Rate Of Heart Transplantation Research Articles

Outcomes and complications of transvenous implantable cardioverter defibrillator systems in the pediatric population: a systematic review and meta-analysis

Abstract Background Implantable cardioverter defibrillators (ICDs) are an important therapy for prevention of sudden cardiac death (SCD). Data on transvenous (TV) ICD therapy rates, complications, and system survival in children ≤ 21 years is limited by absolute numbers and inclusion of adults with congenital heart disease. Purpose To determine ICD therapy rates and complications in pediatric patients ≤ 21 years of age. Methods A systematic review and meta-analysis were conducted following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement. Medline, Embase, PubMed Ahead-of-Print and in-process records, and Cochrane Database of Systematic Reviews were searched for full-text articles on pediatric ICD. Articles were assessed for inclusion by two authors via a consensus process; the exclusion criteria included: case reports, review articles, abstracts, editorials, articles not in English, articles solely on epicardial or subcutaneous leads, articles on unrelated topics, articles with <50% ICD leads, and articles with any patients >21 years old. Data were extracted by 1 author and verified by a 2nd author. Meta-analysis of aggregate data was performed, where possible. Random effects models were used for pooled inferences. All reported rates are annualized rates based on reported follow-up times in each study. Results Of 1325 articles, 23 met all inclusion and no exclusion criteria (Figure) representing 1700 patients implanted from 1987-2020. The average age of patients was 12.8 years (95% confidence interval [CI]: 11.9 – 13.7) with weight of 51.1 kg (95% CI: 42.2 – 60.0) (Table). Primary prevention was the indication in 56.4 % (95% CI: 45.0% – 67.7%). Only 14.8% (95% CI: 10.6% – 19.0%) of the patients had congenital heart disease while 22.6% (95% CI: 15.8% – 29.3%) had long QT syndrome. The annualized rate of appropriate shock was 7.0% (95% CI: 5.6% – 8.8%) and the rate of inappropriate shock was 4.8% (95% CI: 3.8% – 6.0%). Annualized rates of reported adverse events included lead failure: 2.3% (95% CI: 1.6% – 3.3%) and infection: 0.8% (95% CI: 0.4% – 1.6%), which could have led to system revision: 4.4% (95% CI: 2.1% – 9.4%). The all-cause mortality rate was 0.8% (95% CI: 0.6% – 1.2%) across 17 studies. In 9 studies with 719 patients, the heart transplant rate was 1.6% (95% CI: 0.6% – 3.9%). Conclusion In this first systematic review and meta-analysis of ICDs in pediatric patients, ICD therapy was effective for prevention of SCD with a 7.0% annualized rate of appropriate ICD shocks. This is comparable to the annualized rate of appropriate ICD shock for secondary prevention of SCD in adult randomized controlled trials. Most common adverse events in the pediatric population were lead failure and system revision. Further refinements in patient selection criteria for implantation and improvements in lead and device technology are needed for pediatric patients.FigureTable

Prevalence, Clinical Manifestations, and Adverse Outcomes of Left Ventricular Noncompaction in Adults: A Systematic Review and Meta-Analysis.

Left ventricular noncompaction (LVNC) is recognized within the spectrum of adult cardiomyopathies for its unique pathophysiologic features and clinical challenges. This condition exhibits a wide range of clinical manifestations, from asymptomatic states to severe cardiovascular complications, making its diagnosis and management challenging. This study aimed to synthesize current data on the prevalence, diagnostic methods, clinical outcomes, and treatment efficacy of LVNC in adults to address gaps in understanding and management strategies. A systematic review and meta-analysis of research from 2000 to March 2024 was conducted, focusing on studies involving adults diagnosed with LVNC. This approach aimed to collect data on the prevalence of LVNC, the diagnostic accuracy of different imaging modalities, clinical manifestations, and the impact of different treatment strategies. The study showed a prevalence of LVNC of 0.5%, with cardiovascular magnetic resonance outperforming echocardiography in diagnosis with a detection rate of 1.3%. Mortality and heart transplantation rates were 12% and 7%, respectively. Significant predictors of adverse outcomes included New York Heart Association (NYHA) class III or IV, ventricular tachycardia, and reduced left ventricular ejection fraction (LVEF), guiding a nuanced approach in tailoring therapeutic strategies to optimize patient care and outcomes. This study advances the understanding of LVNC by refining diagnostic criteria and evaluating management strategies, highlighting the superiority of cardiovascular magnetic resonance. It identifies predictors of adverse outcomes and assesses treatment efficacy, urging precision in diagnosis and tailored treatments. Its comprehensive analysis and methodological rigor make it a key resource advocating a multidisciplinary approach to improve patient outcomes in LVNC.

