Direct Link Between Cardiac Failure and Global Cerebral Atrophy in a Young Adult: A Case Report on Reduced Cerebral Artery Blood Flow.

BACKGROUND Heart failure is associated with structural brain abnormalities, including atrophy of multiple brain regions. Previous studies have reported brain atrophy in middle-aged patients with systolic heart failure. In this report, we present the case of a 21-year-old woman with idiopathic dilated cardiomyopathy, cardiac failure, and global cerebral atrophy due to reduced cerebral artery blood flow. We also discuss the impact of brain atrophy in this young adult patient with severe heart failure and no risk factors for atherosclerosis. CASE REPORT A 21-year-old woman with dyspnea and leg edema was admitted to our hospital. After several examinations, an endomyocardial biopsy led to a diagnosis of idiopathic dilated cardiomyopathy, and transthoracic ultrasound cardiography revealed that her left ventricular ejection fraction was 36%. One year after the first hospitalization, her heart failure was classified as New York Heart Association Class III. Magnetic resonance imaging showed severe global brain atrophy, and single-photon emission computed tomography combined with brain computed tomography showed reduced blood flow to the entire brain. She had no risk factors for atherosclerosis and no atherosclerotic changes to her brain or carotid arteries, but her neuropsychological and neurological findings indicated more pronounced brain and cognitive dysfunction. CONCLUSIONS This young adult patient with idiopathic dilated cardiomyopathy, cardiac failure, and global cerebral atrophy showed reduced cerebral artery blood flow and cognitive impairment. The findings of this report indicate that low cardiac output may directly cause brain atrophy in patients with systolic heart failure.

The efficacy of neutralizing monoclonal antibodies in transplant recipients with mild-to-moderate COVID-19.

INTRODUCTION: Transplant recipients (TRs) are at high risk for severe coronavirus disease 2019 (COVID-19). Neutralizing monoclonal antibodies (mAbs) are used for treating mild-to-moderate COVID-19. However, reports comparing the efficacy of COVID-19 treatment without/with mAbs in TRs are limited. We assessed the efficacy of casirivimab/imdevimab against mild-to-moderate COVID-19 in TRs. METHODS: Forty-one patients were retrospectively evaluated. The duration until defervescence, oxygen (O2) requirement ≥5 L, and neutralizing antibody levels were compared in TRs with COVID-19 without/with casirivimab/imdevimab. RESULTS: Casirivimab/imdevimab was correlated with shorter duration until defervescence and non-requirement of O2 ≥ 5 L in TRs with COVID-19 [mean: without/with: 6 vs. 2; P = 0.0002, hazard ratio (HR) = 0.3333, 95% confidence interval (CI) = 0.1763-0.6301; 15 vs. 8; P < 0.0001, HR = 0.5333, 95% CI = 0.2878-0.9883; P = 0.0377, HR = 0.1502, 95% CI = 0.02511-0.8980]. Casirivimab/imdevimab was associated with early defervescence after adjusting for sex and age (P = 0.013, HR = 0.412, 95% CI = 0.205-0.826). The antibody levels between patients without/with casirivimab/imdevimab on the day of hospitalization were not significantly different (P = 0.1055), including 13 TRs with vaccination. Antibody levels were higher in patients with casirivimab/imdevimab at 3-5 days after hospitalization than in those without, at 7-9 days after hospitalization (P < 0.0001, mean, without/with: 414.9/40000 AU/mL). CONCLUSION: Casirivimab/imdevimab was effective and increased the neutralizing antibody in TRs with mild-to-moderate COVID-19, it may contribute toward preventing the progression.

High-normal diastolic blood pressure as a risk factor for left ventricular diastolic dysfunction in healthy postmenopausal women.

Left ventricular (LV) diastolic dysfunction is associated with heart failure with preserved ejection fraction, and metabolic syndrome (MetS) is a risk factor. However, there is limited knowledge regarding the metabolic factors that contribute to LV dysfunction in postmenopausal women without comorbidities. This study aimed to analyze the relationship between LV diastolic dysfunction and MetS, as well as other cardiovascular risk factors, and to determine risks for LV diastolic dysfunction. Postmenopausal women without hypertension, diabetes mellitus, LV systolic dysfunction, or other heart diseases underwent physical examinations, including echocardiography. The study participants were diagnosed with LV diastolic dysfunction based on several echocardiographic parameters. Logistic regression analyses of LV diastolic dysfunction and cardiovascular risk factors were performed. Of the 269 postmenopausal women examined, 29 (10.7%) and 40 (14.9%) had MetS and LV diastolic dysfunction, respectively. Abnormal diastolic blood pressure (odds ratio, 3.6; 95% confidence interval, 1.16-10.9; P < 0.05) and age (odds ratio, 1.1; 95% confidence interval, 1.07-1.19; P < 0.01) were predictors of LV diastolic dysfunction. In healthy postmenopausal women, high-normal diastolic blood pressure was the only independent risk factor for LV diastolic dysfunction, and it thus may be a useful predictor of diastolic heart failure during routine physical examinations.

