Sex-Related Differences in Patients With Hypertrophic Cardiomyopathy Undergoing Alcohol Septal Ablation.

BACKGROUND: Previous studies have shown that women with hypertrophic obstructive cardiomyopathy (HCM) have worse long-term outcomes irrespective of intervention. However, the outcomes of patients undergoing alcohol septal ablation (ASA) based on sex have not been described. Hence, this study aimed to evaluate pressure changes and long-term mortality in patients with HCM undergoing ASA based on sex. METHODS AND RESULTS: This is a single-center retrospective study evaluating hemodynamic changes and long-term mortality in patients with HCM treated with ASA according to sex. A total of 259 patients were included (aged 68.4±11.9 years, 62.2% women). Women had higher age and baseline pressures at the time of ASA, with a greater percent reduction in mean left atrial pressure (men versus women: 2.2% versus 15.9%, respectively; P=0.02). Women had better survival (median survival rate of men versus women: 8.6 versus 12.5 years, respectively; P=0.011). On Cox multivariable regression, predictors of mortality were age (per group change <60 years, 61-70 years, 71-80 years, and >80 years; hazard ratio [HR], 1.45 [95% CI, 1.10-1.91], P=0.008), female sex (HR, 0.59 [95% CI, 0.35-0.99], P=0.048), chronic kidney disease (HR, 1.88 [95% CI, 1.06-3.33], P=0.031), and left ventricular outflow tract gradient reduction ≤86% (HR, 1.91 [95% CI, 1.14-3.19], P=0.014). CONCLUSIONS: Women with HCM undergoing ASA are older and have higher left-sided baseline pressures compared with men yet have better survival. Further studies exploring the mechanisms of differential outcomes according to sex in patients with HCM undergoing ASA are needed.

Clinical characteristics and outcomes of alcohol septal ablation in the era of transcatheter valve interventions.

BACKGROUND: The clinical efficacy and safety of alcohol septal ablation (ASA) for obstructive hypertrophic cardiomyopathy (HCM) have been well-established; however, less is known about outcomes in patients undergoing preemptive ASA before transcatheter mitral valve replacement (TMVR). AIMS: The goal of this study is to characterize the procedural characteristics and examine the clinical outcomes of ASA in both HCM and pre-TMVR. METHODS: This retrospective study compared procedural characteristics and outcomes in patient who underwent ASA for HCM and TMVR. RESULTS: In total, 137 patients were included, 86 in the HCM group and 51 in the TMVR group. The intraventricular septal thickness (mean 1.8 vs. 1.2 cm; p < 0.0001) and the pre-ASA LVOT gradient (73.6 vs. 33.8 mmHg; p ≤ 0.001) were higher in the HCM group vs the TMVR group. The mean volume of ethanol injected was higher (mean 2.4 vs. 1.7 cc; p < 0.0001). The average neo-left ventricular outflow tract area increased significantly after ASA in the patients undergoing TMVR (99.2 ± 83.37 mm2 vs. 196.5 ± 114.55 mm2; p = <0.0001). The HCM group had a greater reduction in the LVOT gradient after ASA vs the TMVR group (49.3 vs. 18 mmHg; p = 0.0040). The primary composite endpoint was higher in the TMVR group versus the HCM group (50.9% vs. 25.6%; p = 0.0404) and had a higher incidence of new permanent pacemaker (PPM) (25.5% vs. 18.6%; p = 0.3402). The TMVR group had a higher rate of all-cause mortality (9.8% vs. 1.2%; p = 0.0268). CONCLUSIONS: Preemptive ASA before TMVR was performed in patients with higher degree of clinical comorbidities, and correspondingly is associated with worse short-term clinical outcomes in comparison to ASA for HCM patients. ASA before TMVR enabled percutaneous mitral interventions in a small but significant minority of patients that would have otherwise been excluded. The degree of LVOT and neoLVOT area increase is significant and predictable.

Trends of hypertrophic cardiomyopathy-related mortality in United States young adults: a nationwide 20-year analysis.

