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Abstract Lymphocytic myocarditis (LM) is primarily triggered by various factors including viral infections and subsequent immune responses. While rare, some patients with LM experience recurrence with a life‐threatening fulminant form. Although combining steroids and immunosuppressants, such as azathioprine and mycophenolate mofetil, has demonstrated favourable outcomes in patients with LM, their efficacy is limited to the chronic phase. Indeed, various immunosuppressants have been used for LM with fulminant manifestation; however, their evidence remains lacking. In our case series, two patients with LM experienced fulminant relapses during steroid tapering, and another presented persistent cardiac enzymes elevation despite steroid therapies. Consequently, we initiated calcineurin inhibitors alongside steroids, resulting in well‐controlled clinical courses without further recurrence of LM and significant adverse effects. Our cases suggest calcineurin inhibitors as therapeutic options for managing steroid‐resistant LM with fulminant relapse.

Backgrounds: Remote cardiac rehabilitation has proven useful in patients with cardiovascular disease; however, the methodology had not been fully validated. This study aimed to investigate the efficacy and safety of remote cardiac rehabilitation (RCR) with real-time monitoring and an ergometer using a bidirectional communication tool during the recovery phase of cardiovascular diseases. Methods: This multicenter, nonrandomized, interventional study was conducted at 29 institutions across Japan and enrolled patients with cardiovascular diseases who met indications for cardiac rehabilitation (CR) after receiving in-hospital treatment. The RCR group exercised at home using an ergometer and was monitored in real-time using interactive video and monitoring tools for 2–3 months. Educational instructions were provided concurrently through e-learning approaches. The safety of the RCR protocol and the improvement in peak oxygen consumption (VO2) were compared with those of the historical control group that participated in center-based CR. Results: Fifty-three patients from the RCR group were compared with 103 historical controls having similar background characteristics. No patients in RCR experienced significant cardiovascular complications while engaging in exercise sessions. After 2–3 months of RCR, the peak VO2 improved significantly, and the increases in the RCR group did not exhibit any significant differences compared to those in the historical controls. During follow-up, the proportion of patients whose exercise capacity increased by 10% or more was also evaluated; this finding did not indicate a statistically significant distinction between the groups. Conclusions: RCR during the recovery phase of cardiovascular diseases proved equally efficient and safe as center-based CR.

Background: IgG4-related disease (IgG4-RD) is a systemic disease characterized by serum IgG4 upregulation, massive infiltration of IgG4-positive plasma cells, and storiform fibrosis, which results in nodules or thickening of the involved organs. Cardiologists have recently recognized that IgG4-RD can be complicated by coronary artery events (CAEs); however, the mechanisms and clinical characteristics of this phenomenon are unknown. We evaluated the clinical signs of patients with coronary periarteritis (CP), aortic periarteritis (AP), and pericardial thickening, which are complications of IgG4-RD, to determine the contributing factors. Methods: We retrospectively examined 19 patients with IgG4-RD who attended or consulted a cardiologist in our department at the University of Tokyo Hospital between January 1, 2004, and December 31, 2021. Results: The frequency of CAEs was significantly higher in the CP group than in the non-CP group. Furthermore, the CP group had significantly lower event-free survival than the non-CP group (log-rank test, P = 0.008). However, the frequency of incidents and event-free survival for CAEs after IgG4-RD diagnosis did not differ significantly between the AP and non-AP groups. Although no statistically significant difference was present between the frequency of CAEs for those with vs without pericardial thickening, the group with pericardial thickening had significantly worse event-free survival than the group without pericardial thickening (log-rank test, P = 0.017). Conclusions: The incidence and clinical course of CAEs complicated by IgG4-RD could be predicted by identifying CP and pericardial thickening in IgG4-RD but not AP. Résumé: Contexte: La maladie liée aux immunoglobulines de type G4 (ML-IgG4) est une maladie généralisée caractérisée par une augmentation du taux sérique d’IgG4, par une infiltration massive de plasmocytes exprimant les IgG4 et par une fibrose storiforme, qui produit des nodules ou un épaississement des organes touchés. Les cardiologues ont récemment reconnu que la ML-IgG4 peut être compliquée par des événements coronariens; les mécanismes et caractéristiques cliniques de ce phénomène demeurent cependant inconnus. Nous avons évalué les signes cliniques chez des patients atteints de périartérite coronarienne (PC), de périaortite (PA) et d’épaississement du péricarde, des complications de la ML-IgG4, pour tenter d’établir les facteurs contributifs. Méthodologie: Nous avons examiné de manière rétrospective les dossiers de 19 patients atteints de ML-IgG4 qui ont été admis à notre service de l’Hôpital de l’Université de Tokyo ou qui ont consulté un cardiologue du service entre le 1er janvier 2004 et le 31 décembre 2021. Résultats: La fréquence des événements coronariens était significativement plus élevée dans le groupe PC que dans les autres groupes. Par ailleurs, le groupe PC avait une survie sans événement significativement plus courte que les autres groupes (test logarithmique par rangs; p = 0,008). En outre, la fréquence des événements coronariens et la survie sans événement coronarien après un diagnostic de ML-IgG4 ne variaient pas de manière significative entre le groupe PA et les autres groupes. Bien qu’aucune différence statistiquement significative n’ait été constatée quant à la fréquence des événements coronariens entre les patients présentant un épaississement du péricarde et les autres patients, le premier groupe affichait une survie sans événement significativement plus courte que l’autre (test logarithmique par rangs; p = 0,017). Conclusions: L’incidence et le déroulement clinique des événements coronariens compliqués par la ML-IgG4 pouvaient être anticipés dans les cas de ML-IgG4 en présence de PC et d’un épaississement du péricarde, mais pas de PA.

