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Biomedical subjects

Barry J Maron

Publications and source records attributed to Barry J Maron.

At least 19 recordsLinked to original sources

Commotio cordis--sudden cardiac death with chest wall impact.

Commotio cordis (CC), sudden death as a result of a blunt, often innocent-appearing chest wall blow, is being reported with increasing frequency. The clinical spectrum is diverse; however, a substantial number of cases occur in youth athletics. In events that occur during sport, victims are struck by projectiles regarded as standard implements of the game. Sudden death is instantaneous and victims are most often found in ventricular fibrillation (VF). Overall survival is poor; however, successful resuscitation can be achieved with early defibrillation. Autopsy is notable for the absence of any significant cardiac or thoracic injury. Development of an experimental model has allowed for substantial insights into the underlying mechanisms of sudden death. In anesthetized juvenile swine, induction of VF is instantaneous following chest wall blows occurring during a vulnerable window before the T wave peak. Crucial variables including the velocity of impact, impact location, and hardness of the impact object have been identified. Rapid left ventricular (LV) pressure rise following chest impact likely results in activation of ion channels via mechano-electric coupling. The generation of inward current via mechano-sensitive ion channels likely results in augmentation of repolarization and nonuniform myocardial activation, and is the cause of premature ventricular depolarizations that are triggers of VF in CC. While softer-than-standard safety baseballs reduce the risk of CC, commercially available chest protectors are ineffective in preventing CC. The development of more effective chest protectors and more widespread use of automated external defibrillators at youth sporting events are needed.

Death, Sudden, Cardiac↗

Importance of the autonomic nervous system in an experimental model of commotio cordis.

BACKGROUND: Young athletes may die suddenly when they are struck in the chest (commotio cordis). Proposed mechanisms of sudden death in commotio cordis include hypervagatonia and activation of the sympathetic nervous system. In an experimental model of commotio cordis, the importance of the sympathetic and parasympathetic nervous system in the initiation of ventricular fibrillation was evaluated. MATERIAL/METHODS: Juvenile swine weighing between 8 and 12 kg were anesthetized with ketamine and isoflurane and placed prone in a sling. Twenty animals were randomized to pretreatment with placebo or sympathetic and parasympathetic blockade. Chest blows were guided by echocardiography to the center of the left ventricle and animals received 1-3 strikes with a regulation baseball propelled at 30 mph and timed to impact 10-30 ms prior to the peak of the T-wave. RESULTS: With 17 impacts in 10 autonomically blocked animals, 6 episodes of ventricular fibrillation were seen; with 15 impacts in 10 control animals, ventricular fibrillation occurred 6 times. There was also no significant difference between the groups in occurrence of nonsustained polymorphic ventricular tachycardia or ST elevation. Transient complete heart block was less commonly seen in animals treated with autonomic blockade, although this did not achieve statistical significance. CONCLUSIONS: In this experimental model of commotio cordis, autonomic blockade did not affect the frequency of sudden cardiac death, polymorphic ventricular tachycardia or ST segment elevation. Thus, vagotonic and sympathetic surges likely do not contribute to the syndrome of sudden death due to chest blows in young people and athletes.

Animals↗

Hypertrophic cardiomyopathy is predominantly a disease of left ventricular outflow tract obstruction.

BACKGROUND: Nonobstructive hypertrophic cardiomyopathy (HCM) has been regarded as the predominant hemodynamic form of the disease on the basis of assessment of outflow gradient under resting conditions. We sought to prospectively define the prevalence, clinical profile, and significance of left ventricular (LV) outflow tract obstruction under resting conditions and with physiological exercise in a large HCM cohort. METHODS AND RESULTS: We prospectively analyzed 320 consecutive HCM patients (age, 47+/-17 years), measuring LV outflow gradient at rest, with Valsalva maneuver, and with exercise echocardiography. LV outflow obstruction was present at rest and/or with exercise in 225 patients (70%); 119 had rest gradients > or = 50 mm Hg and were not exercised. Of the other 201 patients with gradients < 50 mm Hg at rest (average, 4+/-9 mm Hg), 106 developed mechanical obstruction to LV outflow resulting from mitral valve-septal contact after exercise (80+/-43 mm Hg), including 76 with marked gradients > or = 50 mm Hg and 46 with heart failure symptoms. The remaining 95 patients (30%) had no or small gradients (< 30 mm Hg) both at rest and with exercise. Valsalva maneuver underestimated the presence and magnitude of exercise-induced obstruction. CONCLUSIONS: Among those patients who come to clinical evaluation, HCM is a predominantly obstructive disease in which LV outflow gradients, frequently associated with heart failure symptoms and often identified only with exercise, are evident in most patients (ie, 70%). Identification of LV outflow obstruction with exercise echocardiography may broaden management options in HCM by identifying symptomatic patients not otherwise regarded as potential candidates for septal reduction therapy. Assessment of subaortic gradients with exercise should be a routine component of the evaluation of HCM patients without outflow obstruction under resting conditions.

