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Danon disease (DD) is a rare X-linked dominant cardioskeletal myopathy caused by mutations in the lysosome-associated membrane protein-2 (LAMP-2) gene that is usually lethal without cardiac transplantation. The purpose of this study was to characterize post-transplant outcomes in a large cohort of patients with DD who underwent cardiac transplantation.The clinical phenotype and outcome data of patients with DD who underwent cardiac transplantation (n = 38; 19 males and 19 females) were obtained from 8 centers. Study outcomes included graft survival, defined as death or retransplantation, and episodes of acute cellular and antibody-mediated rejection and cardiac allograft vasculopathy at 1 year.Median follow-up time after transplantation for the entire cohort was 4.4 years (IQR: 1.5-12.8 years). The median age at transplant for the cohort was 20.2 years (15.8-27.9 years), with no difference in age between sexes. Median pretransplant left-ventricular ejection fraction for the entire cohort was 30% (range 11%-84%). Males had higher pretransplant aspartate aminotransferase, alanine aminotransferase and creatine phosphokinase levels than females (P0.001). There were 2 deaths in the entire cohort and 2 retransplants. There was no difference in actuarial graft survival between males and females (P = 0.8965); the estimated graft survival was 87.1% (95%CI: 63.6%-95.9%) at 5 years. One episode (2.7%) of antibody-mediated rejection, grade 2, and 7 episodes (19%) of acute cellular rejection, grade 2 or 3, were reported in patients who survived to discharge (6 females and 1 male; P = 0.172).Heart transplantation outcomes are acceptable in DD with high probabilities of 5-year graft survival for males and females suggesting that cardiac transplantation is an effective treatment option for DD patients.

A 40-year-old man with history of prior coronavirus disease 2019 (COVID-19) infection developed pleuritic chest pain 3 days after receiving the first dose of the BNT162b2 mRNA vaccine. Echocardiography results were significant for mild dysfunction and left ventricular hypertrophy. Cardiac magnetic resonance imaging showed myocardial edema as well as delayed enhancement in the inferior wall of the basal left ventricular myocardium, suggestive of acute myocarditis. This case describes the work-up, diagnosis, risk-stratification, and management of acute myocarditis post BNT162b2 mRNA vaccine.

Danon disease (DD) is a rare X-linked autophagic vacuolar myopathy that affects the lysosome-associated membrane protein ( LAMP ) 2 gene and causes severe cardiac manifestations, frequently progressing to end-stage heart failure ([1][1]). The differential diagnosis includes many different diseases

BACKGROUND Fulminant myocarditis (FM) is a form of acute myocarditis characterized by severe left ventricular systolic dysfunction requiring inotropes and/or mechanical circulatory support. A single-center study found that a patient with FM had better outcomes than those with acute nonfulminant myocarditis (NFM) presenting with left ventricular systolic dysfunction, but otherwise hemodynamically stable. This was recently challenged, so disagreement still exists. OBJECTIVES This study sought to provide additional evidence on the outcome of FM and to ascertain whether patient stratification based on the main histologic subtypes can provide additional prognostic information. METHODS A total of 220 patients (median age 42 years, 46.3% female) with histologically proven acute myocarditis (onset of symptoms

Background Given conflicting findings of previous studies, much remains to be understood regarding a volume-outcomes relationship (VOR) in transcatheter aortic valve replacement (TAVR). Objectives The purpose of this study was: 1) to determine if, after the initial learning curve (LC), a VOR for balloon-expandable (BE) TAVR persisted; and 2) to determine if LCs and VORs differed across different device generations. Methods Data collected by the TVT registry for BE valve implants from November 2011 through January 2017 were included in this analysis (n = 61,949). Primary outcomes included 30-day all-cause mortality, stroke, and major vascular complications. For each center, all implants were ordered chronologically according to case sequence number (CS#). To determine where the learning curve terminated (LCT), a grid search analysis was applied across a range of CS# from 10 to 300 by increments of 1. After LCT, the VOR was assessed by examining case volume/month by center. This analysis was performed separately for: 1) all BE valve types; 2) Sapien 3 (S3) only; and 3) S3 in BE valve naive sites. Results In experience with all commercially available BE valve types, there was an initial LC that terminates around case #201. After the initial LC, a volume–outcomes relationship was no longer evident. In analysis limited to S3, there was no demonstrable LC or VOR. Likewise, there was no demonstrable LC or VOR with S3 for BE valve naive sites. Conclusions After a case experience of 200 cases, there was LCT; subsequent to initial learning, a VOR was no longer evident. In the S3-only analysis, there was no LC or no demonstrable VOR. With current-generation BE-TAVR, centers should expect to achieve consistently excellent outcomes even during early case experience.

