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Background: Recent studies have reported atrial involvement and coexistence of aortic stenosis in transthyretin (ATTR) cardiac amyloidosis (CA). However, pathological reports of extraventricular ATTR amyloid deposits in atrial structures or heart valves are limited, and the clinical implications of ATTR amyloid deposits outside the ventricles are not fully elucidated., Case Presentation: We report 3 cases of extraventricular ATTR amyloid deposits confirmed in surgically resected aortic valves and left atrial structures, all of which were unlikely to have significant ATTR amyloidosis infiltrating the ventricles as determined by multimodality evaluation including 99m technetium-pyrophosphate scintigraphy, cardiac magnetic resonance, endomyocardial biopsy and their mid-term clinical course up to 5 years. These findings suggested that these were extraventricular ATTR amyloid deposits localized in the aortic valve and the left atrium., Conclusions: While long-term observation is required to fully clarify whether these extraventricular ATTR amyloid deposits are truly localized outside the ventricles or are early stages of ATTR-CA infiltrating the ventricles, our 3 cases with multimodality evaluations and mid-term follow up suggest the existence of extraventricular ATTR amyloid deposits localized in the aortic valve and left atrial structures., (© 2023. The Author(s).)

The clinical relevance of transcranial Doppler (TCD) detection of micro-embolic signals (MES) in patients with ventricular assist devices (VADs) has been described. However, all of the previous studies concerning TCD in patients with VADs were conducted in patients with old devices; the clinical relevance of TCD in patients with newer devices has not been fully elucidated. We recently encountered a patient with a continuous-flow VAD with hemolysis. TCD monitoring was useful for the direct evaluation of micro-emboli in this patient. A 50-year-old male who underwent HeartMate II(®) VAD (Thoratec Corporation; Pleasanton, CA) implantation with a diagnosis of ischemic cardiomyopathy 15 months prior was admitted to our institution because of findings suggestive of hemolysis, such as elevated lactate dehydrogenase (LDH) and total bilirubin. Unfractionated heparin was started after admission and hemolysis gradually improved. On admission, TCD detected 146 MES during 30 min of monitoring. During the hospital course, the MES count decreased to 20 signals on hospital day 4 and further decreased to 2 signals on hospital day 15 along with decreases in LDH and total bilirubin. Since hemolysis in VAD patients is thought to be associated with thromboembolic outcomes, MES detected by TCD indicate subclinical micro-emboli. TCD monitoring may be useful for assessing the risk of thromboembolic events in newer continuous-flow VAD patients through direct visualization of micro-emboli.

Background: The purpose of the present study was to determine the impact of preoperative percutaneous cardiopulmonary support (PCPS) on long-term survival following implantation of a left ventricular assist device (LVAD)., Methods and Results: Between 1999 and 2010, we used implantable (n=12) and paracorporeal (n=91) LVADs in 103 consecutive cardiomyopathy patients as a bridge to transplantation. Prior to LVAD implantation, all patients received inotropes, and 25 patients (24%) received PCPS because of cardiogenic shock. Postoperatively, there were no early mortalities within 30 days after surgery, and patients survived on LVAD for 560±391 days, of whom 9 patients recovered and 32 underwent heart transplantation after 711±360 days of LVAD support. More patients with preoperative PCPS required nitric oxide inhalation and prolonged inotropic support to maintain adequate LVAD flow. In addition, bilirubin level at 1 month after LVAD implantation was significantly higher in patients with preoperative PCPS. Cox regression analysis identified preoperative PCPS support as the only significant predictor for death after LVAD implantation and overall survival was significantly better in patients without preoperative PCPS., Conclusions: Despite adequate hemodynamic support after LVAD implantation, patients with preoperative PCPS had significantly worse survival. LVAD should be used for patients with end-stage heart failure, before PCPS is required for hemodynamic support.