Granulocyte and monocyte apheresis therapy for patients with active ulcerative colitis associated with COVID-19: a case report.

Coronavirus disease 2019 (COVID-19), caused by infection with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), is now a pandemic. Although several treatment guidelines have been proposed for patients who have both inflammatory bowel disease and COVID-19, immunosuppressive therapy is essentially not recommended, and the treatment options are limited. Even in the COVID-19 pandemic, adjuvant adsorptive granulocyte and monocyte apheresis may safely bring ulcerative colitis (UC) into remission by removing activated myeloid cells without the use of immunosuppressive therapy. Our patient was a 25-year-old Japanese male with UC and COVID-19. This is the first case report of the induction of UC remission with granulocyte and monocyte apheresis treatment for active UC associated with COVID-19.

Polymerase-chain reaction testing to prevent hospital-acquired severe acute respiratory syndrome coronavirus 2 infection in Shinjuku, an epicenter in Tokyo: The Tokyo Women's Medical University model.

Hospital-acquired severe acute respiratory virus coronavirus 2 (SARS-CoV-2) infection is a healthcare challenge. We hypothesized that polymerase chain reaction testing of symptomatic triaged outpatients and all inpatients before hospitalization in Shinjuku, a coronavirus disease 2019 (COVID-19) epicenter in Tokyo, using the Tokyo Women's Medical University (TMWU) model would be feasible and efficient at preventing COVID-19. This retrospective study enrolled 2981 patients from March to May 2020. The prevalence of SARS-CoV-2 infection was 1.81% (95% credible interval [CI]: 0.95-3.47%) in triaged symptomatic outpatients, 0.04% (95% CI: 0.0002-0.2%) in scheduled asymptomatic inpatients, 3.78% (95% CI: 1.82-7.26%) in emergency inpatients, and 2.4% (95% CI: 1.49-3.82%) in symptomatic patients. There were no cases of hospital-acquired SARS-CoV-2 infection. This shows that the TWMU model could prevent hospital-acquired SARS-CoV-2 infection and is feasible and effective in reducing the impact of SARS-CoV-2 infection in the hospitals.

Emergency upper gastrointestinal endoscopy performed safely in a patient with COVID-19 with suspected hemorrhagic shock.

Coronavirus disease 2019 caused by severe acute respiratory syndrome coronavirus 2 has spread explosively throughout the world and has since been declared a pandemic by the World Health Organization. Although it is recommended that upper gastrointestinal endoscopies either be postponed or canceled during the pandemic because of their high risk of aerosol generation, this does not apply in emergency cases, which may include patients with coronavirus disease. In this case report, we describe the safe undertaking of an emergency upper gastrointestinal endoscopy in a patient with suspected hemorrhagic shock who tested positive for the severe acute respiratory syndrome coronavirus 2 using the polymerase chain reaction. We performed the procedure in the contamination zone of a specialized coronavirus disease ward with prespecified zones. Full personal protective equipment was worn during the procedure, as recommended by various academic societies, and careful attention was paid to the sterilization of all equipment after the procedure. Thus, emergency endoscopies can be performed safely in patients with coronavirus disease in a suitable environment by using appropriate personal protective equipment and by handling the equipment appropriately.

Arousal index as a marker of carotid artery atherosclerosis in patients with obstructive sleep apnea syndrome.

