The efficacy and safety of sacubitril/valsartan in heart failure with reduced ejection fraction patients with hypotension.

Despite previous studies showing that patients with low systolic blood pressure (sBP) in heart failure with reduced ejection fraction (HFrEF) has a poor prognosis, it has few treatment options. This study aimed to investigate the efficacy and safety of sacubitril/valsartan (S/V) in HFrEF patients with hypotension. We included 43 consecutive HFrEF patients with sBP < 100 mmHg despite guideline-directed medical therapy for at least 3 months and who received S/V between September 2020 and July 2021. Patients admitted for acute heart failure were excluded and 29 patients were evaluated for safety endpoints. Furthermore, patients who performed non-pharmacological therapy or died within 1 month were excluded, finally, 25 patients were evaluated for efficacy endpoints. The mean initial S/V dose was 53.0 ± 20.5 mg/day and the mean dosage was increased to 84.0 ± 34.5 mg/day in 1 month. Serum N-terminal pro-B-type natriuretic peptide (NT-proBNP) values significantly decreased from 2200 [interquartile range (IQR): 1462-3666] pg/ml to 1409 (IQR: 964-2451) pg/ml. (p < 0.0001). No significant change in sBP occurred (pre-sBP: 93.2 ± 4.9 mmHg, post-sBP: 93.4 ± 9.6 mmHg, p = 0.91), and no patients discontinued the S/V due to symptomatic hypotension in 1 month after S/V initiation. S/V can be safely introduced in HFrEF patients with hypotension to reduce serum NT-proBNP values. Thus, S/V may be useful for the treatment of HFrEF patients with hypotension.

Glucose-dependent diuresis in relation to improvements in renal-tubular markers of sodium-glucose cotransporter-2 inhibitors in hospitalized heart failure patients with diabetes.

Clinical parameters with correlation to diuretic effects after initiation of sodium-glucose cotransporter-2 (SGLT2) inhibitors are unclear. We aimed to identify the factors associated with the diuretic effect observed following the initiation of SGLT2 inhibitors in patients with diabetes having an acute heart failure (HF). Fifty-six patients included were hospitalized for acute HF with diabetes and started on SGLT2 inhibitors. Changes in urine volume (ΔUV) and blood/urine laboratory parameters before and during the first 4 days of therapy were evaluated. Data were prospectively obtained under clinically stable conditions after initial HF treatment. UV increased following the initiation of SGLT2 inhibitors [UV at baseline (BL): 1383 ± 479 mL/day; ΔUV over 4 days: + 189 ± 358 mL/day]. Multivariate analysis revealed no association between BL-hemoglobin A1c or BL-estimated glomerular filtration rate and ΔUV. Conversely, higher BL-fasting plasma glucose (FPG) and higher BL-urine N-acetyl-ß-D-glucosaminidase (NAG) were associated with a higher ΔUV. ΔUV was inversely associated with ΔFPG and ΔNAG, and positively associated with Δurinary sodium excretion. Elevated FPG and NAG both improved over 4 days of treatment. In conclusion, the diuretic effect of SGLT2 inhibitors was glycemia-dependent, and was associated with a reduction in elevated renal-tubular markers in hospitalized HF complicated with diabetes.

Initiation of peritoneal dialysis in a patient with chronic renal failure associated with tetralogy of Fallot: a case report.

BACKGROUND: Tetralogy of Fallot is the most common cyanotic congenital heart disease. Patients with the condition have a high risk of developing chronic kidney disease. Treatment of kidney disease in patients with complex hemodynamics presents unique challenges. However, there are very few reports on the treatment of end-stage renal failure in patients with tetralogy of Fallot. CASE PRESENTATION: We present a rare case of peritoneal dialysis in a 47-year-old man with tetralogy of Fallot who had not undergone intracardiac repair. Peritoneal dialysis successfully removed fluids and solutes without adversely affecting the patient's hemodynamics. Our patient was managed with peritoneal dialysis for 5 years before he succumbed to sepsis secondary to digestive tract perforation. CONCLUSIONS: In this paper, we discuss the importance of monitoring acid-base balance, changes in cyanosis, and hyperviscosity syndrome during peritoneal dialysis in patients with tetralogy of Fallot. Lower leg edema and B-type natriuretic peptide level were useful monitoring parameters in this case. This case illustrates that with attention to the patient's unique requirements, peritoneal dialysis can provide successful renal replacement therapy without compromising hemodynamics in patients with tetralogy of Fallot.

