scholarly journals Baseline clinical features in a large-scale registration survey of patient with hypertrophic cardiomyopathy throughout Japan: J-HCM registry study

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
T Kubo ◽  
H Takano ◽  
M Takayama ◽  
Y.L Doi ◽  
Y Minami ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Clinical Features ◽  
Clinical Characteristics ◽  
Large Scale ◽  
Risk Group ◽  
Nyha Class ◽  
Left Ventricular ◽  
Registry Study ◽  
Icd Implantation ◽  
Successful Recovery

Abstract Background Hypertrophic cardiomyopathy (HCM) is a most prevalent primary myocardial disorder with heterogeneous clinical features. However, there have been few studies on clinical features of HCM as a prospective cohort. In 2015, we established a large-scale registration survey of patients with HCM throughout Japan, named J-HCM registry study. Purpose The aim of this study was to clarify the clinical features of Japanese patients with HCM. Methods J-HCM registry study is a prospective, multicenter investigation, consisting of 24 hospitals. This time, we present the baseline clinical characteristics in this survey. Results Total 1484 patients were registered. The ages at registration and at diagnosis were 65±15 and 56±17 years, respectively, and 806 patients (54%) were men. Majority of the patients (95%) was NYHA class I or II. With regard to subtypes of HCM, there were 526 patients (36%) in the HCM with left ventricular (LV) outflow tract obstruction, 126 patients (8%) in the mid-ventricular obstruction, 57 patients (4%) in the end-stage phase characterized by LV ejection fraction <50%, and 197 patients (14%) in apical HCM. At registration, 80 patients (6%) had prior successful recovery from sustained ventricular tachycardia or ventricular fibrillation, 162 patients (11%) suffered from heart failure hospitalization, and 64 patients (4%) had history of embolic event. Regarding invasive treatment, 160 patients (10%) had prior septal reduction therapy and 162 patients (11%) had ICD implantation. According to the 2014 European Society of Cardiology Guidelines on sudden cardiac death (SCD) prevention, the study patients were divided into 3 categories by the HCM Risk-SCD calculator: patients distribution, 4% in the high risk group (≥6% calculated HCM Risk-SCD at 5 years), 7% in the intermediate risk group (4% to <6%), 69% in the low risk group (<4%), and 16% in the patients with extreme characteristics (Figure 1). Conclusions In this multicenter registration survey of patients with HCM, the baseline clinical characteristics were almost similar to several retrospective large-scale cohorts in Western countries except older age and less symptomatic state. This study will provide important knowledge regarding management of HCM. Figure 1 Funding Acknowledgement Type of funding source: None

P887Atrial fibrillation is associated with sudden cardiac death risk factors in patients with hypertrophic cardiomyopathy

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
I A Marcuschamer ◽  
O Zusman ◽  
S Schwartzenberg ◽  
M Vaturi ◽  
Y Shapira ◽  
...  
Keyword(s):  
Risk Factors ◽  
Ventricular Tachycardia ◽  
Hypertrophic Cardiomyopathy ◽  
Sudden Cardiac Death ◽  
Cardiac Death ◽  
Nearest Neighbor ◽  
Nyha Class ◽  
Left Ventricular ◽  
Similar Distribution ◽  
Icd Implantation