Haemodynamic effects of intra-aortic balloon pumps stratified by baseline pulmonary artery pulsatility index.

Intra-aortic balloon pump (IABP) devices are commonly used in patients with heart failure related cardiogenic shock (HF-CS), including those with out-of-proportion right ventricular (RV) dysfunction. Pulmonary artery pulsatility index (PAPi) is a haemodynamic surrogate for RV performance. We aimed to assess short-term haemodynamic changes in patients with HF-CS following IABP support stratified by baseline PAPi. This is a single-centre study of 67 consecutive patients with HF-CS who underwent IABP placement between 2020 and 2022. The primary aim was haemodynamic changes of specific variables on pulmonary artery catheter monitoring over 72h following IABP placement. Secondary aims were clinically significant changes in diuretic regimens, changes in inotropes or vasopressors at 72h following IABP, along with clinical outcomes. Prior to IABP placement, 57% of the total cohort (median age 59years [48, 69], 31% female) had Society of Cardiovascular Angiography and Interventions Stage C HF-CS. Thirty-eight (56%) patients had a PAPi <2.0. Following 72h of IABP support, the PAPi <2.0 group had an observed significant decrease in central venous pressure (CVP; 20 to 12mmHg, P<0.001) and mean pulmonary artery pressure (mPAP; 37.5 to 28.5mmHg, P=0.001), and an increase in PAPi (1 to 1.6, P=0.001). No significant change in cardiac index (CI; 2 to 2.1L/min/m2, P=0.31) was observed. The PAPi ≥2.0 group (N=29) had no observed significant change in CVP (10 to 8mmHg, P=0.47), or PAPi (2.6 to 2.8, P=0.92), but there was a significant improvement in CI (1.9 to 2.5L/min/m2, P=0.004) along with reduction in mPA (37 to 29mmHg, P=0.03). The PAPi <2.0 group had a significant increase in diuretic requirement (52.6% vs. 20.7%, P=0.01) and numerically greater addition of inotropes/vasopressors (47.3% vs. 34.4%, P=0.07) compared with the PAPi ≥2.0 group at 72h following IABP placement. Significantly more patients in the PAPi ≥2.0 group underwent left ventricular assist device (55.2% vs. 26.3%, P=0.02), with no overall significant differences observed in escalation to veno-arterial extracorporeal membrane oxygenation, 30-day mortality, renal replacement therapy post-IABP, or rates of heart transplantation. IABP devices in those with HF-CS and low or abnormal PAPi may provide modest short-term haemodynamic benefits without significant improvement in CI, along with greater need for adjustment in medical therapeutics to achieve haemodynamic optimization.

Catheter ablation vs advanced therapy for patients with severe heart failure and ventricular electrical storm

BackgroundCurrent data on outcomes of an initial strategy of catheter ablation vs advanced therapy in patients with severe heart failure (HF) and electric storm (ES) are limited. ObjectiveThe purpose of this study was to evaluate the outcomes of ventricular tachycardia (VT) ablation vs left ventricular assist device (LVAD) or heart transplantation (HT) in patients with severe HF and ventricular ES. MethodsPatients with severe HF and ES who underwent VT ablation, LVAD, or HT between 2012 and 2022 at our medical center were reviewed. Severe HF was defined as ejection fraction ≤ 35% or presence of severe restrictive, valvular, or genetic cardiomyopathy. We assessed in-hospital adverse events and 1-year outcomes between the 2 groups. ResultsOf the 73 patients, 43 (58.9%) underwent VT ablation and 30 (41.1%) received advanced therapy (21 HT (70%) and 9 LVAD (30%)). One-year survival was similar (76.7% vs 86.7%; log-rank, P = .308). However, 10 patients (23.3%) in the ablation group underwent HT during follow-up. After multivariable analysis, United Network for Organ Sharing status 1 or 2 according to VT criteria (hazard ratio 5.52; 95% confidence interval 1.27–24.12; P = .023) and early VT recurrence (hazard ratio 5.67; 95% confidence interval 1.68–19.09; P = .005) were associated with HT or mortality in patients who underwent VT ablation. ConclusionPatients with severe HF and ES who underwent VT ablation had similar overall survival to patients who directly proceeded with advanced therapy, although rates of HT were high during follow-up. Predictors of HT or mortality after catheter ablation include United Network for Organ Sharing status 1 or 2 according to VT criteria and early VT recurrence.