Abstract Aims Anti‐mitochondrial antibody (AMA)‐positive myositis is frequently associated with various cardiac involvements, such as arrhythmia and left ventricular (LV) dysfunction. However, the efficacy of immunosuppressive therapy in these complications remains unknown. This study aimed to investigate the cardiac response to immunosuppressive therapy in patients with AMA‐positive myositis. Methods and results The clinical data of 15 AMA‐positive myositis patients with cardiac involvement were retrospectively collected at our centre. To evaluate the effects of immunosuppressive therapy, echocardiographic and laboratory data of patients who received glucocorticoid therapy with additional immunosuppressants (n = 6) and those who did not (n = 6) were compared. Also, the characteristics of patients with or without >5% LV ejection fraction (LVEF) decline during the follow‐up period (n = 5 vs. n = 7) were compared. Thirteen patients (87%) had arrhythmias, and eight patients (53%) had LV wall motion abnormalities. Although arrhythmias decreased after treatment, reduced LVEF and LV wall motion abnormalities persisted. Further investigation revealed an increased LV end‐systolic dimension and reduced LVEF in patients without additional immunosuppressive therapy, while those in patients with additional immunosuppressive therapy were maintained. Six of seven patients (86%) without LVEF decline received additional immunosuppressive therapy, whereas no patients with LVEF decline had additional immunosuppressive therapy. Conclusions Cardiac involvement in AMA‐positive myositis may worsen even with glucocorticoid monotherapy, and there might be some associations between the change of LV function and additional immunosuppressive therapy.

Abstract Diastolic stiffness coefficient (β) and end‐diastolic elastance (Eed) are ventricular‐specific diastolic parameters. However, the diastolic function of right ventricle had not been investigated sufficiently due to the lack of established evaluation method. We evaluated the validity of these parameters calculated using only data of right heart catheterization (RHC) and assessed it in patients with restrictive cardiomyopathy (RCM) and cardiac amyloidosis. We retrospectively analyzed 46 patients with heart failure who underwent RHC within 10 days of cardiac magnetic resonance (CMR). Right ventricular end‐diastolic volume and end‐systolic volume were calculated using only RHC data, which were found to be finely correlated with those obtained from CMR. β and Eed calculated by this method were also significantly correlated with those derived from conventional method using CMR. By this method, β and Eed were significantly higher in RCM with amyloidosis group than dilated cardiomyopathy group. In addition, the β and Eed calculated by our method were finely correlated with E/A ratio on echocardiography. We established an easy method to estimate β and Eed of right ventricle from only RHC. The method finely demonstrated right ventricular diastolic dysfunction in patients with RCM and amyloidosis.

The co-occurrence of dilated cardiomyopathy (DCM) and aortic dissection has been rarely reported. Here, we present the case of a patient with co-occurrence of DCM and aortic dissection, wherein multivessel coronary artery dissection eventually occurred, thereby leading to advanced heart failure. She suffered from co-occurrence of DCM and aortic dissection 6 years ago. After the heart failure had briefly stabilized, the myocardial infarction due to coronary artery dissection led to worsening mitral regurgitation and decreased right ventricular function, thereby worsening the status of her heart failure. In addition to cardiovascular abnormalities, the patient was also complicated by short stature (145 cm), mild scoliosis, nonfunctioning pituitary adenoma of 1 cm in size, and retinitis pigmentosa. Coronary artery dissection is a possible complication in patients with co-occurrence of DCM and aortopathy, which could dramatically affect the clinical course of advanced heart failure.

Abstract Aims The removal of left ventricular assist device (LVAD) after myocardial recovery can provide survival benefits with freedom from LVAD‐associated complications. However, in the absence of standardization, the weaning evaluation and surgical strategy differ widely among centres. Therefore, we analysed the experiences of LVAD explantation with our protocol in dilated cardiomyopathy (DCM) patients and investigated the validity of our weaning evaluation and surgical strategy from the perspective of optimal long‐term survival. Methods and results All LVAD explantation patients in our institution between May 2012 and May 2020 were enrolled. All patients were evaluated by our three‐phase weaning assessment: (i) clinical stability with improved cardiac function under LVAD support; (ii) haemodynamic stability shown by ramp‐loading and saline‐loading test; (iii) intraoperative pump‐off test. Explant surgery involved removal of the whole system including driveline, pump, sewing ring and outflow‐graft, and closure of an apical hole. Intra‐operative, peri‐operative, and post‐operative outcomes, including all‐cause mortality and LVAD associated major complications, were retrospectively analysed. A total of 12 DCM patients (DuraHeart, n = 2; EVAHEART, n = 2; HeartMate II, n = 6; HeartMate 3, n = 2) had myocardial recovery after a median 10 months [interquartile range (IQR); 6.3–15 months] support and qualified for our LVAD explantation study protocol [median age: 37 y, IQR; 34–41 years; 83% men]. The median left ventricular ejection fraction was 20% (IQR; 12–23%) at LVAD‐implantation and 54% (IQR: 45–55%) before LVAD explantation (P

