{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,17]],"date-time":"2026-09-17T21:05:34Z","timestamp":1789679134346,"version":"4.0.1"},"reference-count":41,"publisher":"Oxford University Press (OUP)","issue":"12","license":[{"start":{"date-parts":[[2011,12,1]],"date-time":"2011-12-01T00:00:00Z","timestamp":1322697600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2011,12,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Aims<\/jats:title>\n                    <jats:p>Next to left ventricular (LV) hypertrophy, hypertrophic cardiomyopathy (HCM) is characterized by microvascular dysfunction and reduced myocardial external efficiency (MEE). Insights into the presence of these abnormalities as early markers of disease are of clinical importance in risk stratification, and development of therapeutic approaches. Therefore, the aim was to investigate myocardial perfusion and energetics in genotype-positive, phenotype-negative HCM subjects (carriers).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods and results<\/jats:title>\n                    <jats:p>Fifteen carriers of an MYBPC3 mutation underwent [15O]water positron emission tomography (PET) to assess myocardial blood flow (MBF). [11C]acetate PET was performed to obtain myocardial oxygen consumption (MVO2). By use of cardiovascular magnetic resonance imaging, LV volumes and mass were defined to calculate MEE, i.e. the ratio between external work and MVO2. Eleven healthy, genotype-negative, family relatives underwent similar scanning protocols to serve as a control group. Left ventricular mass was comparable between carriers and controls (93 \u00b1 25 vs. 99 \u00b1 21 g, P= 0.85), as was MBF at rest (1.19 \u00b1 0.34 vs. 1.18 \u00b1 0.32 mL min\u22121 g\u22121, P= 0.92), and during hyperaemia (3.87 \u00b1 0.75 vs. 3.96 \u00b1 0.86 mL min\u22121 g\u22121, P= 0.77). Myocardial oxygen consumption averaged 0.137 \u00b1 0.057 mL min\u22121 g\u22121 in carriers and was not significantly different from controls (0.125 \u00b1 0.043 mL min\u22121 g\u22121, P= 0.29). Cardiac work, however, was slightly reduced in carriers (7398 \u00b1 1384 vs. 9139 \u00b1 2484 mmHg mL in controls, P= 0.08). As a consequence, MEE was significantly decreased in carriers (27 \u00b1 10 vs. 36 \u00b1 8% in controls, P= 0.02).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Carriers display reduced myocardial work generation in relation to oxygen consumption, in the absence of hypertrophy and flow abnormalities. Hence, impaired myocardial energetics may constitute a primary component of HCM pathogenesis.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1093\/eurjhf\/hfr135","type":"journal-article","created":{"date-parts":[[2011,10,21]],"date-time":"2011-10-21T21:23:45Z","timestamp":1319232225000},"page":"1283-1289","source":"Crossref","is-referenced-by-count":56,"title":["Carriers of the Hypertrophic Cardiomyopathy MYBPC3 Mutation are Characterized by Reduced Myocardial Efficiency in the Absence of Hypertrophy and Microvascular Dysfunction"],"prefix":"10.1093","volume":"13","author":[{"given":"Stefan A.J.","family":"Timmer","sequence":"first","affiliation":[{"name":"Department of Cardiology, 5F, VU University Medical Center , De Boelelaan 1117, 1081 HV, Amsterdam ,","place":["The Netherlands"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tjeerd","family":"Germans","sequence":"additional","affiliation":[{"name":"Department of Cardiology, 5F, VU University Medical Center , De Boelelaan 1117, 1081 HV, Amsterdam ,","place":["The Netherlands"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wessel P.","family":"Brouwer","sequence":"additional","affiliation":[{"name":"Department of Cardiology, 5F, VU University Medical Center , De Boelelaan 1117, 1081 HV, Amsterdam ,","place":["The Netherlands"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mark","family":"Lubberink","sequence":"additional","affiliation":[{"name":"Department of Nuclear Medicine & PET Research, VU University Medical Center , Amsterdam ,","place":["The Netherlands"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jolanda","family":"van der Velden","sequence":"additional","affiliation":[{"name":"Department of Cardiology, 5F, VU University Medical Center , De Boelelaan 1117, 1081 HV, Amsterdam ,","place":["The Netherlands"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arthur 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