AIMS: Data regarding hypertrophic cardiomyopathy (HCM)-related mortality in United States young adults, defined as those aged between 25 and 44 years, are lacking. We sought to assess the trends in HCM-related mortality among US young adults between 1999 and 2019 and determine differences by sex, race, ethnicity, urbanization and census region. METHODS: Mortality data were retrieved by the Centers for Disease Control and Prevention (CDC) Wide-Ranging Online Data for Epidemiologic Research (WONDER) dataset from January 1999 to December 2019. Age-adjusted mortality rates (AAMRs) were assessed using the Joinpoint regression modeling and expressed as estimated average annual percentage change (AAPC) with relative 95% confidence intervals (95% CIs). RESULTS: Over 20-year period, the AAMR from HCM in US young adults linearly decreased, with no differences between sexes [AAPC: -5.3% (95% CI -6.1 to -4.6), P  < 0.001]. The AAMR decrease was more pronounced in Black patients [AAPC: -6.4% (95% CI -7.6 to -5.1), P  < 0.001], Latinx/Hispanic patients [AAPC: -4.8% (95% CI -7.2 to -2.36), P  < 0.001] and residents of urban areas [AAPC: -5.4% (95% CI -6.2 to -4.6), P  < 0.001]. The higher percentages of HCM-related deaths occurred in the South of the country and at the patient's home. CONCLUSION: HCM-related mortality in US young adults has decreased over the last two decades in the United States. Subgroup analyses by race, ethnicity, urbanization and census region showed ethnoracial and regional disparities that will require further investigation.

Demographics and Trends of Hypertrophic Cardiomyopathy-Related Mortality in the United States, 1999-2020.

There are limited data on the mortality trends of HCM in the United States. To study the demographics and trends of mortality in patients with HCM, a retrospective cohort analysis was done with mortality data of patients with HCM listed as an underlying cause of death in the US Centers for Disease Control and Prevention Wide-Ranging Online Data for Epidemiologic Research database (CDC-WONDER) from January 1999 to December 2020. The analysis took place in February 2022. First, we measured HCM-related age-adjusted mortality rate (AAMR) per 100,000 US population stratified by sex, race, ethnicity, and geographic area. We then calculated the Annual Percentage Change (APC) for AAMR for each. A total of 24,655 HCM-related deaths occurred between 1999 and 2020. The AAMR for HCM-related deaths declined from 0.5/100,000 patients in 1999 to 0.2 in 2020. The APC changes are as follows: -6.8 (95% CI: -11.8 to -1.5) from 2002 to 2009, -1.23 (95% CI -13.8 to 13.2) from 2009 to 2014, -6.71 (95% CI -46.2 to 61.7) from 2014 to 2017 and remained at 2.07 (95% CI -26.1 to 41.1) from 2017 to 2020. Men had consistently higher AAMR than women. Overall, AAMR in men was 0.4 (95% CI: 0.4-0.5), and in women was 0.3 (95% CI: 0.3-0.3). A similar trend was noticed in men and women over the years, starting from 1999 (AAMR men: 0.7 and women: 0.4) to 2020 (AAMR men: 0.3 and women: 0.2). AAMRs were highest among black or African American patients 0.6 (95% CI: 0.5-0.6), followed by non-Hispanic and Hispanic white 0.3 (95% CI 0.3-0.3) and Asian or Pacific Islander 0.2 (95% CI 0.2-0.2). There was substantial variation in each region in the US. States such as California, Ohio, Michigan, Oregon, and Wyoming had the highest AAMR. Large metropolitan cities had higher AAMR than non-metropolitan cities. During the study period from 1999 to 2020, HCM-related mortality steadily decreased. The highest AAMR was observed among men, black patients, and residents of metropolitan areas. States such as California, Ohio, Michigan, Oregon, and Wyoming had the highest AAMR.

Management of Hypertrophic Cardiomyopathy: JACC State-of-the-Art Review.

