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Objectives: Cardiac remodeling is a life-long process in hypertrophic cardiomyopathy (HCM), and if uncontrolled, would cause substantial morbidity and mortality. Sleep apnea (SA) is a common comorbidity in HCM. This study aimed to investigate the relationship between SA and cardiac remodeling in a large series of patients with HCM., Methods: A total of 606 patients with HCM who underwent sleep evaluations at Fuwai Hospital were included. Parameters of cardiac remodeling were evaluated by echocardiographic studies., Results: SA was present in 363 (59.9%) patients. Left ventricular (LV) end-diastolic diameter (P < 0.001), left atrial (LA) diameter (P = 0.024), ascending aortic diameter (P < 0.001) all increased and maximal end-diastolic wall thickness (P < 0.001) decreased with the severity of SA. After adjustment for sex, age, body mass index, hypertension, hyperlipidemia, diabetes, coronary artery disease and cigarette use, log (apnea-hypopnea index+1) was independently correlated with increasing LV end-diastolic diameter (β = 0.729, P = 0.003) and deceasing maximal end-diastolic wall thickness (β = -0.503, P = 0.009). Log (percentage of total sleep time spent with oxygen saturation<90% + 1) was independently correlated with increasing LV end-diastolic diameter (β = 0.609, P = 0.004) and LA diameter (β = 0.695, P = 0.006). Severity of SA (severe SA with odds ratio, 2.38; 95% CI, 1.20-4.70; P = 0.013), log (apnea-hypopnea index+1) (OR, 1.28; 95% CI, 1.01-1.63; P = 0.045) and log (percentage of total sleep time spent with oxygen saturation<90% + 1) (OR, 1.31; 95% CI, 1.08-1.59; P = 0.006) were also independently associated with LV enlargement., Conclusions: Severity of SA is independently associated with cardiac remodeling indicating a trend toward enlarged chamber size and thinned wall. Clinical trials are required to determine whether treatment of SA improves cardiac remodeling and long-term outcomes in patients with HCM., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024. Published by Elsevier B.V.)

Background: Myocardial bridging (MB) is common in patients with hypertrophic cardiomyopathy (HCM). There are sparse data on the impact of MB on myocardial fibrosis in HCM. This study was designed to evaluate the relationship between MB and myocardial fibrosis in patients with obstructive HCM., Methods: In this cohort study, retrospective data were collected from a high-volume HCM center. Patients with obstructive HCM who underwent septal myectomy and preoperative cardiac magnetic resonance (CMR) were screened from 2011 to 2018., Results: Finally, 492 patients were included in this study, with an average age of 45.7 years. Of these patients, 76 patients had MB. MB occurred mostly in the left anterior descending artery (73/76). The global extent of late gadolinium enhancement (LGE) was correlated with the degree of systolic compression (r = 0.33, p = 0.003). Multivariable linear regression analysis revealed that the degree of systolic compression was an independent risk factor for LGE (β = 0.292, p = 0.007). The LGE fraction of basal and mid anteroseptal segments in patients with severe MB (compression ratio ≥ 80%) was significantly greater than that in patients with mild to moderate MB (compression ratio < 80%). During a median follow-up of 28 (IQR: 15-52) months, 15 patients died. Kaplan-Meier analysis did not identify differences in all-cause death (log-rank p = 0.63) or cardiovascular death (log-rank p = 0.72) between patients undergoing MB-related surgery and those without MB., Conclusions: MB with severe systolic compression was significantly associated with a high extent of fibrosis in patients with obstructive HCM. Concomitant myotomy or coronary artery bypass grafting might provide excellent survival similar to that of patients without MB. Identification of patients with severe MB and providing comprehensive management might help improve the prognosis of patients with HCM., (© 2024. The Author(s).)

