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Ilan Goldenberg

Publications and source records attributed to Ilan Goldenberg.

At least 19 recordsLinked to original sources
PubMed2007

Risk factors for sudden cardiac death in patients with chronic renal insufficiency and left ventricular dysfunction.

BACKGROUND: Patients with ischemic left ventricular dysfunction have a high risk of sudden cardiac death (SCD). It is, however, unclear if the risk and risk factors of SCD in these patients is modulated by the coexistence of mild chronic renal insufficiency. METHODS: We performed a post-hoc analysis of the outcome associated with mild renal dysfunction, as defined by an estimated glomerular filtration rate (eGFR) of <75 ml/min/1.73 m2 in patients allocated to the conventional medical therapy arm of the Multicenter Automatic Defibrillator Implantation Trial-II. RESULTS: In multivariable analysis, renal dysfunction was independently associated with significant increased risks for all-cause mortality (hazard ratio [HR] = 1.86; 95% CI 1.13-3.05) and SCD (HR = 2.00; 95% CI 1.01-4.02), but not non-SCD, compared to patients without renal dysfunction. Independent predictors of SCD in patients with renal dysfunction were: increased resting heart rate (HR = 2.40; 95% CI 1.50-3.86); low diastolic blood pressure (HR = 3.23; 95% CI 1.52-6.66), and a prolonged QRS duration (HR = 1.63; 95% CI 1.02-2.61). Beta-blocker therapy was independently associated with a significant reduction in the risk of SCD in patients with an eGFR of <75 ml/min/1.73 m2 (HR = 0.61; 95% CI 0.38-0.99). CONCLUSION: These findings suggest that renal dysfunction significantly increases the risk for SCD in patients with left ventricular dysfunction, and that beta-blocker therapy reduces the risk of arrhythmic mortality in heart failure patients with coexisting renal insufficiency.

Adrenergic beta-Antagonists↗
PubMed2007

Treatment of arrhythmias and use of implantable cardioverter-defibrillators to improve survival in elderly patients with cardiac disease.

Patients who have left ventricular dysfunction and heart failure(HF) are at high risk for ventricular tachyarrhythmias and sudden cardiac death. Randomized clinical trials have demonstrated that pharmacologic management with antiarrhythmic drugs has limited efficacy for the prevention of arrhythmic mortality in this high-risk population, whereas implantation of a defibrillator was shown to be associated with a significant survival benefit. Data on the efficacy of defibrillator therapy in elderly patients, in whom comorbidities are common, are limited. In this article we outline current information on therapeutic modalities for the prevention of arrhythmic mortality in elderly patients who have left ventricular dysfunction and HF, focusing mainly on data on the benefit of device therapy in the older age group.

Aged↗
PubMed2006

Effectiveness of the implantable cardioverter defibrillator in blacks versus whites (from MADIT-II).

The aim of this study was to examine the effectiveness of implantable cardioverter-defibrillator (ICD) therapy in whites and blacks who were enrolled in the Multicenter Automatic Defibrillator Implantation Trial-II (MADIT-II). The study population involved 1,232 subjects, with 1,073 white patients (87%) and 102 black patients (8%). Univariate analyses revealed a significant reduction in mortality in white patients treated with ICD therapy (p <0.02), but not in black patients (p = 0.96). White patients had a significant reduction of sudden cardiac death (p <0.01), but no benefit was evident in black patients (p = 0.62). Adjusting for relevant covariates, the ICD therapy/conventional therapy hazard ratios for total mortality were favorable in whites (0.75, 95% confidence interval [CI] 0.55 to 1.02), but not in blacks (1.25, 95% CI 0.42 to 3.60); the hazard ratios for sudden cardiac death were significantly (p = 0.04) lower in whites (0.29, 95% CI 0.17 to 0.49) than in blacks (1.71, 95% CI 0.33 to 8.84). Interaction analyses revealed a pattern of progressive improvement in ICD efficacy in whites as the end point became more specific for arrhythmic death, with exactly the reverse for blacks. In conclusion, ICD therapy in MADIT-II was associated with a reduction in total mortality, cardiac death, and sudden cardiac death in whites but not in blacks.