Abstract Background Atrial Fibrillation (AF) is prevalent in a fourth of patients with Hypertrophic Cardiomyopathy (HCM), but its clinical impact in these patients remains ill-defined. Aim To compare clinical characteristics in HCM patients with vs without AF and assess indirectly potential sudden cardiac death (SCD) risk. Methods Retrospective study in a single tertiary referral HCM center. Patients with HCM and AF were compared with matched controlled HCM patients without AF. NYHA class was assessed by a single physician. Propensity score matching was performed with a ratio of 2:1 by nearest neighbor with adjustment for age, sex, and left ventricular tract obstruction (LVOTO). Ordinal regression was used with NYHA as outcome. Results Among 298 patients with HCM, 68 patients (22.8%) had AF. After propensity matching, 66 patients with AF and 112 without AF had similar distribution of age (67.1 vs. 65.1 years), gender (57.6% vs. 61% males) and Basal Surface Area (1.88 vs. 1.87 m2) respectively. The prevalence of LVOTO (57.6% vs. 58.5%) and apical hypertrophy (19.7% vs 19.5%) was similar in the two groups. Cardiac risk factors including Hypertension (60.6% vs. 60.2%) and Diabetes Mellitus (15.2 vs. 20.3%) were similar in both groups. AF patients were diagnosed with HCM at a younger age than patients without HCM (48.5 vs. 55 years; p=0.01). HCM patients with AF had significantly lower LVOT gradients compared with patients without AF (28.1 mmHg vs 47.4 mmHg, p=0.005), had a higher prevalence of non-sustained ventricular tachycardia (39.4% vs. 9.4%; p<0.01), and ventricular tachycardia (9.1% vs 1.7%; p<0.04) and were more likely to have undergone implantation of an internal cardioverter defibrillator (ICD) (23.1% vs. 8.5%; p=0.001), respectively. Dyspnea was the most prevalent symptom in both groups (51.1% and 46.6% in AF and non-AF respectively). NYHA Class was similar in both groups: 1.88±0.69 in patients with AF vs. 1.73±0.74 in patients without AF (p=0.17). NYHA class did not differ in 26 patients with chronic persistent AF vs. 42 patients with paroxysmal AF (being in sinus rhythm at evaluation). Conclusion AF does not seem to impact functional level class in patients with HCM, but carries a higher burden of sudden cardiac death prognostic factors, incurring a higher rate of ICD implantation. Acknowledgement/Funding None

Abstract 18973: Left Ventricular Function Improvement After Recent MI in Patients Using a Wearable Cardioverter Defibrillator

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Chingping Wan ◽  
Steven J Szymkiewicz
Keyword(s):  
Heart Failure ◽  
Left Ventricular Function ◽  
Ventricular Function ◽  
Clinical Characteristics ◽  
Treated Group ◽  
Left Ventricular ◽  
Icd Implantation ◽  
Cardioverter Defibrillator ◽  
Wearable Cardioverter Defibrillator

Introduction: The wearable cardioverter defibrillator (WCD) has been used to protect AMI patients with reduced LVEF (≤35%) until ICD evaluation is recommended. The rate of EF improvement (e.g. EF>35%) over the initial 8-12 weeks after AMI has not been reported. METHODS: The manufacturer-maintained registry was searched for AMI patients who received a WCD shock for VT/VF between 05/2008 and 02/2013. The treated group was matched (1: ~4) with event-free WCD patients by ICD-9 code (410.*), gender, age and prescription date. Chart notes were reviewed for clinical characteristics. Follow-up was assessed through the registry and Social Security Death Master File. RESULTS: There were 992 (age=63±12, female=20.2%) AMI patients included in the final analysis, 206 treated by WCD and 786 event-free patients. Median follow-up was 334 days. Mean length of WCD use was 67±506 (median=38) days. Subgroup clinical characteristics are presented in Table 1. In the event-free group, 289 (38.9%) patients showed EF improvement to >35%. Nine (4.5%) in the treated group continued wearing the WCD until EF recovery, while 125 (60.7%) received ICD. Absence of recorded heart failure and/or diabetes were associated with LVEF recovery (p<.0001). CONCLUSION: In our study, almost 40% of AMI patients with initial EF ≤35% had EF improvement in two months. The EF recovery group had lower rates of heart failure and diabetes. WCD allows time for left ventricular function recovery in low EF post MI patients, optimizing ICD implantation decisions.

Abstract 19346: Peak Oxygen Consumption Predicts Outcome in Hypertrophic Cardiomyopathy

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Carla Contaldi ◽  
Raffaella Lombardi ◽  
Alessandra Giamundo ◽  
Sandro Betocchi
Keyword(s):  
Heart Failure ◽  
Oxygen Consumption ◽  
Hypertrophic Cardiomyopathy ◽  
Nyha Class ◽  
Left Ventricular ◽  
Exercise Intolerance ◽  
Peak Oxygen Consumption ◽  
Class Iii ◽  
Asymptomatic Patients