Objective: Preserved myocardial function remote from surgical site is crucial for good outcome after surgical ventricular restoration in ischemic cardiomyopathy. We hypothesized that left ventricular scarring untouched by operation would negatively affect postoperative outcome., Methods: In 15 consecutive patients (mean age 61 +/- 12 years, mean left ventricular ejection fraction 20% +/- 7.5%), left ventricular assessments by magnetic resonance imaging and right heart catheterization were performed before and after operation. Left ventricular basal scarring remote from surgical exclusion site was quantified from hyperenhancement area on preoperative delayed-enhancement magnetic resonance imaging as percentage of fibrosis (total infarct size relative to ventricular mass)., Results: Calculated percentage of fibrosis varied from 0% to 29.9% (mean 12% +/- 9.6%). Percentage of fibrosis linearly correlated with significantly worse postoperative hemodynamic variables and left ventricular function recovery: left ventricular ejection fraction (P = .0005, R = -0.79), left ventricular end-systolic volume index (P = .05, R = 0.51), mean pulmonary arterial pressure (P = .004, R = 0.70), pulmonary capillary wedge pressure (P = .009, R = 0.65), and cardiac index (P = .005, R = -0.69). At mean 30-month follow-up, 4 patients with recurrent heart failure had significantly greater percentage of fibrosis than did those without recurrence (19% +/- 8.2% vs 8.8% +/- 8.6%, P = .04)., Conclusion: Amount of myocardial scarring at left ventricular base affected postoperative left ventricular function and hemodynamic improvements. Preoperative quantitative assessment of remote myocardial status with delayed-enhancement magnetic resonance imaging may predict outcomes for patients undergoing surgical ventricular restoration.

Heart transplantation started in Japan in 1999. Since then, 50 transplants have been performed at our center. We performed histopathological analyses of the 50 explanted hearts and the post-transplant biopsy specimens. The median age of recipients was 39 years. The primary diseases before transplant were idiopathic dilated cardiomyopathy in 33 patients (66%), hypertrophic cardiomyopathy in seven (14%), restrictive cardiomyopathy in one, arrhythmogenic right ventricular cardiomyopathy in one, and secondary cardiomyopathy in eight (16%). Before transplantation, 47 patients (94%) had left ventricular assist devices. No severe cardiovascular failure due to allograft rejection occurred. The post-transplant survival rate was 97.6% at 1 year and 93.1% at 10 years. One recipient was lost to sepsis from myelodysplastic syndrome in the fourth year, one died of multiple organ failure and peritonitis 8 months after transplant. Another patient died of recurrent post-transplant lymphoproliferative disorders ( PTLD). Mild cardiac dysfunction occurred in seven recipients in the early postoperative period. Moderate acute cellular rejection occurred in six patients (12%), and antibody-mediated rejection occurred in three (6%). The number of heart transplants performed in Japan is very small. However, the outstanding 10-year survival rate is due to donor evaluation and post-transplant care resulting in low grade rejection. Pathological evaluation has also greatly contributed to the results. [ABSTRACT FROM AUTHOR]

Dilated cardiomyopathy (DCM) is a heart muscle disease characterized by progressive heart failure, and is a leading cause of mortality and morbidity. Recently, cellular therapy for end-stage heart failure has been emerging. We herein report a 56-year-old male who received a transplant of autologous myoblast sheets manufactured in temperature-responsive culture dishes. His clinical condition improved markedly, leaving him without any arrhythmia and able to discontinue using a left ventricular assist system and avoid cardiac transplantation. These findings suggest that cellular therapy using myoblast sheets is a promising new strategy for treating patients with end-stage DCM. This method might be an effective alternative to heart transplantation in the near future. [ABSTRACT FROM AUTHOR]