Aged↗
PubMed2006

Risk of aborted cardiac arrest or sudden cardiac death during adolescence in the long-QT syndrome.

CONTEXT: Analysis of predictors of cardiac events in hereditary long-QT syndrome (LQTS) has primarily considered syncope as the predominant end point. Risk factors specific for aborted cardiac arrest and sudden cardiac death have not been investigated. OBJECTIVE: To identify risk factors associated with aborted cardiac arrest and sudden cardiac death during adolescence in patients with clinically suspected LQTS. DESIGN, SETTING, AND PARTICIPANTS: The study involved 2772 participants from the International Long QT Syndrome Registry who were alive at age 10 years and were followed up during adolescence until age 20 years. The registry enrollment began in 1979 at 5 cardiology centers in the United States and Europe. MAIN OUTCOME MEASURES: Aborted cardiac arrest or LQTS-related sudden cardiac death; follow-up ended on February 15, 2005. RESULTS: There were 81 patients who experienced aborted cardiac arrest and 45 who had sudden cardiac death; 9 of the 81 patients who had an aborted cardiac arrest event experienced subsequent sudden cardiac death. Significant independent predictors of aborted cardiac arrest or sudden cardiac death during adolescence included recent syncope, QTc interval, and sex. Compared with those with no syncopal events in the last 10 years, patients with 1 or 2 or more episodes of syncope 2 to 10 years ago (but none in the last 2 years) had an adjusted hazard ratio (HR) of 2.7; (95% confidence interval [CI], 1.3-5.7; P<.01) and an adjusted HR of 5.8 (95% CI, 3.6-9.4; P<.001), respectively, for life-threatening events; those with 1 syncopal episodes in the last 2 years had an adjusted HR of 11.7 (95% CI, 7.0-19.5; P<.001) and those with 2 or more syncopal episodes in the last 2 years had an adjusted HR of 18.1 (95% CI, 10.4-31.2; P<.001). Irrespective of events occurring more than 2 years ago, QTc of 530 ms or longer was associated with increased risk (adjusted HR, 2.3; 95% CI, 1.6-3.3; P<.001) compared with those having a shorter QTc. Males between the ages of 10 and 12 years had higher risk than females (HR, 4.0; 95% CI, 1.8-9.2; P = .001), but there was no significant risk difference between males and females between the ages of 13 and 20 years. Among individuals with syncope in the past 2 years, beta-blocker therapy was associated with a 64% reduced risk (HR, 0.36; 95% CI, 0.18-0.72; P<.01). CONCLUSIONS: In LQTS, the timing and frequency of syncope, QTc prolongation, and sex were predictive of risk for aborted cardiac arrest and sudden cardiac death during adolescence. Among patients with recent syncope, beta-blocker treatment was associated with reduced risk.

Adolescent↗
PubMed2006

Polymorphism in the angiotensinogen gene, hypertension, and ethnic differences in the risk of recurrent coronary events.

The M235T mutation of the human angiotensinogen gene has been shown to be associated with elevated circulating angiotensinogen concentrations and essential hypertension. The frequencies of the 235T allele are significantly different in black and white subjects. We analyzed the independent contribution of the angiotensinogen M235T mutation to the development of recurrent coronary events (coronary-related death, nonfatal myocardial infarction, or unstable angina) in a cohort of 916 black (n=145) and white (n=771) postmyocardial infarction patients who were prospectively studied during an average follow-up of 28 months. The frequency of the 235T allele was significantly higher among black (82%) than among white (44%) patients (P<0.001). There was no evidence for Hardy-Weinberg disequilibrium. During follow-up, 41 cardiac events (28%) occurred in blacks and 197 (26%) in whites (P=0.49). Multivariate Cox proportional hazards regression analysis demonstrated that 235T homozygosity was independently associated with increased risk of coronary events among black (hazard ratio: 2.37; P=0.04) but not white (hazard ratio: 0.93; P=0.68) patients, with a significant ethnic-related interaction effect (P for the difference=0.04). Among hypertensive black patients, the TT genotype was associated with a 3.3-fold (P=0.02) increase in the risk of coronary events. Our findings suggest that homozygosity for the 235T mutation in the angiotensinogen gene is an independent risk factor for coronary events in black postmyocardial infarction patients. The presence of hypertension significantly augments the risk associated with this genetic mutation.

