Usefulness of tissue Doppler-derived left ventricular isovolumic contraction velocity in patients with heart failure with preserved ejection fraction.

BACKGROUND: Heart failure with preserved ejection fraction (HFpEF) is a group of diseases classified by left ventricular (LV) EF, a measure of pump function. However, LVEF does not reflect LV contractility. Previous studies have shown that tissue Doppler-derived LV isovolumic contraction velocity (IVCv) correlates well with the LV peak dP/dt, an index of LV contractility. We explored whether LV IVCv is associated with 1-year post-discharge outcomes in HFpEF. METHODS: We enrolled 113 patients (median age, 86 years, 45 male) with HFpEF (EF on admission ≥ 50%) who were admitted to our hospital for the treatment of acute HF. Clinical characteristics including echocardiographic data were obtained before discharge. IVCv was obtained from the tissue Doppler waveforms of both the septal and lateral mitral annulus of the apical 4-chamber view and averaged data were used. Primary outcomes were all-cause death or unplanned hospitalization due to HF within the first year. RESULTS: Among all patients, median LVEF was 61%, left atrial diameter was 47 mm, E/e' was 17.5, and IVCv was 4.5 cm/sec; mean tricuspid regurgitation velocity was 2.6 m/sec. Regarding laboratory data, the median plasma B-type natriuretic peptide level was 185 pg/mL. Thirty-four events occurred (15 deaths, 19 unplanned hospitalizations due to HF) within the first year. In multivariate Cox proportional hazards analyses, IVCv was significantly associated with outcomes (hazard ratio .68, 95% confidence interval .50-.89, p = .0095), independent of general characteristics, echocardiographic measures and pertinent laboratory parameters. CONCLUSION: LV IVCv was independently associated with 1-year outcomes in patients with HFpEF.

Changes in prognostic nutritional index during hospitalization and outcomes in patients with acute heart failure.

Although the prognostic nutritional index (PNI) is reported as a prognosticator in patients with heart failure (HF), that is evaluated usually on one occasion, and any changes in PNI during hospitalization are not considered. This study aimed to assess between changes in the PNI during hospitalization and outcomes in patients with acute HF. We enrolled 141 patients (median age, 84 years, 75 male) admitted to our hospital for the treatment of acute HF. The PNI was calculated on admission and at discharge based on the original report. According to the PNI change during hospitalization, patients were classified as either improved (PNI at discharge ≥ PNI on admission) or deteriorated (PNI at discharge < PNI on admission). Primary outcomes were all-cause death or unplanned hospitalization due to HF within the first year. Forty-nine events occurred (19 deaths, 30 HF hospitalizations). The event-free survival rate determined by Kaplan-Meier analysis was significantly higher in patients in the improved group (log-rank test, P < 0.0001), regardless of the PNI value on admission. Multivariate analysis showed that younger age (HR 1.06, 95% CI 1.01-1.11, P = 0.016), higher body mass index (HR 0.90, 95% CI 0.82-0.98, P = 0.021) and the PNI in the improved group (HR 0.30, 95% CI 0.14-0.57, P = 0.0006) were independently associated with favorable outcomes. In conclusion, changes in nutritional status during hospitalization, evaluated using the PNI on admission and at discharge, were independently associated with 1-year outcomes in patients with acute HF.

Factors influencing inferior vena cava diameter and its respiratory variation: Simultaneous comparison with hemodynamic data.