Diabetes, hyperlipidemia, and hypertension are modifiable risk factors that predict cardiovascular disease events. The effect of these risk factors on incident cardiovascular disease increases with progressively higher levels of glucose, low-density lipoprotein cholesterol, and blood pressure. The thresholds for initiating treatment of these modifiable risk factors and the optimal goals of risk factor modification are a focus of primary prevention research. Although an aggressive approach is appealing, adverse events may occur, and potential physiological barriers may exist. This paper discusses primary prevention of coronary heart disease that may be achieved through modification of diabetes, hyperlipidemia, and hypertension by summarizing current guidelines and pertinent clinical trial data from intervention trials that included a primary prevention cohort.

Purpose Danon Disease is a rare X-linked myopathy caused by defects in the lysosome-associated membrane 2 (LAMP2) gene. It is strongly associated with hypertrophic cardiomyopathy as well as Wolff-Parkinson-White (WPW) syndrome, but the prevalence of WPW in Danon is unclear. This study examined the prevalence of WPW and associated outcomes in a cohort of patients with Danon Disease. Methods Records were reviewed for 41 patients with confirmed Danon Disease enrolled in a previously described retrospective registry, 31 of which had at least one electrocardiogram (EKG) on file. EKGs were assessed for the presence of WPW, as well as left and right ventricular hypertrophy (LVH, RVH), or both. WPW was defined as a PR interval ≤ 120 milliseconds, QRS interval ≥120 milliseconds, and delta wave. Charts were reviewed for internal cardiac defibrillator (ICD) placement, heart transplantation, and death. These outcomes were evaluated between patients with and without WPW on EKG using chi-square analysis, with p Results EKG criteria for LVH, RVH or both was met in 10 of the 11 WPW patients, compared to 9 of the 20 without WPW (p=0.012). ICD devices were present in 9 of the 11 WPW patients, 3 of which were placed for secondary prevention, compared to 9 of the 20 patients without WPW (p=0.119). Six of the 11 WPW patients eventually underwent heart transplant, compared to 4 of the 20 without WPW (p=0.058). Only one death was noted in this cohort, and was determined to not be cardiac related. Conclusion In a cohort of patients with Danon Disease, approximately one-third had WPW syndrome on EKG. Those with WPW on EKG had higher rates of EKG criteria for LVH, RVH or both and were more likely to require advanced therapies such as ICD placement and cardiac transplant. Larger studies involving WPW in Danon Disease should seek to better characterize these outcomes.

PURPOSE: 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). METHODS: 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. RESULTS: Patients with an ICD at the time of LVAD (n=350) implantation were older (56 vs. 53 years, p

Danon Disease is a rare X-linked myopathy associated with hypertrophic cardiomyopathy and Wolff-Parkinson-White (WPW) syndrome. The prevalence of WPW in Danon Disease is unclear. This study examined the prevalence of WPW and associated outcomes in a cohort of Danon Disease patients. EKGs of 28

Danon Disease (DD) is a rare X-linked disorder due to mutations in the Lysosomal Associated Membrane Protein 2 (LAMP-2) gene and causes severe cardiac manifestations. Longitudinal strain (LS) has emerged as a diagnostic and prognostic tool in various cardiomyopathies. A retrospective, international