Aged↗
PubMed2006

Clinical course and risk stratification of patients affected with the Jervell and Lange-Nielsen syndrome.

INTRODUCTION: Data regarding risk factors and clinical course of patients affected with Jervell and Lange-Nielsen syndrome (JLNS), an autosomal recessive form of the congenital long-QT syndrome (LQTS), are limited to several reported cases and a retrospective analysis. METHODS AND RESULTS: We prospectively followed-up 44 JLNS patients from the U.S. portion of the International LQTS Registry and compared their clinical course with 2,174 patients with the phenotypically determined dominant form of LQTS (Romano-Ward syndrome [RWS]) and a subgroup of 285 patients with type 1 LQTS (LQT1). Mean (+/-SD) corrected QT interval (QTc) in the JLNS, RWS, and LQT1 groups were 548 +/- 73, 500 +/- 48, and 502 +/- 46 msec, respectively (P < 0.001). The cumulative rates of cardiac events from birth through age 40 among JLNS and RWS patients were 93% (mean [+/-SD] age: 5.0 +/- 7.0 years) and 54% (mean [+/-SD] age: 14.2 +/- 9.3 years), respectively (P < 0.001). The JLNS:RWS and JLNS:LQT1 adjusted hazard ratios (HR) for cardiac events were highest among patients with a baseline QTc > or = 550 msec (HR = 15.83 [P < 0.001] and 13.80 [P < 0.001], respectively). Among JLNS patients treated with beta-blockers, the cumulative probability of LQTS-related death was 35%; defibrillator therapy was associated with a 0% mortality rate during a mean (+/-SD) follow-up period of 4.9 +/- 3.4 years. CONCLUSIONS: Patients with JLNS experience a high rate of cardiac and fatal events from early childhood despite medical therapy. Defibrillator therapy appears to improve outcome in this high-risk population, although longer follow-up is needed to establish its long-term efficacy.

Adolescent↗
PubMed2006

Corrected QT variability in serial electrocardiograms in long QT syndrome: the importance of the maximum corrected QT for risk stratification.

OBJECTIVES: We evaluated the incremental prognostic information provided by multiple corrected QT (QTc) measurements on serial electrocardiograms (ECGs) in patients with the inherited long QT syndrome (LQTS). BACKGROUND: A baseline QTc of > or =500 ms has been shown to be associated with increased risk of cardiac events among LQTS patients. However, the value of QTc measurements on follow-up ECGs in risk assessment has not been determined. METHODS: The risk of a first LQTS-related cardiac event during adolescence was assessed in 375 patients enrolled in the International LQTS Registry for whom serial follow-up ECGs were recorded before age 10. RESULTS: The mean +/- SD difference between the minimum and maximum QTc values on serial ECGs recorded in study patients was 47 +/- 40 ms. The maximum QTc interval recorded before age 10 was the strongest predictor of cardiac events during adolescence (adjusted hazard ratio [HR] = 2.74; p < 0.001). Other follow-up QTc measures, including the baseline, the mean, and the most recent QTc interval recorded before age 10, were less significant risk factors. After adjusting for the maximum QTc value during follow-up, no significant association remained between the baseline QTc value and the risk of subsequent cardiac events (HR = 1.04; p = 0.91). CONCLUSIONS: In LQTS patients, there is a considerable variability in QTc measures in serial follow-up ECGs. The maximum QTc interval provides incremental prognostic information beyond the baseline measurement. We suggest that risk stratification in LQTS patients should include follow-up ECG data.