Effect of Preoperative Mitral Regurgitation on LVAD Outcomes in Patients with Elevated Pulmonary Vascular Resistance.

In patients with end-stage heart failure who undergo left ventricular assist device (LVAD) implantation, higher pulmonary vascular resistance (PVR) is associated with higher right heart failure rates and ineligibility for heart transplant. Concomitant mitral regurgitation (MR) could potentially worsen pulmonary hemodynamics and lead to worse outcomes; however, its effects in this patient population have not been specifically examined. Using an institutional database spanning November 2003 to August 2017, we retrospectively identified patients with elevated PVR who underwent LVAD implantation. Patients were stratified by concurrent MR: moderate/severe (PVR + MR) vs. mild/none (PVR - MR). Cumulative incidence functions and Fine-Gray competing risk regression were performed to assess the effect of MR on heart transplant rates and overall survival during index LVAD support. Of 644 LVAD recipients, 232 (171 HeartMate II, 59 HeartWare, 2 HeartMate III) had baseline PVR > 3 Woods units; of these, 124 (53%) were INTERMACS 1-2, and 133 (57%) had moderate/severe MR (≥ 3 +). Patients with PVR + MR had larger a baseline left ventricular end-diastolic diameter than patients with PVR - MR (87.9 ± 38.2 mm vs. 75.9 ± 38.0 mm; P = 0.02). Median clinical follow-up was 18.8 months (interquartile range: 4.7-36.4 months). Moderate/severe MR was associated with lower mortality rates during index LVAD support (adjusted hazard ratio 0.64, 95% CI 0.41-0.98; P = 0.045) and higher heart transplant rates (adjusted odds ratio 2.86, 95% CI 1.31-6.25; P = 0.009). No differences in stroke, gastrointestinal bleeding, or right heart failure rates were observed. Among LVAD recipients with elevated preoperative PVR, those with moderate/severe MR had better overall survival and higher transplant rates than those with mild/no MR. These hypothesis-generating findings could be explained by incremental LVAD benefits resulting from reduction of MR and better LV unloading in a subset of patients with larger ventricles at baseline. In patients with preoperative elevated PVR, MR severity may be a prognostic sign that can inform patient selection for end-stage heart failure therapy.

Worse outcomes in patients with cardiac sarcoidosis and involvement of the right ventricle in cardiac magnetic resonance

Abstract Background Cardiac sarcoidosis (CS) can manifest with variety of unspecific symptoms. It can mimic all types of cardiomyopathies but most often it resembles dilated cardiomyopathy. Involvement of right ventricle (RV) is rare and may lead to a false diagnosis of arrhythmogenic right ventricular cardiomyopathy and delay of the immunosuppressive therapy. Purpose The aim of this research was to study the characteristics and the outcomes of this peculiar subtype of patients. Methods We analyzed 112 patients diagnosed with CS after elaborate work-up with cardiac magnetic resonance (CMR), FDG PET and endomyocardial biopsy (EMB). All patients underwent follow-up for occurrence of combined endpoint of death, LV assist device implantation or heart transplantation. Patients with evidence of late gadolinium enhancement (LGE) involving RV (RV group) were identified and compared to the rest (DCM group) for baseline clinical characteristics, arhythmic outcomes and combined endpoint. Results Involvement of the RV in CMR was observed in 19 (17%) patients. Patients in the RV group were more frequently male: 84% vs. 57%, P = 0.032. EMB confirmed CS in 63% vs. 19.4% in the DCM group; P= 0.0001. LV ejection fraction in the RV group was comparable to the DCM group: 42±14% vs. 45±14%; P = 0.3. Furthermore, in the RV group the rates of sustained ventricular tachycardia were significantly higher than the DCM: 68% vs. 34%; P = 0.009. Long-term immunosuppressive therapy was started in 74% in RV and in 70% of the DCM group; P=0.77. Despite this, during follow-up, the rate of death, LVAD implantation or heart transplantation was significantly higher in the RV than in the DCM group; 26.3% vs. 7.5%; P = 0.03. In binary logistic analysis, the OR for the combined endpoint was 4.4; 95% CI 1.2-15.7. Conclusions Patients with CS and RV involvement in CMR have worse outcomes as compared to patients presenting as DCM, despite the comparable LV EF and immunosuppressive therapy.