Dilated cardiomyopathy with re-worsening left ventricular ejection fraction.

Re-worsening left ventricular ejection fraction (LVEF) is observed in some patients with dilated cardiomyopathy (DCM) despite initial improvements in LVEF. We analyzed cardiac outcomes and clinical variables associated with this re-worsening LVEF. A total of 180 newly diagnosed DCM patients who received only pharmacotherapy were enrolled. Echocardiography was performed after 6, 12, 24, and 36 months after initiation of pharmacotherapy. Patients were divided into three groups: (1) Improved: (n = 113, 63%), defined as those > 10% increase in LVEF after 12 months and no decrease (> 10%) between 12 and 36 months; (2) Re-worse: (n = 12, 7%), those with > 10% increase in LVEF after 12 months but with decrease (> 10%) between 12 and 36 months; and (3) Not-improved: (n = 55: 30%), those with no increase in LVEF (> 10%) after 12 months. Patients with re-worse group were older (P = 0.04) and had higher brain natriuretic peptide (BNP) levels after 12 months (P = 0.002) than those in the Improved group. Major cardiac events (sudden death, implantation of a ventricular assist device, and death due to heart failure,) were observed in 13 (7%) patients after 36 months of pharmacotherapy. Multivariate analysis revealed that the Re-worse group had a higher risk for cardiac events (hazard ratio 11.7, 95% confidence interval 1.9-90.7, P = 0.01) than the Improved group, but had a similar risk compared with the Not-improved group. Re-worsening LVEF was associated with poor cardiac outcomes in newly diagnosed DCM patients. Age and persistently high-BNP levels after improvement in LVEF were significantly associated with re-worsening LVEF.

Portal congestion and intestinal edema in hospitalized patients with heart failure.

An interaction between the intestine and cardiovascular disease has been suggested. We thought to clarify the association between intestinal conditions and clinical outcomes in patients with heart failure (HF). Hemodynamic parameters in intestinal vessels [superior mesenteric artery (SMA), inferior mesenteric artery (IMA), and portal vein (PV)] and average colon wall thickness (aCWT) from the ascending colon to sigmoid colon were evaluated in 224 hospitalized HF patients. Echocardiographic parameters and composite event rates (all-cause mortality, readmission for HF deterioration, major ventricular arrhythmias) were also examined. Higher PV congestion index (CI) and aCWT were observed in patients with New York Heart Association (NYHA) class III/IV. Higher PVCI [hazard ratio (HR) per + 1 standard deviation (SD) 1.50, p < 0.01] and aCWT (HR per + 1 SD 1.45, p < 0.01) were independently associated with higher composite event rates during the follow-up of 122 ± 68 days. None of SMA/IMA hemodynamic parameters were associated with NYHA class or composite event rates. Higher right ventricular end-diastolic dimension (38 ± 7 vs 34 ± 9 mm, p < 0.01) and lower tricuspid annual plane systolic excursion (15 ± 5 vs 19 ± 5 mm, p < 0.001) were observed in patients with higher PVCI (> 0.031 cm s) and aCWT (> 2.8 mm) relative to those in others. In conclusion, increased portal congestion and intestinal edema were associated with severe HF symptoms and poor outcomes in hospitalized HF patients, in addition to being associated with impaired right-sided cardiac function.

Early Safety and Efficacy of Sapien 3 20 mm Transcatheter Heart Valve Implantation in Small Japanese Body Size.

BACKGROUND: Transcatheter aortic valve implantation (TAVI) is effective in treating severe aortic stenosis in inoperable or high-risk surgical patients, however, the little is known about outcomes after Sapien 3 20 mm transcatheter heart valve (THV) implantation. The purpose of this study was to investigate the short term outcomes of Sapien 3 20 mm THV implantation in Japanese people with a small body size. METHODS: We retrospectively collected the hospital records of consecutive patients who underwent TAVI using the Sapien 3 20 mm THV between October 2016 and March 2017. Clinical and echocardiographic data from before and one week after TAVI were collected. RESULTS: Six Japanese patients (all female, mean age 89 ± 5 years, body surface area [BSA] 1.29 ± 0.16m2) received a Sapien 3 20 mm THV. All the procedures were feasible and successful, and the 30-day mortality rate was 0%. The functional class and the echocardiographic findings significantly improved (aortic valve area, 0.5 ± 0.1 cm2 to 0.8 ± 0.1cm2; mean pressure gradient, 55 ± 15 mmHg to 19 ± 7 mmHg; P = .043, respectively). However, the values of the indexed effective orifice area in all patients after Sapien 3 20 mm THV implantation were less than 0.85 cm2/m2, suggesting prosthesis-patient mismatch (PPM). CONCLUSIONS: The implantation of a Sapien 3 20 mm THV was safe and effective in high surgical risk elderly Japanese patients with a small body size. PPM after Sapien 3 20mm THV may be prevalent among Asians with small body sizes. Careful clinical follow-up will be necessary after Sapien 3 20 mm THV implantation.

