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Biomedical subjects

Barry J Maron

Publications and source records attributed to Barry J Maron.

At least 55 records · Page 3Linked to original sources
PubMed2005

Role of implantable cardioverter defibrillators in the treatment of hypertrophic cardiomyopathy.

Hypertrophic cardiomyopathy (HCM) is an important cardiovascular disease with sudden cardiac death as the most devastating presentation. Implantable cardioverter defibrillators (ICD) are the optimal therapy for prevention of sudden death from ventricular tachycardia or fibrillation of any cause. While there is no controversy with implanting ICDs in patients who have already survived a cardiac arrest, identifying high-risk patients for primary prevention in this disease remains a challenge. Implanting ICDs in patients with HCM is an important clinical consideration since many individuals could achieve normal or near-normal lifespans with this protection.

Editorial↗
PubMed2005

Spectrum and prognostic significance of arrhythmias on ambulatory Holter electrocardiogram in hypertrophic cardiomyopathy.

OBJECTIVES: The goal of this study was to assemble a profile and assess the significance of arrhythmias in a nontertiary-based hypertrophic cardiomyopathy (HCM) cohort. BACKGROUND: Hypertrophic cardiomyopathy is associated with arrhythmia-related consequences, particularly sudden death. Ventricular tachyarrhythmias on Holter electrocardiograms (ECG) have been reported as markers for sudden death in highly selected HCM populations. METHODS: We assessed the profile of ventricular and supraventricular ectopy and bradyarrhythmia on ambulatory 24-h Holter ECG and also related these findings to clinical outcome in 178 HCM patients. RESULTS: Of the 178 study patients, 157 (88%) had premature ventricular complexes (PVCs), including 21 (12%) with >/=500 PVCs, 74 (42%) had couplets, 67 (37%) had supraventricular tachycardia (SVT), and 56 (31%) had nonsustained ventricular tachycardia (NSVT). Mean number of PVCs was 330 +/- 763 (range 1 to 5,435) and increased with age (p < 0.01); NSVT was associated with greater left ventricular hypertrophy (p = 0.01) and severe symptoms (New York Heart Association functional classes III and IV) (p = 0.04); SVT occurred more commonly in patients with outflow obstruction (p = 0.02). Over a follow-up of 5.5 +/- 3.4 years, 11 (6%) patients died suddenly (annual mortality rate, 1.1%) including 5 patients with NSVT. For sudden death, NSVT on Holter ECG had negative and positive predictive values of 95% and 9%, and sensitivity and specificity of 45% and 69%, respectively. CONCLUSIONS: In this nontertiary-based HCM cohort, ventricular and supraventricular tachyarrhythmias were particularly frequent and demonstrated a broad spectrum on ambulatory (Holter) ECG. Paradoxically, despite such a highly arrhythmogenic substrate, sudden death events proved to be relatively uncommon. Ventricular tachyarrhythmias had a low positive and relatively high negative predictive value for sudden death in this HCM population.

Adolescent↗
PubMed2005

Acute and reversible cardiomyopathy provoked by stress in women from the United States.

BACKGROUND: A clinical entity characterized by acute but rapidly reversible left ventricular (LV) systolic dysfunction and triggered by psychological stress is emerging, with reports largely confined to Japan. METHODS AND RESULTS: Over a 32-month period, 22 consecutive patients with this novel cardiomyopathy were prospectively identified within a community-based practice in the Minneapolis-St. Paul, Minn, area. All patients were women aged 32 to 89 years old (mean 65+/-13 years); 21 (96%) were > or =50 years of age. The syndrome is characterized by (1) acute substernal chest pain with ST-segment elevation and/or T-wave inversion; (2) absence of significant coronary arterial narrowing by angiography; (3) systolic dysfunction (ejection fraction 29+/-9%), with abnormal wall motion of the mid and distal LV, ie, "apical ballooning"; and (4) profound psychological stress (eg, death of relatives, domestic abuse, arguments, catastrophic medical diagnoses, devastating financial or gambling losses) immediately preceding and triggering the cardiac events. A significant proportion of patients (37%) had hemodynamic compromise and required vasopressor agents and intra-aortic balloon counterpulsation. Each patient survived with normalized ejection fraction (63+/-6%; P<0.001) and rapid restoration to previous functional cardiovascular status within 6+/-3 days. In 95%, MRI identified diffusely distributed segmental wall-motion abnormalities that encompassed LV myocardium in multiple coronary arterial vascular territories. CONCLUSIONS: A reversible cardiomyopathy triggered by psychologically stressful events occurs in older women and may mimic evolving acute myocardial infarction or coronary syndrome. This condition is characterized by a distinctive form of systolic dysfunction that predominantly affects the distal LV chamber and a favorable outcome with appropriate medical therapy.