BACKGROUND: Ultrasound assessment of inferior vena cava (IVC) diameter and its respiratory variation is widely used to estimate right atrial pressure (RAP). Generally, the IVC distends as the RAP rises; however, there may be discrepancies between the values. Therefore, it is critical to recognize clinical factors other than RAP that may influence IVC measurements. METHODS: We obtained the IVC maximum diameter and IVC collapsibility index (IVCCI) simultaneously during right-heart catheterization in 71 consecutive patients. Then, we assessed various clinical and hemodynamic factors to elucidate the independent determinants of IVC measurements. Moreover, we tried to generate the regression equation to estimate mean RAP from the IVC maximum diameter and IVCCI. RESULTS: The mean IVC maximum diameter and IVCCI were 15 ± 4 mm and 51 ± 15%, respectively. In stepwise multivariate analysis, the higher mean RAP (ß = 0.52; p < 0.0001), the presence of significant tricuspid regurgitation (ß = 0.31; p = 0.0005), a larger body surface area (ß = 0.22; p = 0.0017), and younger age (ß = -0.18; p = 0.049) were independently associated with the IVC maximum diameter. Only the mean RAP was independently associated with the IVCCI (ß = -0.45; p < 0.0001). The regression equation (R2 = 0.43, p < 0.0001) was as follows: estimated mean RAP = 3.7 + 0.62 × maximum IVC diameter / BSA - 0.07 × IVCCI. CONCLUSION: Distension of the IVC mainly occurs with elevated RAP. However, the presence of significant tricuspid regurgitation, a larger body surface area, and younger age are associated with the IVC maximum diameter, independently of RAP. Interestingly, IVCCI is influenced only by RAP.

Combined evaluation of right ventricular function using echocardiography in non-ischaemic dilated cardiomyopathy.

AIMS: Although comprehensive assessment of right ventricular (RV) function using multiple echocardiographic parameters is recommended for management of patients with non-ischaemic dilated cardiomyopathy (DCM), it is unclear which RV parameters to combine. Additionally, normalization of RV parameters by estimated pulmonary artery systolic pressure (PASP), in consideration of RV-pulmonary artery coupling, may be clinically significant. The aim of our study was to elucidate the best combination of echocardiographic RV functional parameters, with or without indexing for PASP, to predict outcome in patients with heart failure with reduced ejection fraction secondary to DCM. METHODS AND RESULTS: We retrospectively analysed 109 DCM patients with left ventricular ejection fraction <40%. RV size was assessed by RV end-diastolic area (RVEDA) and RV end-systolic area (RVESA) from RV-focused apical four-chamber view. RV function was assessed by fractional area change (FAC) and tricuspid annular plane systolic excursion (TAPSE) and by RV longitudinal strain (RVLS) using two-dimensional speckle-tracking echocardiography. All functional parameters were also indexed for estimated PASP. Cox analyses were used to evaluate the association of RV morphology and functional parameters with 1 year outcome (composite of left ventricular assist device implantation and all-cause death). Area under the curve was used to compare prognostic values. Mean age was 44 ± 14 years, and 76 (69.7%) were men. Mean left ventricular ejection fraction was 21.9%, median RVEDA was 22.1 cm2 , FAC was 27.0%, TAPSE was 15.0 mm, and RVLS was -12.5%. Forty-one (37.6%) patients experienced the primary outcome. Multivariate Cox analysis revealed that RVEDA, RVESA, FAC, TAPSE, RVLS, FAC/PASP, and RVLS/PASP were independent predictors for primary outcome (all P < 0.05). However, normalization with PASP did not improve area under the curve for any RV functional parameters. When we evaluate hazard ratios according to the combination of two echocardiographic parameters of RV function, patients with impairment of both FAC (<27%) and RVLS (>-8.6%) had significantly higher hazard ratio than those with either impairment alone (11.3 vs. 3.4, P < 0.001); the other combinations did not improve prognostic value. CONCLUSIONS: Normalizing echocardiographic RV parameters for PASP did not improve the prognostic values for our population. Meanwhile, combined evaluation of FAC and RVLS improved risk stratification in patients with heart failure with reduced ejection fraction secondary to DCM.

Association Between Albumin-Bilirubin Score at Admission and In-Hospital Mortality in Patients with Acute Heart Failure.

