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The Cardiac Amyloidosis Registry Study (CARS): Rationale, Design and Methodology

Stern, Lily K; Grodin, Justin L; Maurer, Mathew S; Ruberg, Frederick L; Patel, Ayan R; Khouri, Michel G; Roth, Lori R; Aras, Mandar A; Bhardwaj, Anju; Bhattacharya, Priyanka; Brailovsky, Yevgeniy; Drachman, Brian M; Ebong, Imo A; Fine, Nowell M; Gaggin, Hanna; Gopal, Deepa; Griffin, Jan; Judge, Daniel; Kim, Paul; Mitchell, Joshua; Mitter, Sumeet S; Mohan, Rajeev C; Ramos, Hannia; Reyentovich, Alex; Sheikh, Farooq H; Sperry, Brett; Carter, Spencer; Urey, Marcus; Vaishnav, Joban; Vest, Amanda R; Kittleson, Michelle M; Patel, Jignesh K
BACKGROUND:CARS (Cardiac Amyloidosis Registry Study) is a multicenter registry established in 2019 that includes patients with transthyretin (ATTR, wild-type and variant) and light chain (AL) cardiac amyloidosis (CA) evaluated at major amyloidosis centers between 1997 and 2025. CARS aims to describe the natural history of CA with attention to clinical and diagnostic variables at the time of diagnosis, real-world treatment patterns, and associated outcomes of patients in a diverse cohort that is more representative of the at-risk population than that described in CA clinical trials. METHODS AND RESULTS/RESULTS:This article describes the design and methodology of CARS, including procedures for data collection and preliminary results. As of February 2023, 20 centers in the United States enrolled 1415 patients, including 1155 (82%) with ATTR and 260 (18%) with AL CA. Among those with ATTR, wild-type is the most common ATTR (71%), and most of the 305 patients with variant ATTR have the p.V142I mutation (68%). A quarter of the total population identifies as Black. More individuals with AL are female (39%) compared to those with ATTR (13%). CONCLUSIONS:CARS will answer crucial clinical questions about CA natural history and permit comparison of different therapeutics not possible through current clinical trials. Future international collaboration will further strengthen the validity of observations of this increasingly recognized condition.
PMID: 37907148
ISSN: 1532-8414
CID: 5628152

Cardiac allograft vasculopathy in heart transplant recipients from hepatitis C viremic donors

Kadosh, Bernard S; Birs, Antoinette S; Flattery, Erin; Stachel, Maxine; Hong, Kimberly N; Xia, Yuhe; Gidea, Claudia; Aslam, Saima; Razzouk, Louai; Saraon, Tajinderpal; Goldberg, Randal; Rao, Shaline; Pretorius, Victor; Moazami, Nader; Smith, Deane E; Adler, Eric D; Reyentovich, Alex
BACKGROUND:Recent studies suggest the transplantation of Hepatitis C (HCV) hearts from viremic donors is associated with comparable 1 year survival to nonviremic donors. Though HCV viremia is a known risk factor for accelerated atherosclerosis, data on cardiac allograft vasculopathy (CAV) outcomes are limited. We compared the incidence of CAV in heart transplant recipients from HCV viremic donors (nucleic acid amplification test positive; NAT+) compared to non-HCV infected donors (NAT-). METHODS:We retrospectively reviewed annual coronary angiograms with intravascular ultrasound from April 2017 to August 2020 at two large cardiac transplant centers. CAV was graded according to ISHLT guidelines. Maximal intimal thickness (MIT) ≥ 0.5 mm was considered significant for subclinical disease. RESULTS:Among 270 heart transplant recipients (mean age 54; 77% male), 62 patients were transplanted from NAT+ donors. CAV ≥ grade 1 was present in 8.8% of the NAT+ versus 16.8% of the NAT- group at 1 year, 20% versus 28.8% at 2 years, and 33.3% versus 41.5% at 3 years. After adjusting for donor age, donor smoking history, recipient BMI, recipient, hypertension, and recipient diabetes, NAT+ status did not confer increased risk of CAV (HR.80; 95% CI.45-1.40, p = 0.43) or subclinical IVUS disease (HR.87; 95% CI.58-1.30, p = 0.49). Additionally, there was no difference in the presence of rapidly progressive lesions on IVUS. CONCLUSION/CONCLUSIONS:Our data show that NAT+ donors conferred no increased risk for early CAV or subclinical IVUS disease following transplantation in a cohort of heart transplant patients who were treated for HCV, suggesting the short-term safety of this strategy to maximize the pool of available donor hearts.
PMID: 38545881
ISSN: 1399-0012
CID: 5645082