Acute Disease↗
PubMed2005

Glycogen storage diseases presenting as hypertrophic cardiomyopathy.

BACKGROUND: Unexplained left ventricular hypertrophy often prompts the diagnosis of hypertrophic cardiomyopathy, a sarcomere-protein gene disorder. Because mutations in the gene for AMP-activated protein kinase gamma2 (PRKAG2) cause an accumulation of cardiac glycogen and left ventricular hypertrophy that mimics hypertrophic cardiomyopathy, we hypothesized that hypertrophic cardiomyopathy might also be clinically misdiagnosed in patients with other mutations in genes regulating glycogen metabolism. METHODS: Genetic analyses performed in 75 consecutive unrelated patients with hypertrophic cardiomyopathy detected 40 sarcomere-protein mutations. In the remaining 35 patients, PRKAG2, lysosome-associated membrane protein 2 (LAMP2), alpha-galactosidase (GLA), and acid alpha-1,4-glucosidase (GAA) genes were studied. RESULTS: Gene defects causing Fabry's disease (GLA) and Pompe's disease (GAA) were not found, but two LAMP2 and one PRKAG2 mutations were identified in probands with prominent hypertrophy and electrophysiological abnormalities. These results prompted the study of two additional, independent series of patients. Genetic analyses of 20 subjects with massive hypertrophy (left ventricular wall thickness, > or =30 mm) but without electrophysiological abnormalities revealed mutations in neither LAMP2 nor PRKAG2. Genetic analyses of 24 subjects with increased left ventricular wall thickness and electrocardiograms suggesting ventricular preexcitation revealed four LAMP2 and seven PRKAG2 mutations. Clinical features associated with defects in LAMP2 included male sex, severe hypertrophy, early onset (at 8 to 17 years of age), ventricular preexcitation, and asymptomatic elevations of two serum proteins. CONCLUSIONS: LAMP2 mutations typically cause multisystem glycogen-storage disease (Danon's disease) but can also present as a primary cardiomyopathy. The glycogen-storage cardiomyopathy produced by LAMP2 or PRKAG2 mutations resembles hypertrophic cardiomyopathy but is distinguished by electrophysiological abnormalities, particularly ventricular preexcitation.

AMP-Activated Protein Kinases↗
PubMed2005

Cardioverter-defibrillator implantation in high-risk patients with hypertrophic cardiomyopathy.

BACKGROUND: Implantable cardioverter-defibrillators (ICDs) are used with increasing frequency in hypertrophic cardiomyopathy (HCM) patients of all ages for primary and secondary sudden death prevention. Concerns may arise regarding the safety of device implantation because of unique clinical and phenotypic expressions of HCM. OBJECTIVES: The purpose of this study was to assess the efficacy and safety of ICD placement in high-risk patients with HCM. METHODS: We analyzed the experience with ICDs and transvenous lead systems in 75 consecutive HCM patients at the Minneapolis Heart Institute from 1993 to 2004. RESULTS: The age of the study group patients was 12 to 79 years (mean 36 +/- 16). Patients received ICDs for secondary (n = 4, after cardiac arrest) or primary prevention (n = 71, with > or = 1 risk factor). Thirty-one patients demonstrated disease features that potentially impacted methodology and safety of the implant procedure, most commonly massive left ventricular (LV) hypertrophy and outflow obstruction > or = 50 mmHg. There were no procedure-related deaths; defibrillator implants were successful and uneventful in 71 of 75 patients (95%). In 3 of the 75 patients (4%), defibrillation was unsuccessful because of high thresholds, associated with extreme hypertrophy (wall thickness > 45 mm) and/or ongoing amiodarone therapy. In two of these patients, thoracotomy with epicardial lead placement achieved successful defibrillation; ICD therapy was abandoned in the other patient. CONCLUSION: ICD placement in children and adults with HCM is generally safe and effective. However, in some patients with massive LV hypertrophy and/or prior administration of amiodarone, transvenous defibrillation proved difficult, and epicardial lead placement was required. High-energy ICD devices and defibrillation threshold testing are recommended for most high-risk HCM patients.

Adolescent↗
PubMed2005

Efficacy of bystander cardiopulmonary resuscitation and out-of-hospital automated external defibrillation as life-saving therapy in commotio cordis.