Hypertrophic cardiomyopathy (HCM), a relatively common, globally distributed, and often inherited primary cardiac disease, has now transformed into a contemporary highly treatable condition with effective options that alter natural history along specific personalized adverse pathways at all ages. HCM patients with disease-related complications benefit from: matured risk stratification in which major markers reliably select patients for prophylactic defibrillators and prevention of arrhythmic sudden death; low risk to high benefit surgical myectomy (with percutaneous alcohol ablation a selective alternative) that reverses progressive heart failure caused by outflow obstruction; anticoagulation prophylaxis that prevents atrial fibrillation-related embolic stroke and ablation techniques that decrease the frequency of paroxysmal episodes; and occasionally, heart transplant for end-stage nonobstructive patients. Those innovations have substantially improved outcomes by significantly reducing morbidity and HCM-related mortality to 0.5%/y. Palliative pharmacological strategies with currently available negative inotropic drugs can control symptoms over the short-term in some patients, but generally do not alter long-term clinical course. Notably, a substantial proportion of HCM patients (largely those identified without outflow obstruction) experience a stable/benign course without major interventions. The expert panel has critically appraised all available data and presented management insights and recommendations with concise principles for clinical decision-making.

Hypertrophic cardiomyopathies requiring more monitoring for less atrial fibrillation-related complications: a clustering analysis based on the French registry on hypertrophic cardiomyopathy (REMY).

AIMS: Defining the risk of atrial fibrillation (AF) in hypertrophic cardiomyopathy (HCM) patients is an important clinical and prognostic challenge. The aim of this study is to determine HCM phenogroups with different risk of AF occurrence at 5 years. METHODS AND RESULTS: We applied retrospectively the Bayesian method, which can analyze a large number of variables, to differentiate phenogroups of patients with different risks of AF and prognoses across a French prospective on-going hospital-based registry of adult HCM patients (REMY). Clinical and imaging data were prospectively recorded, and patients were followed for 5 years. A total of 1431 HCM patients were recruited, including 1275 analyzed in the present study after exclusion criteria. The population included 412 women, 369 patients with obstructive HCM, and 252 implanted with an ICD. AF occurred in 167 (11.6%) patients during the 5 year follow-up. Three phenogroups were defined according to their common clinical and echocardiographic characteristics. Patients at the highest risk were oldest, more often female, with more frequent comorbidities, anteroposterior diameter of the left atrium was significantly greater, with diastolic dysfunction, outflow-tract obstruction, and mitral valve abnormality, and presented higher pulmonary artery pressure and/or right-ventricular dysfunction. These also had a higher risk of all-cause hospitalizations and death. CONCLUSION: Based on a clustering analysis, three phenogroups of HCM according to the risk of AF occurrence can be identified. It can indicate which patients should be more monitored and/or treated, particular to prevent the risk of stroke.

Early post-septal myectomy outcomes for hypertrophic obstructive cardiomyopathy.

BACKGROUND: We aimed to evaluate early outcomes of septal myectomy in patients with hypertrophic cardiomyopathy. METHODS: We retrospectively analyzed data collected over a 9-year period from 583 patients who underwent septal myectomy for hypertrophic cardiomyopathy at our institution. RESULTS: The mean age was 55.7 ± 13.1 years, and 338 (58%) patients were in New York Heart Association class III or IV. There were 11 (1.9%) early deaths, including 3 (0.5%) intraoperative deaths. Early mortality was lowest after isolated septal myectomy (0.8%) and highest after concomitant mitral valve replacement (6.1%). There were 4 (0.7%) and 9 (1.5%) patients with left ventricular wall rupture and ventricular septal defect, respectively, after myectomy. New pacemaker implantation caused by atrioventricular disturbances was required in 29 (5.0%) patients, and was associated with previous alcohol septal ablation (odds ratio 3.34, 95% confidence interval 1.02-11.0, P = 0.047). Left ventricular wall rupture, intraoperative residual (15.5% moderate, 0.3% severe) mitral regurgitation, and pre-discharge residual outflow tract gradient >30 mm Hg (4.6%) occurrences were surgeon-dependent. CONCLUSIONS: The early results are consistent with example targets reported in the 2020 American College of Cardiology/American Heart Association guidelines for septal reduction therapy outcomes. Septal myectomy safety and efficacy are surgeon-dependent. Previous alcohol septal ablation increases the risk of permanent pacemaker implantation due to postoperative complete atrioventricular block. Therefore, continuous education, mentoring, and learning by doing may play an important role in achieving reasonable septal myectomy safety and efficacy.

