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Background: End-stage (ES) hypertrophic cardiomyopathy (HCM) has been considered a particularly grim and unfavorable disease complication, associated with substantial morbidity and mortality, frequently requiring heart transplant. Previous reports have included small numbers of patients with relatively short follow-up, predominantly in prior treatment eras.Objectives: The purpose of this study was to re-evaluate clinical profile and prognosis for end-stage heart failure in a large HCM cohort with contemporary management strategies.Methods: Patients at Tufts HCM Institute, from 2004 to 2017, were identified with ES and systolic dysfunction (ejection fraction [EF] <50%), followed for 5.8 ± 4.7 years (up to 18 years).Results: Of the 2,447 patients, 118 (4.8%) had ES-HCM (EF 39 ± 9%; range 12% to 49%) at age 48 ± 15 years. Notably, over follow-up, 57 patients (48%) achieved clinical stability in New York Heart Association functional classes I/II with medical treatment (or cardiac resynchronization therapy), including 6 patients ≥10 years from ES diagnosis (up to 14 years). In total, 61 other patients (52%) developed refractory heart failure to disabling New York Heart Association functional classes III/IV (5.2%/year); 67% have survived, including 31 with heart transplant. Of the 118 ES patients, 21 had appropriate implantable cardioverter-defibrillator (ICD) therapy terminating potentially lethal tachyarrhythmias, with no difference in frequency of events in patients with EF 35% to 49% versus EF <35% (17% vs. 19%; p = 0.80). With all available treatment modalities, ES-related mortality was 1.9%/year, with 10-year survival of 85% (95% confidence interval: 77% to 94%). Mortality was 4-fold lower than previously reported for ES (8.0%/year), but exceeded 10-fold HCM with preserved EF (0.2%/year; p < 0.001).Conclusions: Although ES remains an important complication of HCM, contemporary treatment strategies, including ICDs and heart transplant, are associated with significantly lower mortality than previously considered. Primary prevention ICDs should be considered when EF is <50% in HCM. Rapid heart failure progression is not an inevitable consequence of ES, and some patients experience extended periods of clinical stability. [ABSTRACT FROM AUTHOR]

The Framingham Heart Study (FHS) has conducted seminal research defining cardiovascular disease (CVD) risk factors and fundamentally shaping public health guidelines for CVD prevention over the past five decades. The success of the Original Cohort, initiated in 1948, paved the way for further epidemiological research in preventive cardiology. Due to the keen observations suggesting the role of shared familial factors in the development of CVD, in 1971 the FHS began enroling the second generation cohort, comprising the children of the Original Cohort and the spouses of the children. In 2002, the third generation cohort, comprising the grandchildren of the Original Cohort, was initiated to additionally explore genetic contributions to CVD in greater depth. Additionally, because of the predominance of White individuals of European descent in the three generations of FHS participants noted above, the Heart Study enrolled the OMNI1 and OMNI2 cohorts in 1994 and 2003, respectively, aimed to reflect the current greater racial and ethnic diversity of the town of Framingham. All FHS cohorts have been examined approximately every 2-4 years since the initiation of the study. At these periodic Heart Study examinations, we obtain a medical history and perform a cardiovascular-focused physical examination, 12-lead electrocardiography, blood and urine samples testing and other cardiovascular imaging studies reflecting subclinical disease burden.The FHS has continually evolved along the cutting edge of cardiovascular science and epidemiological research since its inception. Participant studies now additionally include study of cardiovascular imaging, serum and urine biomarkers, genetics/genomics, proteomics, metabolomics and social networks. Numerous ancillary studies have been established, expanding the phenotypes to encompass multiple organ systems including the lungs, brain, bone and fat depots, among others. Whereas the FHS was originally conceived and designed to study the epidemiology of cardiovascular disease, it has evolved over the years with staggering expanded breadth and depth that have far greater implications in the study of the epidemiology of a wide spectrum of human diseases. The FHS welcomes research collaborations using existing or new collection of data. Detailed information regarding the procedures for research application submission and review are available at [http://www.framinghamheartstudy.org/researchers/index.php]. [ABSTRACT FROM AUTHOR]

The article offers information about the association between race and clinical profile of patients diagnosed with hypertrophic cardiomyopathy (HCM), a genetic heart disease that affects patients globally, with an estimated prevalence of at least 1:500.Other information on reports about the specific role of race in clinical course, and presentation as well as phenotypic features using echocardiography and cardiovascular magnetic resonance is presented.