We report a child who sustained commotio cordis after being struck by a baseball, and offer documentation of the advantages of having readily available access to bystander cardiopulmonary resuscitation (CPR) and an automated external defibrillator (AED). We suggest that communities and school districts reexamine the need for accessible AEDs and CPR-trained coaches at organized sporting events for children.

Adolescent↗
PubMed2005

Role of streptomycin-sensitive stretch-activated channel in chest wall impact induced sudden death (commotio cordis).

INTRODUCTION: Deaths secondary to low-energy impacts to the precordium in young individuals (commotio cordis) have been reported with increasing frequency. In a swine model, baseball impacts induce ventricular fibrillation when directed at the center of the left ventricle during the vulnerable portion of repolarization just prior to the T-wave peak. It has been hypothesized that activation of stretch-sensitive channels could be crucial for this electrophysiological phenomenon. In this study, a nonselective stretch-activated cation channel was pharmacologically blocked prior to chest blows to determine whether this channel represents a possible pathway by which commotio cordis events occur. METHODS: In a randomized and blinded experiment, 12 swine (mean 17.1 +/- 2.5 kg) received either 2-g streptomycin intramuscularly (mean serum concentration 115 +/- 18 muM) or sterile water prior to chest impact. Each animal received six precordial impacts with a baseball propelled at 40 mph. RESULTS: There was no significant difference in the frequency of induced VF in the animals administered streptomycin (10 of 19 impacts: 53%) compared to those control animals receiving only sterile water (10 of 31: 32%) (P = 0.15). However, the magnitude of ST segment elevation was less in the streptomycin-treated animals (19 +/- 19 mV) versus controls (61 +/- 46 mV) (P = 0.015). CONCLUSION: Streptomycin did not alter the frequency of ventricular fibrillation in our commotio cordis model, indicating that the stretch-activated channel is not implicated in the genesis of chest blow-induced cardiac arrest. However, streptomycin did reduce ST elevation following impact suggesting that the stretch-activated channel may play a role in ST segment elevation following chest wall blows.

Animals↗
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↗
PubMed2004

Proposal for contemporary screening strategies in families with hypertrophic cardiomyopathy.

Screening families with hypertrophic cardiomyopathy (HCM) presents a common clinical problem to practicing cardiologists, internists, and pediatricians. The traditional recommended strategy for screening relatives in most HCM families calls for such evaluations with echocardiography (and electrocardiogram [ECG]) on a 12- to 18-month basis, usually beginning at about age 12 years. If such tests show no evidence of left ventricular hypertrophy, i.e., without one or more segments of abnormally increased wall thickness by the time full growth and maturation is achieved (at the age of about 18 to 21 years), it has been customary practice to conclude that HCM is probably absent and reassure family members accordingly that further echocardiographic testing is unnecessary. However, novel developments in the definition of the genetic causes of HCM have defined both substantial molecular diversity and heterogeneity of the disease expression including (in some relatives) incomplete phenotypic penetrance and delayed, late-onset left ventricular hypertrophy well into adulthood. These observations have unavoidably reshaped the customary practice of genetic counseling and established a new proposed paradigm for clinical family screening of HCM families. Therefore, in the absence of genetic testing, strong consideration should be given to extending diagnostic serial echocardiography past adolescence and into mid-life for those family members with a normal echocardiogram and ECG. Of note, recent developments in laboratory DNA-based diagnosis for HCM could potentially avoid the necessity for serial echocardiography in many such relatives.

Age of Onset↗
PubMed2004

The case for surgery in obstructive hypertrophic cardiomyopathy.

Relief of left ventricular (LV) outflow obstruction in patients with hypertrophic cardiomyopathy (HCM) and disabling symptoms refractory to maximum medical management has historically been a surgical problem. Surgical septal myectomy permanently abolishes systolic anterior motion of the mitral valve and mitral regurgitation, while normalizing LV pressures and wall stress. Also, these salutary goals are achieved without encumbering patients with post-procedural devices (e.g., pacemakers or defibrillators) or creating potentially arrhythmogenic substrates, as may occur with alcohol septal ablation. Procedural morbidity and mortality risk with myectomy is similar to, and in some institutions less than those for alcohol septal ablation. Over four decades, reports from numerous centers worldwide have consistently and unequivocably documented the benefits of surgery on hemodynamic and functional state, restoring normal and acceptable quality of life to patients of all ages by largely reversing the complications of heart failure. Long-term survival after myectomy is similar to that of the general population and superior to non-operated patients with obstruction. The LV outflow tract morphology in HCM is heterogeneous and not uncommonly includes congenital anomalies of the mitral valve apparatus for which the surgeon has the flexibility to adapt the repair, often employing an extended myectomy. In the current atmosphere of increasing and perhaps excessive enthusiasm for newer catheter-based interventions, it is a critical time to promote and re-emphasize that surgery is the time-honored (and presently the most effective) treatment strategy for relieving heart failure-related disability resulting from dynamic LV outflow obstruction in HCM, and is the primary treatment option for this subgroup of severely symptomatic drug-refractory patients.