Liver dysfunction is one of the most recognized complications in patients with acute heart failure (HF) and therefore a liver function score may be useful for risk-stratification in those patients. Recently, the albumin-bilirubin (ALBI) score was developed as a new model to assess liver function in liver disease. We explored the association between the ALBI score at admission and in-hospital mortality in patients with acute HF.We enrolled 262 patients (median age, 86 years, 137 males) who were admitted to our hospital for treatment of acute HF. The following data were recorded: vital signs, laboratory data including B-type natriuretic peptide (BNP) level, echocardiographic data at admission, demographic and clinical characteristics, and treatment and prognostic information. The Get With the Guidelines-Heart Failure (GWTG-HF) risk score was calculated as an established risk model for each patient. The primary outcome was all-cause in-hospital mortality.During hospitalization, 37 patients (14.1%) died. The in-hospital mortality rate was significantly higher in patients with ALBI scores > -2.25 compared with patients with ALBI scores ≤ -2.25 (21.1% versus 4.5%, respectively; P = 0.0001). Multivariate analysis revealed that the GWTG-HF score (odds ratio [OR] 1.16, 95% confidence interval [CI] 1.08-1.25, P < 0.0001), BNP level (OR 1.0007, 95% CI 1.0003-1.001, P = 0.0003) and ALBI score (OR 6.0, 95% CI 1.8-19.6, P = 0.0017) were independently associated with in-hospital mortality.Our results indicated that the ALBI score was independently associated with in-hospital mortality in patients hospitalized for acute HF.

Mitral Valvular Coaptation-Zone Area Is Associated with the Severity of Atherosclerosis Assessed by Cardio-Ankle Vascular Index.

Preservation of the mitral valve (MV) size is essential for valve function, and a reduced MV coaptation-zone area increases the risk of developing functional mitral regurgitation (FMR). We aimed to determine if the MV leaflet and coaptation-zone areas were associated with the severity of atherosclerosis assessed by cardio-ankle vascular index (CAVI) in patients with normal left ventricle (LV) systolic function and size by real-time 3D echocardiography (RT3DE).We performed RT3DE analysis in 66 patients with normal LV size and ejection fraction who underwent 2D echocardiography and CAVI. MV coaptation-zone areas were measured by custom 3D software and indexed by body surface area (BSA). The associations of clinical factors and mean CAVI with MV leaflet and coaptation-zone areas were evaluated by univariable and multivariable linear regression analyses.On univariable analysis, MV leaflet area/BSA was significantly associated with age (r = -0.335, P = 0.0069) and mean CAVI (r = -0.464, P < 0.001), and MV coaptation-zone area was significantly associated with age (r = -0.626, P < 0.001), hypertension (r = -0.626, P < 0.001), dyslipidemia (r = -0.626, P < 0.001), E/e' (r = -0.626, P < 0.001), and CAVI (r = -0.740, P < 0.001). On multivariable analysis, mean CAVI was independently associated only with MV leaflet area/BSA (standardized coefficient = -0.611, P < 0.001) and MV coaptation-zone area/BSA (standardized coefficient = -0.74, P < 0.001).In patients with normal LV systolic function and size, MV leaflet and coaptation-zone areas might be reduced according to advancing atherosclerosis. Patients with atherosclerosis might be at increased risk of developing FMR.

Relationship Between Mitral Leaflet Size and Coaptation and Their Associated Factors in Patients with Normal Left Ventricular Size and Systolic Function.

Enlargement of the mitral valve (MV) has gained attention as a compensatory mechanism for functional mitral regurgitation (FMR). We aimed to determine if MV leaflet area is associated with MV coaptation-zone area and identify the clinical factors associated with MV leaflet size and coaptation-zone area in patients with normal left ventricle (LV) systolic function and size using real-time 3D echocardiography (RT3DE).We performed RT3DE in 135 patients with normal LV size and ejection fraction. MV leaflet and coaptation-zone areas were measured using custom 3D software. The clinical factors associated with MV leaflet and coaptation-zone areas were evaluated using univariate and multivariate linear regression analyses.There was a significant relationship between MV leaflet and coaptation-zone areas (r = 0.499, P < 0.001). MV leaflet area was strongly associated with body surface area (BSA) (r = 0.905, P < 0.001) rather than LV size and age. MV leaflet area/BSA was independently associated with male gender (P = 0.002), lower diastolic blood pressure (P = 0.042), and LV end-diastolic volume (LVEDV) index (P = 0.048); MV coaptation-zone area/BSA was independently associated with lower LVEDV index (P = 0.01).In patients with normal LV systolic function and size, MV leaflet size has a significant impact on competent MV coaptation. MV leaflet area might be intrinsically determined by body size rather than age and LV size, and the MV leaflet area/BSA is relatively constant. On the other hand, some clinical factors might also influence MV leaflet and coaptation-zone area.

