Microvascular function is selectively impaired in patients with hypertrophic cardiomyopathy and sarcomere myofilament gene mutations.
作者： Iacopo Olivotto , Francesca Girolami , Roberto Sciagrà , Michael J. Ackerman , Barbara Sotgia
DOI: 10.1016/J.JACC.2011.05.018
关键词:
摘要: Objectives The purpose of this study was to assess myocardial blood flow (MBF) using positron emission tomography in patients with hypertrophic cardiomyopathy (HCM) according genetic status. Background Coronary microvascular dysfunction is an important feature HCM, associated ventricular remodeling and heart failure. We recently demonstrated the increased prevalence systolic HCM sarcomere myofilament gene mutations postulated association between status coronary dysfunction. Methods Maximum MBF (intravenous dipyridamole, 0.56 mg/kg; Dip-MBF) measured 13N-labeled ammonia 61 (age 38 ± 14 years), genotyped by automatic DNA sequencing 8 myofilament-encoding genes (myosin-binding protein C, beta-myosin heavy chain, regulatory essential light chains, troponin T, I, alpha-tropomyosin, alpha-actin). In 35 patients, cardiac magnetic resonance imaging performed. Results Fifty-three were identified 42 (genotype positive; 69%). Despite similar clinical profiles, genotype-positive showed substantially lower Dip-MBF compared that genotype-negative (1.7 0.6 ml/min/g vs. 2.4 1.2 ml/min/g; p Conclusions Patients are characterized more severe impairment function fibrosis, individuals. These findings suggest a direct link adverse microcirculation accounting for long-term failure patients.
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