Introduction: Peak oxygen consumption (VO 2 ) has a strong and independent prognostic value in systolic heart failure; in contrast no data support its prognostic role in hypertrophic cardiomyopathy (HCM). Hypothesis: We assess if peak VO 2 is a long-term predictor of outcome in HCM. Methods: We studied 92 HCM patients (40±15 years). Peak VO 2 was expressed as percentage (%) of the predicted value. Follow up was 76±57 months. The primary composite endpoint (CE) was atrial fibrillation, progression to NYHA class III or IV, myotomy-myectomy (MM), heart transplantation (HT) and cardiac death. An ancillary endpoint (HFE) included markers of heart failure (progression to NYHA class III or IV, MM and HT). Results: At baseline, 62% of patients were asymptomatic, 35% NYHA class II and 3% NYHA class III; 26% had left ventricular outflow tract obstruction. During follow up, 30 patients met CE with 43 events. By multivariate Cox survival analysis, we analyzed 2 models, using the CE, and in turn HFE. For CE, maximal left atrial diameter (LAD) (HR: 1.12; 95% CI: 1.04 to 1.22), maximal wall thickness (MWT) (HR: 0.14; 95% CI: 1.04 to 1.23) and % predicted peak VO 2 (HR: -0.03; 95% CI: 0.95 to 0.99) independently predicted outcome (overall, p<0.0001). For HFE, maximal LAD (HR:0.31; 95% CI: 1.09 to 1.70), MWT (HR: 0.35; 95% CI: 1.08 to 1.84) and % predicted peak VO 2 (HR: -0.06; 95% CI: 0.89 to 0.98) independently predicted outcome (overall, p<0.0001). Only 19% of mildly symptomatic or asymptomatic patients with % predicted peak VO 2 >80% had events, as opposed to 53% of them with % predicted peak VO 2 < 55% (p= 0.04). Event-free survival for both endpoints was significantly lower in patients with % predicted peak VO 2 < 55% as compared to those with it between 55 and 80 and >80% , Figure. Conclusion: In mildly or asymptomatic patients severe exercise intolerance may precede clinical deterioration. In HCM, peak VO 2 provides excellent risk stratification with a high event rate in patients with % predicted value <55%.

scholarly journals P1291 Are we ready for machine learning in HCM risk stratification?

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
D M Adamczak ◽  
M Bednarski ◽  
A Rogala ◽  
M Antoniak ◽  
T Kiebalo ◽  
...  
Keyword(s):  
Machine Learning ◽  
High Risk ◽  
Risk Group ◽  
Ventricular Myocardium ◽  
Left Ventricular ◽  
High Risk Group ◽  
Machine Learning Algorithms ◽  
Left Ventricular Myocardium ◽  
Risk Calculator ◽  
Icd Implantation

Abstract BACKGROUND Hypertrophic cardiomyopathy (HCM) is a heart disease characterized by hypertrophy of the left ventricular myocardium. The disease is the most common cause of sudden cardiac death (SCD) in young people and competitive athletes due to fatal ventricular arrhythmias, but in most patients, however, HCM has a benign course. Therefore, it is of the utmost importance to properly evaluate patients and identify those who would benefit from a cardioverter-defibrillator (ICD) implantation. The HCM SCD-Risk Calculator is a useful tool for estimating the 5-year risk of SCD. Parameters included in the model at evaluation are: age, maximum left ventricular wall thickness, left atrial dimension, maximum gradient in left ventricular outflow tract, family history of SCD, non-sustained ventricular tachycardia and unexplained syncope. Patients’ risk of SCD is classified as low (&lt;4%), intermediate (4-&lt;6%) or high (≥6%). Those in the high-risk group should have an ICD implantation. It can also be considered in the intermediate-risk group. However, the calculator still needs improvement and machine learning (ML) has the potential to fulfill this task. ML algorithm creates a model for solving a specific problem without explicit programming - instead it relies only on available data - by discovering patterns and relations. METHODS 252 HCM patients (aged 20-88 years, 49,6% were men) treated in our Department from 2005 to 2018, have been enrolled. The follow-up lasted 0-13 years (average: 3.8 years). SCD was defined as sudden cardiac arrest (SCA) or an appropriate ICD intervention. All parameters from HCM SCD-Risk Calculator have been obtained and the risk of SCD has been calculated for all patients during the first echocardiographic evaluation. ML model with variables from HCM SCD-Risk Calculator has been created. Both methods have been compared. RESULTS 20 patients reached an SCD end-point. 1 patient died due to SCA and 19 had an appropriate ICD intervention. Among them, there were respectively 6, 7 and 7 patients in the low, intermediate and high-risk group of SCD. 1 patient, who died, had a low risk. The ML model correctly assessed the SCD event only in 1 patient. According to ML, the risk of SCD ≤2.07% was a negative predictor. CONCLUSIONS The study did not show an advantage of ML over HCM SCD-Risk Calculator. Because of the characteristic of the dataset (approximately the same number of features and observations), the selection of machine learning algorithms was limited. Best results (evaluated using LOOCV) were achieved with a decision tree. We expect that bigger dataset would allow improving model performance because of strong regularization need in the current setup.

