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Although elevated B-type natriuretic peptide (BNP) levels predict outcome in patients with hypertrophic cardiomyopathy (HCM), the association between BNP levels and outcome in patients with the apical phenotype of HCM remains unclear. We evaluated the impact of elevated BNP levels on outcome in a cohort of apical HCM patients.Among 432 HCM patients, 144 with an apical phenotype were examined. Plasma BNP levels were measured at the time of the initial evaluation.The median (interquartile range) BNP level at initial evaluation in these patients was 188.5 (72.0-334.4) pg/mL. During a median follow-up period of 9.5 years, 34 patients experienced HCM-related adverse outcomes, including 2 patients with sudden death, 5 with appropriate implantable defibrillator shocks, 3 with stroke-related death, 8 with non-fatal stroke, and 16 with heart failure hospitalization. Receiver operating characteristic (ROC) curve analysis of the prognostic value of BNP for the combined endpoint gave an area under the ROC curve of 0.756, and optimal BNP cut-off point of 226.0pg/mL. Patients with high BNP levels (≥226.0pg/mL) were at significantly greater risk of the combined endpoint (log-rank p0.001) than patients with low BNP levels. Multivariable analysis that included BNP levels and potential confounders showed that high BNP levels were an independent determinant of the combined endpoint (adjusted hazard ratio: 3.71; p=0.002).Measuring BNP may help stratify the risk of HCM-related adverse outcome in apical HCM patients.

Anomalous left coronary artery arising from the noncoronary cusp (LCANCC) is a rare congenital disorder. We herein describe a 17-year-old female patient with sudden cardiac arrest followed by refractory cardiogenic shock. LCANCC-induced acute myocardial infarction with left main coronary artery involvement was subsequently diagnosed, and the patient required a durable left ventricular assist device.

AIMS: There are regional differences in the patient characteristics, management, and outcomes of hospitalized patients with heart failure (HF). The aim of this study was to evaluate the clinical characteristics and outcomes of Japanese patients who are hospitalized with HF on the basis of the left ventricular ejection fraction (LVEF) stratum. METHODS AND RESULTS: We retrospectively conducted a multicentre cohort study of 1245 hospitalized patients with decompensated HF between 2013 and 2014. Of these patients, 36% had an LVEF < 40% [HF with reduced ejection fraction (HFrEF), median age 72 years, 71% male], 21% had an LVEF 40-49% [HF with mid-range EF (HFmrEF), 77 years, 56% male], and 43% had an LVEF ≥ 50% [HF with preserved EF (HFpEF), 81 years, 44% male]. The primary outcome was death from any cause, and the secondary outcomes were cardiac death and re-hospitalization due to worsened HF after hospital discharge. There were high proportions of non-ischaemic cardiomyopathy (32%) in HFrEF patients, coronary artery disease (44%) in HFmrEF patients, and valvular disease (39%) in HFpEF patients. The frequencies of intravenous diuretic and natriuretic peptide administration during hospitalization were 66% and 30%, respectively. The median hospital stay for the overall population was 19 days, and the length of stay was >7 days for >90% of patients. In-hospital mortality was 7%, but was not different among the LVEF groups (HFrEF 7%, HFmrEF 6%, and HFpEF 8%). After a median follow-up of 19 months (range, 3-26 months), 192 (17%) of the 1156 patients who were discharged alive died, and 534 (46%) were re-hospitalized after hospital discharge. There were no significant differences in mortality after hospital discharge among the three LVEF groups (HFrEF 18%, HFmrEF 16%, and HFpEF 16%). There were no differences in cardiac death or re-hospitalization due to worsened HF after hospital discharge among the LVEF groups (cardiac death: HFrEF 8%, HFmrEF 7%, and HFpEF 7%; re-hospitalization due to worsened HF: HFrEF 19%, HFmrEF 16%, and HFpEF 17%). Multivariable-adjusted analyses showed that the HFmrEF and HFrEF groups, compared with the HFpEF group, were not associated with an increased risk for in-hospital death or death after hospital discharge. Non-cardiac causes of death and re-hospitalization after hospital discharge accounted for 35% and 38%, respectively. CONCLUSIONS: Our results revealed different clinical characteristics but similar mortality rates in the HFrEF, HFmrEF, and HFpEF groups. The most common cause of death and re-hospitalization after hospital discharge was HF, but non-cardiac causes also contributed to their prognosis. Integrated management approaches will be required for HF patients. peerReviewed