Hemodilution after Initial Treatment in Patients with Acute Decompensated Heart Failure.

Decongestion is an important goal of heart failure (HF) management. Blood cell concentration is a recognized indicator for guiding decongestive treatment for HF. We aimed to assess the clinical impact of hemodilution and hemoconcentration after initial treatment in acute decompensated HF (ADHF) patients. We retrospectively evaluated hemoglobin levels and body weight obtained before admission, on admission, 3 days after admission, and at discharge in 102 consecutive patients admitted with ADHF. Patients were then stratified into hemodilution (n = 55) and hemoconcentration (n = 47) groups based on whether their hemoglobin levels decreased or increased, respectively, during the first 3 days after admission. From before admission to admission, hemoglobin levels decreased less in the hemodilution group (-0.16 ± 0.98 g/dL) than in the hemoconcentration group (-0.88 ± 1.11 g/dL) (P < 0.001); however, there was no significant difference in body weight (P≥ 0.05). More patients in the hemodilution group (85%) had grade III/IV pulmonary edema (Turner's criteria) compared with the hemoconcentration group (63%) (P < 0.01). Rate of readmission for HF within 180 days of discharge was higher in the hemodilution group (34%) compared with the hemoconcentration group (9%) (P < 0.01). Hemodilution after initial treatment for ADHF was associated with severe pulmonary edema at admission and higher readmission rates.

Importance of Early Diagnosis of Cardiac Sarcoidosis in Patients with Complete Atrioventricular Block.

Our aim is to clarify the factors for early diagnosis of cardiac sarcoidosis (CS) in patients with complete atrioventricular block (CAVB) and its impact on cardiac function after corticosteroid therapy.A total of 15 CS patients with CAVB who underwent corticosteroid therapy were retrospectively analyzed. Patients were divided into two groups according to the time from the first CAVB onset to the diagnosis of CS. Clinical characteristics and outcomes were compared between the early diagnosis group (within 1 year; group E, n = 10) and the late diagnosis group (over 1 year; group L, n = 5).The history of extracardiac sarcoidosis (60 versus 0%, P = 0.0440) and abnormal findings on echocardiography (70 versus 0%, P = 0.0256) at the CAVB onset were significantly more frequent in group E than in group L. The change of left ventricular ejection fraction (LVEF) and brain natriuretic peptide (BNP) levels was significantly better in group E than in group L (0.8 ± 2.8 versus -32.4 ± 3.9%, P < 0.0001; -11.1 ± 16.0 versus 161.8 ± 35.8 pg/mL, P = 0.0013, respectively). After corticosteroid therapy, the LVEF and BNP levels were also significantly better in group E than in group L (53.3 ± 10.7 versus 37.0 ± 9.3%, P = 0.0128; 63.0 ± 46.4 versus 458.8 ± 352.0 pg/mL, P = 0.0027).The diagnosis may be delayed in CS patients with CAVB without history of extracardiac sarcoidosis. Abnormal findings on echocardiography contributed to the early diagnosis of CS. Therefore, the diagnosis of CS may be missed or delayed in patients without them. Time delay from the CAVB onset to the CS diagnosis may exacerbate the cardiac function.

Tafamidis for the Treatment of Hereditary Transthyretin Amyloid Cardiomyopathy: A Case Report.