Abstract Pulmonary arterial hypertension (PAH) is a progressively life‐threatening disease that causes right heart failure (RHF). Renal dysfunction frequently complicates PAH with RHF and is associated with a worse prognosis. Renal replacement therapy (RRT) may be a therapeutic option, although its efficacy and safety are unclear. We describe a 30‐year‐old male with severe PAH who developed renal insufficiency and diuretic‐refractory volume overload complicated with RHF but was successfully managed with intermittent RRT via a subcutaneously fixed superficial artery for 4 years. RRT led to haemodynamic stability, which enabled us to carefully de‐titrate parenteral PAH drugs without worsening RHF. This case highlights that RRT may be a potential alternative for haemodynamic and volume control of refractory fluid retention complicated with RHF in severe PAH cases. Further studies are warranted to gain more insight into patient selection and the optimal timing of RRT in PAH patients with deteriorating RHF.

Cardiac fibrosis is a hallmark of heart failure. Here the authors use single-cell RNA-sequencing, spatial transcriptomics, and genetic manipulations, to show that Htra3 regulates cardiac fibrosis by keeping fibroblasts quiescent and by degrading TGF-beta.

Abstract Aims Heart transplantation (HT) is an effective therapeutic option for end‐stage heart failure. Infection is a major cause of morbidity and mortality after HT. Sarcopenia, defined as the loss of muscle mass and strength, is a common comorbidity in HT candidates with end‐stage heart failure. However, the effects of sarcopenia on the occurrence of post‐HT infections are not well understood. Therefore, we explored the association between the skeletal muscle mass and post‐transplant infections in adult HT recipients. Methods and results We retrospectively examined the records of 135 patients who underwent HT between August 2007 and November 2019 at our institution. Pre‐transplant computed tomography was used to calculate the skeletal muscle index (SMI) at the level of the third lumbar vertebra. Muscle wasting was defined as the SMI of the lowest sex‐based tertiles. The primary endpoint was infections within 6 months of HT. The study included 109 patients (80 men, mean age: 41.6 ± 12.0 years): 37 patients in the muscle wasting group and 72 patients in the non‐muscle wasting group. The mean SMI values in the muscle wasting and non‐muscle wasting groups were 29.9 ± 4.8 cm2/m2 and 40.7 ± 6.7 cm2/m2, respectively. Prior to HT, 108 (99.1%) patients were on left ventricular assist device support, and during that support, the rate of late right heart failure was significantly higher in the muscle wasting group than non‐muscle wasting group (P = 0.012). Sixteen infections occurred within 6 months of HT. The most common infection sites included the respiratory tract (n = 5) and the upper gastrointestinal tract (n = 5), followed by the urinary tract (n = 4). Overall, 10 patients experienced infections in the muscle wasting group (27.0%) and 6 in the non‐muscle wasting group (8.3%) (P = 0.009). Two patients in the muscle wasting group required intensive care unit admission, compared to none in the non‐muscle wasting group. Low skeletal muscle mass was associated with infections in the univariate and multivariate logistic regression models (hazard ratio: 3.68, 95% confidence interval: 1.19–11.3; P = 0.023). However, the duration of all‐cause mortality within 3 years did not differ between the groups (P = 0.56). Conclusions Low skeletal muscle mass is a predictor of post‐HT infections within 6 months of HT.

Abstract 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 −8.6%) had significantly higher hazard ratio than those with either impairment alone (11.3 vs. 3.4, P

AimsWe compared hemodynamics and clinical events after heart transplantation (HTx) in patients stratified by the severity of residual pulmonary vascular resistance (PVR) after left ventricular assist device (LVAD) implantation for bridge to transplantation.MethodsWe retrospectively analyzed patients who had undergone HTx at the University of Tokyo Hospital. We defined the high PVR group as patients with PVR of >3 Wood Units (WU) as measured by right heart catheterization performed 1 month after LVAD implantation.ResultsWe included 85 consecutive HTx recipients, 20 of whom were classified in the high PVR group and 65 in the low PVR group. The difference in PVR between the two groups became apparent at 2 years after HTx (the high PVR group: 1.77 ± 0.41 WU, the low PVR group: 1.24 ± 0.59 WU, p = 0.0009). The differences in mean pulmonary artery pressure (mPAP), mean right arterial pressure (mRAP), and mean pulmonary capillary wedge pressure (mPCWP) tended to increase from the first year after HTx, and were all significantly higher in the high PVR group at 3 years after HTx (mPAP: 22.7 ± 9.0 mm Hg vs. 15.4 ± 4.3 mm Hg, p = 0.0009, mRAP: 7.2 ± 3.6 mm Hg vs. 4.1 ± 2.1 mm Hg, p = 0.0042, and mPCWP: 13.4 ± 4.5 mm Hg, 8.8 ± 3.3 mm Hg, p = 0.0040). In addition, pulmonary artery pulsatility index was significantly lower in the high PVR group than in the low PVR group at 3 years after HTx (2.51 ± 1.00 vs. 5.21 ± 3.23, p = 0.0033). The composite event including hospitalization for heart failure, diuretic use, and elevated intracardiac pressure (mRAP ≥ 12 mm Hg or mPCWP ≥ 18 mm Hg) between the two groups was significantly more common in the high PVR group. Residual high PVR was still an important predictor (hazard ratio 6.5, 95% confidence interval 2.0–21.6, and p = 0.0023) after multivariate Cox regression analysis.ConclusionOur study demonstrates that patients with residual high PVR under LVAD implantation showed the increase of right and left atrial pressure in the chronic phase after HTx.