Inflammation and malnutrition are common problems in patients who are hospitalized for acute heart failure (AHF). C-reactive protein (CRP) is an acute-phase reactant and nonspecific marker for evaluating systemic inflammation. There has been growing interest in prealbumin for nutritional assessment. Additionally, prealbumin is a negative acute-phase protein because its synthesis is suppressed in the inflammatory setting in which cytokines stimulate hepatic production of acute-phase proteins (e.g. CRP). Therefore, the CRP to prealbumin ratio (CP ratio) may be a comprehensive marker of inflammation and malnutrition. We evaluated the relationship of the CP ratio with mortality in patients with AHF.We analyzed 257 hospitalized patients with AHF who had CRP and prealbumin levels examined on admission.The median CP ratio on admission was 0.57, with an interquartile range of 0.11 to 1.94. In receiver operating characteristic curve analysis, the area under the curve was 0.729 and the optimal cut-off point of the CP ratio for all-cause death was1.60 (sensitivity: 67.5%; specificity: 77.6%; p = 0.003). Kaplan-Meier survival curves showed that patients with a high CP ratio (1.60) had a significantly greater risk of all-cause, cardiac, and non-cardiac death (log-rank test, all p0.001) than patients with a low CP ratio (≤1.60). Multivariable analysis adjusted for imbalanced baseline variables showed that a high CP ratio was independently associated with higher all-cause mortality (adjusted hazard ratio 3.88; 95% confidence interval 1.91-7.86; p0.001).The ratio of two hepatic proteins, CRP and prealbumin, may be useful in risk stratification of patients with AHF.

In hypertrophic cardiomyopathy (HCM), one of the main pathophysiological features is diastolic dysfunction. According to the 2016 American Society of Echocardiography (ASE)/European Association of Cardiovascular Imaging (EACVI) recommendations, diastolic function is assessed with echocardiographic variables. However, the association between the ASE/EACVI recommendations and the outcome in patients with HCM remains unclear. We evaluated the prognostic implications of the ASE/EACVI recommendations in patients with HCM.This study included 290 patients with HCM. We evaluated four variables for identifying diastolic dysfunction using the following abnormal cutoff values: septal e'7 cm/sec, septal E/e' ratio15, left atrial volume index34 mL/mThe prevalence of an ASE/EACVI score ≥3 was 37.2%. Over a median follow-up of 9.7 (6.9-12.9) years, 26 (24.1%) patients with an ASE/EACVI score ≥3 and 25 (13.7%) patients with an ASE/EACVI score3 experienced a combination of HCM-related adverse outcomes. Patients with an ASE/EACVI score ≥3 had a significantly higher incidence of the combined endpoint than those with an ASE/EACVI score3 (log-rank, P = .010). An ASE/EACVI score ≥3 was an independent determinant of the combined endpoint in multivariate analysis (adjusted hazard ratio = 1.92; 95% CI, 1.05-3.49; P = .033).The score for identifying diastolic dysfunction by following ASE/EACVI recommendations may be associated with an adverse outcome in patients with HCM.

Renal dysfunction is a known risk of sudden cardiac death in patients with ischemic heart disease. However, the association between renal dysfunction and sudden death in hypertrophic cardiomyopathy (HC) patients remains unknown. This study investigated the significance of an impaired renal function for the sudden death risk in a cohort of patients with HC. We included 450 patients with HC (mean age 52.9 years, 65.1% men). The estimated glomerular filtration rate (eGFR) was evaluated at the time of the initial evaluation. Renal dysfunction was defined as an eGFR60 ml/min/1.73 m