Effect of hypokintic transformation on the clinical phenotype and functional capacity in hypertrophic cardiomyopathy

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
Y Wasserstrum ◽  
E Itelman ◽  
R Barriales-Villa ◽  
X Fernandez-Fernandez ◽  
Y Adler ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Nyha Class ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Ventricular Tachyarrhythmias ◽  
Ventricular Ejection Fraction ◽  
Icd Therapy ◽  
Medical Centers ◽  
Increased Risk

Abstract Background Advanced hypertrophic cardiomyopathy (HCM) may be complicated by a dilated hypokinetic transformation. Reduced left ventricular ejection fraction (HFrEF) has been described in terms of specific risks of morbidity and mortality, and specifically in terms of increased risk for fatal arrhythmias. Nevertheless, recent publications have casted doubt regarding the role of arrhythmia in non-ischemic HFrEF and questioned the role of primary prevention strategies in these cases. Methods We've reviewed clinical characteristics of 883 patients age ≥40, diagnosed with HCM who were evaluated in the cardiomyopathy clinic in two tertiary medical centers in Israel and Spain. Results Forty-five patients (5%) suffered from hypokinetic transformation. They were younger at diagnosis (median 32 [IQR 24–55] vs. 49 [35–60], p&lt;0.001), had a lower body-mass index (28.4 [±4.7] vs. 26.0 [±3.9], p&lt;0.001), and suffered more from strokes (19% vs 6%, p&lt;0.001). They had lower had a lower NYHA class (p=0.001) and lower exercise capacity (7.3 [4.5–10.8] vs. 9.6 [6.7–12.0] METS, p&lt;0.001). Patients with hypokinetic HCM had higher rates of pacemaker and implanted defibrillator (ICD) implantations (41% vs 11%, p&lt;0.001) and (43% vs 13%, p&lt;0.001) respectively. These patients had a higher incidence of sustained ventricular tachyarrhythmias (14% vs 2%, p&lt;0.001). Among patients who had an ICD, patients suffering from hypokinetic transformation had received more appropriate ICD therapy (27% vs 12%, p&lt;0.001). These patients received more heart transplantations (13% vs 1%, p&lt;0.001), and had a trend for higher incidence rate of Sudden cardiac death (6% vs 2% p=0.06) and a higher 5-year mortality rates (21% vs. 5%, p&lt;0.001). Conclusions HCM patients suffering from hypokinetic transformation have lower functional and exercise capacities, are more likely to suffer from ventricular tachyarrhythmias and experience appropriate ICD therapy, and undergo heart transplantation. They also have a significantly lower 5-year survival. Five-year survival Funding Acknowledgement Type of funding source: None

Hypertrophic cardiomyopathy: prevention of sudden cardiac death

2018 ◽  
Author(s):  
Constantinos O’Mahony
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Sudden Cardiac Death ◽  
Risk Stratification ◽  
Cardiac Death ◽  
Clinical Care ◽  
Psychological Impact ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Icd Implantation ◽  
European Society Of Cardiology

