs. The principal advantages related to patients' attending a structured hospital-based outpatient rehabilitation program, compared to a home program, included increased exercise adherence for males and females, increased knowledge about the condition for males, and increased stress control for females. Return to work, self-efficacy, and aspects of self-care such as adherence to a medication regimen and smoking cessation were not significantly different, regardless of type of program, for either sex. In general, females at home fared the worst regarding lifestyle changes, showing decreased exercise adherence, decreased ability to control stress, and no significant increase in knowledge about their medical condition.     

Therapy of ventricular tachyarrhythmia with implantable cardioverters/defibrillators--mortality and complications using epicardial electrodes

Technical improvements of third generation implantable cardioverter defibrillators (ICD) like antitachycardia pacing modalities lead to an extended use of ICDs, not only in patients with aborted sudden cardiac death, but also in patients with hemodynamically tolerable ventricular tachycardia. In addition, anticipated results of current prospective studies might indicate the prophylactic use of ICDs in patients with high risk for sudden cardiac death, but without documented ventricular tachyarrhythmias. This report reviews mortality and complications associated with the ICDs with epicardial defibrillation leads. Mortality is separated in cardiac death, sudden cardiac, arrhythmogenic "not so sudden" cardiac death, and overall mortality. Pulmonary complications and infections are related to the underlying disease and the surgical procedure. Device- and lead-related complications, high defibrillation thresholds, pacemaker interactions, inappropriate shocks, arrhythmic effects, syncope, and psychosocial problems are reported, respectively.     

Prognosis of completely asymptomatic adult patients with hypertrophic cardiomyopathy

OBJECTIVES: We investigated the long-term prognosis of completely asymptomatic adult patients with hypertrophic cardiomyopathy (HC). Diagnosis of HC was suspected because of an abnormal electrocardiogram and/or cardiac murmur and confirmed by echocardiography and/or left ventricular angiography, and hemodynamic investigation. BACKGROUND: Hypertrophic cardiomyopathy shows marked heterogeneity in clinical expression and prognosis. The prognosis of asymptomatic patients with HC has not been fully defined. METHODS: Of 128 consecutive adult patients with HC, 58 asymptomatic patients (Group 1, mean age 42.8 years) and 70 symptomatic patients (Group 2, mean age 50.4 years) were studied to assess cardiac mortality. Mean follow-up periods were 11.0 years for Group 1 and 9.1 years for Group 2. RESULTS: At presentation, Group 1 patients were younger and had smaller left atrial dimensions than did Group 2 patients. The annual cardiac mortality rate and the rate for sudden death alone in Group 1 were significantly lower than in Group 2 (0.9% vs. 1.9%, p < 0.05, 0.1% vs. 1.4%, p < 0.05, respectively). Although about one-third of the survivors in Group 1 had cardiac symptoms at their most recent evaluation, only one patient died suddenly compared with eight in Group 2. The annual mortality rate due to heart failure was similar in each group. Only a syncopal episode was associated with both cardiac death and sudden death for both groups combined. CONCLUSIONS: The cardiac mortality rate for completely asymptomatic adult patients with HC was very low, significantly lower than that of symptomatic patients, and there was a disproportionately low incidence of sudden death.     

Trends along the debt-income axis: implications for medical students'' selections of family practice careers

Even elderly patients > or = 75 years of age with coronary artery disease have modest improvements in lipid levels and marked improvements in exercise capacity, behavioral characteristics, and quality-of-life parameters after cardiac rehabilitation and exercise training programs. These data support that even very elderly patients with coronary artery disease should be routinely referred to and vigorously encouraged to pursue formal outpatient cardiac rehabilitation and exercise training programs following major coronary events.     

Peroxidase activity of liver microsomal vitamin D 25-hydroxylase and cytochrome P450 1A2 catalyzes 25-hydroxylation of vitamin D3 and oxidation of dopamine to aminochrome