A mitral L-wave indicates advanced diastolic dysfunction with elevated left ventricular filling pressure. Previous studies have reported that the presence of a mitral L-wave is associated with a poor prognosis in patients with heart failure. However, whether the L-wave can predict adverse events in patients with hypertrophic cardiomyopathy (HC) is still unclear. Therefore, we aimed to investigate the prevalence of a mitral L-wave in patients with HC, and the prognosis of patients with or without an L-wave. We analyzed 445 patients with HC. The end points of this study were HC-related death, such as sudden death or potentially lethal arrhythmic events, heart failure-related death, and stroke-related death. A mitral L-wave was defined as a distinct mid-diastolic flow velocity after the E wave with a peak velocity20 cm/s. The prevalence of an L-wave was 32.4% in patients with HC. Patients with an L-wave were significantly younger, more likely to be women, had higher New York Heart Association functional class, and had a higher prevalence of atrial fibrillation than did patients without an L-wave. Patients with an L-wave had a significantly higher incidence of HC-related death compared with those without an L-wave (log-rank, p0.001). The L-wave was an independent determinant of HC-related death in multivariate analysis adjusted for imbalanced baseline variables (adjusted hazard ratio 2.38; 95% confidence interval 1.42 to 4.01; p = 0.001). In conclusion, the presence of a mitral L-wave may be associated with adverse outcome in patients with HC.

Background The association between first‐degree atrioventricular block ( AVB ) and life‐threatening cardiac events in patients with hypertrophic cardiomyopathy ( HCM ) remains unclear. This study sought to investigate whether presence of first‐degree AVB was associated with HCM ‐related death in patients with HCM . Methods and Results We included 414 patients with HCM (mean age, 51±16 years; 64.5% men). The P‐R interval was measured at the time of the initial evaluation and patients were classified into those with and without first‐degree AVB , which was defined as a P‐R interval ≥200 ms. HCM ‐related death was defined as a combined end point of sudden death or potentially lethal arrhythmic events, heart failure–related death, and stroke‐related death. First‐degree AVB was noted in 96 patients (23.2%) at time of enrollment. Over a median (interquartile range) follow‐up period of 8.8 (4.9–12.9) years, a total of 56 patients (13.5%) experienced HCM ‐related deaths, including 47 (11.4%) with a combined end point of sudden death or potentially lethal arrhythmic events. In a multivariable analysis that included first‐degree AVB and risk factors for life‐threatening events, first‐degree AVB was independently associated with an HCM ‐related death (adjusted hazard ratio, 2.41; 95% CI , 1.27–4.58; P =0.007), and this trend also persisted for the combined end point of sudden death or potentially lethal arrhythmic events (adjusted hazard ratio, 2.60; 95% CI , 1.28–5.27; P =0.008). Conclusions In this cohort of patients with HCM , first‐degree AVB may be associated with HCM ‐related death, including the combined end point of sudden death or potentially lethal arrhythmic events.

Functional mitral regurgitation (MR) caused by reduced left ventricular ejection fraction (EF) and tethering, termed ventricular functional MR (VFMR), is associated with worse outcomes. Atrial functional MR (AFMR) caused by left atrial enlargement and annular dilatation was also recently described in patients with atrial fibrillation (AF). However, the clinical profiles of AFMR in hospitalized heart failure (HF) patients are unclear. We investigated the prevalence, clinical characteristics, and prognosis of AFMR in hospitalized HF patients with AF.We analyzed 189 hospitalized HF patients with AF. The prevalence, clinical characteristics, and prognosis were compared between 4 groups: patients with EF ≥50% and no/mild MR (pEFnoMR), patients with EF50% and no/mild MR (rEFnoMR), patients with EF ≥50% and moderate/severe MR (AFMR), and patients with EF50% and moderate/severe MR (VFMR).The prevalence of AFMR was 15.9% in hospitalized HF patients with AF. AFMR patients were older and more likely to have an enlarged left atrium, lower tenting height, and moderate/severe tricuspid regurgitation than VFMR patients. There were no differences in all-cause death after discharge among pEFnoMR, rEFnoMR, and AFMR patients. AFMR patients were associated with a higher rate of a composite of cardiac death and readmission for HF compared with pEFnoMR and rEFnoMR patients (log-rank p=0.046 and p=0.004). There were no differences in composite endpoints between AFMR and VFMR patients (log-rank p=0.507).AFMR was present in a proportion of elderly hospitalized HF patients with AF, and was a condition requiring attention because of readmission for HF in a hospitalized HF cohort.

