Late gadolinium enhancement on cardiac magnetic resonance and phenotypic expression in hypertrophic cardiomyopathy

Background: Cardiac magnetic resonance (CMR) imaging with late gadolinium enhancement (LGE) can identify areas of myocardial fibrosis in vivo in patients with hypertrophic cardiomyopathy (HCM). The aim of this study was to examine the association between clinical-morphological variables, risk factor for sudden death, and LGE findings in a consecutive, unselected population of HCM patients. Methods: From January 2005 to August 2009, 124 HCM patients (53 ± 17 years, 86 men) were prospectively evaluated with CMR examination, assessing left ventricular (LV) hypertrophy, function, and LGE. Results: In univariate analysis, patients were divided into tertiles according to the number of segments positive for LGE (first tertile, 0.3 ± 0.4; second tertile, 2.2 ± 0.4; third tertile, 5.2 ± 1.9 segments). Male gender (P = .05), maximum LV wall thickness (P = .002), nonsustained ventricular tachycardia (P = .001), ejection fraction <50% (P = .02), LV mass (P = .02), left atrium dilation (P = .04), perfusion defects (P ≤ .001), and telesystolic volume (P = .04) were all positively related with the number of segments of LGE. In multivariable analysis, male gender (P = .007), maximum LV wall thickness (P = .006), LV mass (P = .031), and perfusion alterations (P = .017) were independent predictors of LGE extent. Conclusions: Our study shows an independent association, even at multivariate analysis, between the entity of LGE and maximum LV wall thickness, mass, and perfusion defects in patients with HCM. Whether the presence and the extent of LGE translates into clinical events later on awaits further long-term follow-up studies. © 2011 Mosby, Inc. All rights reserved

Real-world candidacy to mavacamten in a contemporary hypertrophic obstructive cardiomyopathy population

Aims: In the EXPLORER-HCM trial, mavacamten reduced left ventricular outflow tract obstruction (LVOTO) and improved functional capacity of symptomatic hypertrophic obstructive cardiomyopathy (HOCM) patients. We sought to define the potential use of mavacamten by comparing real-world HOCM patients with those enrolled in EXPLORER-HCM and assessing their eligibility to treatment. Methods and results: We collected information on HOCM patients followed up at 25 Italian HCM outpatient clinics and with significant LVOTO (i.e. gradient ≥30 mmHg at rest or ≥50 mmHg after Valsalva manoeuvre or exercise) despite pharmacological or non-pharmacological therapy. Pharmacological or non-pharmacological therapy resolved LVOTO in 1044 (61.2%) of the 1706 HOCM patients under active follow-up, whereas 662 patients (38.8%) had persistent LVOTO. Compared to the EXPLORER-HCM trial population, these real-world HOCM patients were older (62.1 ± 14.3 vs. 58.5 ± 12.2 years, p = 0.02), had a lower body mass index (26.8 ± 5.3 vs. 29.7 ± 4.9 kg/m2 , p &lt; 0.0001) and a more frequent history of atrial fibrillation (21.5% vs. 9.8%, p = 0.027). At echocardiography, they had lower left ventricular ejection fraction (LVEF, 66 ± 7% vs. 74 ± 6%, p &lt; 0.0001), higher left ventricular outflow tract gradients at rest (60 ± 27 vs. 52 ± 29 mmHg, p = 0.003), and larger left atrial volume index (49 ± 16 vs. 40 ± 12 ml/m2 , p &lt; 0.0001). Overall, 324 (48.9%) would have been eligible for enrolment in the EXPLORER-HCM trial and 339 (51.2%) for treatment with mavacamten according to European guidelines. Conclusions: Real-world HOCM patients differ from the EXPLORER-HCM population for their older age, lower LVEF and larger atrial volume, potentially reflecting a more advanced stage of the disease. About half of real-world HOCM patients were found eligible to mavacamten

Real-world candidacy to mavacamten in a contemporary hypertrophic obstructive cardiomyopathy population

Aims In the EXPLORER-HCM trial, mavacamten reduced left ventricular outflow tract obstruction (LVOTO) and improved functional capacity of symptomatic hypertrophic obstructive cardiomyopathy (HOCM) patients. We sought to define the potential use of mavacamten by comparing real-world HOCM patients with those enrolled in EXPLORER-HCM and assessing their eligibility to treatment.Methods and results We collected information on HOCM patients followed up at 25 Italian HCM outpatient clinics and with significant LVOTO (i.e. gradient >= 30 mmHg at rest or >= 50 mmHg after Valsalva manoeuvre or exercise) despite pharmacological or non-pharmacological therapy. Pharmacological or non-pharmacological therapy resolved LVOTO in 1044 (61.2%) of the 1706 HOCM patients under active follow-up, whereas 662 patients (38.8%) had persistent LVOTO. Compared to the EXPLORER-HCM trial population, these real-world HOCM patients were older (62.1 +/- 14.3 vs. 58.5 +/- 12.2 years, p = 0.02), had a lower body mass index (26.8 +/- 5.3 vs. 29.7 +/- 4.9 kg/m(2), p < 0.0001) and a more frequent history of atrial fibrillation (21.5% vs. 9.8%, p = 0.027). At echocardiography, they had lower left ventricular ejection fraction (LVEF, 66 +/- 7% vs. 74 +/- 6%, p < 0.0001), higher left ventricular outflow tract gradients at rest (60 +/- 27 vs. 52 +/- 29 mmHg, p = 0.003), and larger left atrial volume index (49 +/- 16 vs. 40 +/- 12 ml/m(2), p < 0.0001). Overall, 324 (48.9%) would have been eligible for enrolment in the EXPLORER-HCM trial and 339 (51.2%) for treatment with mavacamten according to European guidelines.Conclusions Real-world HOCM patients differ from the EXPLORER-HCM population for their older age, lower LVEF and larger atrial volume, potentially reflecting a more advanced stage of the disease. About half of real-world HOCM patients were found eligible to mavacamten