Background: Management of native aortic insufficiency (AI) during left ventricular assist device (LVAD) support is challenging. We investigated the occurrence of de novo AI during long-term LVAD support to identify its effect on late clinical and echocardiographic outcomes. Methods: Left ventricular assist devices were implanted in 99 patients with dilated cardiomyopathy, of whom 47 without preoperative AI were investigated using serial echocardiography examinations for more than 1 year after the operation. Results: The mean duration of LVAD support was 838 ± 327 days, and 26 patients (55%) were supported for more than 2 years. Twenty-nine patients (62%) had no AI (group A), whereas de novo AI developed in the remaining 18 (38%; group B) at 1 year after LVAD implantation (≥ grade 2 in 5, grade 1 in 13). The LV end-diastolic diameter was significantly reduced after LVAD implantation in both groups, with no significant difference between them. Overall survival was better in group A (p = 0.0195). Multivariate analysis revealed that preoperative mitral regurgitation of more than grade 2 (odds ratio, 7.8; 95% confidence interval, 1.2 to 48.6; p = 0.028) and an aortic valve that remained closed at 1 month after implantation (odds ratio, 6.7; 95% confidence interval, 1.0 to 43.9; p = 0.048) were significant independent predictors of de novo AI at 1 year after LVAD implantation. Conclusions: Survival was significantly worse when de novo AI developed in patients during long-term LVAD. Our findings indicate that preoperative functional mitral regurgitation and postoperative aortic valve opening are related to the progression of AI during long-term LVAD support. [ABSTRACT FROM AUTHOR]

Purpose: Simultaneous injection of autologous bone marrow cells and skeletal myoblasts has been demonstrated to improve cardiac function in animal models. We evaluated the potential application of this combination cell therapy in patients with severe ischemic cardiomyopathy who required left ventricular assist device (LVAD) implantation. Methods: Four patients (age range, 43-69 years) who required LVAD implantation due to severe ischemic cardiomyopathy were studied. Skeletal myoblasts were obtained from the thigh, while bone marrow mononuclear cells were collected and purified at the time of the operation. These cells were directly injected in a serial manner into the damaged myocardium. Results: No fatal arrhythmias or major complications were observed. The number of injected skeletal myoblasts ranged from 2.7 × 10 to 3.0 × 10, and their purity ranged from 25% to 96%. Two patients showed decreased brain natriuretic peptide levels and echocardiographic improvements in the transplanted areas, as well as increased perfusion revealed by HO positron emission tomography, of whom one was successfully weaned from LVAD. Histological findings at autopsy of the other patient showed a small amount of skeletal muscle in the injected area. Only marginal improvements were observed in the other two patients. Conclusions: Combined cell transplantation is feasible for patients with severe ischemic cardiomyopathy, and functional recovery is anticipated in selected patients. [ABSTRACT FROM AUTHOR]

Background: The feasibility of left ventricular restoration for patients with ischemic cardiomyopathy complicated with cardiogenic shock remains unclear. We report early and mid-term outcomes of surgical interventions including left ventricular restoration for patients with cardiogenic shock. Methods: From April 2001 to June 2007, 10 patients with ischemic cardiomyopathy who developed cardiogenic shock underwent left ventricular restoration combined with mitral annuloplasty or coronary artery bypass grafting. All had been supported by a maximum dose of inotropic agents, 8 had required an intraaortic balloon pump, and 1 had required extracorporeal life support. Mean left ventricular end-diastolic and end-systolic volume indices were 142 ± 33 mL/m2 and 113 ± 28 mL/m2, respectively, and ejection fraction was 0.21 ± 0.059. Results: There was no mortality at 30 days. Five patients demonstrated significant recovery after the operation. Three patients simultaneously underwent left ventricular assist system (LVAS) implantation with left ventricular restoration because of preexisting severe end-organ failure, and 2 of them were subsequently weaned from LVAS, although 1 required reimplantation. The other 2 patients eventually underwent LVAS implantation in the early postoperative period. Two patients who required prolonged LVAS support underwent successful heart transplantation. Seven patients are alive at a mean follow-up of 1.9 years. Patients who required prolonged LVAS support had significantly longer duration of heart failure symptoms (p = 0.04) and higher mean pulmonary artery pressure (p = 0.02) preoperatively. Conclusions: Early combined surgical interventions including left ventricular restoration can be a choice of treatment even in patients with ischemic cardiomyopathy complicated with cardiogenic shock. Additional use of the LVAS followed by bridge to recovery or transplantation should be appropriately applied in these critically ill patients. [Copyright &y& Elsevier]