Obstructive hypertrophic cardiomyopathy (oHCM) and mitral valve (MV) prolapse (MVP) are the 2 conditions which could cause symptomatic heart failure and sudden cardiac death. The clinical characteristics and surgical outcomes of patients with oHCM and MVP have not been well reported. From April 2012 to February 2018, 84 patients with oHCM (28 patients with MVP and 56 gender- and age-matched patients without MVP) who underwent septal myectomy at our institution were enrolled in this study. Information on clinical characteristics and outcomes was obtained from electronic medical records and follow-up surveys. Compared with those without MVP, patients with MVP were more symptomatic (New York Heart Association class III to IV; 96% vs 77%), more often moderate-to-severe mitral regurgitation (86% vs 48%), atrial fibrillation (39% vs 11%) and higher incidence of nonsustained ventricular tachycardia (44% vs 15%). Twenty (71%) had MV repair and 8 (29%) had MV replacement. Compared with patients without MVP, those with MVP had a longer postoperative hospital stay (10.9 ± 6.4 vs 7.8 ± 2.8 days). None of the 84 study patients died during hospital or follow-up. At the most recent echocardiographic evaluation, left ventricular outflow tract gradient significantly decreased from 69.7 ± 35.4 millimeters of mercury to 7.3 ± 5.1 millimeters of mercury and the degree of mitral valve regurgitation improved from grade 2.43 ± 0.69 to grade 0.5 ± 0.69. In conclusion, MVP occurs rarely in oHCM, and was related to atrial fibrillation, ventricular arrhythmia and mitral regurgitation. Mitral valve surgery in combination with myectomy is effective and safe for patients with oHCM and MVP, relieving substantially left ventricular outflow tract gradients and mitral regurgitation., Competing Interests: Declaration of Competing Interest The authors have no competing interests to declare., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Obstructive sleep apnea (OSA) is much common and associated with worse clinical outcomes in patients with hypertrophic cardiomyopathy (HCM), however, the diagnosis of OSA in HCM is still insufficient. We aim to investigate the clinical predictors of OSA in a large series of patients with HCM. A total of 589 patients with HCM who underwent sleep evaluations were retrospectively enrolled. Data from clinical characteristics and polysomnography studies were recorded. OSA was present in 346 patients (58.7%). Patients who had OSA were older, more likely to be male and had more clinical comorbidities such as hypertension, atrial fibrillation and cardiac remodeling. Multivariate logistic analyses showed that male, age, body mass index, hypertension and left ventricular outflow tract obstruction were significant factors associated with OSA. The area under the ROC curve (AUC) was 0.78 (95% CI 0.74-0.82; P < 0.001). These factors were also able to identify moderate to severe OSA with an AUC of 0.77 (95% CI 0.73-0.81; P < 0.001). These findings suggest that identifying HCM patients with high risk for OSA is feasible using characteristics from clinical practices and clinicians should have no hesitate to conduct sleep test in these patients.

Objectives: Metabolic abnormalities have been associated with long-term cardiac mortality in patients with hypertrophic cardiomyopathy (HCM). Obstructive sleep apnea (OSA) is a risk factor for metabolic abnormalities in general populations, but association between OSA and metabolic abnormalities in HCM is still undefined. This study aimed to investigate the relationship between OSA and metabolic dysfunction in a large series of patients with HCM., Methods: A total of 587 patients with HCM who underwent sleep evaluations at Fuwai Hospital were included. Data from clinical characteristics, polysomnography studies, and metabolic measurements were collected., Results: OSA was present in 344 patients (58.6%). Patients with OSA were older, more often male, and had more clinical comorbidities. Body mass index, blood pressure, fasting glucose, and triglycerides all increased (all P < 0.001) and high-density lipoprotein cholesterol decreased (P = 0.046) with the severity of OSA. In multivariate analysis, moderate to severe OSA and Log (apnea-hypopnea index + 1) were independently associated with obesity (odds ratio [OR], 2.42; 95% CI, 1.48-3.95 and OR, 1.60; 95% CI, 1.31-1.95), elevated blood pressure (OR, 1.99; 95% CI, 1.42-3.26 and OR, 1.31; 95% CI, 1.08-1.60), and elevated triglycerides (OR, 1.71; 95% CI, 1.05-2.78 and OR, 1.24; 95% CI, 1.02-1.51 but not elevated fasting glucose (OR, 0.88; 95% CI, 0.50-1.52 and OR, 1.02; 95% CI, 0.82-1.28) or reduced high-density lipoprotein cholesterol (OR, 1.30; 95% CI, 0.83-2.04 and OR, 1.06; 95% CI, 0.89-1.27)., Conclusions: Severity of OSA is independently associated with some profiles of metabolic abnormalities. Clinical trials are required to determine whether OSA treatment improves metabolic abnormalities and long-term outcomes in patients with HCM., (© The Author(s) 2021. Published by Oxford University Press on behalf of the Endocrine Society. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Atrial fibrillation (AF) is the most common arrhythmia and potentially increase the risk of embolic stroke and aggravate progressive heart failure in patients with hypertrophic cardiomyopathy (HCM). Recent studies demonstrated that epicardial adipose tissue (EAT) was closely associated with AF in general population. However, the relationship between EAT and AF in HCM patients remains unclear. A total of 93 consecutive patients with hypertrophic obstructive cardiomyopathy (HOCM) at Fuwai Hospital were enrolled in our study. There were 18 patients with AF and 75 patients without it. Cardiac magnetic resonance (CMR) imaging was performed in all participants. EAT volume (EATV) and left atrial volume (LAV) were determined by E-3D medical model software. HOCM patients with AF had significantly greater EATV index (EATVI, P < 0.001), LAV index (LAVI, P < 0.001) and left ventricular end-systole volume index (LVESVI, P = 0.039), and lower left ventricular ejection fraction (LVEF, P = 0.002). In multivariable logistic regression analysis, EATVI, LAVI, and LVEF remained independent determinants of AF occurrence (OR = 1.023, 95% CI, 1.003-1.043, P = 0.023, OR = 1.043, 95% CI, 1.012-1.075, P = 0.006, and OR = 0.887, 95% CI, 0.818-0.962, P = 0.004, respectively). Furthermore, receiver operating characteristic (ROC) curve analysis demonstrated that integration of EATVI, LAVI and LVEF provided better discriminatory performance for incident AF in HOCM patients with a high sensitivity of 94.4% and a specificity of 69.3% (AUC = 0.864, 95% CI, 0.771-0.958, P < 0.001). EATVI is an independent predictor of the presence of AF, and integration of EATVI, LVEF and LAVI determined by CMR provide greater discriminatory performance for identifying AF in HOCM patients.

