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Barry J Maron

Publications and source records attributed to Barry J Maron.

At least 73 records · Page 4Linked to original sources
PubMed2004

Recommendations for physical activity and recreational sports participation for young patients with genetic cardiovascular diseases.

A group of relatively uncommon but important genetic cardiovascular diseases (GCVDs) are associated with increased risk for sudden cardiac death during exercise, including hypertrophic cardiomyopathy, long-QT syndrome, Marfan syndrome, and arrhythmogenic right ventricular cardiomyopathy. These conditions, characterized by diverse phenotypic expression and genetic substrates, account for a substantial proportion of unexpected and usually arrhythmia-based fatal events during adolescence and young adulthood. Guidelines are in place governing eligibility and disqualification criteria for competitive athletes with these GCVDs (eg, Bethesda Conference No. 26 and its update as Bethesda Conference No. 36 in 2005). However, similar systematic recommendations for the much larger population of patients with GCVD who are not trained athletes, but nevertheless wish to participate in any of a variety of recreational physical activities and sports, have not been available. The practicing clinician is frequently confronted with the dilemma of designing noncompetitive exercise programs for athletes with GCVD after disqualification from competition, as well as for those patients with such conditions who do not aspire to organized sports. Indeed, many asymptomatic (or mildly symptomatic) patients with GCVD desire a physically active lifestyle with participation in recreational and leisure-time activities to take advantage of the many documented benefits of exercise. However, to date, no reference document has been available for ascertaining which types of physical activity could be regarded as either prudent or inadvisable in these subgroups of patients. Therefore, given this clear and present need, this American Heart Association consensus document was constituted, based largely on the experience and insights of the expert panel, to offer recommendations governing recreational exercise for patients with known GCVDs.

Adolescent↗
PubMed2004

Arrhythmogenic right ventricular cardiomyopathy causing sudden cardiac death in boxer dogs: a new animal model of human disease.

BACKGROUND: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a primary familial heart muscle disease associated with substantial cardiovascular morbidity and risk of sudden death. Efforts to discern relevant pathophysiological mechanisms have been impaired by lack of a suitable animal model. METHODS AND RESULTS: ARVC was diagnosed in 23 boxer dogs (12 male; 9.1+/-2.3 years old). Clinical events alone or in combination included sudden death (n=9; 39%), ventricular arrhythmias of suspected right ventricular (RV) origin (n=19; 83%), syncope (n=12, 52%), and heart failure (n=3; 13%). Right ventricular enlargement or aneurysms occurred in 10 (43%). Striking histopathological abnormalities were present in each boxer dog but not in controls, including severe RV myocyte loss with replacement by fatty (n=15, 65%) or fibrofatty (n=8, 35%) tissue. Focal fibrofatty lesions were also present in both atria (n=8) and the left ventricle (LV) (n=11). Fatty replacement occupied substantially greater RV wall area in ARVC dogs than controls (40.4+/-18.8% versus 13.8+/-3.4%, respectively) (P<0.001); residual myocardium was correspondingly reduced (56.6+/-19.2% versus 84.8+/-3.8% in controls) (P<0.001). MRI demonstrated bright anterolateral and/or infundibular RV myocardial signals, confirmed as fat by histopathology. Myocarditis appeared in the RV (n=14, 61%) and LV (n=16, 70%) and in each dog with sudden death, but not in controls. Familial transmission was evident in 10 of the 23. CONCLUSIONS: We describe a novel, spontaneous, and genetically transmitted animal model of ARVC associated with sudden death in the boxer dog, closely resembling the human disease. This model may aid in understanding the pathogenic mechanisms of ARVC.

Animals↗
PubMed2004

Usefulness of B-type natriuretic peptide assay in the assessment of symptomatic state in hypertrophic cardiomyopathy.

BACKGROUND: Hypertrophic cardiomyopathy (HCM) has a diverse clinical spectrum that often includes progressive heart failure symptoms and disability. Assessment of symptom severity may be highly subjective, encumbered by the heterogeneous clinical presentation. Plasma B-type natriuretic peptide (BNP) has been used widely as an objective marker for heart failure severity and outcome, predominantly in coronary heart disease with ventricular dilatation and systolic dysfunction. METHODS AND RESULTS: We prospectively assessed plasma BNP as a quantitative clinical marker of heart failure severity in 107 consecutive HCM patients. BNP showed a statistically significant relationship to magnitude of functional limitation, assessed by New York Heart Association (NYHA) functional class: I, 136+/-159 pg/mL; II, 338+/-439 pg/mL; and III/IV, 481+/-334 pg/mL (P<0.001). Multivariable analysis showed that BNP was independently related to NYHA class as well as age and left ventricular wall thickness (each with a value of P=0.0001). BNP > or =200 pg/mL was the most reliable predictor of heart failure symptoms, with positive and negative predictive values of 63% and 79%, respectively. BNP power in distinguishing patients with or without heart failure symptoms was less than that for differentiating between no (or only mild) and severe symptoms (area under receiver operating characteristic curve=0.75 and 0.83, respectively). CONCLUSIONS: Plasma BNP is independently related to the presence and magnitude of heart failure symptoms in patients with HCM. As a clinical marker for heart failure, BNP is limited by considerable overlap in values between categories of heart failure severity as well as confounding variables of left ventricular wall thickness and age.