Detection of Pulmonary Hypertension with Systolic Pressure Estimated by Doppler Echocardiography.

Pulmonary hypertension (PH) is defined as a mean pulmonary artery pressure (PAP) ≥ 25 mmHg at rest as assessed by right heart catheterization (RHC), and Doppler-derived systolic PAP (sPAPECHO) or tricuspid regurgitation pressure gradient (TRPG) is widely used to screen for PH. However, the cutoff value of sPAPECHO or TRPG for detecting a mean PAP ≥ 25 mmHg that was determined invasively has not been well defined.We studied 189 patients who underwent RHC. Echocardiography was performed within 24 hours of invasive evaluation, and sPAPECHO was defined as the TRPG with right atrial pressure estimated on the basis of the current guideline.From the receiver operating characteristic (ROC) curve analysis, the optimal sPAPECHO, and TRPG cutoffs for detecting PH were 41 mmHg (sensitivity, 92%; specificity, 91%; area under the curve = 0.95) and 36 mmHg (sensitivity, 90%; specificity, 93%; area under the curve = 0.95), respectively. The area under the TRPG ROC curve was similar to the area under the sPAPECHO ROC curve.Given that Doppler echocardiography is required to accurately detect PH rather than to accurately estimate systolic PAP, our results provide useful information with regard to screening patients for PH and recommending further investigations on PH.

Effect of Left Ventricular Reverse Remodeling on Long-term Outcomes After Aortic Valve Replacement.

There have been few studies with a large number of patients on the effect of left ventricular (LV) reverse remodeling and long-term outcomes after aortic valve replacement (AVR). This study aimed to investigate long-term outcomes and the prognostic impact of follow-up echocardiographic parameters after AVR. We evaluated 456 consecutive patients from a retrospective multicenter registry in Japan (J-PROVE-Retro) who underwent AVR for aortic valve diseases (predominantly aortic stenosis [AS]; 326 patients and aortic regurgitation [AR]; 130 patients). Preoperative and follow-up echocardiography at 1 year after AVR was evaluated. The primary outcome measure was a composite of cardiac death or hospitalization due to heart failure. The median follow-up period was 9.2 years in AS group and 9.7 years in AR group. The freedom rate from the primary outcome was 92% at 5 years and 79% at 10 years in AS, and 97% at 5 years, and 93% at 10 years in AR. LV end-diastolic and end-systolic diameters, and the LV mass index decreased and LV ejection fraction increased after AVR in both AS and AR, and LV mass index was normalized in more than half of the patients. In the Cox proportional hazard model, echocardiographic parameters at 1 year after AVR were more strongly related to long-term outcomes than preoperative echocardiographic parameters. In conclusion, echocardiographic parameters at 1 year after AVR are more important as predictors of long-term outcomes than preoperative parameters in both AS and AR. More attention should be paid on early postoperative remodeling for long-term follow-up of patients after AVR.

The Significance of the Effect of Visceral Adiposity on Left Ventricular Diastolic Function in the General Population.

We evaluated the association between visceral adiposity and left ventricular (LV) diastolic function in association with plasma adiponectin levels in 213 subjects without overt cardiac diseases. Abdominal visceral fat area was quantified by computed tomography. Excessive visceral fat was significantly associated with impaired diastolic parameters including E/A, E' and E/E'. Although serum adiponectin levels decreased with increased visceral adiposity, there was no independent association between serum adiponectin levels and diastolic parameters, which suggest that the role of adiponectin in this association might be indirect.