Sudden cardiac death (SCD) secondary to ventricular arrhythmias is the most common mode of death in hypertrophic cardiomyopathy (HCM) and can be effectively prevented with an implantable cardioverter defibrillator (ICD). The risk of SCD in HCM relates to the severity of the phenotype and regular risk stratification is an integral part of routine clinical care. For the primary prevention of SCD, risk stratification involves the assessment of seven readily available clinical parameters (age, maximal left ventricular wall thickness, left atrial diameter, left ventricular outflow tract gradient, non-sustained ventricular tachycardia, unexplained syncope, and family history of SCD) which are used to estimate the risk of SCD within 5 years of clinical evaluation using a statistical risk prediction model (HCM Risk-SCD). The 2014 European Society of Cardiology Guidelines provide a framework to aid clinical decisions and consider patients with a 5-year risk of SCD of less than 4% as low risk and recommend regular assessment while those with a risk of 6% or higher should be considered for an ICD. In patients with an intermediate risk (4% to <6%) ICD implantation may also be considered after taking into account age, co-morbid conditions, socioeconomic factors, and the psychological impact of therapy. Survivors of ventricular fibrillation arrest should receive an ICD for secondary prevention unless their life expectancy is less than 1 year. Following device implantation, patients should be followed up for device- and disease-related complications, particularly heart failure and cerebrovascular disease.

scholarly journals 577 Bisoprolol for the treatment of symptomatic patients with obstructive hypertrophic cardiomyopathy

2021 ◽  
Vol 23 (Supplement_G) ◽  
Author(s):  
Emanuele Monda ◽  
Michele Lioncino ◽  
Giuseppe Palmiero ◽  
Marta Rubino ◽  
Annapaola Cirillo ◽  
...  
Keyword(s):  
New York ◽  
Hypertrophic Cardiomyopathy ◽  
Primary Endpoint ◽  
Nyha Class ◽  
Significant Proportion ◽  
Heart Association ◽  
Ventricular Outflow Tract ◽  
Maximum Tolerated Dose ◽  
Left Ventricular ◽  
Valsalva Manoeuvre

Abstract Aims To evaluate to role of bisoprolol to control symptoms and left ventricular outflow tract obstruction (LVOTO) in a consecutive cohort of adults with hypertrophic cardiomyopathy (HCM). Methods and results In this retrospective study, patients with HCM with an LVOT gradient ≥50mmHg after Valsalva manoeuvre and New York Heart Association (NYHA) class II-III symptoms were assigned to receive bisoprolol (starting at 1.25 mg daily). The initial dose was increased every two weeks to achieve the target in LVOT gradient &lt;30 mmHg or the maximum tolerated dose. The primary endpoint was the achievement of a LVOT gradient &lt;30 mmHg and ≥1 NYHA class improvement. The secondary endpoints were proportion of patients with LVOT gradient &lt;30 mmHg or &lt; 50 mmHg, proportion of patients with ≥1 NYHA class improvement, and change from baseline in LVOT gradient. Between December 2001 and December 2020, 92 patients were enrolled into the study. Sixteen (17%) patients on bisoprolol met the primary endpoint. Bisoprolol reduced the LVOT gradient to less than 30 mmHg in 33 (36%) patients, to less than 50 mmHg in 57 (62%), and improved NYHA class in 30 (33%). The mean reduction of LVOT gradient on bisoprolol was 28 (±14) mmHg and the percentage reduction was 42 (±21)%. In 35 (39%) patients, bisoprolol did not reduce the gradient to less than 50 mmHg requiring disopyramide and/or myectomy to achieve this goal. Conclusions Treatment with bisoprolol was well-tolerated and effective in relieving obstruction and improving symptoms in a significant proportion of patients with symptomatic obstructive HCM.

scholarly journals Mid-regional proatrial natriuretic peptide for predicting prognosis in hypertrophic cardiomyopathy

Heart ◽  
2019 ◽  
pp. heartjnl-2019-314826 ◽  
Author(s):  
Céline Bégué ◽  
Stellan Mörner ◽  
Dulce Brito ◽  
Christian Hengstenberg ◽  
John G F Cleland ◽  
...  
Keyword(s):  
Heart Failure ◽  
New York ◽  
Hypertrophic Cardiomyopathy ◽  
Natriuretic Peptide ◽  
Nyha Class ◽  
Heart Association ◽  
Roc Curves ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Ventricular Ejection Fraction