OBJECTIVES: This study sought to determine the long-term risk of sudden cardiac death in patients with hemodynamically stable sustained ventricular tachycardia complicating coronary artery disease. BACKGROUND: The prognosis and risk of sudden cardiac death in patients with a history of myocardial infarction and ventricular tachyarrhythmias have not been clearly defined. Prior studies are limited by a short follow-up period and by inclusion of patients with heterogeneous cardiac diseases and presenting arrhythmias. METHODS: A retrospective cohort analysis was performed on data from 124 patients, followed up for a mean of 36 +/- 30 months, who received electrophysiologically guided therapy for hemodynamically stable ventricular tachycardia after remote myocardial infarction. RESULTS: Seventy-eight patients were treated pharmacologically (medical group), and 46 patients underwent map-guided subendocardial resection (surgical group). Nine patients (7.3%) died suddenly, 5 (4.0%) died of noncardiac causes, 9 (7.3%) died of a perioperative complication, and 20 (23.4%) died of other cardiac causes. At 1, 2 and 3 years, sudden death occurred at cumulative rates of 2 +/- 1%, 3 +/- 2% and 7 +/- 3%, whereas total mortality was 20 +/- 4%, 28 +/- 4% and 32 +/- 5% (mean +/- SD). Sudden cardiac death (p = 0.047) and total mortality (p = 0.036) were higher in patients with multivessel disease and were similar for both treatment groups. CONCLUSIONS: Although the overall mortality in postinfarction patients presenting with hemodynamically stable ventricular tachycardia treated with electrophysiologically guided antiarrhythmic therapy is high, the risk of sudden death in these patients appears to be low (average 2.4%/year).     

Should survivors of myocardial infarction with low ejection fraction be routinely referred to arrhythmia specialists?

Because survivors of myocardial infarction are at risk for ventricular arrhythmias and sudden death, physicians must decide whether to refer these patients to specialists for arrhythmia assessment and therapy. However, this decision is complex as few randomized data are available concerning either diagnostic or therapeutic options. Therefore, we modeled the potential impact of current arrhythmia detection and management strategies on mortality in survivors of myocardial infarction with reduced left ventricular function who are managed in a contemporary manner. Based on recent data we estimated that the mortality for myocardial infarction survivors with left ventricular ejection fraction less than 0.40 is 20 percent over 3.5 years and that half of the deaths are sudden. The sensitivity and specificity of a Holter electrocardiogram (ECG), a signal-averaged ECG, and an invasive electrophysiology study for predicting sudden death were obtained from a literature review of trials published after 1990 that included more than 300 patients. A series of models were constructed to predict mortality achieved by different arrhythmia management strategies that reduced sudden death by 50 percent and 75 percent--reductions estimated to be within the range for amiodarone and implantable defibrillators. We found that, when routinely applied to all infarct survivors with depressed ventricular function, a therapy that reduces sudden death by 50 percent with 1 percent fatal adverse effects (potentially amiodarone) saves approximately 1 life for every 25 patients treated. Therapy that reduces sudden death by 75 percent with 2 percent fatal adverse effects (potentially implantable defibrillators) saves 1 life for every 14 patients treated. Using Holter ECG recordings, a signal-averaged ECG, or an invasive electrophysiology study to select higher-risk groups, 1 life can be saved for every 4 to 11 patients treated, and the negative impact of adverse effects can be reduced. However, to achieve this benefit, additional and potentially invasive arrhythmia testing must be applied to 28 to 47 patients for each life saved. Thus, with contemporary management of acute myocardial infarction, the risk of sudden death for survivors is sufficiently low that broad application of available antiarrhythmic therapies has limited potential for further improving survival, particularly if therapy also has significant adverse effects. Thus, routine referral to arrhythmia specialists is not warranted for the majority of infarct survivors and should be largely reserved for patients with serious, symptomatic arrhythmias.     

Influence of left ventricular function on survival and mode of death after implantable defibrillator therapy (Cleveland Clinic Foundation and Montefiore Medical Center experience)

To determine the influence of left ventricular (LV) function on survival and mode of death in patients with an implantable cardioverter-defibrillator (ICD), sudden death, surgical mortality, total arrhythmia-related death, total cardiac death and total death were retrospectively evaluated in 377 consecutive patients. The outcomes were also compared between patients with an LV ejection fraction > or = 30% (214 patients, group 1) and < 30% (148 patients, group 2). Surgical mortality was 3.9% (1.8% in group 1, 7% in group 2). During the follow-up of 25 +/- 20 months, actuarial survival rates of all patients at 3 years were 96% for sudden deaths, 81% for total cardiac deaths and 74% for total mortality. When the 2 groups were compared, survival rates of groups 1 and 2 at 3 years, respectively, were 99 and 90% for sudden death (p < 0.05), 97 and 84% for sudden death and surgical mortality (p < 0.01), 94 and 80% for the total arrhythmia-related death (p < 0.001), 88 and 68% for total cardiac death (p < 0.0001), and 81 and 62% for total mortality (p < 0.002). In group 2, 73% of total cardiac deaths within 1 year were causally related to the arrhythmia. Thus, in patients with an ICD, sudden death rates were very low. However, total cardiac death and total death rates were relatively higher. The outcomes of patients with an ICD were strongly influenced by the degree of LV dysfunction.(ABSTRACT TRUNCATED AT 250 WORDS)     