Commentary: United Network for Organ Sharing policies work, but progress only occurs at the speed of a snail: A need for expeditious adjustments [Editorial]

Reyentovich, Alex; Smith, Deane; Moazami, Nader
PMID: 37354946
ISSN: 1097-685x
CID: 5543072

Nonischemic Cardiomyopathy With Myocardial Calcinosis Masquerading as Cardiac Amyloidosis

Singh, Arushi; Kadosh, Bernard S; Grossman, Kelsey; Donnino, Robert; Narula, Navneet; Zhou, Fang; DiVita, Michael; Smith, Deane E; Moazami, Nader; Chang, Stephanie H; Angel, Luis F; Reyentovich, Alex
PMID: 37492988
ISSN: 1941-3297
CID: 5620132

Pig-to-human heart xenotransplantation in two recently deceased human recipients

Moazami, Nader; Stern, Jeffrey M; Khalil, Karen; Kim, Jacqueline I; Narula, Navneet; Mangiola, Massimo; Weldon, Elaina P; Kagermazova, Larisa; James, Les; Lawson, Nikki; Piper, Greta L; Sommer, Philip M; Reyentovich, Alex; Bamira, Daniel; Saraon, Tajinderpal; Kadosh, Bernard S; DiVita, Michael; Goldberg, Randal I; Hussain, Syed T; Chan, Justin; Ngai, Jennie; Jan, Thomas; Ali, Nicole M; Tatapudi, Vasishta S; Segev, Dorry L; Bisen, Shivani; Jaffe, Ian S; Piegari, Benjamin; Kowalski, Haley; Kokkinaki, Maria; Monahan, Jeffrey; Sorrells, Lori; Burdorf, Lars; Boeke, Jef D; Pass, Harvey; Goparaju, Chandra; Keating, Brendan; Ayares, David; Lorber, Marc; Griesemer, Adam; Mehta, Sapna A; Smith, Deane E; Montgomery, Robert A
Genetically modified xenografts are one of the most promising solutions to the discrepancy between the numbers of available human organs for transplantation and potential recipients. To date, a porcine heart has been implanted into only one human recipient. Here, using 10-gene-edited pigs, we transplanted porcine hearts into two brain-dead human recipients and monitored xenograft function, hemodynamics and systemic responses over the course of 66 hours. Although both xenografts demonstrated excellent cardiac function immediately after transplantation and continued to function for the duration of the study, cardiac function declined postoperatively in one case, attributed to a size mismatch between the donor pig and the recipient. For both hearts, we confirmed transgene expression and found no evidence of cellular or antibody-mediated rejection, as assessed using histology, flow cytometry and a cytotoxic crossmatch assay. Moreover, we found no evidence of zoonotic transmission from the donor pigs to the human recipients. While substantial additional work will be needed to advance this technology to human trials, these results indicate that pig-to-human heart xenotransplantation can be performed successfully without hyperacute rejection or zoonosis.
PMID: 37488288
ISSN: 1546-170x
CID: 5595152

HHV-6 Myocarditis Progressing to Ventricular Standstill Requiring Cardiac Transplant

Golob, S; Nazeer, H; Kadosh, B; Goldberg, R; Narula, N; Moazami, N; Rao, S; Reyentovich, A
Human herpesvirus-6 (HHV-6) is an increasingly recognized cause of myocarditis. We present the case of a 46-year-old woman who presented with fulminant HHV-6 myocarditis requiring heart transplantation. (Level of Difficulty: Advanced.)
ISSN: 2666-0849
CID: 5514222

The Society of Thoracic Surgeons Intermacs 2022 Annual Report: Focus on 2018 Heart Transplant Allocation System