The aim of this study was to evaluate the association of functional mitral regurgitation (FMR), preserved or reduced ejection fraction (EF), and ischemic or nonischemic origin with outcomes in patients discharged alive after hospitalization for acute decompensated heart failure (HF). Of the 4,842 patients enrolled in the Acute Decompensated Heart Failure Syndromes (ATTEND) registry, 3,357 patients were evaluated to assess the association of FMR, preserved or reduced EF, and ischemic or nonischemic origin with the primary end point (all-cause death and readmission for HF after discharge). At the time of discharge, FMR was assessed semiquantitatively (classified as none, mild, or moderate to severe) by color Doppler analysis of the regurgitant jet area. According to multivariable analysis, in the ischemic group, either mild or moderate to severe FMR in patients with a preserved EF had a significantly higher risk of the primary end point than patients without FMR (hazard ratio [HR] 1.60; 95% confidence interval [CI] 1.12 to 2.29; p = 0.010 and HR 1.98; 95% CI 1.30 to 3.01; p = 0.001, respectively). In patients with reduced EF with an ischemic origin, only moderate to severe FMR was associated with a significantly higher risk of the primary end point (HR 1.67; 95% CI 1.11 to 2.50; p = 0.014). In the nonischemic group, there was no significant association between FMR and the primary end point in patients with either a preserved or reduced EF. In conclusion, among patients with acute decompensated HF with a preserved or reduced EF, the association of FMR with adverse outcomes may differ between patients who had an ischemic or nonischemic origin of HF.

Echocardiographically estimated pulmonary artery systolic pressure (PASP) is a non-invasive widely available method that is used to estimate pulmonary arterial pressure. Although elevated PASP predicts mortality in patients with hypertrophic cardiomyopathy (HCM), the relationship between PASP and embolic events is unclear. This study aimed to determine whether elevated PASP is associated with stroke and systemic embolic events in a tertiary referral HCM cohort.This study included 374 clinically diagnosed patients with HCM. PASP was estimated from tricuspid regurgitant jet velocity using the modified Bernoulli equation.The median (interquartile range) PASP was 33 (28-37) mm Hg, and elevated PASP (40mmHg) was observed in 66 (17.6%) patients. Seventeen of the 66 (25.8%) patients with elevated PASP and 24 of the 308 (7.8%) patients without elevated PASP experienced stroke and systemic embolic events during the 10.3±7.4years of follow-up (log-rank P0.001). Multivariable analysis showed that age at diagnosis, atrial fibrillation, and PASP40mmHg (adjusted hazard ratio, 2.59; 95% confidence interval, 1.31-5.12; P=0.006) were independently associated with embolic events.In addition to age and atrial fibrillation, PASP estimated by Doppler echocardiography could help embolic risk stratification in patients with HCM.

Aims The association between kinetics of blood urea nitrogen (BUN) levels in hospital and cardiovascular outcomes in patients with acutely decompensated congestive heart failure (HF) is unclear. We aimed to estimate the impact of changes in BUN level during hospitalization on clinical prognosis in patients with acute HF. Methods and results A total of 353 consecutive patients that were urgently hospitalized due to acutely decompensated HF and discharged alive were divided into four subgroups depending on their BUN level at admission and discharge, using a cut-off level of 21.0 mg/dL. Among 206 patients with high baseline BUN level, 46 (22%) and 160 (78%) had normal and persistent high BUN levels at discharge, respectively. In contrast, of the 147 patients with normal baseline BUN level, 55 (37%) and 92 (63%) had high and normal BUN levels at discharge, respectively. During the observational period after discharge, Kaplan–Meier analysis showed the highest rate of combined outcome of cardiovascular death and HF readmission in patients with persistent high BUN (log-rank test: P