PURPOSE: It was shown in a previous cohort study that men with internal carotid artery (ICA) plaque, defined as focal wall thickness of ≥ 1.5 mm, had a threefold higher risk of stroke than those without plaque. We examined the relationship between arousal indices and sleep stages in patients with obstructive sleep apnea syndrome (OSAS) and carotid atherosclerosis. METHODS: Carotid atherosclerosis severity was evaluated using the maximal carotid wall intima-media thickness of the ICA (ICA-maxIMT) and plaque in 83 patients with OSAS. RESULTS: The ICA-maxIMT values were positively correlated with the apnea hypopnea index (AHI) (ρ = 0.294, P = 0.007), arousal index (ρ = 0.289, P = 0.008), oxygen desaturation index (ρ = 0.298, P = 0.006), percentage of visually scored total sleep time spent in nocturnal oxygen saturation < 90% (SpO2 < 90%) (ρ = 0.246, P = 0.025), and the percentage of visually scored total sleep time spent in non-REM sleep stage 1 (ρ = 0.326, P = 0.003) and were negatively correlated with the percentage of visually scored total sleep time spent in non-REM sleep stages 2 and 3. Arousal index, diabetes mellitus, and age were found to be independent predictors of ICA plaque presence (OR 1.052, P = 0.003; OR 8.705, P = 0.026; OR 1.064, P = 0.023, respectively). CONCLUSIONS: Several PSG variables that are indicative of sleep fragmentation, sleep disordered breathing, and poor sleep quality correlated with the occurrence of atherosclerosis, but total arousal index was the only independent predictive factor.

Contrast-Induced Nephropathy and Oxygen Pretreatment in Patients With Impaired Renal Function.

INTRODUCTION: Contrast-induced nephropathy is a complication following coronary angiography and percutaneous coronary intervention. Because contrast-induced nephropathy is a predictor of long-term mortality in patients with ischemic heart disease undergoing percutaneous coronary intervention, preventive strategies are required. We assessed the effects of periprocedural oxygenation on contrast-induced nephropathy among patients with pre-existing renal dysfunction. METHODS: A total of 200 consecutive patients with impaired renal function (estimated glomerular filtration < 60 ml/min per 1.73 m2) undergoing elective cardiovascular angiography were randomly assigned to an oxygenation treatment (n = 100) or control group (n = 100). In oxygenation treatment, pure oxygen (2 L/min) was administered for 10 minutes before exposure to contrast medium. The primary endpoint was the incidence of contrast-induced nephropathy, defined as a ≥ 25% increase in serum creatinine levels from baseline within 48 hours of exposure. RESULTS: In the oxygenation treatment group, partial pressure of arterial oxygen was higher (135 ± 25 mm Hg vs. 84 ± 10 mm Hg, P < 0.001); contrast-induced nephropathy incidence was lower (1% vs. 8%, odds ratio [OR] = 0.12, 95% confidence interval [CI] = 0.01-0.95, P = 0.02); and partial pressure of arterial carbon dioxide and bicarbonate base lactate levels were similar compared with those in the control group. Upon univariate analysis, excess and absence of oxygenation treatment (OR = 9.18, CI = 1.13-74.86, P = 0.03) and anemia (OR = 4.30, CI = 1.04-17.78, P = 0.04) were shown to be associated with contrast-induced nephropathy incidence. CONCLUSION: Oxygenation, a simple, nonpharmacological strategy, may be beneficial when using contrast media in patients with impaired renal function from noninvasive angiography to emergency catheterization.

Effects of Calcium-Channel Blocker Benidipine-Based Combination Therapy on Cardiac Events　- Subanalysis of the COPE Trial.

BACKGROUND: The Combination Therapy of Hypertension to Prevent Cardiovascular Events (COPE) trial was conducted to compare the effects of regimens combining the dihydropyridine calcium-channel blocker benidipine with each of 3 secondary agent types (an angiotensin-receptor blocker (ARB), a ß-blocker and a thiazide) in Japanese hypertensive outpatients who did not achieve target blood pressure (<140/90 mmHg) with benidipine 4 mg/day alone. The analysis included 3,293 patients (ARB, 1,110; ß-blocker, 1,089; thiazide, 1,094) with a median follow-up of 3.61 years. The main results of the COPE trial demonstrated that the incidences of hard cardiovascular composite endpoints and fatal or non-fatal strokes were significantly higher in the benidipine/ß-blocker group than in the benidipine/thiazide group.Methods and Results:We further evaluated the treatment effects on different cardiac events among the 3 benidipine-based regimens.We observed a total of 50 cardiac events, 4.2 per 1000 person-years. The incidences of total cardiac events and each cardiac event were similarly low among the 3 treatment groups. Unadjusted and multi-adjusted hazard ratios for total cardiac events showed no significant difference among the 3 treatment groups. CONCLUSIONS: This subanalysis of the COPE trial demonstrated that blood pressure-lowering regimens combining benidipine with an ARB, ß-blocker or thiazide diuretic were similarly effective for the prevention of cardiac events in Japanese hypertensive outpatients.