Background Strokes are a devastating complication following left ventricular assist device (LVAD). The purpose of this study was to determine if a cerebral vascular accident (CVA) prior to LVAD placement in a bridge-to-transplant population is associated with worse outcomes compared to those without a history of prior CVA. Methods De-identified data were obtained from TRAVIATA (TransAtlantic Registry on VAD and Transplant) a retrospective, observational, multinational registry that included candidates for heart or heart and kidney transplant in whom an LVAD was implanted between January 2008 and April 2017. Only individuals greater than 15 years old who were implanted with either an HVAD (HeartWare, Minnesota, MN, USA) or a Heartmate II (HM2, Abbott, Pleasanton) were included (N=524). Patients were stratified into those with a CVA prior to LVAD ((+)CVA, n=55) and those without a CVA prior to LVAD ((-)CVA, n=469). The primary outcome was survival to transplant. Other outcomes of interest included death on support and post-LVAD CVA. Multivariable regression was performed in order to assess the simultaneous effect of multiple risk factors on transplant. Results The only difference between the preoperative characteristics studied between the study groups was a higher frequency of non-whites in those who had a CVA pre-LVAD (Table1). There were no differences in rates of transplant, death on support, or post-LVAD CVA between the study groups (Table2). In multivariable analysis, older age, higher BMI and preoperative DM were associated with decreased likelihood of transplant. Of interest, in those who were transplanted, time to transplant was longer in the (+)CVA group compared to the (-)CVA group (Table 2). Conclusions Stroke prior to LVAD was not associated with worse outcomes after LVAD, including decreased survival to transplant, death or stroke post-LVAD.

The risk factors for post-operative conduction disturbances after heart valve surgery requiring cardiac implantable electronic devices (CIED) remains poorly characterized. We conducted a retrospective cohort study using a mandatory statewide discharge database, New York's Statewide Planning and

ObjectiveThe goal of this study was to quantify the net increase in resource use associated with complications after isolated mitral valve surgery.MethodsDeidentified patient-level claims data on a random sample of mitral valve operations performed in the United States from January 1, 2006, to December 31, 2007, were obtained from the National Inpatient Sample (n = 16,788). Patients with major concomitant cardiac procedures were excluded from the analysis for a net sample size of 6297 patients. Risk-adjusted median total hospital costs and length of stay were analyzed by major complications, including pneumonia, sepsis, stroke, renal failure requiring hemodialysis, cardiac tamponade, myocardial infarction, gastrointestinal bleed, and venous thromboembolism.ResultsThere were a total of 1323 complication events that occurred in 1089 patients. The most common complication was pneumonia (n = 346, 5.5%), which was associated with a $29,692 increase in hospital costs and a 10.2-day increase in median length of stay (P

Over the past decade, minimally invasive (MI) mitral valve surgery has grown in popularity. The purpose of this study was to compare both short- and long-term outcomes of mitral valve repair and replacement performed through a MI versus traditional sternotomy (ST) incision using a propensity analysis approach to account for differences in baseline risk.From January 2000 to December 2008, a total of 1,121 isolated mitral valve operations were performed at our institution (548 ST, 573 MI). Data were retrospectively collected on all patients, and a logistic regression model was created to predict selection to a MI versus ST approach. Propensity scores were then generated based on the regression model and matched pairs created using 1:1 nearest neighbor matching. There were 382 matched pairs in the analysis for a total sample size of 764, or 68.2% of the original cohort. Major outcomes of interest included cardiopulmonary bypass time, cross-clamp time, hospital length of stay, major in-hospital complications, and both short- and long-term survival.Cardiopulmonary bypass time was 117.1 ± 2.0 minutes in the ST group and 139.7 ± 2.6 minutes in the MI group (p0.0001), and cross-clamp time was 79.6 ± 1.5 minutes in the ST group and 83.7 ± 1.9 in the MI group (p = 0.106). The average hospital length of stay was 9.81 ± 0.61 days among ST patients and 7.76 ± 0.37 days among MI patients (p = 0.0043). There was no significant difference in the frequency of major in-hospital complications between groups. The mean duration of survival follow-up was 4.2 ± 2.4 years. There was no significant difference in mortality at 30 days (p = 0.622) or 1 year (p = 0.599). In addition, there was no significant difference in long-term survival between groups (p = 0.569).Although minimally invasive mitral valve surgery required a slightly longer cardiopulmonary bypass time, there was no difference in cross-clamp time, morbidity, or mortality, and hospital length of stay was significantly shorter when compared with matched sternotomy control patients.