Background Recent clinical trials’ findings have revealed the therapeutic noninferiority of direct oral anticoagulant (DOAC) to standard therapy with vitamin K antagonist (VKA) in patients with pulmonary thromboembolism (PTE). However, few studies have quantitatively analyzed thrombus reduction in the pulmonary artery. Methods This observational study included 38 symptomatic PTE patients with stable hemodynamics. All patients received an intravenous heparin bolus followed by continual heparin injections immediately after the PTE diagnosis. The heparin was discontinued after edoxaban therapy began in the DOAC group (n = 22) or after the therapeutic range for the prothrombin time-international normalized ratio was achieved in the VKA group (n = 16). The thrombus volumes in the pulmonary arteries were quantitatively analyzed using contrast-enhanced computed tomography scans, and they were compared at baseline and at 2 weeks after admission. Results The pulmonary thrombus volumes declined in the VKA and DOAC groups from 7.9 to 4.2 cm3 (P = 0.048) and from 7.1 to 3.7 cm3 (P Conclusions In relation to pulmonary artery thrombus volume reduction, DOAC monotherapy for PTE may be comparable with standard therapy involving VKAs.

We aimed to clarify the relationships between angiographic lesion characteristics and values of fractional flow reserve (FFR) on intermediate coronary artery stenosis. The clinical meaning and assessment for "visual-functional mismatches," including regular-mismatches [defined as angiographic percent diameter stenosis (%DS) ≥50 % and FFR >0.80] and reverse-mismatches (defined as angiographic %DS

Prealbumin is a marker of nutritional and inflammatory status, and low prealbumin level at discharge is associated with poor outcome in hospitalized patients with heart failure. However, the prognostic value of prealbumin level on admission in patients with acute heart failure (AHF) has not been established, especially in an acute care setting. We aimed to clarify the association between prealbumin level on admission and outcome in patients with AHF referred to a cardiac intensive care unit.We analyzed 186 hospitalized patients with AHF who had their prealbumin level examined within 24h of admission.The mean prealbumin level was 16.6±6.5mg/dL. Prealbumin effectively predicted all-cause death during the median follow-up period of 276 days, using receiver operating characteristic (ROC) curve analysis (the area under the ROC curve; 0.722, optimal cut-off point; ≤14.0mg/dL, sensitivity 71.0%; specificity 69.7%; p0.001). The all-cause mortality and the composite endpoints of all-cause death or readmission for AHF in patients with low prealbumin level (≤14.0mg/dL) were significantly higher than in patients with high prealbumin level (log-rank p0.001 and p=0.002). Multivariate analysis adjusted for established markers of AHF severity showed that prealbumin ≤14.0mg/dL was independently associated with higher mortality (hazard ratio 4.79; 95% confidence interval 1.89-12.2; p=0.001) and with the composite endpoints (hazard ratio 2.38; 95% confidence interval 1.30-4.36; p=0.005).Prealbumin level on admission may be useful in the risk stratification of patients with AHF in an acute care setting.

The association between B-type natriuretic peptide (BNP) levels and sudden cardiac death in patients with hypertrophic cardiomyopathy (HCM) remains unclear.This study evaluated the effect of elevated BNP levels on sudden death risk in a cohort of patients with HCM.This study included 346 patients with HCM. Plasma BNP levels were measured at the initial evaluation.The median (interquartile range) BNP level in the study patients was 197.2 (84.4-353.3) pg/mL. During a median (interquartile range) follow-up period of 8.4 (4.2-12.5) years, 37 patients (10.7%) experienced the combined end point of sudden death or potentially lethal arrhythmic events, including 11 patients with sudden death (3.2%), 8 resuscitated after cardiac arrest, and 18 with appropriate implantable defibrillator shocks. Time-dependent receiver operating characteristic curve analysis of the prognostic value of BNP for the combined end point showed that the Harrell's concordance index was 0.748 and the optimal BNP cutoff point was 312 pg/mL. Patients with high BNP levels (312 pg/mL) were at a significantly higher risk of sudden death (Gray test, P = .001) and the combined end point (Gray test, P .001) than were patients with low BNP levels (≤312 pg/mL). Multivariable analysis that included BNP levels and established risk factors for sudden death showed that high BNP levels were an independent determinant of the combined end point (adjusted hazard ratio 5.71; 95% confidence interval 2.86-11.4; P.001).Elevated BNP levels may be associated with sudden death and the combination of sudden death or potentially lethal arrhythmic events in patients with HCM.