Adult↗
PubMed2004

Electrocardiographic changes associated with training and discontinuation of training in an athlete with hypertrophic cardiomyopathy.

The 12-lead electrocardiogram of a 17-year-old African professional soccer player had signs of left ventricular (LV) hypertrophy and deeply inverted T waves in the inferior and precordial leads. Two-dimensional echocardiography showed mild LV hypertrophy with normal cavity size consistent with nonobstructive hypertrophic cardiomyopathy. After 5 months of complete discontinuation of training, the electrocardiogram normalized; however, the echocardiogram was unchanged. Subsequently, and contrary to our advice, the athlete resumed training and professional soccer. One year later the electrocardiogram again showed a similar and markedly abnormal pattern without cardiac dimensional changes.

Adolescent↗
PubMed2004

Extended septal myectomy for hypertrophic obstructive cardiomyopathy with anomalous mitral papillary muscles or chordae.

OBJECTIVES: Transaortic left ventricular septal myectomy yields excellent results for most severely symptomatic patients with hypertrophic obstructive cardiomyopathy. However, associated anomalies of the mitral subvalvular apparatus may prevent complete relief of obstruction, and mitral valve replacement has been advocated. We reviewed our results of procedures designed to relieve obstruction with preservation of the mitral valve. METHODS: Among 291 patients undergoing septal myectomy from 1975 to 2002, 56 (ages 2-77 years) had anomalous mitral subvalvular apparatus including anomalous chordae (n = 28) and papillary muscles with direct insertion into mitral leaflets (n = 13) or fusion to septum (n = 31) or free wall (n = 12); 82% of patients were in New York Heart Association class III or IV. Operation included resection of anomalous chordae (28 patients), relief of papillary muscle fusion (36 patients), and extended septal myectomy, wider at the apex than the base. RESULTS: There were no early deaths and no patients required mitral valve replacement. Mean peak pressure gradients decreased from 70 +/- 28 to 4.9 +/- 8.4 mm Hg and mean mitral regurgitation grade decreased from 2.3 to 1.0 (P <.001). Mean follow-up was 2.8 +/- 2.6 years. Freedom from reoperation at 4 years was 95%. There were 3 late noncardiac deaths; 98% of patients were in New York Heart Association class I or II. CONCLUSIONS: Hypertrophic obstructive cardiomyopathy associated with anomalous mitral papillary muscles or chordae can be successfully treated without mitral valve replacement by surgical relief of the anomalies and an extended septal myectomy; early mortality is low, obstruction and mitral regurgitation are significantly reduced, and late results are excellent.

Adolescent↗
PubMed2004

Hypertrophic cardiomyopathy in childhood.

Hypertrophic cardiomyopathy (HCM) is a heterogeneous and relatively common genetic cardiac disease that has been the subject of intense scrutiny and investigation for over 40 years. HCM is an important cause of disability and death in patients of all ages, although unexpected sudden death in the young is perhaps the most devastating component of the natural history. Therefore, while HCM is uncommon in pediatric cardiology practice, it is nevertheless a disease of great importance to young people and those clinicians charged with their care. Due to marked heterogeneity in clinical expression, natural history and prognosis, diagnostic and management strategies often represent a dilemma (and even the source of controversy) to both primary care clinicians and cardiovascular specialists. Consequently, it is timely to place perspective and clarify many of these relevant clinical issues, and profile the rapidly evolving concepts regarding HCM, especially as they may impact on this disease in childhood.

Cardiomyopathy, Hypertrophic↗
PubMed2003

Comparison of prevalence of apical hypertrophic cardiomyopathy in Japan and the United States.

The morphologic apical form of hypertrophic cardiomyopathy (HC), in which left ventricular (LV) wall thickening is confined to the most distal region at the apex, has been regarded as a phenotypic expression of nonobstructive HC largely unique to Japanese patients. To investigate this question further, we directly compared unselected and regional hospital-based cohorts of adult patients with HC ( > or =18 years of age) from Japan (Kochi; n=100) and from the United States (US) (Minneapolis; n=361). Japanese and American patients with HC had similar clinical features and did not differ significantly with regard to the severity of symptoms and frequency of outflow obstruction. Although Japanese and American patients also showed similar maximum LV thickness, they differed significantly with respect to the distribution of LV hypertrophy. In particular, the segmental form of HC, with hypertrophy confined to the LV apex, was more frequent in Japanese patients (i.e., apical HC, 15% in Japan vs 3% in US, p<0.0001). Giant negative T waves were also more common in Japanese patients with HC (26% vs 2%, p<0.001), including those with the apical form (64% vs. 30%, p<0.05). Each patient with apical HC had either no or only mild symptoms, and all survived. The morphologic form of nonobstructive HC with hypertrophy limited to the LV apex (apical form of HC) was 5 times more common in an unselected Japanese population. These findings document variability in the phenotypic expression of HC between countries and races, which may be due to differences in environmental factors or genetic background. Patients with the apical form of HC had a benign clinical course.