Tafamidis meglumine is a novel medicine that has been shown to slow the progression of peripheral neurological impairment in patients with hereditary transthyretin amyloidosis (ATTR). However, the efficacy of tafamidis against ATTR-related cardiac amyloidosis remains unclear. A 72-year-old woman had cardiac hypertrophy and axonopathy in her lower legs. Endomyocardial biopsy revealed an infiltrative cardiomyopathy consistent with amyloidosis. Immunostaining and genetic studies confirmed the diagnosis of ATTR, and tafamidis was started subsequently. Two years after the initiation of tafamidis treatment, electromyography demonstrated no change in the axonopathy in her lower legs; however, electrocardiography displayed QRS prolongation, and echocardiography disclosed an increase in interventricular septal thickness. Endomyocardial biopsy indicated that transthyretin amyloid infiltration of the myocardium was not reduced. In this case, there was no apparent progression of axonopathy, although there were signs of worsening amyloid cardiomyopathy during the treatment with tafamidis.

Higher hemoglobin A1c levels are associated with impaired left ventricular diastolic function and higher incidence of adverse cardiac events in patients with nonischemic dilated cardiomyopathy.

We aimed to elucidate the relationship between glycated hemoglobin (HbA1c), cardiac systolic/diastolic function, and heart failure (HF) prognosis during guideline-directed medical therapy in patients with nonischemic dilated cardiomyopathy (NIDCM). We evaluated 283 hospitalized NIDCM patients, who were grouped according to baseline (BL) and 1-year (1Y) levels of HbA1c (<6.0, 6.0-6.9, and ≥7.0 %). The primary endpoint was defined as either readmission for HF worsening or cardiac death. Approximately half of the patients had BL- or 1Y-HbA1c ≥6.0 % (31 % at BL, 34 % at 1Y had 6.0-6.9 %; 12 % at BL, 12 % at 1Y had ≥7.0 %). The absolute value of left ventricular ejection fraction (LVEF) and its improvement during 1 year showed no significant difference among the 1Y-HbA1c groups (p = 0.273), whereas a lower absolute value and a more significant reduction in the early diastolic velocity of the mitral annulus (E a) were seen in the group with 1Y-HbA1c ≥7.0 % (both p < 0.001). In multiple regression analysis, higher 1Y-plasma B-type natriuretic peptide and lower 1Y-Ea were independently associated with higher 1Y-HbA1c (both adjusted p < 0.05). The cumulative incidence of the primary endpoint was highest in the group with 1Y-HbA1c ≥7.0 % (log-rank p = 0.001). Multivariate analysis demonstrated that higher 1Y-HbA1c was independently associated with a higher incidence of the primary endpoint (adjusted p = 0.005). In conclusion, hyperglycemia during clinical follow-up is a risk factor for progression of concomitant LV abnormal relaxation, leading to poor HF prognosis in patients with NIDCM.

Prognostic Impact of Segmental Wall Motion Abnormality in Patients With Idiopathic Dilated Cardiomyopathy.

The clinical impact of left ventricular (LV) segmental wall motion abnormalities (SWMA) in patients with idiopathic dilated cardiomyopathy (IDCM) has not been well elucidated.Among 100 consecutive IDCM patients with follow-up visits, we enrolled 85 after excluding those with left bundle branch block and/or ventricular pacemaker implantation. LV wall motion was assessed using left ventriculography scored for 7 segments according to the American Heart Association classification as follows: 0, normokinesis; 1, hypokinesis; 2, akinesis; and 3, dyskinesis. SWMA were defined as a score dispersion of more than 1 degree among the segments.SWMA was exhibited by 26 patients. Kaplan-Meier curves demonstrated that the patients with SWMA (SWMA+) had a significantly higher cardiac event-free rate than the patients without SWMA (P < 0.001). Cox proportional hazards analysis showed that SWMA+ was an independent predictor of cardiac events (P = 0.03; hazard ratio = 3.38; 95% confidence interval [CI], 1.11-10.8). Furthermore, multiple regression analysis showed that SWMA+ was an independent predictor of decreased LV end-systolic dimension index after optimal pharmacotherapy (ß = -0.24; 95%CI, -9.12 to -0.73; P = 0.02).SWMA is common in patients with IDCM and is independently associated with a poor prognosis and less morphometric and functional improvement of LV in response to pharmacotherapy.

Prognostic significance of beta-blocker up-titration in conjunction with cardiac resynchronization therapy in heart failure management.