It remains unclear whether flecainide, a Class I antiarrhythmic drug, improves left ventricular pressure gradient (LVPG) or symptoms in patients with obstructive hypertrophic cardiomyopathy (HCM). Our study evaluated the long-term efficacy of flecainide, compared to disopyramide, when administered orally, on LVPG and symptoms in obstructive HCM patients. Among 164 obstructive HCM patients, 15 were administered oral flecainide therapy and 33 administered oral disopyramide therapy. LVPG declined from 79.8 ± 36.6 to 39.2 ± 36.7 mmHg (p = 0.003) after flecainide therapy and from 74.5 ± 26.4 to 31.4 ± 24.8 mmHg (p

Background Previous studies reported that the presence of midventricular obstruction (MVO) was an independent determinant of sudden death and potentially lethal arrhythmic events in patients with hypertrophic cardiomyopathy (HCM). However, it remains unclear whether implantable cardioverter defibrillator (ICD) improves survival in HCM patients with MVO. In addition, the risk factors for lethal arrhythmic events in MVO-HCM patients are not fully understood. The aim of this study was to provide an overview of the ICD therapy on sudden death prevention, and to determine the risk factors for lethal arrhythmic events in MVO-HCM patients. Methods This study included 593 HCM patients. Left ventricular MVO was diagnosed when the peak midventricular gradient was estimated as ≥30mmHg. Results MVO was identified in 56 patients (9.4%), and 15 of the 56 MVO-HCM patients (26.8%) received an ICD. Six of 15 ICD-implanted patients (40.0%) had appropriate ICD interventions over the follow-up period of 6.5±5.1years after ICD implantation. Although two of 42 patients without an ICD died suddenly, no patients experienced sudden death after ICD implantation in patients with an ICD throughout the follow-up period of 9.0±8.0years after referral to our hospital. By multivariate analysis, maximal wall thickness was an independent determinant of lethal arrhythmic events in MVO-HCM patients. Conclusions A quarter of MVO-HCM patients received an ICD, and the incidence of appropriate ICD intervention was about 6.2%/year. It may be necessary to give careful consideration to the prevention of lethal arrhythmic events in MVO-HCM patients, especially those with severe left ventricular hypertrophy.

The CONUT (CONtrolling NUTritional) score is simple index calculated from the following factors; serum albumin concentration, total peripheral lymphocyte count and total cholesterol concentration, and has been reported to well represent nutritional status in hospitalized patients. We aimed to

It remains unclear whether the combination of dual-chamber (DDD) pacing and disopyramide can achieve prolonged left ventricular outflow tract (LVOT) gradient reduction and symptom relief in patients with obstructive hypertrophic cardiomyopathy (HCM). In an HCM patient with a severe LVOT gradient, the combination of DDD pacing and disopyramide achieved marked improvement of gradient in the catheter laboratory and also after medium-term follow-up. The patient's severe dyspnoea was alleviated during the follow-up period. This combination might enable physicians to treat and manage elderly symptomatic obstructive HCM patients with a severe LVOT gradient more effectively and less invasively.