Cancer antigen-125 plasma level as a biomarker of new-onset atrial fibrillation in postmenopausal women.

OBJECTIVE: Plasma cancer antigen (CA)-125 is a tumour marker recently shown to be associated with systolic heart failure and new-onset atrial fibrillation (AF) after myocardial infarction. However, no reports have described the relationship between CA-125 and new-onset AF in healthy postmenopausal women. The aim of the present study was to evaluate the relationship between CA-125 and new-onset AF in postmenopausal women. METHODS: Between 2005 and 2015, 2086 women, including 1012 postmenopausal women, visited our hospital for annual health check-ups. We excluded patients with systolic dysfunction, chronic inflammatory disease, chronic obstructive pulmonary disease, histories of AF or neoplastic diseases. A total of 746 postmenopausal women underwent thorough physical examinations, including those for biomarkers such as brain natriuretic peptide, high-sensitivity C-reactive protein (hs-CRP) and CA-125. RESULTS: During the 10-year observation period, AF was documented in 31 participants (4.2%). The mean age of participants developing AF (75±6 years) was higher than that of those without AF (68±8 years). Participants developing AF showed significantly higher CA-125 (11.4±6.3 U/mL) and hs-CRP (0.10±0.11 mg/dL) levels than did those without AF (7.7±3.2 U/mL, p<0.01; 0.07±0.08 mg/dL, p<0.05). Cox regression analyses revealed ageing (HR 1.3; 95% CI 1.08 to 1.57; p<0.01) and plasma CA-125 levels (HR 1.29; 95% CI 1.10 to 1.51; p=0.02) as independent predictors of AF. CONCLUSIONS: High CA-125 levels might be associated with new-onset AF in healthy postmenopausal women.

Prognostic predictive value of gene mutations in Japanese patients with hypertrophic cardiomyopathy.

Although some studies have attempted to find useful prognostic factors in hypertrophic cardiomyopathy (HCM), those results are not fully helpful for use in actual clinical practice. Furthermore, several genetic abnormalities associated with HCM have been identified. However, the genotype-phenotype correlation in HCM remains to be elucidated. Here, we attempted to assess patients with different types of gene mutations causing HCM and investigate the prognosis. A total of 140 patients with HCM underwent a screening test for myofilament gene mutations by direct sequencing of eight sarcomeric genes. Patients with a single mutation in cardiac troponin T, cardiac troponin I, α-tropomyosin, and regulatory and essential light chains were excluded from the study because the number of cases was too small. The clinical presentations and outcomes of the remaining 127 patients with HCM, 31 ß-myosin heavy chain (MYH7) mutation carriers, 19 cardiac myosin-binding protein C (MYBPC3) mutation carriers, and 77 mutation non-carriers were analyzed retrospectively. MYBPC3 mutation carriers had a high frequency of ventricular arrhythmia and syncope. Kaplan-Meier curves revealed no significant difference in prognosis among the three groups, but a lack of family history of sudden death (SD) and a past history of syncope were significantly related to poor prognosis. An absence of family history of SD and past history of syncope are useful prognostic factors in patients with HCM. MYH7 and MYBPC3 mutations did not significantly influence prognosis compared to non-carriers. However, patients with the MYBPC3 mutation should be closely followed for the possibility of SD.

Beta-Adrenergic Blockade Therapy for Autonomic Dysfunction is Less Effective for Elderly Patients with Heart Failure and Reduced Left Ventricular Ejection Fraction.

OBJECTIVE: Heart rate variability (HRV) has been reported to be an independent predictor of all-cause and sudden cardiac death in patients with heart failure. In the aging heart, however, both autonomic and cardiac functions appear to be altered. We assessed the relationship between aging and responsiveness of HRV and ventricular remodeling to beta-adrenergic blockade therapy in patients with heart failure and reduced ejection fraction (HFREF). METHODS: Twenty-eight clinically stable patients with chronic heart failure, sinus rhythm, and left ventricular ejection fraction <50% as confirmed by echocardiography were included. At baseline and after carvedilol treatment, 24-hour ambulatory Holter monitor recording was used to analyze HRV indices by the maximum entropy method. Changes in these parameters were compared among three age groups. RESULTS: HR decreased in all groups after carvedilol treatment, but was still highest in the youngest group despite the same treatment doses. Time and frequency domain variables improved. The response of time domain variables (the standard deviation of all normal sinus to normal sinus [NN] intervals and the standard deviation of the averages of NN intervals in all 5-minute or 30-minute segments) to carvedilol therapy significantly decreased with increasing age. Ventricular reverse remodeling induced by carvedilol therapy significantly decreased with increasing age. Increases in time domain variables and a low-frequency domain moderately correlated with left ventricular reverse remodeling. CONCLUSION: Beta-adrenergic blockade therapy improved HRV variables and ventricular remodeling in HFREF patients; however, the response tended to be milder in the elderly. HRV improvement was associated with ventricular reverse remodeling.