Cardiomyopathy, Hypertrophic↗
PubMed2004

Impact of physical deconditioning on ventricular tachyarrhythmias in trained athletes.

OBJECTIVES: The purpose of this research was to evaluate the impact of athletic training and, in particular, physical deconditioning, on frequent and/or complex ventricular tachyarrhythmias assessed by 24-h ambulatory (Holter) electrocardiogram (ECG). BACKGROUND: Sudden deaths in athletes are usually mediated by ventricular tachyarrhythmias. METHODS: Twenty-four hour ambulatory ECGs were recorded at peak training and after a deconditioning period of 19 +/- 6 weeks (range, 12 to 24 weeks) in a population of 70 trained athletes selected on the basis of frequent and/or complex ventricular tachyarrhythmias (i.e., > or =2,000 premature ventricular depolarization [PVD] and/or > or =1 burst of non-sustained ventricular tachycardia [NSVT]/24 h). RESULTS: A significant decrease in the frequency and complexity of ventricular arrhythmias was evident after deconditioning: PVDs/24 h: 10,611 +/- 10,078 to 2,165 +/- 4,877 (80% reduction; p < 0.001) and NSVT/24 h: 6 +/- 22 to 0.5 +/- 2, (90% reduction; p = 0.04). In 50 of the 70 athletes (71%), ventricular arrhythmias decreased substantially after detraining (to <500 PVDs/24 h and no NSVT). Most of these athletes with reduced arrhythmias did not have structural cardiovascular abnormalities (37 of 50; 74%). Over the 8 +/- 4-year follow-up period, each of the 70 athletes survived without cardiac symptoms. CONCLUSIONS: Frequent and/or complex ventricular tachyarrhythmias in trained athletes (with and without cardiovascular abnormalities) are sensitive to brief periods of deconditioning. In athletes with heart disease, the resolution of such arrhythmias with detraining may represent a mechanism by which risk for sudden death is reduced. Conversely, in athletes without cardiovascular abnormalities, reduction in frequency of ventricular tachyarrhythmias and the absence of cardiac events in the follow-up support the benign clinical nature of these rhythm disturbances as another expression of athlete's heart.

Adolescent↗
PubMed2004

Prevalence of hypertrophic cardiomyopathy in a population-based sample of American Indians aged 51 to 77 years (the Strong Heart Study).

Recognition of the frequency with which hypertrophic cardiomyopathy (HC) occurs in the general population is critical to understanding its demographics and public health implications. However, few data are available for estimating HC prevalence in large populations of different age strata and ethnic or racial groups. The Strong Heart Study is a prospective, population-based epidemiologic survey of cardiovascular disease in residents of 13 geographically diverse American Indian communities. The study population was comprised of 3,501 subjects with echocardiograms performed in 1993 and 1995 to determine the prevalence of HC in middle-aged and older adult populations. Evidence of the HC phenotype was present in 8 previously undiagnosed patients (0.23%; 2 of 1,000) based on a left ventricular (LV) wall thickness >/=15 mm and a nondilated cavity that was not associated with another cardiac disease and was sufficient to produce the magnitude of hypertrophy evident. Ages were 51 to 77 years (mean 64 +/- 9). Four subjects were men and 4 were women, with prevalences by gender of 0.3% (3 of 1,000) and 0.18% (1.8 of 1,000), respectively. Maximum LV thicknesses were 19 to 29 mm (mean 21 +/- 3). Two subjects had mitral valve systolic anterior motion, which was sufficient to produce LV outflow obstruction at rest in 1 patient. Different electrocardiographic abnormalities were present in 5 subjects. In conclusion, clinically unrecognized HC was present in 1:500 of an aging American Indian cohort. This prevalence was similar to that reported in other general populations comprised of younger subjects of other races, offering evidence that HC is a relatively common genetic disease with widespread occurrence within the United States.

Aged↗