Yuzefpolskaya, Melana; Schroeder, Sarah E; Houston, Brian A; Robinson, Monique R; Gosev, Igor; Reyentovich, Alex; Koehl, Devin; Cantor, Ryan; Jorde, Ulrich P; Kirklin, James K; Pagani, Francis D; D'Alessandro, David A
The thirteenth annual report from The Society of Thoracic Surgeons (STS) Interagency Registry for Mechanically Assisted Circulatory Support (Intermacs) highlights outcomes for 27,314 patients receiving continuous flow durable left ventricular assist devices (LVAD) over the last decade (2012-2021). In 2021, 2,464 primary LVADs were implanted, representing a 23.5% reduction in the annual volume compared to peak implantation in 2019 and an ongoing trend from the prior year. This decline is likely a reflection of the untoward effects of the COVID-19 pandemic and the change in the US heart transplant allocation system in 2018. The last several years have been characterized by a shift in device indication and type with 81.1% of patients now implanted as destination therapy and 92.7% receiving an LVAD with full magnetic levitation in 2021. However, despite an older, more ill population being increasingly supported pre-implant with temporary circulatory devices in the recent (2017-2021) vs prior (2012-2016) eras, the 1- and 5-year survival continues to improve at 83.0% and 51.9%, respectively. The adverse events profile has also improved, with significant reduction in stroke, gastrointestinal bleeding, and hospital readmissions. Finally, we examined the impact of the change in heart transplant allocation system in 2018 on LVAD candidacy, implant strategy, and outcomes. In the competing outcomes analysis, the proportion of transplant eligible patients receiving a transplant has declined from 56.5% to 46.0% at 3 years, while the proportion remaining alive with ongoing support has improved from 24.1% to 38.1% at 3 years, underscoring the durability of the currently available technology.
PMID: 36462544
ISSN: 1552-6259
CID: 5383792

Donation after circulatory death heart transplantation using normothermic regional perfusion:The NYU Protocol

James, Les; LaSala, V Reed; Hill, Fredrick; Ngai, Jennie Y; Reyentovich, Alex; Hussain, Syed T; Gidea, Claudia; Piper, Greta L; Galloway, Aubrey C; Smith, Deane E; Moazami, Nader
OBJECTIVE/UNASSIGNED:This study aimed to evaluate the impact of cardiopulmonary bypass for thoraco-abdominal normothermic regional perfusion on the metabolic milieu of donation after cardiac death organ donors before transplantation. METHODS/UNASSIGNED:Local donation after cardiac death donor offers are assessed for suitability and willingness to participate. Withdrawal of life-sustaining therapy is performed in the operating room. After declaration of circulatory death and a 5-minute observation period, the cardiac team performs a median sternotomy, ligation of the aortic arch vessels, and initiation of thoraco-abdominal normothermic regional perfusion via central cardiopulmonary bypass at 37 °C. Three sodium chloride zero balance ultrafiltration bags containing 50 mEq sodium bicarbonate and 0.5 g calcium carbonate are infused. Arterial blood gas measurements are obtained every 15 minutes after every zero balance ultrafiltration bag is infused, and blood is transfused as needed to maintain hemoglobin greater than 8 mg/dL. Cardiopulmonary bypass is weaned with concurrent hemodynamic and transesophageal echocardiogram evaluation of the donor heart. The remainder of the procurement, including the abdominal organs, proceeds in a similar controlled fashion as is performed for a standard donation after brain death donor. RESULTS/UNASSIGNED:.001) . On average, donation after cardiac death donors received transfusions of 2.3 ± 1.5 units of packed red blood cells. Of the 18 donors who underwent normothermic regional perfusion, all hearts were deemed suitable for recovery and successfully transplanted, a yield of 100%. Other organs successfully recovered and transplanted include kidneys (80.6% yield), livers (66.7% yield), and bilateral lungs (27.8% yield). CONCLUSIONS/UNASSIGNED:The use of cardiopulmonary bypass for thoraco-abdominal normothermic regional perfusion is a burgeoning option for improving the quality of organs from donation after cardiac death donors. Meticulous intraoperative management of donation after cardiac death donors with a specific focus on improving their metabolic milieu may lead to improved graft function in transplant recipients.
PMID: 36820336
ISSN: 2666-2507
CID: 5509582

Long-term follow-up of acute and chronic rejection in heart transplant recipients from hepatitis C viremic (NAT+) donors