Prognostic Value of a Simple Echocardiographic Parameter, the Right Ventricular Systolic to Diastolic Duration Ratio, in Patients with Advanced Heart Failure with Non-Ischemic Dilated Cardiomyopathy.

The prognostic value of the right ventricular (RV) systolic to diastolic duration ratio (S/D ratio) in patients with advanced heart failure is not clear.We enrolled 45 patients with DCM (40 ± 13 years, 33 male) who were admitted to our hospital for evaluation or treatment of heart failure. The RV systolic and diastolic durations were measured using continuous Doppler imaging of tricuspid regurgitation, and the RV S/D ratio was calculated. Cardiac events were defined as cardiac death or left ventricular assist device implantation within the first year. Twenty-eight cardiac events occurred. The RV S/D ratio was significantly higher in the event group than in the event-free group (1.8 ± 0.8 versus 1.2 ± 0.5, P = 0.008). Univariate analysis showed that the RV S/D ratio, plasma brain natriuretic peptide concentration, left atrial volume index, and mitral deceleration time were associated with these events. Receiver operating characteristic curve analysis revealed that the optimal RV S/D cutoff value to predict events was 1.2 (sensitivity 79%, specificity 65%, area under the curve 0.745). Kaplan-Meier analysis indicated a significantly higher event rate in patients with an RV S/D ratio > 1.2 (log-rank test, P = 0.003). The addition of an RV S/D ratio > 1.2 improved the prognostic utility of a model that included conventional variables (P = 0.014).In patients with advanced heart failure with DCM, the RV S/D ratio was higher in patients with events than in those without events. The addition of the RV S/D ratio to conventional parameters may provide better prognostic information.

Echocardiographic assessment of right ventricular function in routine practice: Which parameters are useful to predict one-year outcome in advanced heart failure patients with dilated cardiomyopathy?

BACKGROUND: Right ventricular (RV) function has recently gained attention as a prognostic predictor of outcome even in patients who have left-sided heart failure. Since several conventional echocardiographic parameters of RV systolic function have been proposed, our aim was to determine if any of these parameters (tricuspid annular plane systolic excursion: TAPSE, tissue Doppler derived systolic tricuspid annular motion velocity: S', fractional area change: FAC) are associated with outcome in advanced heart failure patients with dilated cardiomyopathy (DCM). METHODS: We retrospectively enrolled 68 DCM patients, who were New York Heart Association (NYHA) Class III or IV and had a left ventricular (LV) ejection fraction <35%. All patients were undergoing evaluation for heart transplantation or management of heart failure. Primary outcomes were defined as LV assist device implantation or cardiac death within one year. RESULTS: Thirty-nine events occurred (5 deaths, 32 LV assist devices implanted). Univariate analysis showed that age, systolic blood pressure, heart rate, NYHA functional class IV, plasma brain natriuretic peptide concentration, intravenous inotrope use, left atrial volume index, and FAC were associated with outcome, whereas TAPSE and S' were not. Receiver-operating characteristic curve analysis showed that the optimal FAC cut-off value to identify patients with an event was <26.7% (area under the curve=0.74). The event-free rate determined by Kaplan-Meier analysis was significantly higher in patients with FAC≥26.7% than in those with FAC<26.7% (log-lank, p=0.0003). Moreover, the addition of FAC<26.7% improved the prognostic utility of a model containing clinical variables and conventional echocardiographic indexes. CONCLUSIONS: FAC may provide better prognostic information than TAPSE or S' in advanced heart failure patients with DCM.

Reconsideration of Inferior Vena Cava Parameters for Estimating Right Atrial Pressure in an East Asian Population　- Comparative Simultaneous Ultrasound-Catheterization Study.

