Search

There are conflicting data related to the use of implantable cardioverter-defibrillators (ICDs) in patients supported with continuous-flow ventricular assist devices (LVADs). Several US studies have shown a neutral effect on mortality, whereas a recent European study suggested better outcomes in LVAD patients who have an ICD. We aimed to investigate the benefit of ICDs in patients bridged to transplant with a LVAD in the TRans-Atlantic registry on VAd and TrAnsplant (TRAViATA). Data from consecutive patients that received a LVAD as a bridge to transplantation between January 2008 and April 2017 were collected in 7 EU (n=299) and 3 US centers (n=225). Survival analysis was performed using Kaplan-Meier and Cox proportion hazard regression analysis. Patients were censored at time of transplantation. Patients with an ICD at the time of LVAD (n=350) implantation were older (56 vs. 53 years, p<0.05), less likely to be female (13% vs. 21%, p<0.05) and to have ischemic cardiomyopathy (39% vs. 52%, p<0.05) compared to patients without an ICD (n=174). The proportion of patients with an ICD/LVAD was 66% in the EU and 68% in the US (p=0.49). Overall survival after LVAD was 86% at one year and 76% at two years. There was no difference in survival in patients with and without an ICD (Figure). These results were consistent when analyzing patients from each continent separately (Figure). In univariate Cox-regression age at time of implant and INTERMACS profile were associated with mortality (p<0.05) whereas presence of ICD was not. Sensitivity analysis excluding patients who received their ICD (n=25) after LVAD confirmed the results. In this retrospective study of LVAD patients from the EU and US ICD therapy conferred no survival advantage irrespective of geographic location. A prospective, randomized, trial of LVAD patients who have an existing ICD randomized to tachyarrhythmia therapy discontinuation is needed to validate these findings. [ABSTRACT FROM AUTHOR]

Purpose Highly effective direct-acting antiviral therapy for hepatitis C virus (HCV) allows the use of organs from viremic, nucleic acid test-positive (NAT+) donors in non-HCV-infected recipients. This opportunity potentially expands donor pool and decreases waitlist time. This study aims to examine the impact of utilizing HCV NAT+ donor heart on heart transplant (HTx) waitlist time. Methods We retrospectively analyzed 126 patients who were listed for HTx from April 2016 through April 2018 at our center. These patients were separated into 2 periods centered on April 27, 2017 when transplantation of HCV NAT+ donor hearts into non-HCV-infected recipients began. Results Baseline patient characteristics were similar between both periods. In Period 1, 74 patients were on HTx waitlist with 52 of them ultimately transplanted, whereas in Period 2, 52 patients were listed and 36 of them transplanted. Fifteen patients were transplanted with HCV NAT+ donor hearts since April 27, 2017. Transplant rate increased from 0.83 person-year in Period 1 to 2.31 person-year in Period 2 (p<0.0001). There was no significant difference between the two periods with respect to waitlist mortality rate (p=0.29), length of post-transplant hospital stay (p=0.51), and short-term post-transplant survival (p=0.359). Median waiting time from listing to transplant was 140.5 days in Period 1 and 40 days in Period 2 (p=0.05). Additionally, median waitlist time was reduced for all blood types (p=0.006) and United Network for Organ Sharing (UNOS) listing status (p=0.015) in Period 2. Conclusion Our single-center analysis demonstrates that utilization of HCV NAT+ donor heart has resulted in an increase in transplant rate and a reduction in HTx waitlist time. Further extended follow-up is needed to determine long-term safety and efficacy of this strategy. [ABSTRACT FROM AUTHOR]