Adult↗

Determinants for clinical diagnosis of hypertrophic cardiomyopathy.

Although hypertrophic cardiomyopathy (HC) occurs in 1 of 500 adults, most cardiology practices treat relatively few patients with HC, suggesting that many affected patients evade clinical recognition. Determining the clinical circumstances under which HC is identified will provide clues to its under-recognition. Clinical triggers leading to diagnostic echocardiograms were analyzed in 711 consecutive patients with HC. In most (384 [54%]), HC was initially suspected only after the onset of cardiac symptoms or acute cardiac events. In a substantial minority (327 [46%]), HC was recognized while patients were asymptomatic, including 225 (32%) by routine medical evaluations, in 27 of whom (4%) HC was recognized during preparticipation examinations for competitive sports or other activities. Women, older patients (age > or =50 years), and those with outflow obstruction at rest (gradient > or =30 mm Hg) were more likely suspected to have HC by virtue of cardiac symptoms or events (p <0.0001). Conversely, patients with extreme hypertrophy (wall thickness > or =30 mm) and those at high risk for sudden death were more often asymptomatic and identified by routine or family screenings (p <0.0001 and p = 0.004, respectively). Patients who subsequently died of heart failure or experienced embolic stroke were more often identified by virtue of symptoms or acute events (p = 0.03). In conclusion, although most patients with HC were recognized clinically only after overt disease manifestations, a substantial minority were diagnosed by routine examinations while asymptomatic, including an important subset of patients with HC recognized solely because of findings on sports preparticipation screening. These data underscore the need for heightened awareness and clinical suspicion of HC to increase the number of diagnosed patients, including many who may be at high risk for sudden death.

Adult↗

Brief communication: Cardiovascular screening practices of major North American professional sports teams.

BACKGROUND: Customary preparticipation screening strategies to detect heart disease in professional athletes have not been examined systematically. OBJECTIVE: To describe the current preparticipation cardiovascular screening process for professional athletes. DESIGN: Screening practices surveyed by questionnaire. SETTING: The 122 major professional sports teams in North America. PARTICIPANTS: Athletic trainers and team physicians. MEASUREMENTS: League recommendations for history taking and physical examination and noninvasive testing were compared with screening recommendations from an American Heart Association consensus panel. RESULTS: All 122 teams have team physicians perform annual screening, including family and personal history taking (100%), physical examination (100%), and lipid panels (108 of 122 [89%]). Diagnostic testing by using electrocardiography was substantially more common (112 of 122 [92%]) than exercise testing and stress echocardiography (21 of 122 [17%]) or echocardiography (16 of 122 [13%]). League recommendations for history taking and physical examination were most complete for Major League Baseball and the National Hockey League, meeting 10 of 12 and 8 of 12 American Heart Association recommendations, respectively. The most comprehensive cardiovascular screening using echocardiography is confined to selected, elite professional basketball players. LIMITATIONS: Data were self-reported by team representatives. CONCLUSIONS: A variety of nonstandardized preparticipation screening strategies for the detection of cardiovascular disease, varying considerably in scope, constitute customary practice among professional sports teams.

American Heart Association↗

Prognostic significance of left atrial size in patients with hypertrophic cardiomyopathy (from the Italian Registry for Hypertrophic Cardiomyopathy).

This study assessed left atrial (LA) dimension as a potential predictor of outcome in hypertrophic cardiomyopathy (HC). From the Italian Registry for Hypertrophic Cardiomyopathy, 1,491 patients (mean age 47 +/- 17 years; 61% men; 19% obstructive), followed for 9.4 +/- 7.4 years after the initial echocardiographic evaluation, constituted the study group. The mean LA transverse dimension was 43 +/- 9 mm and was larger in patients with severe symptoms (48 +/- 9 mm for New York Heart Association classes III and IV vs 42 +/- 9 mm for classes I and II, p <0.001), atrial fibrillation (47 +/- 9 vs 42 +/- 8 mm in sinus rhythm, p <0.001), and left ventricular outflow obstruction (46 +/- 9 mm for >or=30 mm Hg at rest vs 42 +/- 9 mm for <30 mm Hg at rest, p <0.001). On univariate analysis, each 5-mm increase in LA size was associated with a hazard ratio (HR) of 1.2 for all-cause mortality (p <0.0001). On multivariate analysis, a LA dimension >48 mm (the 75th percentile) had a HR of 1.9 for all-cause mortality (p = 0.008), 2.0 for cardiovascular death (p = 0.014), and 3.1 for death related to heart failure (p = 0.008) but was unassociated with sudden death (p = 0.81). Similar results were obtained after the exclusion of patients with atrial fibrillation (HR 1.7, p = 0.008) or outflow obstruction (HR 1.8, p = 0.003). The predictive power of LA dimension >48 mm was also validated in an independent HC cohort from the United States, with similar HRs (1.8 for all-cause mortality, p = 0.019). In conclusion, in a large cohort of patients with HC from a nationwide registry, a marked increase in LA dimension were predictive of long-term outcome, independent of co-existent atrial fibrillation or outflow obstruction. LA dimension is a novel and independent marker of prognosis in HC, particularly relevant to the identification of patients at risk for death related to heart failure.