Stachel, Maxine W; Alimi, Marjan; Narula, Navneet; Flattery, Erin E; Xia, Yuhe; Ramachandran, Abhinay; Saraon, Tajinderpal; Smith, Deane; Reyentovich, Alex; Goldberg, Randal; Kadosh, Bernard S; Razzouk, Louai; Katz, Stuart; Moazami, Nader; Gidea, Claudia G
The long-term safety of heart transplants from hepatitis C viremic (NAT+) donors remains uncertain. We conducted a prospective study of all patients who underwent heart transplantation at our center from January 2018 through August 2020. Routine testing was performed to assess for donor-derived cell-free DNA, acute cellular rejection (ACR), antibody-mediated rejection (AMR), and cardiac allograft vasculopathy (CAV). Allograft dysfunction and mortality were also monitored. Seventy-five NAT- recipients and 32 NAT+ recipients were enrolled in the study. All NAT+ recipients developed viremia detected by PCR, were treated with glecaprevir/pibrentasvir at the time of viremia detection, and cleared the virus by 59 days post-transplant. Patients who underwent NAT testing starting on post-operative day 7 (NAT+ Group 1) had significantly higher viral loads and were viremic for a longer period compared with patients tested on post-operative day 1 (NAT+ Group 2). Through 3.5 years of follow-up, there were no statistically significant differences in timing, severity, or frequency of ACR in NAT+ recipients compared with the NAT- cohort, nor were there differences in noninvasive measures of graft injury, incidence or severity of CAV, graft dysfunction, or mortality. There were five episodes of AMR, all in the NAT- group. There were no statistically significant differences between Group 1 and Group 2 NAT+ cohorts. Overall, these findings underscore the safety of heart transplantation from NAT+ donors.
PMID: 36053676
ISSN: 1600-6143
CID: 5332222

Acute Myocarditis Associated With Desmosomal Gene Variants

Ammirati, Enrico; Raimondi, Francesca; Piriou, Nicolas; Sardo Infirri, Loren; Mohiddin, Saidi A; Mazzanti, Andrea; Shenoy, Chetan; Cavallari, Ugo A; Imazio, Massimo; Aquaro, Giovanni Donato; Olivotto, Iacopo; Pedrotti, Patrizia; Sekhri, Neha; Van de Heyning, Caroline M; Broeckx, Glenn; Peretto, Giovanni; Guttmann, Oliver; Dellegrottaglie, Santo; Scatteia, Alessandra; Gentile, Piero; Merlo, Marco; Goldberg, Randal I; Reyentovich, Alex; Sciamanna, Christopher; Klaassen, Sabine; Poller, Wolfgang; Trankle, Cory R; Abbate, Antonio; Keren, Andre; Horowitz-Cederboim, Smadar; Cadrin-Tourigny, Julia; Tadros, Rafik; Annoni, Giuseppe A; Bonoldi, Emanuela; Toquet, Claire; Marteau, Lara; Probst, Vincent; Trochu, Jean Noël; Kissopoulou, Antheia; Grosu, Aurelia; Kukavica, Deni; Trancuccio, Alessandro; Gil, Cristina; Tini, Giacomo; Pedrazzini, Matteo; Torchio, Margherita; Sinagra, Gianfranco; Gimeno, Juan Ramón; Bernasconi, Davide; Valsecchi, Maria Grazia; Klingel, Karin; Adler, Eric D; Camici, Paolo G; Cooper, Leslie T
BACKGROUND:The risk of adverse cardiovascular events in patients with acute myocarditis (AM) and desmosomal gene variants (DGV) remains unknown. OBJECTIVES/OBJECTIVE:The purpose of this study was to ascertain the risk of death, ventricular arrhythmias, recurrent myocarditis, and heart failure (main endpoint) in patients with AM and pathogenic or likely pathogenetic DGV. METHODS:In a retrospective international study from 23 hospitals, 97 patients were included: 36 with AM and DGV (DGV[+]), 25 with AM and negative gene testing (DGV[-]), and 36 with AM without genetics testing. All patients had troponin elevation plus findings consistent with AM on histology or at cardiac magnetic resonance (CMR). In 86 patients, CMR changes in function and structure were re-assessed at follow-up. RESULTS:In the DGV(+) AM group (88.9% DSP variants), median age was 24 years, 91.7% presented with chest pain, and median left ventricular ejection fraction (LVEF) was 56% on CMR (P = NS vs the other 2 groups). Kaplan-Meier curves demonstrated a higher risk of the main endpoint in DGV(+) AM compared with DGV(-) and without genetics testing patients (62.3% vs 17.5% vs 5.3% at 5 years, respectively; P < 0.0001), driven by myocarditis recurrence and ventricular arrhythmias. At follow-up CMR, a higher number of late gadolinium enhanced segments was found in DGV(+) AM. CONCLUSIONS:Patients with AM and evidence of DGV have a higher incidence of adverse cardiovascular events compared with patients with AM without DGV. Further prospective studies are needed to ascertain if genetic testing might improve risk stratification of patients with AM who are considered at low risk.
PMID: 36175056
ISSN: 2213-1787
CID: 5334522