Elsevier

American Heart Journal

Volume 161, Issue 6, June 2011, Pages 1073-1077
American Heart Journal

Clinical Investigation
Congestive Heart Failure
Late gadolinium enhancement on cardiac magnetic resonance and phenotypic expression in hypertrophic cardiomyopathy

https://doi.org/10.1016/j.ahj.2011.03.022Get rights and content

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.

Section snippets

Study patients

From January 2005 to August 2009, 124 HCM patients (53 ± 17 years, 86 men) from 2 centers (Ospedale Mauriziano, Torino, Italy, and Ospedale Cardinal Massaia, Asti, Italy) were prospectively evaluated with CMR.

All patients underwent a complete cardiac evaluation, which included detailed history and clinical examination, 12-lead electrocardiogram, echocardiography, treadmill exercise test (Bruce protocol), and 24-hour electrocardiogram Holter monitoring within 3 months before the CMR study.

Definitions

Patient characteristics

Patient characteristics and distributions of risk factors are listed in Table I. At CMR, study patients were 53 ± 17 years of age; 86 (69%) were male. Of the 124 patients, 36 (29%) had a family history of sudden death, 11 (9%) had at least an episode of unexplained syncope, 9 (7%) had extreme LV hypertrophy, 8 (6%) had abnormal exercise blood pressure response, and 20 (16%) had nonsustained ventricular tachycardia episodes on Holter monitoring. One hundred five patients (85%) had 0 to 1 risk

Discussion

Hypertrophic cardiomyopathy is characterized by the presence of extensive myocardial disarray and various patterns of myocardial fibrosis. Cardiac magnetic resonance is the imaging tool of choice for 3-dimensional assessment of ventricular mass, chamber volume, cardiac function, and pattern and distribution of hypertrophy.10 Cardiac magnetic resonance with LGE represents a unique way to identify areas of fibrosis in different areas of the left ventricle.11, 12 Moreover, the presence of LGE

Conclusions

We found an independent association, even at multivariate analysis, between the entity of LGE and maximum LV wall thickness, mass, and perfusion alterations in patients with HCM. Our data support the potential role of myocardial fibrosis as detected by CMR in the arrhythmogenesis. Whether large areas of LGE translate into clinical events later on awaits further long-term follow-up studies.

Acknowledgements

Marzia Casetta, MD, and Maria Cristina Rosa Brusin, MD (Ospedale degli Infermi, Rivoli, Torino, Italy).

References (26)

  • A.S. Flett et al.

    Evaluation of techniques for the quantification of myocardial scar of differing etiology using cardiac magnetic resonance

    JACC Cardiovasc Imaging

    (2011)
  • R. O'Hanlon et al.

    Prognostic significance of myocardial fibrosis in hypertrophic cardiomyopathy

    J Am Coll Cardiol

    (2010)
  • L. Fananapazir et al.

    Hypertrophic cardiomyopathy: evaluation and treatment of patients at high risk for sudden death

    Pacing Clin Electrophysiol

    (1997)
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