Adult↗

Evidence for efficacy of the Italian national pre-participation screening programme for identification of hypertrophic cardiomyopathy in competitive athletes.

AIMS: Hypertrophic cardiomyopathy (HCM) is a leading cause of sudden death in young athletes, and substantial interest persists in strategies for timely identification. We assessed the diagnostic efficacy of Italian pre-participation screening programme with 12-lead ECG (in addition to history and physical examination) for identification of HCM. METHODS AND RESULTS: Four thousand four hundred and fifty members of the Italian national teams, initially judged eligible for competition as a result of systematic pre-participation screening across Italy, subsequently underwent clinical and echocardiographic examination at the Institute of Sports Medicine and Science (Rome) to assess the presence of previously undetected HCM. None of the 4450 athletes showed clinical evidence of HCM. Other cardiac abnormalities were detected in only 12 athletes, including myocarditis (n=4), mitral valve prolapse (n=3), Marfan's syndrome (n=2), aortic regurgitation with bicuspid valve (n=2), and arrhythmogenic right ventricular cardiomyopathy (n=1). In addition, echocardiography identified four athletes with borderline left ventricular wall thickness (i.e. 13 mm) in the 'grey zone' of overlap between HCM and athlete's heart. In two of these athletes, subsequent genetic analysis or clinical changes over an average 8-year follow-up resulted, respectively, in a definitive or possible diagnosis of HCM. CONCLUSION: The Italian national pre-participation screening programme including 12-lead ECG appears to be efficient in identifying young athletes with HCM, leading to their timely disqualification from competitive sports. These data also suggest that routine echocardiography is not an obligatory component of broad-based screening programmes designed to identify young athletes with HCM.

Adolescent↗

Prevalence, clinical profile, and significance of left ventricular remodeling in the end-stage phase of hypertrophic cardiomyopathy.

BACKGROUND: End stage (ES) is a recognized part of the hypertrophic cardiomyopathy (HCM) disease spectrum. Frequency, clinical profile and course, and treatment strategies in these patients remain incompletely defined. METHODS AND RESULTS: Three HCM cohorts comprised 1259 patients, including 44 (3.5%) characterized as ES with systolic dysfunction (ejection fraction <50% at rest; range 15% to 49%). ES developed at a wide age range (14 to 74 years), with 45% of patients < or = 40 years old. Although 29 patients (66%) died of progressive heart failure, had sudden death events, or underwent heart transplantation, 15 (34%) survived with medical management over 3+/-3 years. Duration from onset of HCM symptoms to ES identification was considerable (14+/-10 years), but ES onset to death/transplantation was brief (2.7+/-2 years). ES occurred with similar frequency in patients with or without prior myectomy (P=0.84). Appropriate defibrillator interventions were 10% per year in patients awaiting donor hearts. Most ES patients (n=23; 52%) showed substantial left ventricular (LV) remodeling with cavity dilatation. Less complete remodeling occurred in 21 patients (48%), including 5 with persistence of a nondilated and markedly hypertrophied LV. Pathology and magnetic resonance imaging showed extensive (transmural) fibrosis in 9 of 11 ES patients. At initial evaluation, patients who developed ES were younger with more severe symptoms, had a larger LV cavity, and more frequently had a family history of ES than other HCM patients. CONCLUSIONS: ES of nonobstructive HCM has an expanded and more diverse clinical expression than previously appreciated, including occurrence in young patients, heterogeneous patterns of remodeling, frequent association with atrial fibrillation, and impaired LV contractility that precedes cavity dilatation, wall thinning, and heart failure symptoms. ES is an unfavorable complication (mortality rate 11% per year) and a sudden death risk factor; it requires vigilance to permit timely recognition and the necessity for defibrillator implantation and heart transplantation.

Adolescent↗

Novel locus for an inherited cardiomyopathy maps to chromosome 7.