The lung allocation score (LAS) was initiated in May 2005 to allocate lungs based on medical urgency and posttransplant survival. The purpose of this study was to determine if there is an association between an elevated LAS at the time of transplantation and increased postoperative morbidity and mortality.The United Network for Organ Sharing provided de-identified patient-level data. Analysis included lung transplant recipients agedor= 12 years who received transplants between April 5, 2006, and December 31, 2007 (n = 3,836). Recipients were stratified into three groups: LAS50 (n = 3,161, 83.87%), LAS 50 to 75 (n = 411, 10.9%), and LASor= 75 (n = 197, 5.23%), referred to as low LAS (LLAS), intermediate LAS (ILAS), and high LAS (HLAS), respectively. The primary outcome was posttransplant graft survival at 1 year. Secondary outcomes included length of stay and in-hospital complications.HLAS recipients had significantly worse actuarial survival at 90 days and 1 year compared with LLAS recipients. When transplant recipients were stratified by disease etiology, a trend of decreased survival with elevated LAS was observed across all major causes of lung transplant. HLAS recipients were more likely to require dialysis or to have infections compared with LLAS recipients (P.001). In addition, length of stay was higher in the HLAS group when compared with the LLAS group (P.001).HLAS is associated with decreased survival and increased complications during the transplant hospitalization. Whereas the LAS has improved organ allocation through decreased waiting list deaths and waiting list times, lower survival and higher morbidity among HLAS recipients suggests that continued review of LAS scoring is needed to ensure optimal long-term transplant survival.

In May 2005, the lung allocation score (LAS) was introduced as a means of allocating donor lungs in order to decrease wait-list mortality and prioritize candidates based on medical urgency and posttransplant survival. The purpose of this study was to assess changes in recipient wait-list times and mean LAS since the introduction of the LAS model.The United Network for Organ Sharing provided de-identified patient-level data. The study population consisted of all patients in the United States with a reported LAS (n = 3529) undergoing lung transplantation between May 7, 2005 and November 7, 2007. The study period was divided into 6-month intervals. The Kruskal-Wallis test was used to assess differences in variables with nonparametric distributions. The nonparametric trends test was used to determine significance of trends over time.There was a significant decrease in wait-list time during the study period, while LAS among transplant recipients increased (p0.001). There was no significant change in FVC (49.3 +/- 17.5%, p = 0.48) or pulmonary capillary wedge pressure (11.1 +/- 5.8 mm Hg, p = 0.23); however, there was a significant increase in age (51.5 +/- 13.9 years, p0.001) during the study period. When stratified by etiology, the LAS increased for both interstitial pulmonary fibrosis and COPD patients (p0.001). Moreover, the overall number of patients listed for transplantation as well as the LAS among transplant candidates increased (p0.001).Two years after initiation of the LAS model, wait-list times continue to decrease while mean LAS continued to increase. This increase in LAS among transplant recipients was observed most notably in patients with interstitial pulmonary fibrosis and COPD, and reflected in an increased mean LAS at the time of listing.

was performed. Results: There was no correlation between pre-transplant DSA and the development of BOS (BOS stage 2/3 vs 0/1) p 0.22, or stages of BOS p 0.48. Similarly, there was no correlation between any of the pretransplant clinical factors and the development of BOS in patients surviving greater than one year in the presence of preformed DSA or non-DSA. Graft ischaemic time, HLA-mismatches, underlying disease and number of infections requiring hospital admission after transplantation did not have significant impact on the presence of BOS. Conclusions: Whilst pre-transplant donor specific HLA antibodies are known to have a deleterious effect on graft outcome in the early posttransplant period, it would appear from this preliminary data that in patients who survive despite having DSA there is no effect on subsequent development of BOS.

Purpose: To describe the frequency of and resource utilization related to rehospitalization among continuous-flow ventricular assist device patients. Methods and Materials: All patients implanted with a continuous-flow left ventricular assist device at a single institution between 7/1/2008 and 11/1/2010. Hospital admissions following discharge from the implant hospitalization were analyzed. Outcome measures included frequency of rehospitalization, hospital length of stay, ICU days, and cost.