Gender differences in predictors of left ventricular myocardial relaxation in non-obese, healthy individuals.

BACKGROUND: Previous studies indicate that individuals with metabolic syndrome (MetS) might be at risk for left ventricular (LV) diastolic dysfunction. However, little is known about which metabolic factors contribute to the development of LV dysfunction in individuals who are not obese or overweight and who do not have diabetes mellitus and/or cardiovascular disease. METHODS: Participants without diabetes mellitus, systolic dysfunction, or other heart diseases underwent a thorough physical examination, including tissue Doppler echocardiography. A peak early mitral annular velocity (e') of <5.0 was designated as indicating abnormal LV myocardial relaxation (LVMR). We performed single and multiple logistic regression analyses of e' and cardiovascular risk factors, including MetS factors and indicators of major organ dysfunction. Normal-weight subjects (body mass index <25 kg/m2) were also analyzed. RESULTS: A total of 1055 individuals (mean age, 63 ± 13 years) participated, of which 307 (29.1%) had MetS and 199 (18.9%) had abnormal LVMR. Multiple logistic regression analysis revealed waist circumference (WC) (odds ratio [OR] 1.04, P < 0.05) and age (OR 1.10, P < 0.05) to be predictors of abnormal LVMR. In normal-weight subjects (n = 806), aging (OR 1.08, P < 0.01), abnormal WC (OR 3.80, P < 0.01), and renal dysfunction (OR 2.14, P < 0.01) were predictors of abnormal LVMR. Among MetS factors, abnormal WC in men (OR 3.70, P < 0.01) and high diastolic blood pressure (DBP) in women (OR 4.00, P = 0.01) were related to abnormal LVMR.

Multicenter, Randomized, Double-Blinded, Placebo-Controlled Phase II Study of Serelaxin in Japanese Patients With Acute Heart Failure.

BACKGROUND: Serelaxin, a recombinant form of human relaxin-2, is in development for treating acute heart failure (AHF) and a Phase II study in Japanese AHF patients was conducted. METHODS AND RESULTS: A randomized, double-blind, placebo-controlled study of serelaxin at 10 and 30 µg·kg(-1)·day(-1)continuous intravenous infusion for up to 48 h, added to standard care for Japanese AHF patients. Primary endpoints were adverse events (AEs) through Day 5, serious AEs (SAEs) through Day 14, and serelaxin pharmacokinetics. Secondary endpoints included changes in systolic blood pressure (SBP) and cardiorenal biomarkers. A total of 46 patients received the study drug and were followed for 60 days. The observed AE profile was comparable between the groups, with no AEs of concern. Dose-dependent increase in the serum concentration of serelaxin was observed across the 2 dose rates of serelaxin. A greater reduction in SBP was observed with serelaxin 30 µg·kg(-1)·day(-1)vs. placebo (-7.7 [-16.4, 1.0] mmHg). A greater reduction in NT-proBNP was noted with serelaxin (-50.8% and -54.9% for 10 and 30 µg·kg(-1)·day(-1), respectively at Day 2). CONCLUSIONS: Serelaxin was well tolerated in this study with Japanese AHF patients, with no AEs of concern and favorable beneficial trends on efficacy. These findings support further evaluation of serelaxin 30 µg·kg(-1)·day(-1)in this patient population.

Impact of myocardial reperfusion status on QT dispersion after successful recanalization of the infarct-related artery in acute myocardial infarction.