Adolescent↗
PubMed2006

Relations among renal function, risk of sudden cardiac death, and benefit of the implanted cardiac defibrillator in patients with ischemic left ventricular dysfunction.

Implanted cardioverter defibrillator therapy has been shown to be associated with a significant reduction in the risk of sudden cardiac death (SCD) in patients with ischemic left ventricular dysfunction. However, data on the relation between renal function and SCD in this population are limited, and the effect of renal dysfunction on the implanted cardioverter defibrillator benefit has not been determined. We performed a retrospective analysis of the outcome associated with renal dysfunction, as determined by the estimated glomerular filtration rate (eGFR), in patients enrolled in the Multicenter Automatic Defibrillator Implantation Trial-II. Multivariate analysis in conventionally treated patients showed that for each 10-U reduction in eGFR, the risk of all-cause mortality and SCD increased by 16% (p = 0.005) and 17% (p = 0.03), respectively. Defibrillator therapy was associated with a survival benefit in each eGFR category of > or = 35 ml/min/1.73 m2 (overall risk reduction for all-cause mortality 32%, p = 0.01 and for SCD 66%, p < 0.001). However, no implanted cardioverter defibrillator benefit was shown among patients with an eGFR < 35 ml/min/1.73 m2 (all-cause mortality hazard ratio 1.09, p = 0.84; SCD hazard ratio 0.95, p = 0.95). In conclusion, in patients with high-risk cardiac disease enrolled in the Multicenter Automatic Defibrillator Implantation Trial-II, a significant increase was found in the risk of SCD with declining renal function. Defibrillator therapy was associated with a significant survival benefit among the study patients with mild to moderate or no renal disease, but no benefit was shown among patients with more advanced renal dysfunction.

Aged↗
PubMed2006

Causes and consequences of heart failure after prophylactic implantation of a defibrillator in the multicenter automatic defibrillator implantation trial II.

BACKGROUND: Implantable cardioverter-defibrillator (ICD) therapy may be associated with an increased risk for heart failure (HF). The present study evaluated the frequency, causes, and consequences of HF after ICD implantation. METHODS AND RESULTS: We performed a retrospective analysis of the clinical factors and outcomes associated with postenrollment HF events in 1218 patients enrolled in the Multicenter Automatic Defibrillator Implantation Trial II. The adjusted hazard ratios (HRs) of ICD:conventional therapy for first and recurrent HF events were 1.39 (P=0.02) and 1.58 (P<0.001), respectively. The risk was increased among patients who received single-chamber or dual-chamber ICDs. Development of HF was associated with an increased mortality risk (HR, 3.80; P<0.001). Among patients who received a single-chamber ICD, there was a similar survival benefit before and after the development of HF (HR, 0.59 and 0.61, respectively; P=0.92 for difference), whereas among patients with dual-chamber devices, there was a significant reduction in survival benefit after HF (HR, 0.26 and 0.83, respectively; P=0.01 for difference). Within the defibrillator arm of the trial, patients who received life-prolonging therapy from the ICD had an increased risk for first and recurrent HF events (HR, 1.90; P=0.01 and 1.74; P<0.001, respectively). CONCLUSIONS: Patients with chronic ischemic heart disease who are treated with either single-chamber or dual-chamber ICDs have improved survival but an increased risk of HF. The present data suggest that ICD therapy transforms sudden death risk to a subsequent HF risk. These findings should direct more attention to the prevention of HF in patients who receive an ICD.

Adrenergic beta-Antagonists↗
PubMed2006

Cigarette smoking and the risk of supraventricular and ventricular tachyarrhythmias in high-risk cardiac patients with implantable cardioverter defibrillators.