Background: There are some patients with advanced heart failure (HF), for whom implantable left ventricular assist device (LVAD) or heart transplantation (HTx) should be considered. Some of them need to be transferred between hospitals. There are few reports on the interhospital transfer of patients with advanced HF and their subsequent clinical course.In this study, we investigated the characteristics and clinical course of patients transferred to a LVAD/HTx center, focusing on the distance between hospitals. Methods: We retrospectively examined 141 patients who were transferred to our hospital, considering the indications of LVAD implantation or HTx. We divided the patients into two groups: those referred

Comprehensive cardiac rehabilitation (CR) is promising strategy for various cardiovascular diseases. Despite these benefits and the recommendation, adherence to outpatient CR remains low. Home-based CR with telemedicine (tele-CR) is emerging concept that is a good alternative to conventional center-based CR. With the development of e-health and e-Cardiology, real-time telemonitoring of patients’ parameters such as vital signs and supervising by healthcare professionals during exercise training via internet might make it possible for safe and effective tele-CR to be performed. Therefore, the present study reviews the literature to summarize the current situation and methodology of patient telemonitoring in tele-CR.

Abstract Heart transplantation is an effective therapy for patients with end‐stage heart failure. In some cases, Takotsubo syndrome (TTS) was seen in the donor heart. We report a case of TTS in a 40‐year‐old woman with a history of epileptic seizures who underwent heart transplantation from a donor with TTS. The donor was brain‐dead due to severe hypoxic encephalopathy during cardiac arrest with TTS. Fifteen months after heart transplantation, she was readmitted for epileptic seizures. Electrocardiogram showed T‐wave inversion, and transthoracic echocardiography showed apical ballooning. Coronary angiography was normal, and endomyocardial biopsy was negative for rejection. Iodine‐123 metaiodobenzylguanidine imaging demonstrated a low heart‐to‐mediastinum ratio and high washout rate. Eighteen days after admission, recovery of left ventricular dysfunction was confirmed, and she was diagnosed with TTS triggered by epileptic seizures. It is important to recognize the risk of recurrent TTS in heart transplantation patients from a donor with TTS.

Heart failure (HF) has various characteristics, such as etiology, clinical course, and clinical characteristics. Several studies reported the clinical findings of the characteristics of non-ischemic cardiomyopathy. There have been issues with genetic, biochemical, or pathophysiological problems. Some studies have been conducted on non-ischemic cardiomyopathy and social factors, for instance, racial disparities in peripartum cardiomyopathy (PPCM) or the social setting of hypertrophic cardiomyopathy. However, there have been insufficient materials to consider the relationship between social factors and clinical course in non-ischemic cardiomyopathies. There were various methodologies in therapeutic interventions, such as pharmacological, surgical, or rehabilitational, and educational issues. However, interventions that could be closely associated with social inequality have not been sufficiently elucidated. We will summarize the effects of social equality, which could have a large impact on the development and progression of HF in non-ischemic cardiomyopathies.

Abstract Aims Nephrotoxicity of calcineurin inhibitors (CNIs) is associated with adverse events in patients undergoing heart transplant (HTx), although studies directly comparing tacrolimus (TAC) versus cyclosporin A (CsA), especially in combination with everolimus and low‐dose CNIs approach, are limited. Thus, we sought to investigate the associations of TAC and CsA with clinical outcomes in HTx recipients, with specific focus on renal function. Methods and results From August 2007 to February 2017, 72 consecutive patients (39 treated with TAC vs. 33 with CsA) receiving de novo HTx in a single transplant centre were retrospectively evaluated. We used the instrumental variable method to account for unmeasured confounding. The study outcomes were percentage change in estimated glomerular filtration rates (eGFR) (safety endpoint) and biopsy‐proven acute rejection (efficacy endpoint) within the first year after HTx. The enrolled patients (median age 40 years) were predominantly men (68%). There were no significant differences in baseline characteristics, including eGFR (64.8 [45.7–96.4] mL/min/1.73 m2 in TAC vs. 65.6 [57.9–83.0] mL/min/1.73 m2 for CsA; P = 0.48), other than sex (male, 49% for TAC vs. 91% for CsA; P