Background: Myocardial fibrosis (MF) is considered a result of microvascular dysfunction in patients with hypertrophic cardiomyopathy. Matrix metalloproteinases (MMPs) and their tissue inhibitors (TIMPs), capable of degrading collagen, directly participate in the development of MF. First we investigated the relationships among MF, microvascular rarefaction, and MMPs. Then we assessed the prognostic value of MF-related circulating biomarkers., Methods: Fifty-five obstructive hypertrophic cardiomyopathy (HOCM) patients were enrolled after surgical myectomy. Myocardial samples were performed with Masson's trichrome staining and immunohistochemical procedures for collagen volume fraction and microvascular density, respectively. Enzyme-linked immunosorbent assays were used to assess myocardial and plasma of MMP-2, MMP-9, and TIMP-1 and plasma C-terminal propeptide of procollagen type Ⅰ (PICP) and C-terminal telopeptide of type Ⅰ collagen (ICTP) levels. The composite cardiovascular endpoint consisted of new-onset atrial fibrillation, heart failure requiring hospitalization, and all-cause death., Results: In HOCM patients microvascular density was associated with the myocardial MMP-2/TIMP-1 ratio (r = -0.348, P = .009), whereas no correlation was found between collagen volume fraction and myocardial MMPs. During the 44-month follow-up 6 patients experienced a cardiovascular endpoint. The plasma PICP/ICTP ratio and MMP-2/TIMP-1 ratio were the 2 strongest prognostic makers. In multivariable analyses high PICP/ICTP and MMP-2/TIMP-1 ratios remained independent predictors of cardiovascular outcomes after adjusting for clinical confounders (hazard ratios, 12.683 [P = .021] and 17.037 [P = .027], respectively)., Conclusions: In HOCM patients the myocardial MMP-2/TIMP-1 ratio was elevated because of microvascular rarefaction but may not be responsible for MF. High plasma PICP/ICTP and MMP-2/TIMP-1 ratios are independent predictors of adverse outcomes in HOCM patients., (Copyright © 2021 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background Collagen cross-linking is covalent bonds among collagen fibers from catalysis of lysyl oxidase (LOX) and advanced glycation end products (AGEs). We aimed to evaluate the formation of enzymatic and nonenzymatic collagen cross-linking and its clinical significance in patients with hypertrophic obstructive cardiomyopathy. Methods and Results Forty-four patients with hypertrophic obstructive cardiomyopathy who underwent surgical myectomy were consecutively enrolled. Cardiovascular magnetic resonance parameters of left atrial/left ventricular function were measured, including peak filling rate (PFR) and early peak emptying rate (PER-E). Total collagen was the sum of soluble and insoluble collagen, which were assessed by collagen assay. The myocardial LOX and AGEs expression were measured by molecular and biochemical methods. Compared with patients without atrial fibrillation, insoluble collagen ( P =0.018), insoluble collagen fraction ( P =0.017), and AGEs ( P =0.039) were higher in patients with atrial fibrillation, whereas LOX expression was similar ( P =0.494). The insoluble collagen fraction was correlated with PFR index (PFR normalized by left ventricular filling volume) (r=-0.44, P =0.005), left atrial diameters (r=0.36, P =0.021) and PER-E index (PER-E normalized by left ventricular filling volume) (r=-0.49, P =0.001).Myocardial LOX was positively correlated with total collagen (r=0.37, P =0.025) and insoluble collagen fraction (r=0.53, P  < 0.001), but inversely correlated with PFR index (r=-0.43, P =0.006) and PER-E index (r=-0.35, P =0.027). In multiple regression analysis, myocardial LOX was independently associated with PFR, while insoluble collagen fraction showed independent correlation with PER-E after adjustment for clinical confounders. Conclusions Collagen cross-linking plays an important role on heart remodeling in hypertrophic obstructive cardiomyopathy. Myocardial LOX expression is independently correlated with left ventricular stiffness, while accumulation of AGEs cross-links might be associated with the occurrence of atrial fibrillation in patients with hypertrophic obstructive cardiomyopathy.

