Leoni, Juan Carlos

AbstractBackgroundThe prognosis in patients with advanced cardiac amyloidosis (CA) remains poor.ObjectivesWe sought to describe survival post heart transplantation (HT) in amyloid compared with non‐amyloid recipients, highlight waitlist times within the new allocation system across three Organ Procurement and Transplantation Network (OPTN) regions, and describe multiorgan transplantation (MOT) in hereditary amyloidosis.MethodsThis is a retrospective review of end‐stage CA patients who underwent HT at Mayo Clinic from January 2007 to December 2020. Wait time was compared in the new versus old OPTN allocation era starting December 18, 2018 by Wilcoxon rank sum test. All‐cause mortality for those with and without CA was compared using Kaplan–Meier estimates with log rank analysis, censoring December 16, 2022.ResultsFifty‐five patients with CA underwent HT between 2007 and 2020, 8 light chain amyloidosis (AL) (14.5%), 28 hereditary transthyretin (ATTRv) (50.9%), 17 wildtype transthyretin (ATTRwt) (30.9%), and 2 hereditary apolipoprotein A1 (AApoA1) amyloidosis patients (3.6%). No significant difference in overall survival post‐transplant was seen in amyloid compared with non‐amyloid (p = 0.816). Median time to HT was shorter in the new system, 45 days (IQR 24, 78) versus 174 days (IQR 76.5, 483.5), p = 0.006. There was a decline in MOT in hereditary amyloidosis over time with the concomitant rise in disease‐targeted therapies.ConclusionsHT survival in CA patients was similar to non‐amyloid patients. The new allocation system benefits this cohort with shorter wait times. There is less MOT in hereditary amyloidosis with increased utilization of disease‐targeted therapy.

Introduction/Background: Patients with heart failure and those waiting for a heart transplant often experience a significant decrease in their ability to perform daily activities and exercise, leading to a lower quality of life. Although exercise-based cardiac rehabilitation (EBCR) is a possible treatment option, its effectiveness has not been well measured, resulting in limited adoption. Research Questions/Hypothesis: Could physical rehabilitation before receiving a heart transplant improve functional status and better outcomes? Goals/Aims: Our objective is to evaluate the effect of pre-transplant physical therapy on the standardized improvement of AM-PAC scores, measured from the earliest pre-transplant assessment to the most recent pre-transplant evaluation. Our second objective is to assess the impact of the latest Pre-Transplant AM-PAC Score on the number of hospital-free days following transplantation. Methods: We conducted a retrospective study on 91 heart transplant patients who received Impella support from January 2019 to April 2023, approved by the Mayo Clinic Institutional Review Board. We collected data on demographics, AMPAC scores, and outcomes at three time points. Continuous variables were compared using the T-test or Mann-Whitney test, and categorical variables using the Chi-square or Fisher's exact test. We used a linear regression model to investigate the relationship between rehabilitation duration and AMPAC scores. Quantile regression was preferred due to the non-normal distribution of the outcome, hospital-free days. Results: We conducted a linear regression analysis and found a statistically significant association between increased hours spent in PT and greater improvement. After one hour of physical therapy, there was an average increase of 1.07 (95% CI 0.1, 2; p=0.03) in AMPAC scoore. We also compared the change in the AMPAC score with hospital-free days, and it was statistically significant in quantile regression analysis, indicating an increase of 0.28 (95% CI 0.2, 0.55; P=0.03) points in the AMPAC Score, corresponding to an additional hospital-free day. Conclusion: Increased hours spent in physical therapy show a significant positive association with greater improvement in standardized AM-PAC Mobility scores and hospital-free days. These findings encourage increasing uptake and support of cardiac rehabilitation programs.

Abstract Background Stiff left atrial syndrome is a well-established cause of heart failure symptoms. A parallel entity involving the right atrium (RA) has not previously been described. We present a case of refractory right heart failure (RHF) 12 years following orthotopic heart transplantation. Case summary Patient underwent annuloplasty ring placement for severe tricuspid regurgitation in 2018 and kidney transplantation in 2020. The use of multimodality imaging and a multidisciplinary approach suggested a stiff RA as a potential etiology to refractory symptoms. Redo-heart and kidney transplantation in March 2021 led to the resolution of symptoms without recurrence. Discussion We propose stiff right atrial syndrome that may need to be considered in the setting of refractory RHF primarily suggested by significant right atrial enlargement and restrictive physiology.