Abstract Background Relapses frequently occur in giant cell arteritis (GCA), and long-term glucocorticoid therapy is required. The identification of associated factors with poor treatment outcomes is important to decide the treatment algorithm of GCA. Methods We enrolled 139 newly diagnosed GCA patients treated with glucocorticoids between 2007 and 2014 in a retrospective, multi-center registry. Patients were diagnosed with temporal artery biopsy, 1990 American College of Rheumatology classification criteria, or large vessel lesions (LVLs) detected by imaging based on the modified classification criteria. Poor treatment outcomes (non-achievement of clinical remission by week 24 or relapse during 52 weeks) were evaluated. Clinical remission was defined as the absence of clinical signs and symptoms in cranial and large vessel areas, polymyalgia rheumatica (PMR), and elevation of C-reactive protein (CRP) levels. A patient was determined to have a relapse if he/she had either one of the signs and symptoms that newly appeared or worsened after achieving clinical remission. Re-elevation of CRP without clinical manifestations was considered as a relapse if other causes such as infection were excluded and the treatment was intensified. Associated factors with poor treatment outcomes were analyzed by using the Cox proportional hazard model. Results Cranial lesions, PMR, and LVLs were detected in 77.7%, 41.7%, and 52.5% of the enrolled patients, respectively. Treatment outcomes were evaluated in 119 newly diagnosed patients who were observed for 24 weeks or longer. The mean initial dose of prednisolone was 0.76 mg/kg/day, and 29.4% received any concomitant immunosuppressive drugs at baseline. Overall, 41 (34.5%) of the 119 patients had poor treatment outcomes; 13 did not achieve clinical remission by week 24, and 28 had a relapse after achieving clinical remission. Cumulative rates of the events of poor treatment outcomes in patients with and without LVLs were 47.5% and 17.7%, respectively. A multivariable model showed the presence of LVLs at baseline was significantly associated with poor treatment outcomes (adjusted hazard ratio [HR] 3.54, 95% CI 1.52–8.24, p = 0.003). Cranial lesions and PMR did not increase the risk of poor treatment outcomes. Conclusion The initial treatment intensity in the treatment algorithm of GCA could be determined based upon the presence or absence of LVLs detected by imaging at baseline.

Background: Implantable continuous-flow left ventricular assist device (LVAD) improve renal function in advanced heart failure. However, the long-term effects of LVAD on renal function have not been investigated thoroughly. We aimed to assess long-term renal function in patients with LVAD support and to identify predictors for late deterioration in renal function (LDRF). Methods: One hundred patients underwent LVAD implantation as a bridge to transplant at the University of Tokyo Hospital between May 2011 and December 2018. We assessed renal function at intervals (preoperative, 1, 6, 12, 18, 24 and 30 months after LVAD implantation). We divided patients into two groups: “with LDRF,” whose renal function at 30 months had decreased by >25% compared with preoperatively (n = 14), and “without LDRF” (n = 55). Results: Renal function improved at 1 month, returned to preoperative levels at 6 months, and remained there up to 30 months after LVAD implantation. However, renal function impairment became evident in patients with LDRF 18 months after LVAD implantation. A ratio of right atrial pressure/pulmonary artery wedge pressure > 0.57 and left ventricular dimension diastole ≤ 67 mm were preoperative independent risk factors for LDRF. In addition, the incidence of perioperative acute kidney injury, ventricular arrhythmia, aortic insufficiency, and late right ventricular failure was significantly higher in patients with LDRF. Conclusion: LDRF after LVAD implantation corresponded to several risk factors, including a small left ventricle and LVAD-related complications, such as right ventricular failure.

This study aimed to investigate the outcomes of surgical interventions for symptomatic moderate-to-severe aortic regurgitation (AR), including aortic valve replacement (AVR) and repair (AVP), in 184 patients who underwent continuous flow-left ventricular assist device (Cf-LVAD) implantation as a bridge-to-transplant (BTT) between November 2007 and April 2020. Ten patients (median age, 34 (25–41) years; 60% men) underwent surgical interventions (AVR, n = 6; AVP, n = 4) late after cf-LVAD implantation. The median duration after the device implantation was 34 (24–44) months. Three patients required additional tricuspid valve repair. Aortic valve suturing resulted in severe recurrent AR 6 months postoperatively, due to leaflet cutting in one patient. Seven patients with AVR survived without regurgitation during the study period, except for one non-survivor complicated by liver failure due to postoperative right heart failure. Therefore, six patients after AVP (n = 4) and AVR (n = 2) underwent successful heart transplantation 7 (4–13) months after aortic intervention. Kaplan–Meier analysis showed no significant difference in overall survival through 5 years after cf-LVAD implantation, regardless of the surgical AV intervention chosen (log-rank test, p = 0.86). In conclusion, surgical interventions (AVR or AVP) for patients with an ongoing cf-LVAD are safe, effective, and viable options.