Background: Obstructive sleep apnea (OSA) is highly prevalent in patients with hypertrophic obstructive cardiomyopathy (HOCM). Inflammatory responses are increased in patients with OSA, meanwhile, inflammation is also associated with adverse outcomes in HOCM., Hypothesis: To investigate the association between severity of OSA and high-sensitivity C-reactive protein (hs-CRP) in patients with HOCM., Methods: Three hundred and ninteen patients with HOCM who underwent sleep evaluations at Fuwai Hospital were retrospectively included between February 2010 and December 2018. Data from baseline clinical characteristics and polysomnography studies were collected., Results: OSA was present in 168 (52.7%). Patients with OSA were older, more likely to be male, had a higher body mass index and more clinical comorbidities. Patients with OSA had enlarged left ventricular diameter and similar left ventricular outflow tract obstruction compared with those without. In multivariate logistic analysis, apnea-hypopnea index (OR, 1.024; 95% CI, 1.005-1.044; P = .014), oxygen desaturation index (OR, 1.025; 95% CI, 1.004-1.046; P = .018) and lowest oxygen saturation (OR, 0.951; 95% CI, 0.915-0.989; P = .011) were independently associated with high risk hs-CRP (>3 mg/L) after adjusting for confounders. In addition, decreasing lowest oxygen saturation (β = -.159, P = .004) was also independently correlated with increasing hs-CRP concentrations in multivariate linear analysis after adjusting for confounders., Conclusions: Severity of OSA was independently associated with elevated hs-CRP levels in patients with HOCM. Further studies are needed to evaluate the effects of treating OSA on hs-CRP as well as clinical outcomes in these patients., (© 2020 The Authors. Clinical Cardiology published by Wiley Periodicals, Inc.)

Background: To investigate the relationship between fragmented QRS (fQRS) quantified by a new method and myocardial fibrosis (MF) and the diagnostic value of quantitative fQRS (Q-fQRS) to detect MF in hypertrophic obstructive cardiomyopathy (HOCM) patients based on histological validation., Methods: We performed a retrospective study that included 69 patients with HOCM who underwent ventricular septal surgery. Nine individuals who died from accidents were studied as a control reference for the histological parameters. Septal myocardium samples were subjected to Masson's trichrome staining to quantify the collagen volume fraction (CVF). An fQRS pattern was defined as the presence of additional R waves or RSR', evidenced by notched R or S wave on electrocardiography (ECG). The Q-fQRS was quantified as the total amount of deflections in the QRS complex in all 12 routine ECG leads together. Cardiac magnetic resonance imaging was conducted, and late gadolinium enhancement (LGE) was measured at 2, 4, 6 and 8 standard deviations (SDs)., Results: Of the 69 patients, fQRS was documented in 38 (55.1%) patients, the mean number of leads with fQRS was 3.7 ± 1.6, and the mean Q-fQRS was 17 ± 7.2. Compared with HOCM patients without fQRS, HOCM patients with fQRS had a higher CVF and more LGE at 6 SD (P < 0.001; P = 0.040). Q-fQRS was correlated with CVF (r = 0.640, P < 0.001), and Q-fQRS showed the best correlation with LGE measured at 8 SD (r = 0.379, P = 0.002). Multivariate regression analyses revealed that Q-fQRS was independently associated with the extent of CVF in HOCM patients after adjusting for age, sex, body surface area and the extent of LGE at 6 SD (P < 0.001). When the patients were divided into subgroups with normal CVF or high CVF according to the CVF in controls, Q-fQRS and LGE at 6SD showed similar diagnostic value in detecting patients with high CVF, with sensitivities of 66.7% vs 68.6%, specificities of 76.7% vs 72.4%, and accuracies of 71% vs 70.3%., Conclusions: HOCM patients with fQRS showed more extensive MF. Q-fQRS was an independent predictor for MF and had a good diagnostic value, with a sensitivity of 66.7% and specificity of 76.7%, in identifying patients with higher fibrotic burden.