Clinical practice guidelines emphasize that optimal pharmacotherapy, including beta-blockers (BB), is a prerequisite before receiving cardiac resynchronization therapy (CRT) in eligible patients with heart failure (HF). However, the optimal dose of BB before CRT implantation cannot be tolerated in a number of patients. Sixty-three consecutive patients who underwent CRT in 2006-2013 were retrospectively investigated. Before receiving CRT, BB could not be introduced in 20 patients (32 %); the daily carvedilol-equivalent dose in other 43 patients was 5.6 ± 7.0 mg because of significant HF and bradycardia. After receiving CRT, BB could be introduced in almost all patients (n = 61, 97 %), and the daily BB dose increased from 5.6 ± 7.0 to 13.2 ± 7.8 mg (P < 0.001). Multivariate analysis indicated that the change of BB dose after CRT was independently associated with improved left ventricular end-systolic volume (LVESV) [ß = -0.36; 95 % confidence interval (CI) -2.13 to -0.45; P < 0.01] after 6-months follow-up. Furthermore, Cox proportional hazard analysis also showed that the change in the BB dose (hazard ratio, 0.92; 95 % CI, 0.87-0.98; P < 0.01) as well as the New York Heart Association functional classification was an independent predictor of cardiac events. After initiating CRT, BB therapy can be introduced and up-titrated in intolerant HF patients. The up-titrated dose of BB after CRT was an independent predictor for the improvement of LVESV and HF prognosis.

Cardiac fibrosis detected by magnetic resonance imaging on predicting time course diversity of left ventricular reverse remodeling in patients with idiopathic dilated cardiomyopathy.

This study aimed to identify the association between the time course of left ventricular reverse remodeling (LVRR) and late gadolinium enhancement in cardiac magnetic resonance imaging (LGE-cMRI) in patients with idiopathic dilated cardiomyopathy (IDCM). We identified 214 IDCM patients treated by optimal pharmacotherapies. LVRR was defined as ≥10 % increment in LV ejection fraction along with ≥10 % reduction in LV end-diastolic dimension. Findings of LGE-cMRI focusing on presence and extent of LGE were evaluated at baseline. Echocardiographic evaluation for detecting LVRR was performed in all patients for 3 years. The primary endpoint was defined as composite events (CEs) including readmission for heart failure, detection of major ventricular arrhythmia, and all-cause mortality. LVRR was found at <1 year in 59 patients (28 %, early responder), ≥1 year in 56 patients (26 %, late responder), and was absent in 99 patients (46 %, non-responder). Multivariate Cox-proportional hazards analysis revealed that both early responders (P = 0.02) and late responders (P < 0.001) had lower incidence of CEs than non-responders. Among 66 subjects (23 %) with complete cMRI evaluation, LGE was detected more often in late and non- than early responders (65, 83 vs. 23 % P < 0.001, respectively), whereas the LGE area was smaller in both early and late than non-responders (2 ± 3, 4 ± 3 vs. 12 ± 10 %, P < 0.001, respectively). In conclusion, evaluating the presence and the extent of LGE is useful for predicting the clinical differences of LVRR time course and subsequent long-term outcomes.

Clinical significance of endomyocardial biopsy in conjunction with cardiac magnetic resonance imaging to predict left ventricular reverse remodeling in idiopathic dilated cardiomyopathy.

Endomyocardial biopsy (EMB) and cardiac magnetic resonance (CMR) are useful modalities to study the characteristics of myocardial tissue. However, the prognostic impact of both diagnostic tools to predict subsequent left ventricular reverse remodeling (LVRR) has not been well elucidated. A total of 187 consecutive patients with idiopathic dilated cardiomyopathy (IDCM) who were treated by optimal pharmacotherapy (OPT) and underwent EMB of the LV wall were investigated. The myocardial specimens were semiquantitatively evaluated measuring cardiomyocyte degeneration (CD), interstitial fibrosis (IF), and hypertrophy. In addition, late gadolinium enhancement (LGE)-CMR was performed in 78 (48 %) patients. Seventy-eight (48 %) patients developed LVRR, defined as a ≥10 % increase in LV ejection fraction with a ≥10 % decrease in indexed LV end-diastolic dimension at 12 months after OPT. Multivariate regression analysis revealed that CD (P = 0.003), but not IF (P = 0.320), was an independent predictor of LVRR. In the patients with not only EMB but also CMR, the CD score and LGE area were independent predictors of LVRR (odds ratios/P values 0.268/0.010, 0.855/<0.001, respectively). The patients with mild CD and negative LGE had a better achievement rate of LVRR than those with severe CD and positive LGE (74 vs. 19 %). A combination of CD score on EMB and LGE-CMR is useful to predict subsequent LVRR in IDCM patients.