BackgroundConventional group-based outpatient cardiac rehabilitation through monitoring and center-based approaches for patients in the recovery phase has shown strong evidence for the prevention of cardiovascular diseases. However, there are some cases in which maintaining attendance of center-based cardiac rehabilitation is difficult. ObjectiveThis study aims to ascertain the safety and efficacy of remote cardiac rehabilitation (RCR) in the recovery phase in patients with cardiovascular disease. MethodsPatients satisfying the study criteria will be recruited from multiple institutions (approximately 30) across Japan. In total, 75 patients (approximately 2 or 3 patients from each institution) are proposed to be recruited. Patients enrolled in the RCR group will be lent devices necessary for RCR (including calibrated ergometers and tablets). Patients will perform anaerobic exercise at home using ergometer for 30-40 minutes at least 3 times weekly. During exercise, an instructor will monitor the patient in real time (using interactive video tools and monitoring tools for various vital data). Moreover, educational instructions will be given 3 times weekly using e-learning methods. ResultsThe primary endpoint is the peak oxygen uptake 2-3 months from the start of exercise or 6-min walk test. The extracted data will be compared between RCR patients and controls without RCR. ConclusionsThe establishment of the system of RCR proposed in this study will lead to the development of more extensive applications, which have been insufficient through conventional interventions. Trial RegistrationUniversity Hospital Medical Information Network—Clinical Trials Registry UMIN–CTR UMIN000042942; https://upload.umin.ac.jp/cgi-open-bin/ctr_e/ctr_view.cgi?recptno=R000048983 International Registered Report Identifier (IRRID)DERR1-10.2196/30725

There have been several reports on the identification of the stage of transthyretin amyloid cardiomyopathy (ATTR-CM); however, a staging system for ATTR-CM has not yet been established. An 83-year-old woman was referred to our department about ten years ago. Recently, she was diagnosed with ATTR-CM. The electrocardiogram showed characteristic changes that take place over the duration of ATTR-CM progression. Among these, the precordial R amplitudes abruptly decreased before the development of increased ventricular thickness. This case suggested that the decrease in the precordial R wave amplitudes may represent a new diagnostic clue reflecting early myocardial damage due to ATTR-CM.

Summary: This study evaluated myocardial nuclear staining for the DNA damage markers poly(ADP-ribose) (PAR) and γ-H2A.X in 58 patients with dilated cardiomyopathy. Patients with left ventricular reverse remodeling (LVRR) showed a significantly smaller proportion of PAR-positive nuclei and γ-H2A.X–positive nuclei in biopsy specimens compared with those without LVRR. Propensity analysis showed that the proportion of both PAR-positive and γ-H2A.X–positive nuclei were independent prognostic factors for LVRR. In conclusion, we showed the utility of DNA damage-marker staining to predict the probability of LVRR, thus revealing a novel prognostic predictor of medical therapy for dilated cardiomyopathy. Key Words: dilated cardiomyopathy, DNA damage, left ventricular reverse remodeling, poly ADP-ribose

Abstract Background Continuous-flow left ventricular assist devices (LVADs) improve survival and morbidity in patients with stage D heart failure. Management of LVADs for longer durations is necessary in some clinical settings, and a better understanding of the hemodynamics of patients using LVADs is warranted. Arrhythmia, including atrial (AA) and ventricular (VAs) arrhythmias, is a modifying factor of hemodynamics that is highly prevalent among patients with LVADs. However, the clinical impact of arrhythmias in various clinical settings in patients with LVAD, in which the hemodynamic load is likely to present as worsening of right heart failure, remains to be completely elucidated. Case presentation We describe the case of a patient under sustained ventricular fibrillation for extraordinarily long duration who was stabilized using LVAD support and in whom newly developed atrial fibrillation led to a significant worsening of right heart failure while using an LVAD. Conclusion This case demonstrates the substantial clinical impact of AAs in the management of right heart failure using an LVAD.

To investigate the effect of atrial fibrillation (AF) on the oxygen uptake and exercise tolerance, we evaluated cardiopulmonary exercise test (CPET) data in AF patients and heart rate-matched controls with sinus rhythm (cSR) who received ambulatory cardiac rehabilitation. We compared CPET data between AF (N = 27) and cSR patients (N = 106) who had similar HRs at rest and the peak points. Oxygen uptake (VO2)/kg and relative O2 pulse (ml/bpm/kg) at rest and the anaerobic threshold (AT) level was not different between AF and cSR patients, but these parameters above the AT level were significantly lower in AF than in cSR patients. Concisely the parallel increase of relative O2 pulse during exercise was blunted above the respiratory compensation level (Rc) in the AF group. In addition, the HR change during exercise was inversely correlated with the increase of the O2 pulse above the AT level and this inverse correlation was more prominent in AF patients than in cSR patients. In conclusion, the value of VO2 was significantly lower above the AT level in AF patients. The trend of O2 pulse above the AT level was strongly associated with the detrimental response of HR increase and the response was markedly exaggerated in the AF patients. Keywords: Atrial fibrillation, Oxygen uptake, O2 pulse, Heart rate response, Hemodynamic trend