As highly sensitive and specific markers of myocardial damage, cardiac troponins were demonstrated to correlate with clinical parameters of patients with hypertrophic cardiomyopathy. However, the relationship between cardiac troponins and presence of non-sustained ventricular tachycardia (NSVT) in hypertrophic cardiomyopathy remains unclear. The aim of our study was to explore the association between serum cardiac troponin I (cTNI) and presence of NSVT in patients with hypertrophic obstructive cardiomyopathy (HOCM). A total of 309 HOCM patients were enrolled in our study. All participants underwent clinical evaluations, including collections of medical history, blood tests, 24-h Holter monitoring, echocardiography, and cardiac magnetic resonance imaging. There were 53 (17.2%) patients with NSVT and 256 patients without it. Compared to patients without NSVT, serum cTNI (P < 0.001) and plasma NT-proBNP (P = 0.042) were significantly higher in patients with NSVT. Moreover, cTNI and NT-proBNP were positively correlated with left atrial diameter, maximum wall thickness (MWT), left ventricular volume index and left ventricular mass index. In multivariable logistic analysis, log cTNI [odds ratio (OR) = 2.408, 95% confidence interval (CI) 1.108-5.325, P = 0.027], left ventricular end-diastole diameter (OR = 0.922, 95%CI 0.856-0.994, P = 0.034), MWT (OR = 1.131, 95%CI 1.035-1.235, P = 0.006) and left ventricular end-systole volume index (OR = 1.060, 95%CI 1.025-1.096, P = 0.001) were independent determinants of NSVT occurrence after adjustment for potential cofounders. Serum cTNI level was elevated in patients with NSVT. And it was independently associated with NSVT in patients with HOCM. Our results suggest that it may be more reasonable for HOCM patients with elevated serum cTNI to extend the time of Holter monitoring.

Background Obstructive sleep apnea (OSA) is common and independently associated with atrial fibrillation (AF) in patients with hypertrophic cardiomyopathy (HCM). This study aimed to investigate the relationship between apnea-hypopnea index (AHI), a measure of OSA severity, and prevalence of AF in a large series of patients with HCM. Methods and Results A total of 555 patients with HCM who underwent sleep evaluations were retrospectively included. Data from polysomnography studies, echocardiography, and baseline clinical characteristics were collected. OSA was present in 327 patients (58.9%). Patients with OSA or higher AHI quartiles were older, more often male, had a higher body mass index, and more clinical comorbidities. The prevalence of AF increased in patients with OSA (23.9% versus 13.6%, P =0.003) or across AHI quartiles (9.4%, 17.3%, 26.6%, and 25.2%, respectively; P for trend <0.001). After adjustment for age, sex, body mass index, New York Heart Association class, left atrial diameter, hypertension, oxygen desaturation index, and obstructive HCM, highest AHI quartile (odds ratio, 4.42; 95% CI, 1.35-14.52 [ P =0.014]) or moderate to severe OSA (odds ratio, 3.03; 95% CI, 1.28-7.20 [ P =0.012]) but not presence of OSA (odds ratio, 1.58; 95% CI, 0.84-2.97 [ P =0.153]) were significantly associated with AF. Higher AHI levels were also factors associated with persistent or permanent AF (highest AHI quartile with odds ratio, 10.96; 95% CI, 1.07-111.85). Conclusions Severity of AHI level is independently associated with AF in patients with HCM. Clinical trials are required to determine the benefits of OSA treatment on AF in patients with HCM.

Aim: To evaluate D-dimer levels in patients with hypertrophic cardiomyopathy (HCM). Patients & methods: A total of 346 patients with HCM were recruited. Plasma D-dimer was determined by clinical laboratory of our hospital. Left ventricular mass, stroke volume, cardiac output and cardiac index were assessed with cardiovascular magnetic resonance. Results: A total of 36 (10.4%) patients had elevated D-dimer levels. Age, female patients and statin therapy were independently associated with increasing D-dimer levels, and predictors of elevated D-dimer. Conclusion: Patients with HCM may have higher plasma D-dimer levels than subjects without HCM. D-dimer levels in patients with HCM are influenced by age, sex, atrial fibrillation, statin therapy and diastolic blood pressure.