Time course of left ventricular reverse remodeling in response to pharmacotherapy: clinical implication for heart failure prognosis in patients with idiopathic dilated cardiomyopathy.

The present study aimed to identify the clinical significance of differences in detection timings of left ventricular reverse remodeling (LVRR) on heart failure (HF) prognosis in patients with idiopathic dilated cardiomyopathy (IDCM). We investigated 207 patients with IDCM who underwent pharmacotherapeutic treatment. LVRR was defined as improvements in both LV ejection fraction ≥10 % and indexed LV end-diastolic dimension (LVEDDi) ≥10 %. Patients were stratified into 3 groups by LVRR timing: patients with LVRR <24 months (Early LVRR), those with LVRR ≥24 months (Delayed LVRR), and those without LVRR during the entire follow-up period (No LVRR). The major endpoint was first detection of composite event including readmission for decompensated HF, major ventricular arrhythmias, or all-cause mortality. LVRR was recognized in 108 patients (52 %): Early LVRR in 83 (40 %), Delayed LVRR in 25 (12 %), and No LVRR in 99 (48 %). The survival rate for the major endpoint was significantly higher for Delayed LVRR than for No LVRR (P = 0.001); there was no significant difference between Early and Delayed LVRR. Among patients without LVRR <24 months (Delayed + No LVRR), receiver operating characteristic curve analysis showed that the area under the curve for improvement in LVEDDi during the first 6 months for predicting subsequent LVRR (Delayed LVRR) [0.822 (95 % confidence interval, 0.740-0.916; P = 0.038)] was greater than that for improvement in LVEF. In conclusion, LVRR was a favorable prognostic indicator in patients with IDCM irrespective of its detection timing. Reduced LVEDDi during the first 6 months was predictive for subsequent LVRR in the later phase.

Clinical significance of heart rate during acute decompensated heart failure to predict left ventricular reverse remodeling and prognosis in response to therapies in nonischemic dilated cardiomyopathy.

Although an increased heart rate (HR) is a strong predictor of poor prognosis in cases of chronic heart failure (HF), the clinical value of HR as a predictor in acute decompensated HF (ADHF) is unclear. Seventy-eight patients with nonischemic dilated cardiomyopathy (NIDCM) with sinus rhythm who were first hospitalized for ADHF from 2002 to 2010 were retrospectively investigated after exclusion of patients with tachycardia-induced cardiomyopathy. The patients were divided into two groups stratified by HR on admission with a median value of 113 beats/min (Group H with HR ≥ 113 beats/min; Group L with HR < 113 beats/min). Despite similar backgrounds, including pharmacotherapy for HF, HR changes responding to titration of ß-blocker (BB) therapy and myocardial interstitial fibrosis, left ventricular (LV) ejection fractions improved more significantly 1 year later in Group H than in Group L (57 % ± 11 % vs. 46 % ± 12 %, P < 0.001). Cardiac event-free survival rates were also significantly improved in Group H (P = 0.038). Multiple regression analysis revealed that only the peak HR on admission was an independent predictor of LV reverse remodeling (LVRR) 1 year later (ß = 0.396, P = 0.005). High HR on first admission for ADHF is a strong predictor of LVRR, with a better prognosis in the event of NIDCM in response to optimal pharmacotherapy, independent of pre-existing myocardial damage and subsequent HR reduction by BB therapy.

Baseline cardiac magnetic resonance imaging versus baseline endomyocardial biopsy for the prediction of left ventricular reverse remodeling and prognosis in response to therapy in patients with idiopathic dilated cardiomyopathy.

