title: Prognostic significance of late gadolinium enhancement quantification in cardiac magnetic resonance imaging of hypertrophic cardiomyopathy with systolic dysfunction.
abstract: Hypertrophic cardiomyopathy (HCM) with systolic dysfunction carries a poor prognosis. Although late gadolinium enhancement (LGE) on cardiac magnetic resonance is associated with adverse cardiac events in HCM and is inversely related to left ventricular ejection fraction (LVEF), it is unknown whether LGE or LVEF more accurately predicts adverse cardiac events in HCM with systolic dysfunction. We retrospectively assessed the extent of LGE with a threshold of 6 standard deviations in 46 consecutive HCM patients with systolic dysfunction defined as LVEF <50 % (average 35 ± 12 %) who underwent cardiac magnetic resonance (35 males, mean age 59 ± 14 years). They were followed up over 1755 ± 594 days. The composite adverse cardiac events end point included cardiovascular death, lethal arrhythmia, cardioembolic stroke, and unplanned heart failure hospitalization. LGE was detected in all patients, and the mean extent was 30 ± 15 %. Twenty-nine patients developed adverse cardiac events. Multivariate Cox proportional hazard analysis revealed the extent of LGE as a good independent predictor of adverse cardiac events. Risk increased with the extent of LGE (hazard ratio = 1.62/10 % increase in LGE, 95 % confidence interval = 1.23-2.15, p < 0.001). LVEF was inversely related to the extent of LGE (r = -0.44; p = 0.002) and was also an independent predictor of adverse cardiac events. Risk decreased with LVEF (hazard ratio = 0.68/10 % increase in LVEF, 95 % confidence interval = 0.51-0.91, p = 0.010). The Akaike information criterion evaluating the fit of a model demonstrated that the extent of LGE was a better independent predictor of MACE than LVEF (Akaike information criterion = 172.20 and 178.09, respectively).The extent of LGE was a good independent predictor of adverse cardiac events and reflected mortality and morbidity more precisely than LVEF in HCM with systolic dysfunction.
authors.affiliation: Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, 5-7-1 Fujishiro-dai, Suita, Osaka, 565-8565, Japan. a-funada@e-mail.jp.
authors.forename: Akira
authors.initials: A
authors.lastname: Funada
authors.affiliation: Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, 5-7-1 Fujishiro-dai, Suita, Osaka, 565-8565, Japan.
authors.forename: Hideaki
authors.initials: H
authors.lastname: Kanzaki
authors.affiliation: Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, 5-7-1 Fujishiro-dai, Suita, Osaka, 565-8565, Japan.
authors.forename: Teruo
authors.initials: T
authors.lastname: Noguchi
authors.affiliation: Department of Radiology, National Cerebral and Cardiovascular Center, Suita, Osaka, Japan.
authors.forename: Yoshiaki
authors.initials: Y
authors.lastname: Morita
authors.affiliation: Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, 5-7-1 Fujishiro-dai, Suita, Osaka, 565-8565, Japan.
authors.forename: Yasuo
authors.initials: Y
authors.lastname: Sugano
authors.affiliation: Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, 5-7-1 Fujishiro-dai, Suita, Osaka, 565-8565, Japan.
authors.forename: Takahiro
authors.initials: T
authors.lastname: Ohara
authors.affiliation: Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, 5-7-1 Fujishiro-dai, Suita, Osaka, 565-8565, Japan.
authors.forename: Takuya
authors.initials: T
authors.lastname: Hasegawa
authors.affiliation: Department of Radiology, National Cerebral and Cardiovascular Center, Suita, Osaka, Japan.|Department of Pathology, National Cerebral and Cardiovascular Center, Suita, Osaka, Japan.
authors.forename: Hiromi
authors.initials: H
authors.lastname: Hashimura
authors.affiliation: Department of Pathology, National Cerebral and Cardiovascular Center, Suita, Osaka, Japan.
authors.forename: Hatsue
authors.initials: H
authors.lastname: Ishibashi-Ueda
authors.affiliation: Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, 5-7-1 Fujishiro-dai, Suita, Osaka, 565-8565, Japan.|Department of Clinical Research and Development, National Cerebral and Cardiovascular Center, Suita, Osaka, Japan.
authors.forename: Masafumi
authors.initials: M
authors.lastname: Kitakaze
authors.affiliation: Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, 5-7-1 Fujishiro-dai, Suita, Osaka, 565-8565, Japan.
authors.forename: Satoshi
authors.initials: S
authors.lastname: Yasuda
authors.affiliation: Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, 5-7-1 Fujishiro-dai, Suita, Osaka, 565-8565, Japan.
authors.forename: Hisao
authors.initials: H
authors.lastname: Ogawa
authors.affiliation: Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, 5-7-1 Fujishiro-dai, Suita, Osaka, 565-8565, Japan.
authors.forename: Toshihisa
authors.initials: T
authors.lastname: Anzai
mesh_terms: D000328:Adult; D000368:Aged; D001706:Biopsy; D002312:Cardiomyopathy, Hypertrophic / Q000000981:diagnostic imaging* / Q000401:mortality / Q000503:physiopathology; D003287:Contrast Media / Q000008:administration & dosage*; D005260:Female; D019786:Gadolinium DTPA / Q000008:administration & dosage*; D006801:Humans; D007564:Japan; D019028:Magnetic Resonance Imaging, Cine; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D011237:Predictive Value of Tests; D011379:Prognosis; D016016:Proportional Hazards Models; D012189:Retrospective Studies; D012307:Risk Factors; D013318:Stroke Volume; D013599:Systole; D018487:Ventricular Dysfunction, Left / Q000000981:diagnostic imaging* / Q000401:mortality / Q000503:physiopathology; D016277:Ventricular Function, Left
chemical_list: D003287:Contrast Media; D019786:Gadolinium DTPA
keywords: Cardiac magnetic resonance; Hypertrophic cardiomyopathy; Late gadolinium enhancement; Prognosis
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