Arrhythmogenic cardiomyopathy (ACM) is an arrhythmogenic disorder of the myocardium not secondary to ischemic, hypertensive, or valvular heart disease. ACM incorporates a broad spectrum of genetic, ...systemic, infectious, and inflammatory disorders. This designation includes, but is not limited to, arrhythmogenic right/left ventricular cardiomyopathy, cardiac amyloidosis, sarcoidosis, Chagas disease, and left ventricular noncompaction. The ACM phenotype overlaps with other cardiomyopathies, particularly dilated cardiomyopathy with arrhythmia presentation that may be associated with ventricular dilatation and/or impaired systolic function. This expert consensus statement provides the clinician with guidance on evaluation and management of ACM and includes clinically relevant information on genetics and disease mechanisms. PICO questions were utilized to evaluate contemporary evidence and provide clinical guidance related to exercise in arrhythmogenic right ventricular cardiomyopathy. Recommendations were developed and approved by an expert writing group, after a systematic literature search with evidence tables, and discussion of their own clinical experience, to present the current knowledge in the field. Each recommendation is presented using the Class of Recommendation and Level of Evidence system formulated by the American College of Cardiology and the American Heart Association and is accompanied by references and explanatory text to provide essential context. The ongoing recognition of the genetic basis of ACM provides the opportunity to examine the diverse triggers and potential common pathway for the development of disease and arrhythmia.
Liver transplant is a widely accepted therapy for end-stage liver disease. With advances in our understanding of transplant, candidates are increasingly older with more cardiac comorbidities. ...Cardiovascular disease also represents a leading cause of morbidity and mortality posttransplant.
Preoperative cardiac risk stratification and treatment may improve short-term and long-term outcomes after liver transplant. Importantly, the appropriate frequency of surveillance has not been defined. Optimal timing of cardiac intervention in end-stage liver disease is likewise uncertain.
The approach to risk stratification of cardiovascular disease in end-stage liver disease is outlined, incorporating the AHA/ACC scientific statement on evaluation of cardiac disease in transplant candidates and more recent expert consensus documents. Further study is needed to clarify the ideal timing and approach for cardiovascular interventions.
Arrhythmogenic cardiomyopathy (ACM) is an arrhythmogenic disorder of the myocardium not secondary to ischemic, hypertensive, or valvular heart disease. ACM incorporates a broad spectrum of genetic, ...systemic, infectious, and inflammatory disorders. This designation includes, but is not limited to, arrhythmogenic right/left ventricular cardiomyopathy, cardiac amyloidosis, sarcoidosis, Chagas disease, and left ventricular noncompaction. The ACM phenotype overlaps with other cardiomyopathies, particularly dilated cardiomyopathy with arrhythmia presentation that may be associated with ventricular dilatation and/or impaired systolic function. This expert consensus statement provides the clinician with guidance on evaluation and management of ACM and includes clinically relevant information on genetics and disease mechanisms. PICO questions were utilized to evaluate contemporary evidence and provide clinical guidance related to exercise in arrhythmogenic right ventricular cardiomyopathy. Recommendations were developed and approved by an expert writing group, after a systematic literature search with evidence tables, and discussion of their own clinical experience, to present the current knowledge in the field. Each recommendation is presented using the Class of Recommendation and Level of Evidence system formulated by the American College of Cardiology and the American Heart Association and is accompanied by references and explanatory text to provide essential context. The ongoing recognition of the genetic basis of ACM provides the opportunity to examine the diverse triggers and potential common pathway for the development of disease and arrhythmia.
BACKGROUNDDonor specific HLA antibodies (DSA) are associated with increased rates of rejection and of graft failure in cardiac transplantation. The goal of this study was to determine the association ...of preformed and posttransplant development of newly detected DSA (ndDSA) with antibody mediated rejection (AMR) and characterize the clinical relevance of complement activating DSA in heart allograft recipients.
METHODSThe study included 128 adult and 48 pediatric heart transplant patients transplanted between 2010 to 2013. Routine posttransplant HLA antibody testing was performed by IgG-Single Antigen Bead (SAB) test. The C3d-SAB assay was used to identify complement activating antibodies. Rejection was diagnosed using ISHLT criteria.
RESULTSIn this study, 22 patients were transplanted with preexisting DSA, and 43 patients developed ndDSA posttransplant. Pretransplant (p<0.05) and posttransplant (p<0.001) ndDSA were associated with higher incidence of AMR. Patients with C3d+DSA had significantly higher incidence of AMR compared to patients with no DSA (p<0.001) or patients with C3d-DSA (p=0.02). Nine out of 25 (36%) patients with AMR developed transplant coronary artery disease (TCAD) compared to 17/107 (15.9%) patients without AMR (p<0.05). Among the 47 patients who received ventricular assistant device (VAD), 7/9 VAD+ patients with preformed DSA experienced AMR compared to 7/38 VAD+ patients without preformed DSA, indicating presensitization to donor HLA significantly increased the risk of AMR (p<0.015).