Concomitant Cox-Maze IV and Septal Myectomy in Patients With Hypertrophic Obstructive Cardiomyopathy.

BACKGROUND: In patients with hypertrophic obstructive cardiomyopathy, atrial fibrillation is associated with heart failure and increased late mortality. However, the role of surgical ablation in these patients is not well defined. The aim of this study was to evaluate the efficacy of the concomitant Cox-Maze IV procedure in patients undergoing septal myectomy for hypertrophic obstructive cardiomyopathy. METHODS: Between 2005 and 2019, 347 patients who underwent septal myectomy at a single institution (Washington University School of Medicine, Barnes-Jewish Hospital, St Louis, MO) were retrospectively reviewed. For patients with hypertrophic obstructive cardiomyopathy and atrial fibrillation who underwent a concomitant Cox-Maze IV procedure, freedom from atrial tachyarrhythmias (ATAs) on or off antiarrhythmic drugs (AADs) was evaluated annually. Predictors of ATA recurrence were identified using Fine-Gray regression, with death as a competing risk. RESULTS: A total of 42 patients underwent concomitant septal myectomy and Cox-Maze IV procedures. The majority of patients, 69% (29 of 42), had paroxysmal atrial fibrillation with a 2.5-year median duration. Operative mortality was 7% (3 of 42). New York Heart Association functional class was reduced after surgery (P < .01). Rates of freedom from recurrent ATAs at 1- and 5-year intervals were 93% (27 of 29) and 100% (14 of 14), respectively. Rates of freedom from ATAs and AADs were 83% (24 of 29) and 100% (14 of 14) at the same time points, respectively. Increased left atrial diameter predicted first ATA recurrence (P < .01). Cerebrovascular accident risk was lower in patients with atrial fibrillation who underwent concomitant Cox-Maze IV and septal myectomy relative to myectomy only (P = .02). CONCLUSIONS: Late freedom from ATAs on or off AADs was excellent after Cox-Maze IV and septal myectomy. Although there was a higher than expected rate of perioperative complications, the study results suggest that concomitant surgical ablation should be considered in selected patients with hypertrophic obstructive cardiomyopathy and atrial fibrillation.

Projecting the Long-term Clinical Value of Mavacamten for the Treatment of Obstructive Hypertrophic Cardiomyopathy in the United States: An Assessment of Net Health Benefit.

PURPOSE: The aim of the study was to project the long-term net health benefits of mavacamten for the treatment of symptomatic obstructive hypertrophic cardiomyopathy (HCM) in the United States. METHODS: A Markov model with 4 mutually exclusive health states (New York Heart Association [NYHA] functional classes I, II, and III/IV and death) was developed to project the life-years (LYs) and quality-adjusted life-years (QALYs) over a lifetime horizon for patients with symptomatic obstructive HCM receiving mavacamten with or without ß-blocker (BB) or calcium channel blocker (CCB) monotherapy or placebo with or without BB or CCB monotherapy. The model simulated a patient cohort with a starting age of 59 years and 41% women. Transition probabilities across NYHA functional classes were estimated using data from the Phase III Clinical Study to Evaluate Mavacamten (MYK-461) in Adults With Symptomatic Obstructive Hypertrophic Cardiomyopathy (EXPLORER-HCM) and the EXPLORER long-term extension (EXPLORER-LTE) cohort from the Long-term Safety Extension Study of Mavacamten in Adults who Have Completed MAVERICK-HCM or EXPLORER-HCM (MAVA-LTE) trial and were extrapolated after week 30. The mortality risks of NYHA functional class I were assumed to be the age- and sex-specific mortality risks of the US general population. The mortality risks for NYHA class II and III/IV were estimated using those for class I in conjunction with the relative mortality risks derived using patients with obstructive HCM from a large real-world registry. Health state utilities for each treatment were estimated from EXPLORER-HCM. Both LYs and QALYs were aggregated over a lifetime for each treatment arm, discounted at 3% annually, and compared between the 2 arms. Sensitivity analyses were conducted to evaluate the robustness of the model findings. FINDINGS: Over a lifetime, treatment with mavacamten with or without BB or CCB monotherapy was associated with 3.67 incremental LYs compared with placebo with or without BB or CCB monotherapy (13.00 vs 9.33 LYs). Compared with individuals in the placebo group, patients in the mavacamten group were projected to spend 6.17 additional LYs in NYHA functional class I and 0.04 and 2.46 fewer LYs in NYHA functional classes II and III/IV, respectively. With utilities incorporated, mavacamten with or without BB or CCB monotherapy was associated with 4.17 additional QALYs compared with placebo with or without BB or CCB monotherapy (11.74 vs 7.57 QALYs). In the sensitivity analyses, incremental benefits ranged from 1.55 to 6.21 LYs and from 2.48 to 6.19 QALYs across the scenarios. IMPLICATIONS: This model projected substantial net health benefits associated with mavacamten for symptomatic obstructive HCM owing to improved patient survival and quality of life. The projected QALY gain underscored the likely long-term clinical value of mavacamten in symptomatic obstructive HCM.