Abstract Background Impella is an advanced ventricular assist device frequently used as a bridge to heart transplantation. The association of Impella with increased rates of gout flares has not been studied. Our primary aim is to determine the rates of gout flares in patients on Impella support. Methodology A retrospective study was conducted between January 2017 and September 2022 involving all patients who underwent heart transplantation. The cohort was divided into groups based on Impella support for statistical analysis. In patients receiving Impella support, outcome measures were compared based on the development of gout flares. Results 213 patients were analyzed, with 19.71% supported by Impella. Impella and non-Impella groups had similar age, race, and BMI, but more males were in the Impella group. Gout and chronic kidney disease were more prevalent in Impella-supported patients, while coronary artery disease was less common. The incidence of gout flare was significantly higher in Impella patients (31% vs. 5.3%). The Impella group had an adjusted odds ratio for in-hospital gout flare of 8.069 (95% CI = 2.13–35.08, P = 0.003). Impella-supported patients with gout flares had longer intensive care unit and hospital stays but no difference in mortality or other outcomes. Conclusion Our study is the first to identify the potential association between Impella support and increased rates of gout flares in hospitalized patients. Future studies are required to confirm this association and further elucidate the biological pathways. It is imperative to consider introducing appropriate measures to prevent and promptly manage gout flares in Impella-supported patients.

Abstract Trigeminal amyloidoma is a rare clinical phenomenon with scarce reports in the medical literature. This report presents a case of biopsy-proven trigeminal amyloidoma in a patient with cardiac amyloidosis. This case report sheds light on the differential diagnoses that may resemble trigeminal amyloidoma and strategies for workup and treatment.

Abstract Aims We sought to investigate the outcomes of heart transplant patients supported with Impella 5.5 temporary mechanical circulatory support. Methods and results Patient demographics, perioperative data, hospital timeline, and haemodynamic parameters were followed during initial admission, Impella support, and post-transplant period. Vasoactive-inotropic score, primary graft failure, and complications were recorded. Between March 2020 and March 2021, 16 advanced heart failure patients underwent Impella 5.5 temporary left ventricular assist device support through axillary approach. Subsequently, all these patients had heart transplantation. All patients were either ambulatory or chair bound during their temporary mechanical circulatory support until heart transplantation. Patients were kept on Impella support median of 19 days (3–31) with the median lactate dehydrogenase level of 220 (149–430). All Impella devices were removed during heart transplantation. During Impella support, patients had improved renal function with median creatinine serum level of 1.55 mg/dL decreased to 1.25 (P = 0.007), pulmonary artery pulsatility index scores increased from 2.56 (0.86–10) to 4.2 (1.3–10) (P = 0.048), and right ventricular function improved (P = 0.003). Patients maintained improved renal function and favourable haemodynamics after their heart transplantation as well. All patients survived without any significant morbidity after their heart transplantation. Conclusions Impella 5.5 temporary left ventricular assist device optimizes care of heart transplant recipients providing superior haemodynamic support, mobility, improved renal function, pulmonary haemodynamics, and right ventricular function. Utilizing Impella 5.5 as a direct bridging strategy to heart transplantation resulted in excellent outcomes.

Abstract Transthyretin cardiac amyloidosis is a progressive disease known to cause heart failure, conduction anomalies, and arrythmias. Due to poor outcomes and mortality from severe cardiomyopathy, prevalence and incident rates are often underreported. As global longevity is increasing and rates of amyloidosis are also increasing, there is a need to improve diagnostic and therapeutic interventions. Previously, symptom management and transplantation were the mainstay of treatment for heart failure symptoms, but studies using RNAi and siRNA technologies have shifted the paradigm of therapeutic strategy in amyloid cardiomyopathy management. Additionally, early detection and clinical monitoring with numerous imaging and non-imaging techniques are being increasingly investigated. Here, we review the epidemiology, pathophysiology, diagnosis, and management of transthyretin amyloid cardiomyopathy.