CONCLUSIONPreformed and posttransplant ndDSA were associated with AMR. C3d+DSA correlates with complement deposition on the graft and higher risk of AMR which may permit the application of personalized immunotherapy targeting the complement pathway.
Primary graft dysfunction (PGD) is common early postheart transplantation; however, use of standardized definitions remains inconsistent. This review focuses on understanding the incidence, ...classification, risk factors, and management of PGD.
The incidence and mortality of PGD in heart transplant varies considerably in the published literature ranging from 1.0% to 31% and 3% to 75%, respectively. There is also considerable variation in management strategies with current data favoring early intervention.
PGD in heart transplantation remains a challenging problem associated with significant mortality and morbidity. There is need for a consistent and accessible definition to better define associated risk factors and optimize management strategies.
Restrictive cardiomyopathy (RCM) represents a spectrum of disorders with a common physiology but divergent etiologies. RCM commonly leads to progressive heart failure and the need for heart ...transplantation (HTx). Pediatric RCM is a more homogeneous disorder with post-HTx outcomes comparable to those for non-RCM patients. However, post-HTx outcomes in adult RCM patients have not been studied to date.
Demographic, clinical and survival outcomes of 38,190 adult HTx-only recipients from 1987 to 2010 were acquired from the registry of the United Network of Organ Sharing. The study population included 544 RCM patients (1.4%) and 37,646 non-RCM patients (98.6%). RCM diagnoses included idiopathic (n = 227, 42%), amyloid (n = 142, 26%), sarcoid (n = 81, 15%), radiation/chemotherapy (XRT) (n = 35, 6%) and other (n = 59, 11%).
Follow-up began at the time of HTx (74±64 months). During the follow-up period, 224 (41%) patients in the RCM group died, whereas 18,791 (50%) in the non-RCM group died. Crude 1-, 5- and 10-year survival for RCM patients was 84%, 66% and 45%, and for non-RCM patients was 85%, 70% and 50%, respectively. The overall unadjusted hazard ratio of RCM vs non-RCM for all-cause mortality was 1.07 (confidence interval CI 0.93 to 1.22). Multivariate Cox proportional hazards regression analysis yielded a hazard ratio of 1.06 (CI 0.91 to 1.25). RCM subgroup analysis showed decreased survival at 1, 5 and 10 years in the XRT (71%, 47% and 32%) and amyloid (79%, 47% and 28%) patient groups. The unadjusted hazard ratio for the XRT and amyloid subgroups vs RCM for all-cause mortality was 1.81 (p = 0.002) and 1.85 (p = 0.0004), respectively.
Outcomes for RCM patients post-HTx are comparable to those of non-RCM patients. However, RCM subgroup analysis suggests increased mortality for XRT and amyloid subgroups. Further analysis is warranted to understand the contributing factors.
Background
Model of End‐Stage Liver Disease eXcluding INR (MELD‐XI) at cardiac transplant has demonstrated prognostic survival utility, but has not been specifically validated in adult congenital ...heart disease (ACHD) in a registry study.
Methods
Adults undergoing first‐time orthotopic heart transplant from 2005 to 2015 in the United Network for Organ Sharing (UNOS) registry were examined in parallel: ACHD (n = 543), ischemic‐dilated cardiomyopathy (IDCM, n = 6954) and valvular heart disease (VHD, n = 355). Our primary endpoint was a composite of death, graft failure, and retransplantation assessed at 3 months (early), and those with freedom from early endpoint were reassessed at 5 years (late). Interactions between hepatorenal indices and waitlist time were examined. Secondary outcomes relating to long‐term morbidity were assessed at late endpoint. Freedom from endpoint analysis in ACHD at clinically relevant endpoints was also conducted.
Results
Model of End‐Stage Liver Disease eXcluding INR score at transplant associated with an increased risk of early endpoint in all cohorts. At late endpoint, bilirubin level associated with increased risk uniquely in ACHD.
Conclusions
Model of End‐Stage Liver Disease eXcluding INR holds prognostic application to ACHD in early time points and demonstrates unique waitlist interactions. Transplant bilirubin level may hold significance in long‐term risk stratification of the ACHD population. Time on waitlist is an important consideration to contextualize these values.
Patients with end-stage heart failure often present with concomitant end-stage renal or end-stage liver disease requiring transplantation. There are limited data regarding the risks, benefits and ...long-term outcomes of heart-kidney (HKT) and heart-liver transplantation (HLT), and guidelines are mainly limited to expert consensus statements.
The incidence of HKT and HLT has steadily increased in recent years with favourable outcomes. Both single-centre and large database studies have shown benefits of HKT/HLT through improved survival, freedom from dialysis and lower rates of rejection and coronary allograft vasculopathy. Current guidelines are institution dependent and controversial due to the ethical considerations surrounding multiorgan transplantation (MOT).
MOT is an effective and necessary option for patients with end-stage heart and kidney/liver failure. MOT is ethically permissible, and efforts should be made to consider eligible patients as early as possible to limit morbidity and mortality. Further research is needed regarding appropriate listing criteria and long-term outcomes.
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