OBJECTIVE: To determine whether adequate myocardial perfusion status after transluminal recanalization is associated with prompt improvement of QT dispersion (QTd). BACKGROUND: Transluminal recanalization of the infarct-related coronary artery in acute myocardial infarction aims to promptly restore myocardial perfusion, to maximize electrical and mechanical recovery. QTd represents the heterogeneity of ventricular repolarization, which may affect electrical stability. METHODS: Forty patients who underwent primary percutaneous coronary intervention for their first anterior acute ST-elevation myocardial infarction were prospectively enrolled. Myocardial reperfusion status was assessed by myocardial blush grade (MBG) on the final angiogram after successful recanalization (Thrombolysis In Myocardial Infarction Grade 3 flow). RESULTS: Preprocedural QTd was similar in patients with final MBG 0-1, 2, and 3 (76 ± 24, 67 ± 13, and 69 ± 13 milliseconds, respectively; P = 0.661). After recanalization, QTd decreased in patients with MBG 3 (39 ± 16 milliseconds, P < 0.001) but not in patients with MBG 0-1 (74 ± 20 milliseconds) or MBG 2 (82 ± 16 milliseconds). Multivariate analysis showed that postprocedural MBG was an independent predictor of QTd after recanalization (standardized regression coefficient = -0.628, P < 0.001). CONCLUSIONS: Adequate tissue perfusion may be crucial for electrical stability of the myocardium after reperfusion.

Estradiol promotes neural stem cell differentiation into endothelial lineage and angiogenesis in injured peripheral nerve.

Neural stem cells (NSCs) differentiate into endothelial cells (ECs) and neuronal cells. Estradiol (E2) is known to exhibit proangiogenic effects on ischemic tissues via EC activation. Therefore, we hypothesized that E2 can promote the therapeutic potential of NSC transplantation for injured nerve repair via the differentiation of NSCs into ECs during neovascularization. NSCs isolated from newborn mouse brains were transplanted into injured sciatic nerves with (NSC/E2 group) or without E2-conjugated gelatin hydrogel (E2 group). The NSC/E2 group exhibited the greatest recovery in motor nerve conduction velocity, voltage amplitude, and exercise tolerance. Histological analyses revealed increased intraneural vascularity and blood perfusion as well as striking NSC recruitment to the neovasculature in the injured nerves in the NSC/E2 group. In vitro, E2 enhanced the NSC migration and proliferation inhibiting apoptosis. Fluorescence-activated cell sorting analysis also revealed that E2 significantly increased the percentage of CD31 in NSCs, and the effect of E2 was completely neutralized by the estrogen receptor antagonist ICI. The combination of E2 administration and NSC transplantation cooperatively improved the functional recovery of injured peripheral nerves, at least in part, via E2-associated NSC differentiation into ECs. These findings provide a novel mechanistic insight into both NSC biology and the biological effects of endogenous E2.

Late gadolinium enhancement in cardiac sarcoidosis: characteristic magnetic resonance findings and relationship with left ventricular function.

PURPOSE: Cardiac involvement is an important prognostic factor in sarcoidosis, and cardiovascular magnetic resonance (CMR) imaging with late gadolinium enhancement (LGE) can facilitate the identification of cardiac sarcoidosis (CS). In patients with CS, we investigated LGE characteristics and their relationship with left ventricular (LV) function to identify those characteristics unique to severely reduced LV function. We also investigated the relationship between LGE and duration of sarcoidosis. MATERIALS AND METHODS: We retrospectively evaluated 19 patients with CS diagnosed according to the 2006 revised guidelines of the Japanese Ministry of Health and Welfare who underwent CMR imaging. We analyzed LGE location using 17-segment and subsegment (subepicardial, midwall, subendocardial, and transmural) models and evaluated the relationship between the characteristics of LGE and LV ejection fraction (LVEF) and duration of sarcoidosis. RESULTS: Images of 17 of the 19 patients showed LGE most frequently in the subepicardial layer. The total number of affected segments with LGE correlated significantly with LVEF (r=-0.84, P<0.0001) and LV-diastolic volumes (r=0.88, P<0.0001). Transmural lesions were significantly more common in patients with LVEF of 35% or lower than in those with LVEF exceeding 35% (P=0.0004). All patients with LVEF of 35% or lower had both subepicardial and transmural lesions. The total number of affected segments with LGE correlated with the duration of sarcoidosis in patients with onset in an extracardiac organ (r=0.76, P=0.005). CONCLUSIONS: Demonstration of a characteristic LGE pattern and location allows diagnosis of CS, and CMR imaging with LGE aids in prediction of LV function.

Clinical implications of midventricular obstruction in patients with hypertrophic cardiomyopathy.

OBJECTIVES: We investigated the prevalence, clinical characteristics, and prognosis of hypertrophic cardiomyopathy (HCM) patients with midventricular obstruction (MVO). BACKGROUND: Previous 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. METHODS: The study population included 490 HCM patients. Left ventricular MVO was diagnosed when the peak midcavitary gradient was estimated to be ≥30 mm Hg. RESULTS: MVO 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). CONCLUSIONS: This 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.