QT interval as a cardiac risk factor in a middle aged population

OBJECTIVE: To evaluate the value of QT interval as a cardiac risk factor in middle aged people. METHODS: The association between QT interval and cardiac risk factors and mortality in a middle aged Finnish population of 5598 men and 5119 women was evaluated over a 23 year follow up. To adjust the QT interval confidently for heart rate, a nomogram was constructed from the baseline electrocardiograms separately for men and women. RESULTS: Nomogram-corrected QT interval (QTNc) prolongation was associated with elevated blood pressure and signs of cardiovascular disease; QTNc shortening was associated with smoking. Over 10% prolongation of QTNc predicted death in men with heart disease: adjusted relative risk (RR) was 2.17 (95% confidence interval 0.67-7.45) for sudden death; 2.12 (1.25-3.59) for total cardiovascular mortality; and 1.92 (1.23-3.00) for all cause mortality. In healthy men the increase in RR was not significant: sudden death, 1.48 (0.67-3.25); total cardiovascular mortality, 1.25 (0.92-1.70); all cause mortality, 1.21 (0.96-1.53). However, healthy men with long QTNc in the lowest heart rate quartile exhibited an RR of 2.75 (1.00-7.40) for sudden death. Over 10% shortened QTNc predicted cardiovascular death in men with heart disease who smoked; RR 3.72 (1.45-9.54). Non-smoking men with short QTNc had low mortality risks irrespective of possible signs of cardiovascular disease. The trends in mortality risks were similar but weaker for women. CONCLUSIONS: In a middle aged population, prolonged QT interval predicts cardiac mortality in men with signs of cardiovascular disease. In women and healthy men this risk is weak and may reflect subclinical heart disease. A shortened QT interval predicts death in men with heart disease who smoke.     

Preoperative evaluation of cardiac failure and ischemia in elderly patients by cardiopulmonary exercise testing

Major surgery in the elderly continues to have a high mortality rate. Preoperative myocardial ischemia is a known risk factor. Cardiac failure is also a risk factor, but is difficult to quantify objectively. One hundred eighty-seven elderly surgical patients were evaluated for cardiac failure by cardiopulmonary exercise testing (CPX). The overall mortality in these patients was 7.5 percent. If three deaths secondary to surgical causes are excluded, mortality was 5.9 percent. There were 55 patients in whom the anaerobic threshold (AT) was less than 11 ml/min/kg; of these, 10 died, a mortality rate of 18 percent. There were 132 patients with an AT of greater than 11 ml/min/kg and of these, 1 patient died, giving a mortality rate of 0.8 percent (p < 0.001). A low AT associated with preoperative ischemia resulted in the death of 8 of 19 patients, a mortality rate of 42 percent. When the ischemia was associated with the higher AT, then 1 patient out of 25 died, a mortality rate of 4 percent (p < 0.01). Both preoperative ischemia and preoperative cardiac failure are independent risk factors for perioperative mortality in the elderly.     

Renaissance of beta blocker therapy in myocardial infarct

Beta-adrenergic blocking drugs have been evaluated for the treatment of arrhythmias and for the prevention of sudden cardia death, particularly in post-myocardial infarction patients. Betablockers have been demonstrated to reduce mortality, reinfarction, ventricular fibrillation and cardiac rupture in acute infarction. Therefore, in patients with suspected myocardial infarction and without contraindications, treatment with betablockers should be initiated early and continued for at least 2 years. Side-effects are mild and occur in approximately 10% of patients. Patients who have contraindications for betablockers use early in myocardial infarction should be reevaluated before discharge from the hospital and considered for such therapy. Because betablockers prevent some of the adverse arrhythmogenic mechanisms seen in chronic heart failure, it may be reasonable to expect that these drugs could have a role in preventing sudden cardiac death in these patients. Analysis of some of the betablocker post-infarction trials indicate that betablockers reduced the risk of sudden death in patients with heart failure at baseline. Some studies demonstrated also the symptomatic improvement following therapy with betablockers in patients with heart failure. But the currently available data are too limited to provide conclusive information.     

Common and specific process factors in cardiac rehabilitation: Independent and interactive effects of the working alliance and self-efficacy.