Danon disease is a rare X-linked dominant cardioskeletal myopathy caused by mutations in the lysosome-associated membrane protein-2 (LAMP-2) gene. Currently, there are no disease modifying therapies for Danon disease. The purpose of this study is to characterize individuals with Danon disease who undergo cardiac transplantation and describe their outcomes. Retrospective data of individuals with Danon disease who underwent cardiac transplantation (N=16) were obtained from 6 centers. Post-transplantation outcomes included survival, re-transplantation, and episodes of acute rejection within the first year. Analysis of preoperative patient characteristics was stratified by sex. Because the study was not limited to adults, z-scores were calculated for echocardiographic parameters to allow for comparisons between groups. Median follow up time in this dataset was 2.35 years (IQR: 0.8 years - 8.3 years). Males had higher preoperative AST and ALT (p=0.02) compared to females and were more likely than females to have muscular symptoms (p=0.037). From a cardiac perspective, pre-transplant EF was lower in females compared to males (p=0.047), while left ventricular hypertrophy was more prominent in males compared to females (p<0.001). [TABLE] There was 1 death (female) in the entire cohort (occurred 5 days post-transplant) and no re-transplants. At 1 year, there were no episodes of antibody mediated rejection, and 5 episodes of acute cellular rejection (33%) in those who survived to discharge after transplantation. Danon disease is a progressive multisystem disease. Understanding the individuals who undergo cardiac transplantation and their outcomes is critical for informing patient selection and guiding decisions regarding organ allocation. Short term post-transplant outcomes are acceptable in Danon disease and suggest that cardiac transplantation is a treatment option for these patients. [ABSTRACT FROM AUTHOR]

Danon Disease (DD) is a rare X-linked disorder due to mutations in the Lysosomal Associated Membrane Protein 2 (LAMP-2) gene. Patients present with cardiac manifestations of hypertrophic cardiomyopathy (HCM) and dilated cardiomyopathy (DCM). The goal of this study is to characterize the longitudinal progression of hypertrophic phenotypes and descriptions of left ventricular strain in DD. We created an international registry including 44 adult and pediatric patients with DD. A retrospective review of pre-transplant echocardiographic data was performed. Medical records were supplied by the patients/families. Of 44 patients with DD, longitudinal echocardiographic data from 30 patients were available. Of these, 15 patients (50%) were male. Mean age of diagnosis of cardiomyopathy was younger in males compared to females (10.6 vs 28.3 yrs, p = 0.0004). Baseline echocardiograms demonstrated generally preserved ejection fraction on initial echocardiogram in both males and females (64.9% vs 60.8%, p=0.458). However, baseline measures of left ventricular wall thickness was abnormal; IVSDd thickness and LVPWd thickness were elevated in both males and females [12.73 vs 10.73 mm (p=0.364) and 11.14 vs 10.82 mm (p=0.871), respectively]. LV strain analysis was performed in 12 patients (50% male) during baseline evaluation. Abnormal strain patterns were present in 10 (83.3%) patients. Global LV strain was reduced (mean 11.8%). Severely reduced strain patterns predominantly affected basal ventricular segments (mean 8.5%) with relative apical sparing (mean 16.6%). Mean longitudinal follow up time with echocardiography was 5.4 yrs. On follow up, males demonstrated greater LV hypertrophy compared to females [IVSDd 19.34 vs 14.74 mm (p=0.27), LVPW thickness 18.3 vs 11.3 mm (p=0.047)]. Our data show DD affects males and females unequally. Males are more prone to develop severe HCM both earlier and more rapidly. Females exhibit variable presentation patterns with HCM or DCM, and disease course appears to be more protracted. Strain pattern in DD is similar to cardiac amyloid with overall reduction in global LV strain and relative apical sparing. Variability in presentation and lack of specific echocardiographic findings necessitates early clinical recognition and confirmatory genetic testing. [ABSTRACT FROM AUTHOR]