N-terminal pro-brain natriuretic peptide and adverse outcomes in Chinese patients with hypertrophic cardiomyopathy.

BACKGROUND: Although numerous studies have suggested that elevated N-terminal pro-brain natriuretic peptide (NT-proBNP) is positively correlated with cardiovascular events, especially the heart failure and heart failure-related death (HFRD), evidence of the association between NT-proBNP and the adverse outcomes of hypertrophic cardiomyopathy (HCM) is still relatively limited. The present study was performed to evaluate the relationship between NT-proBNP and outcomes in patients with HCM. METHODS: Observational cohort methodology was used in the present study, and a total of 227 patients were included. And the patients were followed for 44.97 ± 16.37 months. Patients were categorized into three groups according to these NT-proBNP tertiles: first tertile (≤910 pg/ml, n=68), second tertile (913-2141 pg/ml, n=68), and third tertile (≥2151 pg/ml, n=69). The adverse outcomes of the present study were all-cause death (ACD) and cardiac death (CD). RESULTS: According to the risk category of NT-proBNP, the incidence of ACD (P=0.005) and CD (P=0.032) among the three groups showed significant differences. Multivariate Cox regression analysis suggested that the ACD and CD in the third tertile have 7.022 folds (hazard risk [HR] = 7.022 [95% confidence interval [CI]: 1.397-35.282], P=0.018) and 7.129 folds (HR = 7.129 [95% CI: 1.329-38.237], P=0.022) increased risks as compared with those in the first tertile. Kaplan-Meier survival analyses showed that the cumulative risks of ACD and CD in patients with HCM tended to increase. CONCLUSION: The present study indicated NT-proBNP was a novel biomarker suitable for predicting adverse prognosis in patients with HCM, which may be used for early recognition and risk stratification.

Acute coronary syndrome with non-obstructive coronary arteries (ACS-NOCA) in patients with hypertrophic cardiomyopathy.

OBJECTIVES: Our study aimed to determine the prevalence and prognosis of acute coronary syndrome with non-obstructive coronary artery (ACS-NOCA) in patients with hypertrophic cardiomyopathy (HCM). METHODS AND RESULTS: We enrolled a total of 200 consecutive patients with HCM over a 139-month period from 2002 to 2013. The study found that 28 patients (14% of overall patients, 51% of patients with ACS) had ACS-NOCA, and 18 patients (9% of overall patients, 86% of patients with acute MI) had MINOCA as initial clinical presentations. The highest prevalence of non-obstructive coronary artery disease (NOCA) in patients with HCM was found in acute ST-elevation myocardial infarction (STEMI) (100%), followed by non-STEMI (82%), and unstable angina (29%). Patients with ACS-NOCA had more frequent ventricular tachycardia and lower resting left ventricular (LV) outflow tract gradients than those with no ACS-NOCA (p < 0.05 for all). The ACS-NOCA group had a lower probability of HCM-related death compared with the no ACS-NOCA group and the significant coronary artery disease (CAD) group (p-log-rank = 0.0018). CONCLUSIONS: MINOCA or ACS-NOCA is not an uncommon initial presentation (prevalence rate 9-14%) in patients with HCM. NOCA was highly prevalent (51-86%) in patients with HCM presenting with ACS and had a favorable prognosis. Our findings highlight as a reminder that in an era of rapid reperfusion therapy, ACS in patients with HCM is not only a result of obstructive epicardial CAD, but also stems from the complex cellular mechanisms of myocardial necrosis.