BackgroundThe purpose of this study was to compare posttransplantation morbidity and mortality in orthotopic heart transplant recipients bridged to transplant with a left ventricular assist device with nonbridged recipients. To account for potential differences across device types, we stratified bridge-to-transplant recipients by type of ventricular assist device: extracorporeal (EXTRA), paracorporeal (PARA), and intracorporeal (INTRA).MethodsThe United Network for Organ Sharing provided de-identified patient-level data. The study population included 10,668 orthotopic heart transplant recipients aged 18 years old or older and undergoing transplantation between January 1, 2001, and December 31, 2006. Follow-up data were provided through August 3, 2008, with a mean follow-up time of 3.17 ± 2.15 years (range, 0–8.11 years). The primary outcome was actuarial posttransplant graft survival. Other outcomes of interest included infection, stroke, and dialysis during the transplant hospitalization; primary graft failure at 30 days; transplant hospitalization length of stay; and long-term complications including diabetes mellitus, transplant coronary artery disease, and chronic dialysis. Multivariable Cox proportional hazards regression (backward, P < .15) was used to determine the relationship between groups and overall graft survival, and multivariable logistic regression analysis (backward, P < .15) was used to determine the relationship between groups and secondary outcome measures.ResultsIn multivariable Cox regression analysis, when compared with the nonbridged group, risk-adjusted greater than 90-day graft survival was diminished among the EXTRA group (hazard ratio = 3.54, 2.28–5.51, P < .001), but not the INTRA group (1.04, 0.719–1.51, P = .834) or the PARA group (1.06, 0.642–1.76, P = .809). There were no significant differences in risk-adjusted graft survival across the 4 groups during the 90-days to 1-year or 1- to 5-year intervals. However, at more than 5 years, risk-adjusted graft survival in the INTRA group (0.389, 0.205–0.738, P = .004) was better than in the nonbridged group. The EXTRA, PARA, and INTRA groups all experienced increased risks of infection. The EXTRA group had increased risks of dialysis, stroke, and primary graft failure at 30 days, whereas neither the PARA nor the INTRA group differed from the nonbridged group. Long-term complications did not differ by group.ConclusionThe use of implantable left ventricular assist devices as bridges to transplantation, including both intracorporeal and paracorporeal devices, is not associated with diminished posttransplant survival. However, 90-day survival was diminished in recipients bridged with extracorporeal devices.

ObjectivesThe use of mechanical circulatory support to bridge pediatric patients to cardiac transplantation presents unique challenges because of the difficult anatomy and physiology in these patients.MethodsThe United Network for Organ Sharing provided deidentifed patient-level data. The study population included 2532 transplantations performed on patients less than 19 years old in status 1/1A/1B between 1995 and 2005. Mechanical circulatory support was used in 431 patients: 241 (9.5%) received ventricular assist devices, 171 (6.8%) underwent extracorporeal membrane oxygenation, and 19 (0.8%) received intra-aortic balloon pumps.ResultsPatients supported on ventricular assist devices had similar levels of hospitalization and intensive care use and less need for inotropic support (P < .0002) than had those not needing support. Five- and 10-year posttransplantation survival was better in patients receiving ventricular assist devices and patients not receiving mechanical circulatory support than in patients receiving extracorporeal membrane oxygenation or intra-aortic balloon pumping (P < .0001). Among mechanically supported patients, patients with a body surface area of less than 0.30 (odds ratio, 1.70; 95% confidence interval, 1.18–2.43) and those requiring extracorporeal membrane oxygenation (odds ratio, 1.65; 95% confidence interval, 1.15–2.35) or intra-aortic balloon pumping (odds ratio, 1.91; 95% confidence interval, 1.02–3.56) had higher long-term mortality. The use of a ventricular assist device at transplantation did not predict higher long-term, posttransplantation mortality.ConclusionsPediatric patients requiring a pretransplantation ventricular assist device have long-term survival similar to that of patients not receiving mechanical circulatory support. Early survival among patients undergoing extracorporeal membrane oxygenation and infants is poor, reinforcing the need for improvements in device design and physiologic management of infants and neonates.

THE EFFECT OF BODY MASS INDEX ON SURVIVAL FOLLOWING HEART TRANSPLANTATION: A CRITIQUE OF THE ISHLT’S REVISED LISTING CRITERIA FOR HEART TRANSPLANTATION M.J. Russo, J.M. Chen, A. Moskowitz, K.N. Hong, R.R. Davies, F. Cheema, D.D. Ascheim, M. Argenziano, M.C. Oz, A.C. Gelijns, Y. Naka, Division of Cardiothoracic Surgery, Columbia University College of Physicians and Surgeons, NY, NY; International Center for Health Outcomes and Innovation Research, Columbia University College of Physicians and Surgeons, NY, NY; Division of Cardiology, Columbia University College of Physicians and Surgeons, NY, NY