Clinical and Pathological Impact of Tissue Fibrosis on Lethal Arrhythmic Events in Hypertrophic Cardiomyopathy Patients With Impaired Systolic Function

Wada, Yuko; Aiba, Takeshi; Matsuyama, Takehisa; Nakajima, Ikutaro; Ishibashi, Keiichiro; Miyamoto, Koji; Yamada, Yuko; Okamura, Hideo; Noda, Takashi; Sato, Kazuhiro; Morita, Yoshiaki; Kanzaki, Hideaki; Kusano, Kengo; Anzai, Toshihisa; Kamakura, Shiro; Ishibashi‐Ueda, Hatsue; Shimizu, Wataru; Horie, Minoru; Yasuda, Satoshi; Ogawa, Hisao

doi:10.1253/circj.cj-15-0104

Cited by 22 publications

(16 citation statements)

References 31 publications

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“…The final 53 patients with HOCM enrolled into the present study showed a heterogeneous extent of fibrosis, and therefore the level of fibrosis was classified as mild (< 10%; n = 26), moderate (10–20%; n = 20), or severe (> 20%; n = 7) [19]. Representative tissue samples including microvessels, fibrosis, and adipose tissue of each group are shown in Figure 2.…”

Section: Resultsmentioning

confidence: 99%

Microvascular Rarefaction and Myocardial Fibrosis in Hypertrophic Obstructive Cardiomyopathy

Tian¹,

Yang²,

Song³

et al. 2018

Cardiology

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Background: Hypertrophic obstructive cardiomyopathy (HOCM) is a myocardial disease characterized by fibrosis and microvascular ischemia. Microvessels play a critical role in myocardial fibrosis in HOCM. However, it remains unclear whether or not myocardial fibrosis is associated with microvascular density (MVD) changes. Objective: The aim of the present study was to investigate whether a reduction in MVD is related to myocardial fibrosis in HOCM cardiac samples. Methods: We analyzed MVD and fibrosis in myectomy left ventricular (LV) septal wall specimens from 53 HOCM patients. Control myocardium from the LV septal wall was collected at autopsy of 9 individuals who died of noncardiac causes. Results: The fibrosis ratio (% area) in HOCM was higher and the MVD was lower than that in control subjects (i.e., 12.7 ± 10.0 vs. 4.0 ± 1.4%, p = 0.012, and 480.9 ± 206.7 vs. 1,425 ± 221/mm², p < 0.001). Patients with mild fibrosis had a higher MVD than patients with moderate fibrosis (i.e., 568.2 ± 214.8 vs. 403.2 ± 167.8/mm², p = 0.006) and patients with severe fibrosis (i.e., 568.2 ± 214.8 vs. 378.6 ± 154.0/mm², p = 0.024). Furthermore, a signiﬁcant negative correlation was found between myocardial ﬁbrosis and MVD in HOCM patients (r = –0.40, p = 0.003), which was also found in mild fibrosis (r = –0.40, p = 0.043), moderate fibrosis (r = –0.50, p = 0.024), and severe fibrosis (r = –0.24, p = 0.61), although no significant differences were observed in severe fibrosis. Additionally, we demonstrated that late gadolinium enhancement was negatively correlated with MVD (r = –0.37, p = 0.03) and positively correlated with fibrosis (r = 0.44, p = 0.01). Conclusion: HOCM patients had a higher myocardial ﬁbrosis ratio and a lower MVD. The severity of myocardial fibrosis was negatively correlated with MVD in HOCM. These findings showed that a reduced MVD may contribute to myocardial ﬁbrosis in HOCM.

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Section: Resultsmentioning

confidence: 99%

Microvascular Rarefaction and Myocardial Fibrosis in Hypertrophic Obstructive Cardiomyopathy

Tian¹,

Yang²,

Song³

et al. 2018

Cardiology

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“…4,9, 10 However, LGE has difficulties in detecting early and small fibrotic lesions. 11 In addition, LGE is limited in identification of diffuse fibrosis because there is no normal myocardium as a reference.…”

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confidence: 99%

Characterization of Compacted Myocardial Abnormalities by Cardiac Magnetic Resonance With Native T1 Mapping in Left Ventricular Non-Compaction Patients　– A Comparison With Late Gadolinium Enhancement –