Purpose Danon Disease (DD) is a rare genetic disorder that affects the cardiac, neurologic, and muscular systems in both men and women. Subjects affected typically develop hypertrophic cardiomyopathy (HCM) and end-stage heart failure requiring transplantation. DD is an X-linked disorder with women typically suffering milder disease presentations that develop later in life. Methods A global retrospective registry for DD was created to understand the disease's natural history. Data including the description of clinical features and age of symptom onset was collected from patients across the world (United States, United Kingdom, and Australia). Information was obtained via medical records and questionnaires that were collected from patients and/or their family. Results The registry includes a total of 33 patients with 54.5% being female. The major demographics of patients were Caucasian (96.9%) and from the United States (78.8%). Impartial of the affected organs, males experienced earlier symptoms compared to females (5.5 (2.0-12.0) vs 16.0 (11.5-27.0) years of age, p = 0.002) which resulted in an earlier DD diagnosis in males at (8.0 (4.0-12.0) vs 26.5 (13.0-41.0) years of age, p = 0.023). The frequency of HCM was higher in males as compared to females, (93.3% vs 44.4%) while females had a higher frequency of tachyarrhythmias compared to males (61.1% vs 13.3%). Dilated cardiomyopathy (DCM) was absent in males, however was the first symptom of cardiac disease in two female patients (11.1%). Four males (26.7%) and seven females (38.8%) underwent heart transplantation. Death occurred in one (5.6%) female due to post-transplant complications. Neurological symptoms, primarily manifesting as cognitive impairment were documented in 86.7% of males and 61.1% of females and had a similar age of onset (5.0 (4.0-10.0) vs 8.0 (7.0-15.0) years of age, p = 1). 87.9% of subjects presented with symptomatic skeletal myopathy which was more frequent in males (86.7% vs 64.3%). Conclusion Although DD is an X-linked disorder, significant cardiac morbidity develops in women. Females have a higher prevalence of DCM, which may be secondary to delayed symptom presentation and diagnosis. While DD is a rare diagnosis, healthcare providers should be aware that later clinical manifestations occur in women and should be considered in the differential diagnosis of heart failure. [ABSTRACT FROM AUTHOR]

Purpose Patients receiving LVADs are at risk for post-operative right ventricular (RV) failure. Cardiac CT angiography (CTA) provides 3D isotropic spatial resolution and blood pool-myocardial delineation and can be used to assess ventricular volumes. We sought to evaluate whether right heart catheterization (RHC) and echocardiographic (echo) parameters of RV structure and function correlate with CT-derived parameters - specifically, RV ejection fraction RVEF, RV stroke volume index RVSVI, RV to LV end diastolic ratio RV-LVEDR, and RV end diastolic volume index RVEDVI. Methods 11 LVAD candidates (age 55 ± 12, 10 males) underwent cardiac CTA between 9/27/2017 and 10/16/2018. All patients had contemporaneous (within 7 days) echo examinations and 7 patients had contemporaneous RHCs. Pulmonary artery pulsatility index PAPI, RV stroke work index RVSWI, and the ratio of right atrial to pulmonary capillary wedge pressure RAP/PCWP were obtained from RHC reports and RV size, global function, and tricuspid annular plane systolic excursion (TAPSE) were obtained from echo reports. Ventricle size was graded as small, normal, mild, moderate, or severely enlarged, and ventricle function as normal or depressed. Pearson's correlation coefficient (ρ) was calculated for each relationship. For echo-derived ventricular function, an unpaired t-test (p<0.05) was performed. Results Structurally, echo derived RV size correlated moderately with CT RV EDVI and RAP/PCWP correlated strongly with RV-LVEDR. Functionally, RV SWI correlated well with RV EF and RV SVI. PAPI poorly correlated to RV EF and RV SVI. Echo-based characterization of global RV function was not associated with any CT-derived measures (p > 0.1). Conclusion CT-derived RV parameters may provide complementary information to hemodynamic and echo assessment, especially RAP/PCWP and 2D echo parameters, and when combined could lead to comprehensive assessment of RV function. Further research is required to examine the relationship between TAPSE and RV EF. [ABSTRACT FROM AUTHOR]