INTRODUCTION: Nicotine elevates serum catecholamine concentration and is therefore potentially arrhythmogenic. However, the effect of cigarette smoking on arrhythmic risk in coronary heart disease patients is not well established. METHODS AND RESULTS: The risk of appropriate and inappropriate defibrillator therapy by smoking status was analyzed in 717 patients who received an implantable cardioverter defibrillator (ICD) in the Multicenter Automatic Defibrillator Implantation Trial-II. Compared with patients who had quit smoking before study entry (past smokers) and patients who had never smoked (never smokers), patients who continued smoking (current smokers) were significantly younger and generally had more favorable baseline clinical characteristics. Despite this, the adjusted hazard ratio (HR) for appropriate ICD therapy for fast ventricular tachycardia (at heart rates >or=180 b.p.m) or ventricular fibrillation was highest among current smokers (HR = 2.11 [95% CI 1.11-3.99]) and intermediate among past smokers (HR = 1.57 [95% CI 0.95-2.58]), as compared with never smokers (P for trend = 0.02). Current smokers also exhibited a higher risk of inappropriate ICD shocks (HR = 2.93 [95% CI 1.30-6.63]) than past (HR = 1.91 [95% CI 0.97-3.77]) and never smokers (P for trend = 0.008). CONCLUSIONS: In patients with ischemic left ventricular dysfunction, continued cigarette smoking is associated with a significant increase in the risk of life-threatening ventricular tachyarrhythmias and inappropriate ICD shocks induced by rapid supraventricular arrhythmias. Our findings stress the importance of complete smoking cessation in this high-risk population.

Aged↗
PubMed2006

Time dependence of defibrillator benefit after coronary revascularization in the Multicenter Automatic Defibrillator Implantation Trial (MADIT)-II.

OBJECTIVES: The study was designed to assess the effect of elapsed time from coronary revascularization (CR) on the benefit of the implantable cardioverter-defibrillator (ICD) and the risk of sudden cardiac death (SCD) in patients with ischemic left ventricular dysfunction. BACKGROUND: The ICD improves survival in appropriately selected high-risk cardiac patients by 30% to 54%. However, in the Coronary Artery Bypass Graft (CABG)-Patch trial no evidence of improved survival was shown among a similar population of patients in whom an ICD was implanted prophylactically at the time of elective CABG. METHODS: The outcome by time from CR was analyzed in 951 patients in whom a revascularization procedure was performed before enrollment in the Multicenter Automatic Defibrillator Implantation Trial (MADIT)-II. RESULTS: The adjusted hazard ratio (HR) of ICD versus conventional therapy was 0.64 (p = 0.01) among patients enrolled more than six months after CR, whereas no survival benefit with ICD therapy was shown among patients enrolled six months or earlier after CR (HR = 1.19; p = 0.76). In the conventional therapy group, the risk of cardiac death increased significantly with increasing time from CR (p for trend = 0.009), corresponding mainly to a six-fold increase in the risk of SCD among patients enrolled more than six months after CR. CONCLUSIONS: In patients with ischemic left ventricular dysfunction, the efficacy of ICD therapy after CR is time dependent, with a significant life-saving benefit in patients receiving device implantation more than six months after CR. The lack of ICD benefit when implanted early after CR may be related to a relatively low risk of SCD during this time period.

Aged↗
PubMed2006

Decrease in triglyceride level by bezafibrate is related to reduction of recurrent coronary events: a Bezafibrate Infarction Prevention substudy.

BACKGROUND: Fibrates were reported to be effective in reducing recurrent coronary events in coronary heart disease patients with elevated triglycerides. It is not known whether this effect is related to the extent of triglyceride reduction. METHODS: Participants comprised 3090 coronary heart disease patients enrolled in the Bezafibrate Infarction Prevention study, which showed a nonsignificant reduction (9.4%; P=0.26) in fatal or nonfatal myocardial infarction and sudden death during a mean follow-up time of 6.2 years. RESULTS: Significant reduction in triglyceride serum level was evident only among patients allocated to bezafibrate, ranging between 0.06 mmol/l (5 mg/dl) in the lowest decile of baseline triglycerides level and 0.68 mmol/l (60 mg/dl) in the highest baseline decile. The extent of triglyceride reduction with bezafibrate was significantly associated with the reduction of risk; relative risk reduction of 55% (hazards ratio: 0.45; 95% confidence interval: 0.24-0.84) was observed among patients with baseline triglycerides>or=2.26 mmol/l who reduced triglyceride level to >0.50 mmol/l (>44.3 mg/dl). In contrast, the risk of recurrent events among patients treated with bezafibrate and achieving less triglyceride reduction or failing to reduce triglyceride level was not significantly different from that of patients treated with placebo. CONCLUSION: Bezafibrate treatment was associated with significant risk reduction among coronary heart disease patients with elevated triglyceride levels that substantially reduced their triglyceride level with treatment.