Background and Purpose— Stroke and systemic embolic events are known to occur as complications of hypertrophic cardiomyopathy (HCM), and these complications are more common in patients with accompanying atrial fibrillation (AF). The diagnosis of AF is sometimes difficult, however, and it is possible that subclinical asymptomatic paroxysmal episodes or a first episode of AF in patients without previously documented AF may lead to embolic events. We investigated the prevalence of embolic events in patients with HCM and evaluated risk factors for these events in patients without documented AF. Methods— This study enrolled 593 patients with clinically diagnosed HCM (age at diagnosis, 51.0±15.6 years) from 1980 to 2010. Results— During a mean follow-up of 10.7±7.5 years, 68 (11.5%) experienced stroke and embolic events. AF had been documented before the event in 29 (42.6%) of them. AF was documented for the first time at the time of the event in 5 (7.4%) and after the event in 10 (14.7%). Among the 431 patients without previously documented AF (39 with events and 392 without events), older age at diagnosis and left atrial dimension ≥48 mm were identified as the independent determinants of the embolic event after adjusting for sex and classic prognostic markers related to HCM. Conclusions— The incidence of stroke and embolic events was about 1.0% per year in the HCM cohort. AF had not been previously documented before the event in more than half of patients with events. Older age and enlarged left atrial dimension are possible risk factors for embolic events in patients with HCM without documented AF.

The relationships among enlarged left atrial dimension (LAD), the presence or absence of atrial fibrillation (AF), and sudden death risk in patients with hypertrophic cardiomyopathy (HCM) remain unclear. The aim of this study was to evaluate the impact of enlarged LAD on sudden death risk in HCM patients with or without documented AF.This study included 564 HCM patients (follow-up period: 10.8±7.4 years). LAD was measured from the parasternal long-axis view as the antero-posterior linear diameter at end-systole. Sudden death was defined as the combined endpoint of sudden cardiac death and potentially lethal arrhythmic events, and log-rank tests and Cox proportional hazards models were applied to evaluate the impact of LAD enlargement on the combined endpoint.The proportions of patients with sudden death and potentially lethal arrhythmic events were significantly higher among patients with enlarged LAD (≥48mm, N=86) compared with those without enlarged LAD (19.8% vs. 8.2%; p=0.002). However, enlarged LAD was not identified as an independent determinant of sudden death risk in multivariate analysis of all study HCM patients [adjusted hazard ratio (HR): 1.83; 95% confidence interval (CI): 0.95-3.53; p=0.071]. Among patients without documented AF during the follow-up periods, enlarged LAD was an independent determinant of sudden death risk (adjusted HR: 5.23; 95% CI: 2.17-12.58; p0.001), although there was no significant difference in sudden death risk between patients with and without enlarged LAD in patients with documented AF (adjusted HR: 0.77; 95% CI: 0.31-1.90; p=0.567).These results suggest that the relationship between LAD and outcome is influenced by the presence or absence of AF in HCM patients. It may thus be necessary to consider the need to prevent sudden death in LAD-enlarged HCM patients without documented AF.

ObjectivesWe investigated the prevalence, clinical characteristics, and prognosis of hypertrophic cardiomyopathy (HCM) patients with midventricular obstruction (MVO).BackgroundPrevious descriptions of patients with MVO have been confined to case reports or small patient series, and this subgroup of HCM patients has therefore remained underrecognized.MethodsThe study population included 490 HCM patients. Left ventricular MVO was diagnosed when the peak midcavitary gradient was estimated to be ≥30 mm Hg.ResultsMVO was identified in 46 patients (9.4%). Patients with MVO were more likely to be symptomatic than those without. MVO was found to be an independent determinant of HCM-related death in multivariate models (hazard ratio [HR]: 2.23, p = 0.016), and this trend was especially pronounced for the combined endpoint of sudden death and potentially lethal arrhythmic events (HR: 3.19, p < 0.001). Apical aneurysm formation was identified in 28.3% of patients with MVO and strongly predicted HCM-related death (HR: 3.47, p = 0.008) and the combined endpoint of sudden death and potentially lethal arrhythmic events (HR: 5.08, p < 0.001). In addition, MVO without apical aneurysm was also identified as an independent determinant of the combined endpoint of sudden death and potentially lethal arrhythmic events (HR: 2.43, p = 0.045).ConclusionsThis analysis identified MVO as an independent predictor of adverse outcomes, especially the combined endpoint of sudden death and potentially lethal arrhythmic events. Our results suggest that longer periods of exposure to MVO might lead to unfavorable consequences. They also support the principle that the presence of MVO in patients with HCM has important pathophysiological implications.