Zhou

Xue

Fang

et al. 2016

Circ J

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Background: Native T1 mapping is an emerging cardiac magnetic resonance technique for quantitative evaluation of cardiomyopathies. This study aimed to investigate the usefulness of native T1 mapping in characterizing myocardial abnormalities in left ventricular non-compaction (LVNC) by comparing it with late gadolinium enhancement (LGE). Methods and Results:The study group of 31 LVNC patients and 8 normal controls underwent cardiovascular magnetic resonance to acquire the native T1 maps and LGE images. Of the 31 LVNC patients, 14 had LGE. The mean native T1 value of the normal controls, LGE(−) and LGE(+) patients was 1,098.8±40.8 ms, 1140.6±32.8 ms, and 1181.4±53.7 ms, respectively. Significant differences were found in native T1 between any 2 groups (F=9.74, P<0.001). In discriminating the presence of LGE in LVNC patients, the odds ratio and corresponding 95% confidence interval (CI) of native T1 were, respectively, 2.966 (95% CI: 1.123-7.835, P=0.028) and 4.348 (95% CI: 1.155-16.363, P=0.030) before and after adjusting for confounding factors with an increment of 1 standard deviation. Conclusions:The finding that LGE(−) patients had elevated native T1 compared with normal controls suggested native T1 mapping can be used earlier than LGE imaging to detect myocardial fibrosis in LVNC patients. Furthermore, higher native T1 values in LGE(+) patients than in the LGE(−) group suggested native T1 mapping is more sensitive than LGE imaging for identifying myocardial fibrosis in LVNC patients. 1211 Native T1 Mapping Evaluates Myocardium Abnormality factors for cardiac disease (diabetes mellitus, hypertension, and smoking) were enrolled as normal controls. The age and sex of the normal volunteers were matched with the LVNC patient group. Clinical information was collected from the medical record. The study protocol was approved by the local Institutional review board and written informed consent was given by all subjects. CMR ProtocolThe CMR imaging was performed with a 3.0-T MR system (Achieva TX, Philips Healthcare, Best, The Netherlands) using a 32-channel phased array heart coil (InVivo, Gainesville, FL, USA). The cine, phase-sensitive inversion recovery (PSIR), and modified look-locker inversion recovery (MOLLI) image sequences were acquired. 16 The ECG-gated balanced steadystate free precession (bSSFP) cine images of the long-and short-axis (SAX) images covering the entire LV were acquired with the following parameters: TR/TE 2.7/1.3 ms with 30 heart phases; voxel size 1.8×1.5×8.0 mm 3 ; slice thickness 8 mm; and slice spacing 2 mm. The PSIR sequence was acquired 15±5 min after the injection of 0.2 mmol/kg of gadolinium-DTPA (Magnetism, Bayer Schering Pharma, Guangzhou, China) with flow rate of 3 ml/s and 20 ml saline for flushing. The LGE images were obtained during mid-diastole in the same SAX orientations as cine, covering the whole LV, with the following imaging

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“…Recently Wada, et al reported that the severity of fibrosis in myocardial biopsy and lower LV ejection fraction were associated with a greater risk of lethal arrhythmic events in HCM patients. 27) Although we did not have pathological data in our study, the D-HCM patients with non-dilated LV size might have more severe fibrosis.…”

Section: Discussionmentioning

confidence: 78%

Prognostic Significance of Non-Dilated Left Ventricular Size and Mitral Regurgitation in Patients With Dilated Phase of Hypertrophic Cardiomyopathy

et al. 2017

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SummaryAlthough a subtype of hypertrophic cardiomyopathy (HCM), dilated phase of HCM (D-HCM) characterized by left ventricular (LV) systolic dysfunction, has been reported to have a poor prognosis, some patients with D-HCM survive for a relatively long period. The degree of LV dilatation and functional mitral regurgitation (MR) are generally thought to be important predictors of poor prognosis in patients with LV systolic dysfunction. However, there is little information available on the relations among LV size, presence of significant MR, and prognosis in D-HCM patients.We retrospectively studied 31 patients with D-HCM to determine whether echocardiographic assessment of LV size and MR provides incremental prognostic information.During a follow-up period of 5.6 ± 4.2 years, there were 13 cardiovascular deaths. When the patients were divided into two groups by LV size at diagnosis of D-HCM, a non-dilated LV group (LV end-diastolic diameter (LVEDD) < 50 mm, n = 9) and a dilated LV group (LVEDD ≥ 50 mm, n = 22), the clinical course in the non-dilated LV group was significantly worse. As for the clinical impact of MR, no patient in the non-dilated LV group showed significant MR and 7 of the patients with dilated LV size showed significant MR during follow-up. Once significant MR was reached, cardiovascular deaths were significantly more frequent in patients with MR.Patients with D-HCM, particularly those with less LV dilatation at diagnosis of dilated phase and with significant MR during follow-up, have a poor prognosis. (Int Heart J 2017; 58: 63-68)

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Clinical and Pathological Impact of Tissue Fibrosis on Lethal Arrhythmic Events in Hypertrophic Cardiomyopathy Patients With Impaired Systolic Function

Cited by 22 publications

References 31 publications

Microvascular Rarefaction and Myocardial Fibrosis in Hypertrophic Obstructive Cardiomyopathy

Microvascular Rarefaction and Myocardial Fibrosis in Hypertrophic Obstructive Cardiomyopathy

Characterization of Compacted Myocardial Abnormalities by Cardiac Magnetic Resonance With Native T1 Mapping in Left Ventricular Non-Compaction Patients　– A Comparison With Late Gadolinium Enhancement –

Prognostic Significance of Non-Dilated Left Ventricular Size and Mitral Regurgitation in Patients With Dilated Phase of Hypertrophic Cardiomyopathy

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Clinical and Pathological Impact of Tissue Fibrosis on Lethal Arrhythmic Events in Hypertrophic Cardiomyopathy Patients With Impaired Systolic Function

Cited by 22 publications

References 31 publications

Microvascular Rarefaction and Myocardial Fibrosis in Hypertrophic Obstructive Cardiomyopathy

Microvascular Rarefaction and Myocardial Fibrosis in Hypertrophic Obstructive Cardiomyopathy

Characterization of Compacted Myocardial Abnormalities by Cardiac Magnetic Resonance With Native T1 Mapping in Left Ventricular Non-Compaction Patients – A Comparison With Late Gadolinium Enhancement –

Prognostic Significance of Non-Dilated Left Ventricular Size and Mitral Regurgitation in Patients With Dilated Phase of Hypertrophic Cardiomyopathy

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Characterization of Compacted Myocardial Abnormalities by Cardiac Magnetic Resonance With Native T1 Mapping in Left Ventricular Non-Compaction Patients　– A Comparison With Late Gadolinium Enhancement –