Aged↗
PubMed2005

Relation between on-treatment increments in serum high-density lipoprotein cholesterol levels and cardiac mortality in patients with coronary heart disease (from the Bezafibrate Infarction Prevention trial).

This study evaluated the association between changes in serum levels of high-density lipoprotein (HDL) cholesterol that occur under bezafibrate therapy and cardiac mortality in patients with coronary heart disease (CHD) who were enrolled in the Bezafibrate Infarction Prevention trial. We compared serum levels of HDL cholesterol in 1,509 patients in tertiles of on-treatment increments with those of 1,517 patients in the placebo group. Long-term follow-up showed that cardiac mortality decreased significantly with increasing tertiles of on-treatment change in HDL cholesterol (9.5%, 6.6%, and 6.3% of patients tertiles 1, 2, and 3, respectively, died of cardiac causes, p for trend = 0.02). In multivariate analysis, the magnitude of on-treatment increment of HDL cholesterol was independently associated with a decreased risk of cardiac death (hazard ratio 1.05, 95% confidence interval 0.74 to 1.47, for tertile 1; hazard ratio 0.73, 95% confidence interval 0.50 to 1.07, for tertile 2; hazard ratio 0.65, 95% confidence interval 0.43 to 0.97, for tertile 3, compared with placebo-allocated patients, p for trend = 0.02). Analyzing the association with change in HDL cholesterol as a continuous variable showed that the risk of cardiac mortality was decreased by 27% for every 5-mg/dl increase in on-treatment change in HDL cholesterol (p <0.001). In conclusion, although a definite secondary prevention effect of bezafibrate could not be found when examining the intervention group as a whole, our findings are consistent with a possible independent association between an increase in HDL cholesterol with bezafibrate therapy and a decrease in cardiac mortality. In appropriately selected patients, monitoring short-term response to bezafibrate therapy through change in HDL cholesterol may indicate the potential long-term benefit of the drug.

Aged↗
PubMed2005

Nephropathy induced by contrast media: pathogenesis, risk factors and preventive strategies.

With the increasing use of contrast media in diagnostic and interventional procedures, nephropathy induced by contrast media has become the third leading cause of hospital-acquired acute renal failure. It is also associated with a significant risk of morbidity and death. The current understanding of the pathogenesis indicates that contrast-medium nephropathy is caused by a combination of renal ischemia and direct toxic effects on renal tubular cells. Patients with pre-existing renal insufficiency, diabetes mellitus and congestive heart failure are at highest risk. Risk factors also include the type and amount of contrast medium administered. Therapeutic prevention strategies are being extensively investigated, but there is still no definitive answer. In this article, we review the current evidence on the causes, pathogenesis and clinical course of contrast-medium nephropathy as well as therapeutic approaches to its prevention evaluated in clinical trials.

Antioxidants↗
PubMed2005

Serologic testing for celiac disease in young adults--a cost-effect analysis.