Objective: Therapeutic processes in cardiac rehabilitation programs are virtually unexamined. Models were tested by which changes in the working alliance between patient and staff (agreement on goals/tasks; emotional bond) may affect outcomes in conjunction with changes in patient self-efficacy to change their diets and increase exercise. Design: Cardiac patients (n = 79) participated in a 12-week program, and completed assessments at early, mid, and late treatment. Main Outcome Measures: Changes in cardiac depression, physical health, perceived exertion during exercise, rate/pressure product at submaximal exercise tolerance, weight loss, return to work, total fat intake. Results: Early-treatment changes in agreement on goals/tasks were related to changes in psychosocial factors and perceived exertion during exercise independent of effects of changes in self-efficacy. Early-treatment changes in goals/tasks and self-efficacy interacted to predict changes in cardiorespiratory fitness, weight loss, and return to work such that patients high on both goals/tasks and self-efficacy showed the most gains. Conclusion: Sound therapeutic relationships between patients and staff may play an important role in facilitating the achievement of a wide-range of salutary outcomes during cardiac rehabilitation. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Paternal uniparental disomy for chromosome 1 revealed by molecular analysis of a patient with pycnodysostosis

OBJECTIVES: We sought to determine the prognostic capabilities of exercise thallium (Tl)-201 tomographic imaging performed relatively early (within 2 years) after coronary artery bypass graft surgery (CABG). BACKGROUND: Exercise testing is commonly performed after CABG, but few data exist demonstrating its prognostic value in this setting. METHODS: Four hundred eleven patients were followed up for a median duration of 5.8 years. Eleven prospectively chosen clinical, exercise and Tl-201 variables were tested for their associations with outcome end points by means of proportional hazards regression models. RESULTS: During follow-up there were 60 deaths from any cause, 53 initial cardiac deaths or nonfatal myocardial infarctions (MIs) and 22 late (>3 months after the Tl-201 study) revascularization procedures. The number of abnormal Tl-201 segments on the postexercise image was the only variable in the multivariate analyses to show a significant association with all three outcome end points: chi-square 7.3, p = 0.007 for overall mortality; chi-square 8.1, p = 0.004 for cardiac death or MI; chi-square 7.8, p = 0.005 for any cardiac event. Other independent predictors of outcome were exercise duration (chi-square 10.7, p = 0.001) and age (chi-square 3.9, p = 0.049) for overall mortality and exercise angina score (chi-square 8.7, p = 0.003) for cardiac death or MI. The 5-year survival rate free of cardiac death or MI was 93% for patients without angina and a normal image or small postexercise perfusion defect versus 71% for patients with angina and a medium or large defect. CONCLUSIONS: Exercise Tl-201 imaging performed within 2 years of CABG can stratify patients into low and high risk subgroups.     

Sudden cardiac death: definition, mechanisms and risk factors

Sudden cardiac death is defined as natural death due to cardiac causes, heralded by abrupt loss of consciousness within one hour after the onset of symptoms. The mechanisms are the following: 1. ventricular fibrillation, 2. ventricular tachycardia and flutter with subsequent ventricular fibrillation, 3. torsade de pointe, 4. bradyarrhythmias and asystolic arrest. White the main risk factor is the presence of coronary artery disease, any organic or functional disease of the heart can predispose for sudden cardiac death. To evaluate the risk of sudden cardiac death noninvasive (Holter, echocardiography, exercise test and signal averaged (ECG) and often invasive (electrophysiological study) tests are necessary. The therapy is based on drugs (mainly beta blockers and amiodarone), coronary revascularization, catheter ablation techniques and the implantation of a cardioverter defibrillator; the latter appears to be the most promising approach.     

Molecular and cellular changes in vein grafts: influence of pulsatile stretch

Sudden cardiac death is frequent in patients with dilated cardiomyopathy. To assess the risk of an arrhythmic event is still difficult. Here the analysis of the heart rate variability offers new possibilities. METHOD: 25 patients (18 males, 7 females, age 53 +/- 9 yrs) with dilated cardiomyopathy were included in the study. Analysis of heart rate variability assessed by time- and frequency-domain measures was determined from Holter recording. The mean follow-up was 18 +/- 5 months. RESULTS: 6 patients died (5 of sudden cardiac death, 1 of heart failure), 1 patient with an implanted defibrillator received an adequate shock. Parameters influenced by low- and mid-frequent oscillations of the heart rate were significantly lower in patients who died suddenly or had adequate shocks. The best predictive parameter was the s.d.RR: all patients with an s.d.RR < 50 ms had lethal arrhythmias whereas the s.d.RR of the surviving patients was > or = 50 ms. No significant difference was found or high frequency parameters, which are mainly influenced by parasympathetic activity. CONCLUSION: The analysis of heart rate variability is of prognostic relevance in patients with dilated cardiomyopathy. Especially the s.d.RR is able to identify patients with a high risk of a sudden cardiac death.