Endomyocardial biopsy (EMB) and late gadolinium enhancement cardiovascular magnetic resonance (LGE-CMR) imaging performed at baseline are both used to evaluate the extent of myocardial fibrosis. However, no study has directly compared the effectiveness of these diagnostic tools in the prediction of left ventricular reverse remodeling (LVRR) and prognosis in response to therapy in patients with idiopathic dilated cardiomyopathy (IDCM). Seventy-five patients with newly diagnosed IDCM who were undergoing optimal therapy were assessed at baseline using LGE-CMR imaging and EMB; the former measured LGE area and the latter measured collagen volume fraction (CVF) as possible predictive indices of LVRR and cardiac event-free survival. Among all the baseline primary candidate factors with P < 0.2 as per univariate analysis, multivariate analysis indicated that only LGE area was an independent predictor of subsequent LVRR (ß = 0.44; 95 % confidence interval (CI) 0.87-2.53; P < 0.001), as indicated by decreasing left ventricular end-systolic volume index over the 1-year follow-up. Kaplan-Meier curves indicated significantly lower cardiac event-free survival rates in patients with LGE at baseline than in patients without (P < 0.01). By contrast, there was no significant difference in prognosis between patients with CVF values above (severe fibrosis) and below (mild fibrosis) the median of 4.9 %. Cox proportional hazard analysis showed that LGE area was an independent predictor of subsequent cardiac events (hazard ratio 1.06; 95 % CI 1.02-1.10; P ≤ 0.01). The degree of myocardial fibrosis estimated by baseline LGE-CMR imaging, but not that estimated by baseline EMB, can predict LVRR and cardiac event-free survival in response to therapy in patients with newly diagnosed IDCM.

Atrial septal defect becoming clinically evident after cardiac surgery for annuloaortic ectasia: a case report.

Background: Right ventricular volume overload is the key finding in a patient with previously undiagnosed atrial septal defect (ASD). Case summary: A 68-year-old female was referred to our hospital due to progressive pulmonary artery dilatation observed on her chest X-ray. Echocardiography revealed a secundum ASD with right ventricular dilatation. She had undergone aortic root replacement and aortic valve replacement for annuloaortic ectasia and aortic insufficiency 12 years prior to the diagnosis. She was also diagnosed with Marfan syndrome, which was supported by family histories. Computed tomography did not show a secundum ASD before the surgery. We finally closed the secundum ASD with catheter closure device. Discussion: Ascending aneurysm might mask the presence of secundum ASD. Monitoring the change in pulmonary artery dilatation overtime is useful for the diagnosing secundum ASD.

Cardiac sarcoidosis presenting with cardiogenic shock successfully recovered by Impella and corticosteroid pulse therapy: a case report.

Background: Cardiac sarcoidosis (CS) occasionally disrupts circulatory haemodynamics due to arrhythmia or cardiac dysfunction. Case summary: A 70-year-old woman was diagnosed with CS, then was admitted for syncope because of complete atrioventricular block and frequently non-sustained ventricular tachycardia. Although a temporary pacemaker and intravenous amiodarone were introduced, she subsequently went into cardiopulmonary arrest by ventricular fibrillation. After the return of spontaneous circulation, Impella cardiac power (CP) was introduced because of sustained hypotension and severely impaired left ventricular contraction. High-dose intravenous corticosteroid therapy was simultaneously introduced. Her atrioventricular conduction and left ventricular contraction drastically improved. Impella CP was successfully removed after 4 days of support. She was eventually administered steroid maintenance therapy and discharged. Discussion: We report a case of CS with fulminant haemodynamic collapse treated with high-dose intravenous corticosteroid therapy under Impella assistance for acute haemodynamic support. Although CS has been known as an inflammatory disease with progressive cardiac dysfunction and rapid deterioration due to fatal arrhythmias, it can be improved with steroid therapy. It was suggested that strong haemodynamic support by Impella could be a bridge to manifest the effects after introducing steroid therapy to patients with CS.

Cardiac sarcoidosis accompanied by a tumor-like blush on coronary angiography.

A 71-year-old female was diagnosed with cardiac sarcoidosis by a history of complete atrioventricular block requiring pacemaker implantation and a focal thinning of basal interventricular septum (IVS) on echocardiography. Coronary angiography showed a tumor-like blush fed by septal branch of left anterior descending artery and right coronary artery concomitant with a coronary-to-right ventricle shunt. Echocardiography showed a low echoic mass at mid-IVS with an abnormal shunt flow into right ventricle. Fluorodeoxyglucose (FDG)-positron emission tomography (PET) showed an uptake at the corresponding site of mid-IVS. Eight months after the initiation of steroid therapy, FDG-PET showed no abnormal uptake at mid-IVS, suggesting decreased activity of cardiac sarcoidosis. We experienced a case of cardiac sarcoidosis with FDG uptake accompanied by a tumor-like blush at mid-IVS. The present case suggests the involvement of microvascular accumulation in the activity of cardiac sarcoidosis. .