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To date, there has not been a large systematic examination of the hypertrophic cardiomyopathy (HC) phenotype in blacks versus whites. In this study, we investigate differences in presentation of HC between blacks and whites. We included 441 consecutive patients with HC seen at the Johns Hopkins HC clinic in the period from February 2005 to June 2012. We compared 76 blacks for clinical presentation, electrocardiogram, exercise capacity, left ventricular morphology, and hemodynamics by echocardiography to 365 whites. Black patients with HC more often presented with abnormal electrocardiogram (93% vs 80%, p = 0.009), driven by a significant difference in repolarization abnormalities (79% vs 56%, p <0.001). Apical hypertrophy was more common in blacks (26% vs 9%, p <0.001); however, blacks had less severe systolic anterior movement of the mitral valve and had significantly lower left ventricular outflow tract gradients at rest (9 mm Hg; interquartile range [IQR] 7 to 19 vs 16 mm Hg; IQR 8 to 40, p <0.001) and during provocation (36 mm Hg; IQR 16 to 77 vs 59 mm Hg; IQR 26 to 110, p = 0.002). Despite the nonobstructive pathophysiology, blacks had lower exercise capacity (adjusted difference 1.45 metabolic equivalents [0.45 to 2.45], p = 0.005). In conclusion, blacks have an HC phenotype characterized by lower prevalence of the well-recognized echocardiographic features of HC such as systolic anterior movement of the mitral valve and left ventricular outflow tract obstruction and display worse exercise capacity., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Aims: To investigate whether hypertrophic cardiomyopathy (HCM) patients with late gadolinium enhancement (LGE) confined to the right ventricular insertion points (RVIP) differ phenotypically from patients without LGE or intramural LGE in the left ventricle (LV)., Methods and Results: Sixty-two HCM patients underwent cardiac magnetic resonance for quantification of LGE (% LV mass) and were classified as group (i) no-LGE (n = 18), group (ii) LGE-RVIP (n = 19), and group (iii) intramural LGE (n = 25). All patients also underwent vasodilator N-13 ammonia PET to quantify myocardial blood flow (MBF) and myocardial flow reserve (MFR), and echocardiography to measure longitudinal LV strain. LGE extent (17 ± 11% vs. 4 ± 4% vs. 0%; P < 0.001) and LV thickness (21.7 ± 3.4 vs. 18.8 ± 3.9 vs. 16.3 ± 2.8 mm; P < 0.001) were significantly greater in group 3, intermediate in group 2, and lower in group 1. In contrast, stress MBF (1.62 ± 0.44 vs. 1.90 ± 0.37 vs. 2.22 ± 0.48 mL/min/g; P < 0.001); MFR (1.92 ± 0.47 vs. 2.15 ± 0.52 vs. 2.71 ± 0.52; P < 0.001), and longitudinal LV strain (-11.4 ± 3.8 vs. -12.6 ± 3.2 vs. -14.4 ± 4.1%; P = 0.04) were lower in group 3, intermediate in group 2, and higher in group 1., Conclusions: From an imaging viewpoint, patients with LGE confined to only the RVIP appear to represent an intermediate-stage phenotype between patients with no LGE and intramural LGE in the LV., (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2015. For permissions please email: journals.permissions@oup.com.)

The exercise heart rate (HR) profile and its relation to cardiac function and arrhythmias were investigated in patients with hypertrophic cardiomyopathy (HC). Chronotropic response (CR) and heart rate recovery (HRR) were computed during and after treadmill exercise testing in 273 patients with HC and 95 age-matched healthy controls. Patients with HC had higher prevalence of chronotropic incompetence and lower HRR1-5min compared with controls. Exercise capacity, diastolic function (assessed by E/e') and left atrial volume index were associated with HRR1min and CR in HC. Septal myectomy was associated with reduction in chronotropic incompetence but did not affect HRR1min. In conclusion, impaired CR and HRR1min are associated with advanced disease and do not appear to be independent clinical markers indicating high-risk status in HC. Improving CR by titrating doses of negative chronotropic agents, myectomy, and atrial pacing may be useful to increase exercise capacity in patients with HC., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Obesity is independently associated with left ventricular (LV) hypertrophy and thus may be an important modifier of the hypertrophic cardiomyopathy (HC) phenotype. We examined if obesity modifies the clinical presentation, LV morphology, outflow hemodynamics, and exercise tolerance in HC. In this cross-sectional study, 88 obese (body mass index [BMI] ≥30 kg/m(2)) and 154 nonobese (BMI <30 kg/m(2)) patients from the Johns Hopkins HC clinic were compared with respect to a variety of clinical and LV echocardiographic measurements. Obese patients (36.4%) were more likely to report exertional dyspnea (p = 0.04) and chest pain (p = 0.002) and had greater prevalence of hypertension (p = 0.008). LV posterior wall thickness (p = 0.01) but not the septal wall (p ≥0.21) was significantly greater in obese patients, resulting in an increased LV mass index (p = 0.003). No significant differences in LV systolic and diastolic functions were observed, but obesity was associated with higher LV stroke volume (p = 0.03), inducible LV outflow tract gradients (p = 0.045), and chance of developing LV outflow tract obstruction during stress (p = 0.035). In multivariate analysis, BMI was associated with increased posterior (but not septal) wall thickness (β = 0.15, p = 0.02) and LV mass index (β = 0.18, p = 0.005), particularly in those with hypertension. Obesity was also associated with reduced exercise time and functional capacity, and BMI independently correlated with reduced exercise tolerance. In conclusion, obesity is associated with larger LV mass, worse symptoms, lower exercise tolerance, and labile obstructive hemodynamics in HC. The association with increased outflow tract gradients has particular importance as contribution of obesity to the pressure gradients may influence clinical decisions in labile obstructive HC., (Copyright © 2013 Elsevier Inc. All rights reserved.)