Truncating Variants in OBSCN Gene Associated With Disease-Onset and Outcomes of Hypertrophic Cardiomyopathy.

BACKGROUND: The presence of variants in OBSCN was identified to be linked to hypertrophic cardiomyopathy (HCM), but whether OBSCN truncating variants were associated with HCM remained unknown. METHODS: Whole-exome sequencing was performed in 986 patients with HCM and 761 non-HCM controls to search for OBSCN truncating variants, and the result was tested in a replication cohort consisting of 529 patients with HCM and 307 controls. The association of the OBSCN truncating variants with baseline characteristics and prognosis of patients with HCM were ascertained. RESULTS: There were 28 qualifying truncating variants in the OBSCN gene detected in 26 (2.6%) patients with HCM and 6 (0.8%) controls. The OBSCN truncating variants were more prevalent in patients with HCM than controls (odds ratio, 3.4, P=0.004). This association was confirmed in the replication cohort (odds ratio, 3.8, P=0.024). The combined effects of the two cohorts estimated the odds ratio to be 3.58 (P<0.001). Patients with or without OBSCN truncating variants shared similar demographic and echocardiographic variables at baseline. During 3.3±2.4 years (4795 patient-years) follow-up, the patients with OBSCN truncating variants were more likely to experience cardiovascular death (adjusted hazard ratio, 3.1 [95% CI, 1.40-6.70], P=0.005) and all-cause death (adjusted hazard ratio, 2.63 [95% CI, 1.21-5.71], P=0.015). CONCLUSIONS: Our data indicated that OBSCN truncating variants contributed to the disease-onset of HCM, and increased the risk of malignant events in patients with HCM.

The modified US heart allocation system improves transplant rates and decreases status upgrade utilization for patients with hypertrophic cardiomyopathy.

BACKGROUND: On October 18, 2018, the US heart allocation policy was restructured to improve transplant waitlist outcomes. Previously, hypertrophic cardiomyopathy (HCM) patients experienced significant waitlist mortality and functional decline, often requiring status exemptions to be transplanted. This study aims to examine changes in waitlist mortality and transplant rates of HCM patients in the new system. METHODS: Retrospective analysis was performed of the United Network for Organ Sharing Transplant Database for all isolated adult single-organ first-time heart transplant patients with HCM listed between October 17, 2013 and September 4, 2020. Patients were divided by listing date into eras based on allocation system. Era 1 spanned October 17, 2013 to October 17th, 2018 and Era 2 spanned October 18th, 2018 to September 4, 2020. RESULTS: During the study period, 436 and 212 HCM patients were listed in Eras 1 and 2, respectively. Across eras, no differences in gender, ethnicity, BMI or functional status were noted (p>0.05). LVAD utilization remained low (Era 1: 3.7% vs Era 2: 3.3%, p = 0.297). Status upgrades decreased from 49.1% to 31.6% across eras (p = 0.001). There was no statistically significant difference in waitlist mortality across eras (p = 0.332). Transplant rates were improved in Era 2 (p = 0.005). Waitlist time among transplanted patients decreased in Era 2 from 97.1 to 63.9 days (p<0.001). There was no difference in one-year survival post-transplant (p = 0.602). CONCLUSIONS: The new allocation system has significantly increased transplant rates, shortened waitlist times, and decreased status upgrade utilization for HCM patients. Moreover, waitlist mortality remained unchanged in the new system.

Prognostic value of red blood cell distribution width for mortality in patients with hypertrophic cardiomyopathy.