The applications of ultrasound in managing the clinical care of athletes have been expanding over the past decade. This review provides an analysis of the research that has been published regarding the use of ultrasound in athletes and focuses on how these emerging techniques can impact the clinical management of athletes by sports medicine physicians. Electronic database literature searches were performed using the subject terms 'ultrasound' and 'athletes' from the years 2003 to 2012. The following databases were searched: PubMed, Web of Science, Cochrane Library, CINAHL, and SPORTDiscus™. The search produced 617 articles in total, with a predominance of articles focused on cardiac and muscuioskeletal ultrasound. 266 of the studies involved application of ultrasound in evaluating the cardiovascular properties of athletes, and 151 studies involved musculoskeletal ultrasound. Other applications of ultrasound included abdominal, vascular, bone density and volume status. New techniques in echocardiography have made significant contributions to the understanding of the physiological changes that occur in the athlete's heart in response to the haemodynamic stress associated with different types of activity. The likely application of these techniques will be in managing athletes with hypertrophic cardiomyopathy, and the techniques are near ready for application into clinical practice. These techniques are highly specialized, however, and will require referral to dedicated laboratories to influence the clinical management of athletes. Investigation of aortic root pathology and pulmonary vascular haemodynamics are also emerging, but will require additional studies with larger numbers and outcomes analysis to validate their clinical utility. Some of these techniques are relatively simple, and thus hold the potential to enter clinical management in a point-of-care fashion. Musculoskeletal ultrasound has demonstrated a number of diagnostic and therapeutic techniques applicable to pathology of the shoulder, elbow, wrist, hand, hip, knee and ankle. These techniques have been applied mainly to the management of impingement syndromes, tendinopathies and arthritis. Many of these techniques have been validated and have entered clinical practice, while more recently developed techniques (such as dynamic ultrasound and platelet-rich plasma injections) will require further research to verify efficacy. Research in musculoskeletal ultrasound has also been helpful in identifying risk factors for injury and, thus, serving as a focus for developing interventions. Research in abdominal ultrasound has investigated the potential role of ultrasound imaging in assessing splenomegaly in athletes with mononucleosis, in an attempt to inform decisions and policies regarding return to play. Future research will have to demonstrate a reduction in adverse events in order to justify the application of such a technique into policy. The role of ultrasound in assessing groin pain and abdominal pain in ultraendurance athletes has also been investigated, providing promising areas of focus for the development of treatment interventions and physical therapy. Finally, preliminary research has also identified the role of ultrasound in addressing vascular disease, bone density and volume status in athletes. The potential applications of ultrasound in athletes are broad, and continuing research, including larger outcome studies, will be required to establish the clinical utility of these techniques in the care of athletes. [ABSTRACT FROM AUTHOR]

Objective To describe the association of osteophytes with concomitant cartilage damage, assessed using semiquantitative magnetic resonance imaging (MRI), and to describe the prevalence of atrophic and hypertrophic phenotypes of tibiofemoral knee osteoarthritis (OA) in a population-based cohort. Methods Participants of the Framingham Knee Osteoarthritis Study were examined with a 1.5T MRI system using triplanar intermediate-weighted fat-suppressed sequences. Cartilage and osteophytes were assessed using the Whole-Organ Magnetic Resonance Imaging Score (WORMS). Overall prevalence of knees with severe cartilage damage and concomitant osteophyte status were described. Odds ratios for the likelihood of having severe cartilage damage according to osteophyte size were estimated using a logistic regression model. An additional analysis assessed knees according to phenotype in relation to radiographic OA status, with the atrophic phenotype being defined as knees with absent or only tiny osteophytes (WORMS grade ≤2 on a 0-7 scale) in all 10 tibiofemoral subregions but exhibiting severe cartilage damage, and the hypertrophic phenotype being defined as knees with large osteophytes (WORMS grade ≥5 on a 0-7 scale) but lacking substantial cartilage damage. Results In this study, 1,597 knees of 1,248 subjects were included. Of the 67 knees with large osteophytes, 54 (80.6%) exhibited severe cartilage damage. The risk of severe cartilage damage increased markedly with increasing osteophyte size. Twenty-one knees (1.3%) showed an atrophic phenotype. Only 3 knees (0.2%) exhibited a hypertrophic phenotype. Conclusion The majority of knees with severe tibiofemoral cartilage damage exhibited moderate to large osteophytes. The larger the osteophyte, the more likely was the presence of severe cartilage damage. A minority of knees exhibited the atrophic phenotype, which also included knees without radiographic OA. The hypertrophic phenotype was extremely rare. [ABSTRACT FROM AUTHOR]

The article discusses a study on the occurrence of impaired mitochondrial biogenesis in right ventricular (RV) hypertrophy in patients with congenital heart disease. The study involved patients at Children's Hospital Boston in Massachusetts. The possibility that impaired mitochondrial deoxyribonucleic acid (mtDNA) depletion serves as a marker of right ventricular (RV) damage was investigated. It was found that impaired mtDNA replication and depletion precedes heart failure in RV hypertrophy.