BACKGROUND: Ultrasound measurements of the inferior vena cava (IVC) diameter (IVCD), together with its respiratory variation, provide a noninvasive estimate of right atrial pressure (RAP). However, there is a paucity of studies that have compared this technique with simultaneous catheterization. We explored the best cut-off values of IVC parameters for elevated RAP in comparison with RAP measured by catheterization.Methods and Results:We prospectively enrolled 120 East Asian patients who were scheduled for catheterization. The IVCD and IVC collapsibility index (IVCCI) were measured according to the current guidelines. The optimal maximum IVCD (IVCDmax) and IVCCI cut-offs for detecting elevated RAP (RAP ≥10 mmHg) were 17 mm and 40%, respectively. When we combined both in proportion to the guidelines, the sensitivity and specificity for detecting elevated RAP were 75% and 94%, respectively. When the cut-off values from the current guidelines (>21 mm and <50%) were applied, the respective sensitivity and specificity were 42% and 99%. Interestingly, the cut-off value of the optimal IVCDmax indexed by body surface area (11 mm/m2) was similar to previous Western population data. When we combined both cut-off values (11 mm/m2and 40%), the sensitivity and specificity were 75% and 95%, respectively. CONCLUSIONS: The optimal absolute IVCDmax and IVCCI cut-offs to detect elevated RAP were smaller than those in the current guidelines. Indexed IVCDmax may be an IVC parameter that can be used internationally.

Estrogen variation during the menstrual cycle does not influence left ventricular diastolic function and untwisting rate in premenopausal women.

BACKGROUND: Although a cardioprotective effect of estrogen has been suggested by experimental studies, clinical data on the influence of estrogen on left ventricular (LV) diastolic function are sparse. The LV untwisting rate obtained by 2D speckle tracking echocardiography (2D-STE) is correlated with the time constant of LV pressure decay (tau), and this correlation is independent of left atrial pressure. Therefore, we used conventional Doppler echocardiography and 2D-STE to investigate changes in LV diastolic function during a single menstrual cycle in premenopausal women. METHODS: Twenty healthy premenopausal woman (mean age, 28.1±2.7 years) were enrolled. Clinical and echocardiographic data were obtained during the follicular phase (F-phase) and luteal phase (L-phase) of a single menstrual cycle. We compared the clinical and echocardiographic data, and estrogen levels between the two phases. RESULTS: There were no significant differences in LV diastolic parameters derived from Doppler echocardiography (E/A, p=0.295; E/e', p=0.449, DcT, p=0.178) or 2D-STE (peak untwisting rate, p=0.892; time-to-peak untwisting, p=0.951) between the two phases of the menstrual cycle. However, there was a significant decrease in estrogen levels between the F- and L-phases (177±119pg/ml vs. 35±12pg/ml, p<0.0001). CONCLUSIONS: LV diastolic function in healthy premenopausal women did not significantly change during the menstrual cycle. Estrogen does not appear to have a significant acute effect on LV diastolic function in premenopausal woman.

Heart Failure Complicated by Alveolar Hemorrhage due to Vascular Collapse and Amyloid Deposits in Wild-Type Transthyretin Amyloidosis.

The main clinical manifestations of wild-type transthyretin (TTR)-related amyloidosis are progressive heart failure and neuropathy. There have been some reports on cerebral hemorrhage due to cerebral amyloid angiopathy in patients with TTR-related amyloidosis, but little is known about the vascular involvement in other organs. A 77-year-old woman experienced heart failure and was admitted for deteriorating heart failure status. Echocardiography showed diffuse hypokinesis of the left ventricle with biventricular wall thickness. On the 12th hospital day, the blood oxygen saturation level suddenly dropped and, despite oxygen supplementation and intensive care, the patient died. An autopsy revealed systemic deposition of amyloids which were immunolabeled by an anti-TTR antibody. Furthermore, gene-sequencing analysis showed no evidence of TTR gene mutations. The patient was diagnosed postmortem with wild-type TTR-related amyloidosis. Pathological findings revealed alveolar hemorrhage of the lung. Massive amyloid deposits were present in the vessels, and collapsed internal elastic laminae with lymphocyte infiltration were observed at the site of amyloid deposits in the bronchial artery, suggesting that deposits with inflammation might cause the collapse of the bronchial artery and lead to hemorrhage. In amyloidosis patients who suffer heart failure, there is the potential for vascular collapse caused by the accumulation of amyloid deposits with inflammation.