BACKGROUND: Red blood cell distribution width (RDW) has emerged as a useful indicator for adverse outcomes in several cardiovascular diseases. The relation between RDW and the prognosis of hypertrophic cardiomyopathy (HCM) remains to be evaluated. We examined the relation between RDW and all-cause mortality and HCM-related death in a population of adult HCM patients. METHODS: We performed clinical evaluation in 414 consecutive adult HCM patients (median age, 57.5 years; male, 54.8%). RESULTS: During a median follow-up of 3.7 years, all-cause mortality and HCM-related death occurred in 75 (18.1%) and 50 (12.1%) patients, respectively. Based on the tertiles of baseline RDW, mortality increased with higher tertile. With the tertile 1 as reference, adjusted all-cause mortality hazard ratios (HRs) were 3.9 for the tertile 2 (95% confidence interval [CI]: 1.5-10.3) and 3.1 for the tertile 3 (95% CI: 1.1-8.2). Adjusted HCM-related death HRs were higher in the tertile 2 (HR: 5.5; 95% CI: 1.2-24.4) and tertile 3 (HR: 6.6; 95% CI: 1.5-29.0) compared with the tertile 1. Further smooth curve fitting exhibited a saturation effect after adjusting for confounders, and there were a two-stage change and an inflection point. Two-piecewise Cox model suggested mortality significantly increased with RDW level up to the inflection point (about 14.0% for both all-cause mortality and HCM-related death), and RDW was not associated with mortality after the point. CONCLUSION: In adult HCM patients, we found increased RDW was a significant risk predictor for all-cause mortality and HCM-related death, and a saturation effect was observed.

Septuagenarians with hypertrophic obstructive cardiomyopathy undergoing myectomy: the experience of a single center.

PURPOSES: A previous study reported that the ventricular septum is more fragile in geriatric patients, and thus myectomy may expose geriatric patients to a higher risk of serious complications. This single-center study evaluates the impacts of the advanced age on the results following myectomy for symptomatic hypertrophic obstructive cardiomyopathy (HOCM). METHODS: All eligible patients were divided into an elderly group (septuagenarians, n = 35) and a control group (patients under 70 years, n = 197). The patients were followed up for a median of 10 months. Thereafter, the perioperative and follow-up results were compared. RESULTS: Thirteen patients (8.6% for the elderly group and 5.1% for the control group, p = 0.422) underwent immediate repeat surgery. Surgical mortality did not differ between groups (0 vs. 0.5%, p > 0.05). There was no significant difference in the incidence of complete atrioventricular block between groups (5.7 vs. 3.0%, p = 0.346). No follow-up deaths occurred in either group. The maximum gradients at the latest follow-up were significantly lower than the preoperative values in either group. Additionally, grouping (septuagenarians vs. patients under 70 years of age) was not an independent risk factor for surgical complications and results via multivariable logistic regression. CONCLUSIONS: Septuagenarians with HOCM may obtain favorable results following septal myectomy, the same as did HOCM patients under 70 years of age.

Prognostic value of fast semi-automated left atrial long-axis strain analysis in hypertrophic cardiomyopathy.

BACKGROUND: The prognostic value of left atrial (LA) size and function in hypertrophic cardiomyopathy (HCM) is well recognized, but LA function is difficult to routinely analyze. Fast LA long-axis strain (LA-LAS) analysis is a novel technique to assess LA function on cine cardiovascular magnetic resonance (CMR). We aimed to assess the association between fast LA-LAS and adverse clinical outcomes in patients with HCM. METHODS: 359 HCM patients and 100 healthy controls underwent routine CMR imaging. Fast LA-LAS was analyzed by automatically tracking the length between the midpoint of posterior LA wall and the left atrioventricular junction based on standard 2- and 4-chamber balanced steady-state free precession cine-CMR. Three strain parameters including reservoir strain (εs), conduit strain (εe), and active strain (εa) were assessed. The endpoint was set as composite adverse events including cardiovascular death, resuscitated cardiac arrest, sudden cardiac death aborted by appropriate implantable cardioverter-defibrillator discharge, and hospital admission related to heart failure. RESULTS: During an average follow-up of 40.9 months, 59 patients (19.7%) reached endpoints. LA strains were correlated with LA diameter, LA volume index (LAVI) and LA empty fraction (LAEF) (all p < 0.05). In the stepwise multivariate Cox regression analysis, εs and εe (hazard ratio, 0.94 and 0.89; p = 0.019 and 0.006, respectively) emerged as independent predictors of the composite adverse events. Fast LA εs and LA εe are stronger prognostic factors than LA size, LAVI and the presence of left ventricular late gadolinium enhancement. CONCLUSIONS: Fast LA reservoir and conduit strains are independently associated with adverse outcomes in HCM.