In recent years, there has been a marked increase in the diagnostic workups for celiac disease among military personnel, thereby significantly increasing overall laboratory testing expenditures and burden. We evaluated the serologic testing procedure in symptomatic young adults, using a "cost-effect" approach. We evaluated the serologic screening policy for celiac disease among serologically tested military personnel. The study population was divided into subgroups according to the clinical presentation prior to screening: isolated (low-risk) and combined complaints (high-risk). Sensitivity, specificity, and predictive values of serologic markers for celiac disease were evaluated. Cost analyses were based on diagnostic expenditures. Cost-effect ratio is expressed as cost per newly diagnosed patients, and cost minimization as cost per screened individuals. Five hundred thirty-eight military personnel were serologically tested for celiac disease. Eight new cases of celiac were diagnosed, all of whom belonged to the high-risk subgroup and tested positive for at least two positive serologic tests (tTG + EMA or tTG + AGA IgG + EMA). EMA Ab measured the highest sensitivity, specificity, and predictive values. Average screening expenditure was U.S. $287 per patient. The lowest cost-effect and cost minimization ratios were achieved by implementing a two-step single-marker screening protocol for high-risk subjects and one-step follow-up for low-risk subjects. Among patient population of young adults, selective diagnostic workup could result in cost-minimization without risking quality of diagnosis. From a cost-effect perspective, implemented screening procedures need to be dependent on subgroup: low-risk, clinical follow-up; and high-risk, serological testing for EMA and, only if positive, possibly a small-bowel biopsy.

Adult↗
PubMed2005

Sudden cardiac death without structural heart disease: update on the long QT and Brugada syndromes.

The long QT syndrome (LQTS) and the Brugada syndrome (BrS) are the most common genetic causes of malignant ventricular arrhythmias and sudden cardiac death in young patients with normal cardiac morphology. To date, more than 250 different mutations in seven genes have been identified as causing LQTS, whereas the only gene identified to be linked to BrS is SCN5A. In both syndromes, gene-specific mutations have been shown to be associated with specific phenotypic expressions. Risk stratification in LQTS and BrS is based mainly upon a constellation of electrocardiographic findings and a history of prior symptoms. In patients identified as high risk for arrhythmic mortality, the implantable cardioverter defibrillator is the most effective treatment and has been shown to provide near-complete protection during long-term follow-up.

Bundle-Branch Block↗
PubMed2005

Cost-effectiveness of implanted defibrillators in young people with inherited cardiac arrhythmias.

BACKGROUND: The implanted cardioverter-defibrillator (ICD) has been shown to improve survival in adult patients with high risk acquired cardiac disease, with a cost-effectiveness ratio in the range of $30,000 to $185,000 per quality-adjusted-life-year saved. However, data on the benefit and cost-effectiveness of device therapy in high-risk patients with inherited cardiac disorders are limited. METHODS: We developed two separate computer-based analytical models to compare non-ICD with ICD therapy in patients (age range: 10-75 years) with long QT syndrome (LQTS) and hypertrophic cardiomyopathy (HCM). In each disease entity patients were stratified into low-risk (no known risk factors); high-risk (known risk factors [primary prevention]); and very high-risk (prior near-fatal events [secondary prevention]). Net costs were defined as the difference between costs resulting from treatment of the disease and savings due to gained productivity attributable to prevention of sudden cardiac death. Outcome was defined as costs per quality-adjusted life-years saved. RESULTS: In LQTS, defibrillator therapy was shown to be cost effective in high-risk male patients (incremental cost-effectiveness ratio [ICER]=$3328 per quality-adjusted-life-year saved), and cost saving in high-risk females (ICER=$7102 gained per quality-adjusted-life-year saved) and very high-risk males and females (ICER=$15,483 and 19,393 gained per quality-adjusted-life-year saved, respectively). In HCM, defibrillator therapy was cost saving in both male and female high-risk (ICER=$17,892 and $17,526 gained per quality-adjusted-life-year saved, respectively) and very high-risk (ICER $22,944 and $22,329 gained per quality-adjusted-life-year saved, respectively) patients. Defibrillator therapy was not shown to be cost effective in low-risk patients with either LQTS or HCM (ICER in the range of $400,000 to $600,000 lost per quality-adjusted-life-year saved). Sensitivity analyses were consistent with the results in each risk group. CONCLUSIONS: In appropriately selected patients with inherited cardiac disorders, early intervention with ICD therapy is cost-effective to cost saving due to added years of gained productivity when the lifespan of an individual at risk is considered.

Arrhythmias, Cardiac↗