BackgroundLittle evidence has been presented about the association between previous atopic/allergic disease and graft rejection after solid organ transplantation. Thus, we present a case wherein acute cellular rejection (ACR) after heart transplantation (HTx) was noted along with exacerbation of atopic disease.Case SummaryA 32-year-old man was admitted at our hospital for regular monitoring of graft rejection. He had undergone heart transplant 3 years prior due to dilated cardiomyopathy. Echocardiogram revealed good biventricular function, and no abnormal findings were found in blood sampling tests. However, biopsy showed moderate ACR [Grade 2R(ISHLT 2004)/3A(ISHLT 1990)], which required twice-repeated steroid pulses with intensified immunosuppression. Meanwhile, his atopic dermatitis, which was diagnosed before having heart failure, was getting worse for the past 6 months. The exacerbation of atopic dermatitis was presumed to be related to the development of the intractable cellular rejection.DiscussionThis case suggested the association of atopic disease and graft rejection after HTx. We examined 76 patients from a cohort of previous studies who underwent HTx at our hospital, which suggested that patients with atopic/allergic disorders such as atopic dermatitis and asthma tended to have a significantly higher frequency of moderate rejection than non-allergic patients. (p = 0.012; Fisher’s exact test). Our case also suggests that exacerbation of atopic dermatitis might cause graft rejection of the transplanted organ, so that it is important to carefully evaluate the risk of graft rejection if there is a previous history of atopic/allergic disease.

Abstract Background Central venous oxygen saturation (ScvO2) reflects the balance of oxygen delivery and consumption. Low ScvO2 indicates the presence of inadequate oxygen delivery, while high ScvO2 indicates reduced oxygen consumption and is sometimes associated with a high mortality rate in critically ill patients from dysoxia. Thiamine is an essential cofactor in cellular aerobic metabolism. Thiamine deficiency is more prevalent than was previously thought, and underlies severe conditions in critically ill patients. However, currently, there is no rapid diagnostic test for thiamine deficiency. Considering oxygen flux, high ScvO2 might be associated with thiamine deficiency. Case presentation A 70-year-old man admitted to the hospital with chief complaint of malaise and edema. He was diagnosed with heart failure with preserved ejection function and was treated with loop diuretics, which resulted in shock. Venoarterial extracorporeal membrane oxygenation and intra-aortic balloon pumping was indicated. The right heart catheter showed high ScvO2, normal cardiac output, and low systemic vascular resistance. Thiamine deficiency was suspected and we started the thiamine infusion. His hemodynamic status improved after thiamine replacement. After his recovery, it was discovered that he had a 1-month history of anorexia and thiamine deficiency. His final diagnosis was beriberi. Conclusions The current case showed the relation between thiamine deficiency and high ScvO2. A literature review also suggested that thiamine deficiency is associated with high ScvO2. Thiamine deficiency causes impaired tissue oxygen extraction, which could lead to high ScvO2. In this context, high ScvO2 might serve as a predictor of thiamine deficiency in critically ill patients.

Abstract Background Fulminant myocarditis is a life-threatening disease, and myocardial damage expands the right ventricle as well as the left ventricle in some cases. There is a mortality rate of over 40% in patients with fulminant myocarditis who need mechanical circulatory support by peripheral venoarterial extracorporeal membrane oxygenation. Case presentation We report a case of a 27-year-old Japanese woman who was successfully bridged to recovery by using a biventricular assist device. She was diagnosed with fulminant myocarditis, and peripheral venoarterial extracorporeal membrane oxygenation was established on the same day. Her left ventricular ejection fraction rapidly decreased from 40% to 5% in 3 days and weaning from venoarterial extracorporeal membrane oxygenation was deemed difficult. Therefore, we performed a ventricular assist device implantation on day 4. A left ventricular assist device was implanted first. However, adequate blood flow did not circulate to the left side of her heart because of right-sided heart failure. Thus, an additional implant of a right ventricular assist device was performed during the operation. Her left ventricular ejection fraction recovered to 50% on day 10. The biventricular assist device was successfully removed on day 14. She has not experienced worsening of biventricular function during her follow-ups for 4 years. Conclusions Ventricular assist device therapy should be considered if there is no improvement in cardiac function in patients with fulminant myocarditis regardless of several days of support by venoarterial extracorporeal membrane oxygenation. A right ventricular assist device should always be implemented when necessary because biventricular involvement is not uncommon in fulminant myocarditis.

Abstract Background Sarcopenia is generally complicated with patients with chronic heart failure (CHF) and its presence negatively affects the course of heart failure, however effective nutritional intervention had not been elucidated yet. The primary objective of this study is to explore whether the addition of a branched-chain amino acid (BCAA) preparation for cardiac rehabilitation (CR) of patients with CHF further improves cardiopulmonary functions, skeletal muscle functions, and metabolism in comparison with conventional CR. Methods This is a randomized, parallel-group comparative study. The elderly patients that were participated in CR and complicated with left ventricular systolic or diastolic dysfunction are randomized into two groups, CR + BCAA and CR. 20 weeks later, the second randomization is performed, which divide subjects into two groups with and without BCAA intervention without CR. Primary outcome measure is the rate of change of the anaerobic threshold workload from baseline to post-intervention. Secondary outcome include parameters of exercise capacity, cardiac function and psychological status. Discussion In the current study the effect of a promising new intervention, BCAA, will be assessed to determine whether its addition to CR improve exercise capacity in patients with heart failure, who are generally complicated with sarcopenia. Trial registration This clinical trial was registered with the University Hospital Medical Information Network—Clinical Trials Registry (UMIN–CTR; JPRN–UMIN R000022440 ).