Sex differences in the prognosis of patients with hypertrophic cardiomyopathy.

We investigated sex-related differences in the prognosis of patients with hypertrophic cardiomyopathy (HCM) using the Korea National Health Insurance Service database. From 2010 to 2016, 9524 patients diagnosed with HCM and had more than 1-year follow-up period were analyzed. The primary endpoint was the composite of cardiovascular death or new-onset heart failure (HF) admission. Propensity score-matching analysis was performed to adjust for different baseline characteristics. With a 4.4-years' median follow-up interval (range 2.0-6.6 years) and male predominance (77.6%), women with HCM were older (52.6 ± 9.7 vs. 51.4 ± 9.1, p < 0.001), had lower incomes, more comorbidities based on Charlson comorbidity index. Women with HCM had a higher incidence of the primary endpoint than men (incidence rate: 34.15 vs. 22.83 per 1000 person-years, log-rank p < 0.001). Multivariable Cox analysis showed that female sex was a poor prognostic factor for the primary endpoint (HR 1.43, 95% CI 1.24-1.64, p < 0.001). This was mainly driven by a higher incidence of new-onset HF admission (HR 1.55, 95% CI 1.34-1.80). However, there was no difference in the incidence of cardiovascular death between the sexes. This result was concordant in the propensity score-matched cohort. In conclusion, women with HCM have worse prognosis, which was mainly driven by a higher new-onset HF admission.

Management and outcomes of hypertrophic cardiomyopathy in young adults.

BACKGROUND: Management of young adults with hypertrophic cardiomyopathy (HCM) is challenging. AIMS: To evaluate the profile of young adults (16-25 years) with HCM included in the French prospective HCM registry. METHODS: Patients were compared according to occurrence of major adverse cardiac events (MACE), comprising sudden cardiac death (SCD) events (implantable cardioverter defibrillator [ICD] discharge, SCD, sustained ventricular tachycardia), atrial fibrillation/embolic stroke, heart failure hospitalisation and unexplained syncope, at a mean follow-up of 4.4±2.2 years. RESULTS: At baseline, among 61 patients (20.5±3.0 years; 16 women, 26.2%), 13 (21.3%) had a prophylactic ICD, 24.6% a family history of SCD, 29.5% obstruction, 86.0% magnetic resonance imaging myocardial fibrosis, 11.8% abnormal exercise blood pressure and 52.8% a European Society of Cardiology (ESC) 5-year SCD score<4% (24.5%≥6%). At follow-up, 15 patients (24.6%; seven women; all with fibrosis) presented 17 MACE, comprising: SCD events (n=7, 41.2%; including three patients with an ICD, five with at least one SCD major classical risk factor and an ESC score≥5% and two with no risk factors and an ESC score<4%); atrial fibrillation/stroke (n=6, 35.3%); heart failure (n=1, 5.9%); syncope (n=3, 17.6%). An ICD was implanted in 11 patients (four for secondary prevention), but in only 61.5% of patients with a score≥6%. Only obstruction significantly increased MACE risk (odds ratio 3.96; P=0.035), with a non-significant trend towards a lower risk in men (OR 0.29; P=0.065). CONCLUSIONS: In young adults with HCM, MACE are common in the short term, especially in obstructive HCM and women, mostly arrhythmic in origin. Prophylactic ICD implantation is frequent and does not strictly follow the guidelines, while the use of European/USA guidelines is helpful but imperfect in identifying SCD risk.