Adult↗
PubMed2003

Clinical course of hypertrophic cardiomyopathy with survival to advanced age.

OBJECTIVES: This study was designed to clarify and resolve the clinical profile of older patients with hypertrophic cardiomyopathy (HCM). BACKGROUND: Adverse consequences of HCM such as sudden death and incapacitating symptoms have been emphasized for the young and middle-aged. METHODS: Long-term outcome of HCM was assessed in a community-based cohort not subject to tertiary center referral bias. RESULTS: Of 312 patients, 73 (23%) achieved normal life expectancy (> or =75 years; range to 96); 44 (14%) were > or =80 years old. Most patients > or =75 years (47; 64%) experienced no or only mild limiting symptoms and lived virtually their entire lives with few HCM-related clinical consequences; 26 patients (36%) experienced severe progressive symptoms. In elderly patients with HCM, diagnosis and symptom onset were considerably delayed to 74 +/- 8 and 70 +/- 11 years, respectively. For patients > or =50 years at diagnosis, the probability of survival for 5, 10, and 15 years was 85 +/- 3%, 74 +/- 4%, and 57 +/- 6%, respectively, and did not significantly differ from a matched general population (p = 0.20). Patients > or =75 years were predominantly women, and had less marked wall thickness and more frequently showed basal outflow obstruction > or =30 mm Hg (compared with those <75 years; p < 0.01 and 0.001, respectively). CONCLUSIONS: Hypertrophic cardiomyopathy is frequently well tolerated and compatible with normal life expectancy, and may remain clinically dormant for long periods of time with symptoms and initial diagnosis deferred until late in life. These observations afford a measure of reassurance to many patients with HCM, a disease for which clinical course is often unfavorable and unpredictable.

Actuarial Analysis↗
PubMed2003

Relation of left ventricular thickness to age and gender in hypertrophic cardiomyopathy.

Left ventricular (LV) wall thickening is the most consistent clinical marker of hypertrophic cardiomyopathy (HC), and characteristically increases substantially during adolescence. In this study, we used 2-dimensional echocardiography to develop a cross-sectional profile of LV wall thicknesses in adult patients with HC. We studied a regional community-based cohort of 239 consecutively enrolled patients (aged 18 to 91 years). On average, maximum LV wall thickness decreased relative to increasing age (p = 0.007) within 4 age groups: 22.8 +/- 5.1 mm (18 to 39 years) to 22.1 +/- 5.1 mm (40 to 59 years) to 21.1 +/- 3.7 mm (60 to 74 years) to 20.8 +/- 3.6 mm (>or=75 years). The LV thickness index (summation of wall thicknesses in all 4 segments) also decreased with age (p = 0.017): 63.0 +/- 12.2 mm to 59.8 +/- 11.9 mm to 58.3 +/- 10.4 mm to 57.9 +/- 9.8 mm. Decreasing magnitude of LV hypertrophy was independently associated with increasing age, but not with other relevant disease variables, such as symptoms and outflow obstruction. However, when separated by gender, this inverse relation between age and LV wall thickness was statistically significant only for women (p = 0.007). In conclusion, in an unselected HC cohort, cross-sectional analysis showed a modest but statistically significant inverse relation between age and LV hypertrophy that was largely gender-specific for women. This association constitutes another facet of the natural history of this complex and heterogenous disease and may reflect disproportionate occurrence of premature death in young patients with HC with marked hypertrophy or possibly gradual LV remodeling.

Adolescent↗
PubMed2003

Relationship of race to sudden cardiac death in competitive athletes with hypertrophic cardiomyopathy.

OBJECTIVES: The goal of this study was to determine the impact of race on identification of hypertrophic cardiomyopathy (HCM). BACKGROUND: Sudden death in young competitive athletes is due to a variety of cardiovascular diseases (CVDs) and, most commonly, HCM. These catastrophes have become an important issue for African Americans, although HCM has been previously regarded as rare in this segment of the U.S. population. METHODS: We studied the relationship of race to the prevalence of CVDs causing sudden death in our national athlete registry, and compared these findings with a representative multicenter hospital-based cohort of patients with HCM. RESULTS: Of 584 athlete deaths, 286 were documented to be due to CVD at ages 17 +/- 3 years; 156 (55%) were white, and 120 (42%) were African American. Most were male (90%), and 67% participated in basketball and football. Among the 286 cardiovascular deaths, most were due to HCM (n = 102; 36%) or anomalous coronary artery of wrong sinus origin (n = 37; 13%). Of the athletes who died of HCM, 42 (41%) were white, but 56 (55%) were African American. In contrast, of 1,986 clinically identified HCM patients, only 158 (8%) were African American (p < 0.001). CONCLUSIONS: In this autopsy series, HCM represented a common cause of sudden death in young and previously undiagnosed African American male athletes, in sharp contrast with the infrequent clinical identification of HCM in a hospital-based population (i.e., by seven-fold). This discrepancy suggests that many HCM cases go unrecognized in the African American community, underscoring the need for enhanced clinical recognition of HCM to create the opportunity for preventive measures to be employed in high-risk patients with this complex disease.

Adolescent↗