Background: The clinical condition of hypertrophic obstructive cardiomyopathy (HOCM) and concomitant systemic hypertension is growing more and more prevalent, and it brings about a challenging diagnostic and therapeutic dilemma. However, whether systemic hypertension has an impact on HOCM, and whether sex-related differences exist in this impact, remains unclear., Methods: A total of 453 HOCM patients (age 48.7 ± 12.8 years, 252 [55.6%] males) were recruited in this study. There were 150 patients (33.1%, 81 males and 69 females) with a history of controlled systemic hypertension. Cardiac magnetic resonance (CMR) imaging was performed in all patients. Left ventricular (LV) remodeling index (LVRI) was determined by CMR. LVRI >1.3 g/mL was defined as pathological LV remodeling., Results: Men had significantly greater LVRI (1.40 ± 0.54 vs. 1.15 ± 0.38 g/mL, p < 0.001) and LVRI >1.3 g/mL (p = 0.002), compared with women. The incidence of syncope and 5-year sudden cardiac death risk score were significantly lower in HOCM with hypertension than those without hypertension. LVRI (p = 0.003) and LVRI >1.3 g/mL (p = 0.007) were significantly smaller in males with hypertension, but not in females with hypertension. However, log cardiac troponin I and log N-terminal pro-B-type natriuretic peptide were positively correlated with LVRI in men and women. On multivariable logistic analysis, hypertension (OR 0.172, 95% CI 0.056-0.528, p = 0.002) remained an independent determinant of pathological LV remodeling in males, whereas not in females., Conclusions: There were significant sex differences in the impact of systemic hypertension on LV remodeling in patients with HOCM. Controlled systemic hypertension may contribute to improving LV remodeling in male patients with HOCM, but not in females., (© 2020 S. Karger AG, Basel.)

Lymphatic microvessel density (LMVD) contributes to fibrosis in patients with myocardial infarction. However, the role of LMVD in the process of myocardial fibrosis in hypertrophic obstructive cardiomyopathy (HOCM) patients is unclear. We studied LMVD in ventricular septal (VS) samples from 52 individuals (42 was HOCM patients who underwent a transaortic extended septal myectomy, and 10 traffic accident victims), and examined the relationships between the LMVD stained immunohistochemically with lymphatic vessel endothelial hyaluronan receptor (LYVE-1) antibodies, collagen volume fraction (CVF), and clinical characteristics. Compared with traffic accident victims, LMVD was significantly increased in VS of HOCM patients (132.0 ± 49.0 VS 57.8 ± 48.8/mm 2 , p = 0.000). HOCM patients with syncope had higher level of LMVD than without syncope [166.7 (131.0-201.1) VS 116.4 (80.7-152.1)/mm 2 , p = 0.017], and LMVD were positively correlated with Log (CVF) (r = 0.431, p = 0.004). On multiple variables regression analysis, LMVD was independently associated with Log (CVF) (r = 0.379, p = 0.009) and syncope (r = 0.335, p = 0.020). In conclusions, the LYVE-1-positive lymphatics have close associations with VS fibrosis in HOCM patients.

Background: Atrial fibrillation (AF) is the most common complication in hypertrophic cardiomyopathy (HCM). The mechanisms of AF is associated with left atrial (LA) structural remodeling in HCM patients. However, the impact of left ventricular (LV) remodeling on the presence of AF in HCM patients has not been evaluated yet. We sought to investigate effect of LV remodeling on the presence of AF assessed by cardiovascular magnetic resonance (CMR) in HCM patients., Methods: A total of 394 HCM patients were enrolled into this study, including HOCM patients (n = 293) and NOHCM patients (n = 101). Patients were divided into HCM with AF (50) and HCM without AF (n = 344). Data were collected from hospital records., Results: LA diameter and LV remodeling index (LVRI) were significantly higher in HCM patients with AF than that of HCM patients without AF (46.6 ± 7.4 mm versus 39.9 ± 8.0 mm, p < 0.001, and 1.46 ± 0.6 versus 1.2 ± 0.4, p = 0.002, respectively). HCM patients with AF were older than HCM patients without AF (53.6 ± 11.7 years versus 47.7 ± 13.6 years, p = 0.002). Additionally, LVRI positively correlated to LA size (r = 0.12, p = 0.02). In a multivariable logistic regression analysis, when adjusting for age and LV end diastolic mass index, LVRI and LA size remained an independent determinant of AF in HCM patients (OR = 4.7, p = 0.001 and OR = 1.13, P < 0.001)., Conclusion: HCM patients with AF showed significantly more LA diameter, LVRI and age than HCM patients without AF. LVRI and LA size were strong independent predictor of AF in HCM, suggesting LV remodeling may contribute to the occurrence of AF in HCM patients.