Objective: --To determine the relationship of varying degrees of obesity with left ventricular mass and geometry.Design: --Survey.Setting: --Population-based epidemiologic study.Participants and Methods: --M-mode echocardiograms, which were adequate for estimation of left ventricular mass, were obtained in 3922 healthy participants of the Framingham Heart Study. Measured height and weight were used to calculate body-mass index, a measure of obesity.Results: --Body-mass index was strongly correlated with left ventricular mass. After adjusting for age and blood pressure, body-mass index remained a strong independent predictor of left ventricular mass, left ventricular wall thickness, and left ventricular internal dimension (P less than .01 for all). Body-mass index was associated with prevalence of echocardiographic left ventricular hypertrophy, particularly in subjects with a body-mass index exceeding 30 kg/m2.Conclusions: --Obesity is significantly correlated with left ventricular mass, even after controlling for age and blood pressure. The increase in left ventricular mass associated with increasing adiposity reflects increases in both left ventricular wall thickness and left ventricular internal dimension. [ABSTRACT FROM AUTHOR]

The clinical implications of newly acquired left bundle-branch block (LBBB) were examined prospectively in the Framingham Study population. During 18 years of observation 55 people developed LBBB. The mean age at the onset of LBBB was 62; LBBB occurred largely in people with antecedent hypertension, cardiac enlargement, coronary heart disease, or a combination of these. Coincident with or subsequent to the onset of LBBB, 48% developed clinical coronary disease or congestive failure for the first time. Throughout the entire period of observation only 11% remained free of clinically apparent cardiovascular abnormalities. Within 10 years of the onset of LBBB, 50% had died from cardiovascular diseases. In men, the appearance of LBBB contributed independently to an increased risk of cardiovascular disease mortality. Comparison with age- and sex-matched control subjects free from LBBB confirmed that in the general adult population, newly acquired LBBB is most often a hallmark of advanced hypertensive or ischemic heart disease, or both. [ABSTRACT FROM AUTHOR]

Background: The implantable cardioverter-defibrillator (ICD) is effective for preventing sudden death in patients with hypertrophic cardiomyopathy. However, data on performance and complications of implanted ICDs over particularly long time periods to inform clinical practice is presently incomplete.Methods: The study cohort comprises 217 consecutive hypertrophic cardiomyopathy patients with primary prevention ICDs implanted before 2008 and followed for ≥10 years (mean 12±4; range to 31).Results: Patients were 38±17 years at implant and 45 (21%) experienced appropriate interventions terminating ventricular tachycardia/ventricular fibrillation. The majority of ICD discharges occurred ≥5 years after implant (29 patients; 64%), including ≥10 years in 16 patients (36%). Initial device therapy increased in frequency from 2.3% of patients at <1 year to 8.5% of patients at ≥10-years after implant (P=0.005). Inappropriate ICD shocks in 39 patients occurred most commonly <5 years after implant (54%) and decreased in frequency with increasing time from implant (from 9.7% of patients at <5 years to 3.8% at ≥10 years, P=0.02). Other major device complications including infection and lead fractures and dislodgement occurred in 27 patients (12%) but did not increase in frequency over follow-up after implant (P=0.47). There were no arrhythmic sudden death events among the 217 patients with ICD.Conclusions: In hypertrophic cardiomyopathy, after a primary prevention implant, ICD therapy often followed prolonged periods of device dormancy and increased progressively in frequency over time, including one-third of patients with initial therapy after 5 to 9 years, and an additional one-third of patients at ≥10 years. Frequency of inappropriate shocks decreased over follow-up, likely reflecting standard changes in device programming, while occurrence of device complications, such as lead fractures/infection, did not increase during follow-up. [ABSTRACT FROM AUTHOR]

Background: Racial differences in electrocardiographic (ECG) characteristics and prognostic significance among Whites and Asians are not well described., Methods and Results: We studied 2677 White Framingham Heart Study participants (57% women) and 2972 Asian (64% women) Singapore Longitudinal Aging Study participants (mean age 66 years in both) free of myocardial infarction or heart failure. Racial differences in ECG characteristics and effect on mortality were assessed. In linear regression models, PR interval was longer in Asians compared with Whites (multivariable-adjusted β±SE 5.0±1.4 ms in men and 6.6±0.9 ms in women, both P<0.0006). QT interval was shorter in Asian men (β±SE -6.2±1.2 ms, P<0.0001) and longer in Asian women (β±SE 3.6±0.9 ms, P=0.02) compared to White men and women, respectively. Asians had greater odds of having ECG left ventricular hypertrophy (LVH) compared with Whites (odds ratio [OR] 3.56, 95% confidence interval [CI] 1.36-9.35 for men, OR 1.93, 95% CI 1.35-2.76 for women, both P<0.02). Over a mean follow-up of 11±3 years in Framingham and 8±3 years in Singapore, mortality rates were 24.5 and 13.4 per 1000 person-years among Whites and Asians, respectively. In Cox models, the presence of LVH had a greater effect on all-cause mortality in Asians compared with Whites (hazard ratio [HR] 2.66, 95% CI 1.83-3.88 vs HR 1.30, 95% CI 0.90-1.89, P for interaction=0.02)., Conclusion: Our findings from two large community-based cohorts show prominent race differences in ECG characteristics between Whites and Asians, and also suggest a differential association with mortality. These differences may carry implications for race-specific ECG reference ranges and cardiovascular risk., (© 2016 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley Blackwell.)