BACKGROUND: Genetic mutations are the most common cause of hypertrophic cardiomyopathy (HCM) and an increasingly recognized cause of dilated cardiomyopathy. Autosomal dominant HCM is caused by mutations in sarcomere proteins; such mutations are not universally present, however, and fail to account for &40% of cases of phenotypic HCM. To add further complexity, other genetic origins can mimic the gross clinical phenotype of HCM, and mutations in sarcomere genes have been demonstrated to cause dilated cardiomyopathy. METHODS AND RESULTS: To explore novel genetic causes of inherited cardiomyopathies, genome-wide linkage analysis was used to study one kindred (4 generations, 32 individuals) with predominant clinical features of left ventricular hypertrophy in addition to cardiac dilation, end-stage heart failure, and sudden death. Of note, histopathology from 2 family members did not demonstrate myocyte disarray and fibrosis, indicating that this phenotype is not typical sarcomere mutation HCM. Direct DNA sequencing was performed on sarcomere genes known to cause HCM and dilated cardiomyopathy, and no mutations were identified. Linkage was then established to a novel locus on chromosome 7 (7p12.1-7q21). A maximum 2-point logarithm of odds score of 4.11 was obtained. Recombination events refine the disease interval between D7S506 and D7S3314, corresponding to a distance of 27.2 megabases. CONCLUSIONS: The discovery of a novel genetic locus in this family provides more evidence that molecular pathways leading to inherited cardiac hypertrophy extend beyond the sarcomere. Identification of the causal gene mutation and additional genotype-phenotype correlation studies will provide fundamental insight into mechanisms of cardiac remodeling.

Alleles↗

Contemporary definitions and classification of the cardiomyopathies: an American Heart Association Scientific Statement from the Council on Clinical Cardiology, Heart Failure and Transplantation Committee; Quality of Care and Outcomes Research and Functional Genomics and Translational Biology Interdisciplinary Working Groups; and Council on Epidemiology and Prevention.

Classifications of heart muscle diseases have proved to be exceedingly complex and in many respects contradictory. Indeed, the precise language used to describe these diseases is profoundly important. A new contemporary and rigorous classification of cardiomyopathies (with definitions) is proposed here. This reference document affords an important framework and measure of clarity to this heterogeneous group of diseases. Of particular note, the present classification scheme recognizes the rapid evolution of molecular genetics in cardiology, as well as the introduction of several recently described diseases, and is unique in that it incorporates ion channelopathies as a primary cardiomyopathy.

American Heart Association↗

Failure of commercially available chest wall protectors to prevent sudden cardiac death induced by chest wall blows in an experimental model of commotio cordis.

OBJECTIVE: Sudden cardiac death that results from chest wall blows (commotio cordis) the second leading cause of death in young athletes. Most events are caused by blows from projectiles, such as baseballs or lacrosse balls, with a substantial proportion occurring despite the use of a chest protector. In the present experiment, we tested the effectiveness of commercially available chest protectors in preventing ventricular fibrillation (VF) that results from chest wall strikes with baseballs and lacrosse balls. METHODS: Twelve different baseball or lacrosse chest protectors were evaluated in juvenile swines that were subjected to 40-mph baseball or lacrosse ball blows to the precordium during the vulnerable period of repolarization for VF and were compared with control impacts without chest protectors. Seven baseball chest protectors were hit by regulation baseballs, and 5 lacrosse chest protectors were tested by blows with standard lacrosse balls. Each animal received 2 chest blows for each protector and 2 control impacts without a chest protector, with the sequence of impacts assigned randomly. RESULTS: VF was elicited by 12 (32%) of 37 strikes in control animals without baseball chest protectors. None of the baseball chest wall protectors tested were shown to decrease significantly the occurrence of VF when compared with controls. VF was elicited by 11 (46%) of 24 strikes in control animals without lacrosse chest protectors. None of the lacrosse chest wall protectors tested decreased significantly the occurrence of VF when compared with controls. CONCLUSION: In our experimental animal model of commotio cordis, commercially available baseball and lacrosse chest wall protectors were ineffective in protecting against VF that was triggered by chest blows and, by inference, sudden cardiac death. Improvements in materials and design of chest wall barriers are necessary to reduce the occurrence of these tragic events and make the athletic field safer for youths.

Adolescent↗

Identical twins with hypertrophic cardiomyopathy and apical aneurysm.

Left ventricular apical aneurysms, in absence of coronary artery disease, occur in approximately 1% of patients with hypertrophic cardiomyopathy (HC). Identical twins, age 44 years, are presented with HC and identical LV morphology, including apical aneurysms. These cases demonstrate a genetic predisposition to the development of apical aneurysm, as well as overall LV morphology, in patients with HC.

Adult↗

The congenital long QT syndrome and implications for young athletes.

The congenital long QT syndrome (LQTS) is caused by cardiac ion channel mutations, which predispose young individuals to sudden cardiac death often related to exercise. The issue of LQTS and sports participation has received significant publicity due to reports of sudden death in young competitive athletes. This article reviews the pathophysiology, clinical characteristics, and management of LQTS in the physically active and athletic population.

Adolescent↗