Intermediate-term outcomes of heart transplantation for cardiac amyloidosis in the current era.

BACKGROUND: Cardiac amyloidosis (CA) has been historically noted with poor outcomes after heart transplant (HTx). However, strict patient selection, appropriate multi-organ transplant, and aggressive post-transplant therapy can result in favorable outcomes. We present the experience in the largest single-center cohort of CA patients post-HTx in the recent era. METHODS: Between January 2010 and December 2018, 51 CA patients underwent HTx-13 light-chain amyloidosis (AL) and 38 transthyretin amyloidosis (ATTR), 49 were included. Endpoints included 3-year survival, freedom from cardiac allograft vasculopathy (CAV), and freedom from non-fatal major adverse cardiac events (NF-MACE). RESULTS: Overall 3-year survival was 81.6% (69.2% for AL and 86% for ATTR) and was comparable to survival for patients transplanted for non-amyloid restrictive cardiomyopathy (RCM) in the same period (89%, p = .46). Three-year freedom from CAV (84% vs. 89%, p = .98), NF-MACE (82% vs. 83%, p = .96), and any-treated rejection (95% vs. 89%, p = .54) were also comparable in both groups. No recurrence in amyloid was noted in endomyocardial biopsies. Six patients (46%) with AL amyloidosis underwent autologous stem cell transplant 1-year post-HTx, and two patients (8%) with variant ATTR-CA underwent combined heart-liver transplant due to cardiac cirrhosis. CONCLUSION: In the current era, both AL and ATTR cardiac amyloidosis patients have acceptable outcomes after heart transplantation.

Mechanical circulatory support in pre and postheart transplant period.

PURPOSE OF REVIEW: Progression of heart failure (HF) and its unpredictable and volatile nature, often requires advanced therapies including heart transplant. Mechanical circulatory support plays an integral part in the advanced treatment options. This technology can be deployed in several ways, particularly in the preparation and patient optimization for heart transplants. This article discusses the use of temporary and durable devices and their deployment strategies in the pre and posttransplant period. RECENT FINDINGS: Recently temporary mechanical support devices have allowed us to improve survival to transplant as well as posttransplant. Early implementation of temporary devices both for stabilization of advanced HF patients being considered for transplant as well as those with posttransplant primary graft dysfunction (although utilization of extracorporeal membrane oxygenation has repeatedly shown to be associated with worse outcomes compared to the other devices discussed), is reflective of the degree of disease progression in these patients. The outcomes of patients supported with durable devices have significantly improved with advancing technology. HeartMate 3 device has not only been shown to improve survival as well as the quality of life but in comparison to its predecessor, has been shown to decrease the morbidity associated with this technology. SUMMARY: Both temporary and durable devices are now associated with improved survival and allow us to transplant patients in a more stable and safer manner with fewer adverse events. Based on the new United Network of Organ Sharing allocation system, it allows us to upgrade those who do not have the luxury of time to wait for a transplant. Primary graft dysfunction now also can be assisted with those devices, which is reflected in improved survival of posttransplant patients.

The overestimation of concentric hypertrophy in patients with HFpEF as determined by 2D-echocardiography.

Background: Heart failure with preserved ejection fraction continues to pose multiple challenges in terms of accurate diagnosis, treatment, and associated morbidity. Accurate left ventricular (LV) mass calculation yields essential prognostic information relating to structural heart disease. Two-dimensional (2D) echocardiography-based calculations are solely limited to LV geometric assumptions of symmetry, whereas three-dimensional (3D) echocardiography could overcome these limitations. This study aims to compare the performance of 2D and 3D LV mass calculations. Methods: A prospective review of echocardiography findings at the University of Louisville, Kentucky, was conducted and assessed. Normal ejection fraction (EF) was defined as >=52% in males and >=54% in females. The following calculations were performed: relative wall thickness (RWT) = 2x posterior wall thickness/LV internal diastolic dimension (LVIDd) and 2D LV mass = 0.8{1.04([LVIDd + IVSd +PWd]3 - LVIDd3)} + 0.6. Concentric hypertrophy was RWT >0.42 and LV mass >95 kg/m2 in females or >115 kg/m2 in males. The same cut-offs were used for 2D and 3D echocardiography. Results: Echocardiographic findings for a total number of 154 patients in the study were investigated. There was a weak positive correlation between 2D and 3D LV mass indices (R = 0.534, r2 = 0.286, p = 0.001). Seventy patients had 3D EF >=45% with clinical heart failure (HFpEF). Among HFpEF patients, LV hypertrophy (LVH) was present in 74% of patients by 2D echocardiography and 30% by 3D echocardiography (McNemar test p = 0.001). Using 3D echocardiography as the reference, 68% of normal patients were misdiagnosed with LV hypertrophy by 2D echocardiography. Two-thirds of the patients with concentric remodeling by 3D echocardiography were misclassified as having concentric hypertrophy by 2D echocardiography (p = 0.001). Conclusion: Adapting necropsy-proven LV mass index cutoffs, 2D over-diagnosed LV hypertrophy through overestimation of the mass, compared to 3D echocardiography. In turn, the majority of HFpEF patients showed no structural hypertrophy of the LV on 3D imaging. This suggests that the majority of patients with HFpEF may qualify for pharmacological prevention to prevent further progression to LV remodeling or LVH.

A Peek at LVADs Pumping to Recovery.

Left ventricular assist devices (LVADs) have revolutionized the management of patients with advanced heart failure refractory to medical therapy. Current indications of LVADs include Bridge to Transplantation (BTT), Destination Therapy (DT) for long-term use, Bridge to the Decision (BTD) used as a temporary measure, and lastly Bridge to Recovery (BTR). Here, we briefly review the clinical evidence and the molecular mechanisms behind myocardial recovery following LVAD placement. We also share institutional protocols used at 2 major medical centers in the USA.

High Prevalence of Cardiac Amyloidosis in Clinically Significant Aortic Stenosis: A Meta-Analysis.

Background: There is growing evidence of coexistence of aortic stenosis (AS) and transthyretin cardiac amyloidosis (CA). Not screening AS patients at the time of hospital/clinic visit for CA represents a lost opportunity. Methods: We surveyed studies that reported the prevalence of CA among AS patients. Studies that compared patients with aortic stenosis with cardiac amyloidosis (AS-CA) and AS alone were further analyzed, and meta-regression was performed. Results: We identified nine studies with 1,321 patients of AS, of which 131 patients had concomitant CA, with a prevalence of 11%. When compared to AS-alone, the patients with AS-CA were older, more likely to be males, had higher prevalence of carpal tunnel syndrome, right bundle branch block. On echocardiogram, patients with AS-CA had thicker interventricular septum, higher left ventricular mass index (LVMI), lower myocardial contraction fraction, and lower stroke volume index. Classical low-flow low-gradient (LFLG) physiology was more common among patients with AS-CA. Patients with AS-CA had higher all-cause mortality than patients with AS alone (33% vs. 22%, P = 0.02) in a follow-up period of at least 1 year. Conclusions: CA has a high prevalence in patients with AS and is associated with worse clinical, imaging, and biochemical parameters than patients with AS alone.