Relationship of Left Ventricular Diastolic Function to Obesity and Overweight in a Japanese Population With Preserved Left Ventricular Ejection Fraction.

BACKGROUND: Obesity has been found to be associated with future development of diastolic heart failure. Other evidence has indicated that the effect of obesity on left ventricular (LV) mass varies among ethnicities. However, there are few data on the relationship between body mass index (BMI) and LV diastolic dysfunction in the Japanese population. METHODS AND RESULTS: We performed echocardiography in 788 subjects without valvular disease or LV systolic dysfunction. They were divided into 3 groups by BMI: normal weight, overweight, and obese. We used multivariable linear regression analysis to assess the clinical variables associated with diastolic parameters, including BMI. We also assessed the risk of diastolic dysfunction associated with BMI using multivariable logistic models. Overweight and obese subjects had significantly worse LV diastolic function and greater LV mass than normal weight subjects. In the multivariable analysis, BMI was independently associated with diastolic parameters. Furthermore, after adjusting for clinical factors, the increased risks of diastolic dysfunction in overweight subjects (adjusted odds ratio: 2.02, 95% confidence interval 1.21-3.36) and obese subjects (4.85, 3.36-16.27) were greater than those previously observed in Western populations. CONCLUSIONS: The Japanese population might be more susceptible than Western subjects to the effect of BMI on LV diastolic function. Differences between ethnicities should be taken into consideration in strategies for the prevention of diastolic heart failure. (Circ J 2016; 80: 1951-1956).

Utility of Cystatin C for Estimating Glomerular Filtration Rate in Patients With Muscular Dystrophy.

Emerging concerns regarding heart failure, arrhythmia, and sudden death in patients with muscular dystrophy are of significant clinical importance. On the other hand, little attention has been paid to renal dysfunction because these patients have low serum creatinine levels. Serum cystatin C, unaffected by muscle quantity, is a potentially superior marker for estimating renal function. Here, we present cases with muscular dystrophy in which estimated glomerular filtration rate (GFR) by cystatin C (eGFRcys) provided good agreement with simultaneously measured GFR by inulin renal clearance (differences less than 20%). Sudden death with acute heart failure occurred in a patient with underlying renal dysfunction and elevated BNP. Neurologists and cardiologists should evaluate renal function using GFR with cystatin C in patients with muscular dystrophy.

Factors influencing left atrial volume in a population with preserved ejection fraction: Left ventricular diastolic dysfunction or clinical factors?

BACKGROUND: Increased left atrial volume (LAV) predicts a higher incidence of cardiovascular events and is widely recognized as a major surrogate marker of left ventricular (LV) diastolic dysfunction (DD). Although the pathophysiology of LA enlargement is probably multifactorial, few studies have examined comprehensively the clinical factors that lead to LA enlargement in the absence of valvular disease or LV systolic dysfunction. Therefore, we investigated associations between LAV and several clinical and echocardiographic parameters including DD. METHODS: We enrolled 557 subjects without significant valve disease or LV systolic dysfunction from the health check-up clinic retrospectively. We performed univariable and multivariable linear regression using lnLAV index as the dependent variable and the following independent variables: gender, age, smoking status, drinking habit, hypertension, diabetes, body mass index (BMI), LV ejection fraction, DD, LV mass index, hemoglobin, serum creatinine, serum total cholesterol, serum uric acid, serum sodium, and serum iron. RESULTS: In multivariable analysis, LAV index was independently associated with BMI, lower hemoglobin, and moderate and severe DD compared with normal diastolic function (p<0.001), but not with mild DD (p=0.70). CONCLUSIONS: LA enlargement was independently associated with moderate and severe DD, but not with mild DD. Furthermore, obesity and lower hemoglobin were associated with LAV independently of DD.