Objective: ABO blood type is associated with cardiovascular diseases. Several studies have suggested sex-related differences in both hypertrophic cardiomyopathy (HCM) clinical features and ABO blood type. However, few data are available regarding the relationship between ABO blood type and HCM clinical features. We aimed to analyze the relationship between ABO blood type and HCM clinical features, and the potential effects of sex on these relationship., Methods: A total of 549 patients with HCM were enrolled consecutively. Left ventricular outflow tract gradients at rest (LOVTG-R) were measured by echocardiography. Left ventricular end-diastolic dimension, interventricular septum, left ventricular ejection fraction (LVEF), left ventricular end-diastolic volume (LVEDV), left ventricular end-systolic volume (LVESV), and left ventricular mass (LVM) were assessed using cardiovascular magnetic resonance imaging., Results: Compared with the non-B antigen group, patients with B antigen had significantly higher LOVTG-R and LVEF values, worse New York Heart Association (NYHA) classification, lower left ventricular volume index values, as well as no difference in LVM index values. After adjustments for sex, male patients with B antigen still had higher LOVTG-R values and frequency of NYHA classification III/IV as well as lower LVEDV and LVESV index values. These differences were not present in female patients. Additionally, patients with NYHA classification III/IV had lower LVEDV index values., Conclusion: In males, not females, patients with HCM with blood type B antigens exhibited worse cardiac functional capacity, higher LOVTG-R values, and lower left ventricular volume index values. These relationships are a potential indicator for clinical prevention. We speculate that rehydration is more efficient in relieving symptoms in male patients with HCM with B antigens.

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., (© 2019 S. Karger AG, Basel.)

Background: Both high-sensitivity cardiac troponin T and B-type natriuretic peptide are useful in detecting myocardial fibrosis, as determined by late gadolinium enhancement (LGE) cardiovascular magnetic resonance (CMR), in patients with non-obstructive hypertrophic cardiomyopathy. However, their values to predict myocardial fibrosis in hypertrophic obstructive cardiomyopathy (HOCM) remain unclear. We investigated the role of N-Terminal Pro-B-Type Natriuretic Peptide (NT-proBNP) and cardiac troponin I (cTnI) to identify LGE-CMR in patients with HOCM., Methods: Peripheral concentrations of NT-proBNP and cTnI were determined in patients with HOCM (n = 163; age = 47.2 ± 10.8 years; 38.7% females). Contrast-enhanced CMR was performed to identify and quantify myocardial fibrosis., Results: LGE was detected in 120 of 163 patients (73.6%). Patients with LGE had significantly higher levels of NT-proBNP and cTnI than those without LGE (1386.2 [904.6-2340.8] vs. 866.6 [707.2-1875.2] pmol/L, P = 0.003; 0.024 [0.010-0.049] vs. 0.010 [0.005-0.021] ng/ml, P <0.001, respectively). The extent of LGE was positively correlated with log cTnI (r = 0.371, P <0.001) and log NT-proBNP (r = 0.211, P = 0.007). On multivariable analysis, both log cTnI and maximum wall thickness (MWT) were independent predictors of the presence of LGE (OR = 3.193, P = 0.033; OR = 1.410, P < 0.001, respectively), whereas log NT-proBNP was not. According to the ROC curve analysis, combined measurements of MWT ≥21 mm and/or cTnI ≥0.025 ng/ml indicated good diagnostic performance for the presence of LGE, with specificity of 95% or sensitivity of 88%., Conclusions: Serum cTnI is an independent predictor useful for identifying myocardial fibrosis, while plasma NT-proBNP is only associated with myocardial fibrosis on univariate analysis. Combined measurements of serum cTnI with MWT further improve its value in detecting myocardial fibrosis in patients with HOCM.