ObjectivesN-terminal probrain natriuretic peptide (NT-proBNP) predicts mortality and the development of heart failure in hypertrophic cardiomyopathy (HCM). Mid-regional proatrial natriuretic peptide (MR-proANP) is a stable by-product of production of atrial natriuretic peptide. We sought to compare the prognostic value of MR-proANP and NT-proBNP in HCM.MethodsWe prospectively enrolled a cohort of patients with HCM from different European centres and followed them. All patients had clinical, ECG and echocardiographic evaluation and measurement of MR-proANP and NT-proBNP at inclusion.ResultsOf 357 patients enrolled, the median age was 52 (IQR: 36–65) years. MR-proANP and NT-proBNP were both independently associated with age, weight, New York Heart Association (NYHA) class, left ventricular ejection fraction (LVEF), wall thickness and left atrial dimension. During a median follow-up of 23 months, 32 patients had a primary end point defined as death (n=6), heart transplantation (n=8), left ventricular assist device implantation (n=1) or heart failure hospitalisation (n=17). Both NT-proBNP and MR-proANP (p<10–4) were strongly associated with the primary endpoint, and the areas under the receiver operating characteristic (ROC) curves for both peptides were not significantly different. However, in a multiple stepwise regression analysis, the best model for predicting outcome was NYHA 1–2 vs 3–4 (HR=0.35, 95% CI 0.16 to 0.77, p<0.01), LVEF (HR=0.96, 95% CI 0.94 to 0.98, p=0.0005) and MR-proANP (HR=3.77, 95% CI 2.01 to 7.08, p<0.0001).ConclusionsMR-proANP emerges as a valuable biomarker for the prediction of death and heart failure related events in patients with HCM.

Long-term outcomes and left ventricular diastolic function of sarcomere mutation-positive and mutation-negative patients with hypertrophic cardiomyopathy: a prospective cohort study

2020 ◽  
Author(s):  
Ching-Yu Julius Chen ◽  
Mao-Yuan Marine Su ◽  
Ying-Chieh Liao ◽  
Fu-Lan Chang ◽  
Cho-Kai Wu ◽  
...  
Keyword(s):  
New York ◽  
Hypertrophic Cardiomyopathy ◽  
Nyha Class ◽  
Global Longitudinal Strain ◽  
Heart Association ◽  
Left Ventricular Diastolic Function ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Class Iii ◽  
Ventricular Ejection Fraction

Abstract Aims Hypertrophic cardiomyopathy (HCM) is an inheritable disease that leads to sudden cardiac death and heart failure (HF). Sarcomere mutations (SMs) have been associated with HF. However, the differences in ventricular function between SM-positive and SM-negative HCM patients are poorly characterized. Methods and results  Of the prospectively enrolled 374 unrelated HCM patients in Taiwan, 115 patients underwent both 91 cardiomyopathy-related gene screening and cardiovascular magnetic resonance (45.6 ± 10.6 years old, 76.5% were male). Forty pathogenic/likely pathogenic mutations were identified in 52 patients by next-generation sequencing. The SM-positive group were younger at first cardiovascular event (P = 0.04) and progression to diastolic HF (P = 0.02) with higher N-terminal pro-brain natriuretic peptide (NT-proBNP) [New York Heart Association (NYHA) Class III/IV symptoms with left ventricular ejection fraction &gt; 55%] than the SM-negative group (P &lt; 0.001). SM-positive patients had a greater extent of late gadolinium enhancement (P = 0.01), larger left atrial diameter (P = 0.03), higher normalized peak filling rate (PFR) and PFR ratio, and a greater reduction in global longitudinal strain than SM-negative patients (all P ≤ 0.01). During mean lifelong follow-up time (49.2 ± 15.6 years), SM-positive was a predictor of earlier HF (NYHA Class III/IV symptoms) after multivariate adjustment (hazard ratio 3.5; 95% confidence interval 1.3–9.7; P = 0.015). Conclusion SM-positive HCM patients had a higher extent of myocardial fibrosis and more severe ventricular diastolic dysfunction than those without, which may contribute to earlier onset of advanced HF, suggesting the importance of close surveillance and early treatment throughout life.

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