Hyperammonemic encephalopathy secondary to heart failure is rare and there had been little reports about effective treatment. Organ hypoperfusion or congestion by heart failure may lead to various organ dysfunctions, and liver and intestinal circulatory impairment might cause ammonia metabolic failure. Here, we report on the case of a patient with hyperammonemic encephalopathy that was secondary to heart failure, which was effectively treated by lactulose.

BACKGROUND:The prevalence of pulmonary arterial hypertension (PAH) in primary Sjögren's syndrome (SS) had been reported to be rare. However, recent studies using echocardiography as a screening method showed conflicting results, and the true prevalence is still unclear. Since diagnosing primary SS is difficult because of its heterogeneous nature, a number of patients with primary-SS-associated PAH may be misdiagnosed with idiopathic PAH, losing their chance to undergo immunosuppressive therapy. Therefore, we sought to elucidate the prevalence of primary SS among patients who initially present with PAH. METHODS:From our prospective institutional PAH database, 40 consecutive patients without any obvious cause of PAH at the time of PAH diagnosis were identified. We retrospectively evaluated the prevalence of primary SS diagnosed during or after the initial assessment of PAH. RESULTS:During the initial assessment, one patient was diagnosed with primary-SS-associated PAH. Among the 25 patients who were initially diagnosed with idiopathic PAH, five were diagnosed with primary SS during their course of the disease. Of the five patients, three had key signs suggesting primary SS and were probably underdiagnosed at the time of initial evaluation. The remaining two patients, who were finally diagnosed with primary SS, did not have any specific signs suggesting primary SS at the time of initial evaluation but showed positive conversion of their autoantibodies during the course of PAH. CONCLUSION:The prevalence of primary-SS-associated PAH may be relatively high among patients who undergo initial evaluation for PAH. Furthermore, primary-SS-associated PAH may be underdiagnosed with routine evaluation for the primary cause of PAH. Clinicians should pay specific attention and carefully evaluate the possibility of primary SS in patients with PAH.

Exercise-based rehabilitation programs have been reported to have beneficial effects for patients with heart failure. However, there is little evidence about whether this is the case in patients with more severe heart failure. In particular, there is a question in the clinical setting whether patients with advanced heart failure and continuous inotropic infusion should be prescribed exercise training. In contrast, many studies conclude that prolonged immobility associated with heart failure profoundly impairs physical function and promotes muscle wasting that could further hasten the course of heart failure. By contrast, exercise training has various effects not only in improving exercise capacity but also on vascular function, skeletal muscle, and autonomic balance. In this review, we summarize the effectiveness and discuss methods of exercise training in patients with advanced heart failure receiving continuous inotropic agents such as dobutamine.

We compared the contribution of physical activity to the change in arterial stiffness between patients with and without diabetes in ischemic heart disease.We studied 96 (diabetes) and 109 (without diabetes) patients with ischemic heart disease treated by percutaneous coronary intervention (PCI). Arterial stiffness was assessed by cardio-ankle vascular index (CAVI) at the first diagnosis of significant coronary ischemia and 6 months after PCI and optimal medical therapy. Physical activity was evaluated using the long form of the International Physical Activity Questionnaire (IPAQ).CAVI values increased more for diabetic patients than for non-diabetic. The IPAQ scores did not differ between the two groups. During follow-up, CAVI values did not significantly change in either group. In diabetic patients, the CAVI score for 48 patients did not change (NC-group) and 48 patients improved (Improved-group). Physical activity scores were 937.9 ± 923.2 and 1524.6 ± 1166.2 in the NC- and Improved-groups, respectively. IPAQ scores and uric acid levels significantly affect CAVI improvement after adjusting for age, sex, baseline CAVI, total cholesterol, and estimated glomerular filtration rate.Determining factors influencing CAVI improvement during follow-up were significantly different between patients with and without diabetes. IPAQ scores and uric acid levels were significantly correlated with CAVI changes.

The physiological role of vasomotion, rhythmic oscillations in vascular tone or diameter, and its underlying mechanisms are unknown. We investigated the characteristics of brachial artery vasomotion in patients with ischemic heart disease (IHD).We performed a retrospective study of 37 patients with IHD. Endothelial function was assessed using flow-mediated dilation (FMD), and power spectral analysis of brachial artery diameter oscillations during FMD was performed. Frequency-domain components were calculated by integrating the power spectrums in three frequency bands (in ms2) using the MemCalc (GMS, Tokyo, Japan): very-low frequency (VLF), 0.003-0.04 Hz; low frequency (LF), 0.04-0.15 Hz; and high frequency (HF), 0.15-0.4 Hz. Total spectral power (TP) was calculated as the sum of all frequency bands, and each spectral component was normalized against TP.Data revealed that HF/TP closely correlated with FMD (r = -0.33, p = 0.04), whereas VLF/TP and LF/TP did not. We also explored the relationship between elevated C-reactive protein (CRP) levels and vasomotion. HF/TP was significantly increased in subjects with high CRP levels (CRP;>0.08 mg/dL) compared with subjects with low CRP levels (0.052±0.026 versus 0.035±0.022, p