Serum cardiac troponins have been demonstrated to have important clinical implications in patients with hypertrophic cardiomyopathy (HC). However, little is known about their roles in patients with obstructive HC. The aim of this study was to explore the clinical significance and determinants of serum cardiac troponin I (cTnI) in patients with obstructive HC using cardiovascular magnetic resonance imaging. We investigated the relations between serum cTnI levels and clinical, echocardiographic, and cardiovascular magnetic resonance parameters and assessed the determinants of serum cTnI in 149 consecutive patients with obstructive HC. The median level of serum cTnI was 0.019 ng/ml (interquartile range 0.009 to 0.044). CTnI was elevated (≥0.04 ng/ml) in 42 (28%) of the overall cohort. Patients with elevated cTnI had greater maximum wall thickness (p <0.001), larger left ventricular mass index (LVMI, p <0.001), more frequency of left atrium diameter ≥50 mm (p = 0.020), higher plasma values of N-terminal pro-B-type natriuretic peptide (p <0.001), and less hypertension (p = 0.014). Serum cTnI levels were positively correlated with maximum wall thickness (r = 0.444, p <0.001), LVMI (r = 0.556, p <0.001), N-terminal pro-B-type natriuretic peptide (r = 0.305, p <0.001), left ventricular end-diastolic volume index (r = 0.246, p = 0.002), and left ventricular end-systolic volume index (r = 0.272, p = 0.001) but negatively with left ventricular ejection fraction (r = -0.180, p = 0.028). On multivariate analysis, LVMI was independently associated with both elevated cTnI (odds ratio 1.032, p = 0.001) and increasing serum cTnI levels (β = 0.556, p <0.001). In addition, the presence of hypertension was independently related to less likely elevated cTnI (odds ratio 0.307, p = 0.029) and decreasing levels of serum cTnI (β = -0.165, p = 0.015). In conclusion, levels of serum cTnI are elevated in a significant proportion of our patients. Serum cTnI is associated with multiple parameters of disease severity, suggesting its great significance in assessing cardiac remodeling in patients with obstructive HC. Left ventricular hypertrophy, as indicated by LVMI, is the major determinant of serum cTnI levels., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Objective: We investigated the effect of ovariectomy (OVX) and 17β-estradiol (E2) replacement on both mitochondrial and myocardial function in cTnT-Q92 transgenic mice generated by cardiac-restricted expression of a human hypertrophic cardiomyopathy (HCM) mutation., Methods: The cTnT-Q92 mice were ovariectomized at twenty weeks of age and were treated with either placebo (OVX group) or E2 (OVX+E2 group) for twelve weeks before being sacrificed. Wild-type and cTnT-Q92 female mice receiving sham operation were used as controls. Indices of diastolic function such as mitral early (E) and late (A) inflow as well as isovolumic relaxation time (IVRT) were measured by echocardiography. A Clark-type electrode was used to detect respiratory control, and ATP levels were determined at the mitochondrial level using HPLC. Key components related to mitochondrial energy metabolism, such as peroxisome proliferator-activated receptor α (PPARα), PPARγ coactivator 1α (PGC-1α) and nuclear respiratory factor-1 (NRF-1), were also analyzed using Western blot and RT-PCR. The levels of oxidative stress markers were determined by measuring malondialdehyde (MDA) using the thiobarbituric acid assay., Results: The cTnT-Q92 mice had impaired diastolic function compared with wild-type mice (E/A ratio, 1.39 ± 0.04 vs. 1.21 ± 0.01, p<0.001; IVRT, 19.17 ± 0.85 vs. 22.15 ± 1.43 ms, p=0.028). In response to ovariectomy, cardiac function further decreased compared with that observed in cTnT-Q92 mice that received the sham operation (E/A ratio, 1.15 ± 0.04 vs. 1.21 ± 0.01, p<0.001; IVRT, 28.31 ± 0.39 vs. 22.15 ± 1.43 ms, p=0.002). Myocardial energy metabolism, as determined by ATP levels (3.49 ± 0.31 vs. 5.07 ± 0.47 μmol/g, p<0.001), and the mitochondrial respiratory ratio (2.04 ± 0.10 vs. 2.63 ± 0.11, p=0.01) also decreased significantly. By contrast, myocardial concentrations of MDA increased significantly in the OVX group, and PGC-1α, PPARα and NRF-1decreased significantly. E2 supplementation significantly elevated myocardial ATP levels (4.55 ± 0.21 vs. 3.49 ± 0.31 μmol/g, p=0.003) and mitochondrial respiratory function (3.93 ± 0.05 vs. 2.63 ± 0.11, p=0.001); however, it reduced the MDA level (0.21 ± 0.02 vs. 0.36 ± 0.03 nmol/g, p<0.001), which subsequently improved diastolic function (E/A ratio, 1.35 ± 0.06 vs. 1.15 ± 0.04, p<0.001; IVRT, 18.22 ± 1.16 vs. 28.31 ± 0.39 ms, p=0.007)., Conclusions: Our study has shown that 17β-estradiol improved myocardial diastolic function, prevented myocardial energy dysregulation, and reduced myocardial oxidative stress in cTnT-Q92 mice., (Copyright © 2014 Elsevier Ltd. All rights reserved.)