Examining a 12-year experience within Kazakhstan’s national heart transplantation program

Kazakhstan has one of the lowest heart transplantation (HTx) rates globally, but there are no studies evaluating the outcomes of HTx. This study aimed to provide a comprehensive analysis of the national HTx program over a 12-year period (2012–2023). Survival analysis of the national HTx cohort was conducted using life tables, Kaplan‒Meier curves, and Cox regression methods. Time series analysis was applied to analyze historical trends in HTx per million population (pmp) and to make future projections until 2030. The number of patients awaiting HTx in Kazakhstan was evaluated with a regional breakdown. The pmp rates of HTx ranged from 0.06 to 1.08, with no discernible increasing trend. Survival analysis revealed a rapid decrease in the first year after HTx, reaching 77.0% at 379 days, with an overall survival rate of 58.1% at the end of the follow-up period. Among the various factors analyzed, recipient blood levels of creatinine and total bilirubin before surgery, as well as the presence of infection or sepsis and the use of ECMO after surgery, were found to be significant contributors to the survival of HTx patients. There is a need for public health action to improve the HTx programme.

Donor heart refusal after circulatory death: An analysis of United Network for Organ Sharing refusal codes

ObjectiveDonor hearts procured after circulatory death (DCD) may significantly increase the number of hearts available for transplantation. The purpose of this study was to analyze current DCD and brain-dead donor (DBD) heart transplantation rates and characterize organ refusal using the most up-to-date United Network for Organ Sharing (UNOS) and Organ Procurement and Transplantation Network data. MethodsWe analyzed UNOS and Organ Procurement and Transplantation Network DBD and DCD candidate, transplantation, and demographic data from 2020 through 2022 and 2022 refusal code data to characterize DCD heart use and refusal. Subanalyses were performed to characterize DCD donor demographics and regional transplantation rate variance. ResultsDCD hearts were declined 3.37 times more often than DBD hearts. The most frequently used code for DCD refusal was neurologic function, related to concerns of a prolonged dying process and organ preservation. In 2022, 92% (1329/1452) of all DCD refusals were attributed to neurologic function. When compared with DBD, DCD donor hearts were more frequently declined as the result of prolonged warm ischemic time (odds ratio, 5.65; 95% confidence interval, 4.07-7.86) and other concerns over organ preservation (odds ratio, 4.06; 95% confidence interval, 3.33-4.94). Transplantation rate variation was observed between demographic groups and UNOS regions. DCD transplantation rates are currently experiencing second order polynomial growth. ConclusionsDCD donor hearts are declined more frequently than DBD. DCD heart refusals result from concerns over a prolonged dying process and organ preservation. Heart transplantation rates may be substantially improved by ex situ hemodynamic assessment, adoption of normothermic regional perfusion guidelines, and quality initiatives.

Mortality and Heart Transplantation After Hybrid Palliation of Hypoplastic Left Heart Syndrome: A Systematic Review and Meta-Analysis.

Newborns with hypoplastic left heart syndrome (HLHS) who are considered at increased risk for death following Norwood/Sano surgery often undergo hybrid palliation (HP) as initial surgery. We aimed to compile the HP experience in HLHS and its variants and assess the rates of, and risk factors for, death and heart transplantation. CINAHL, CINAHL PLUS, PubMed/MEDLINE, and SCOPUS were systematically searched for HP outcome studies of death or heart transplantation in HLHS between 1998 and 2022. Pooled incidence was estimated, and potential risk factors were identified using random-effects meta-analysis and reconstructed time-to-event data from Kaplan-Meier curves. Thirty-three publications were included in our review. Overall, of 1,162 patients 417 died and 57 underwent heart transplantation, resulting in a combined outcome of 40.7%, (474/1,162). There was a trend toward decreasing mortality risk across the stages of palliation. Pooled mortality between HP and comprehensive stage 2 palliation was 25%, after stage 2 up to Fontan palliation was 16%, and 6% post-Fontan. The incidence of death or heart transplantation was higher in high-risk patients-43% died and 10% received heart transplantation. Our systematic review and meta-analysis found high rates of death or heart transplantation in HP of HLHS patients between HP and Fontan surgeries. All patients should be closely followed during the initial interstage period, which is associated with the highest hazard. Prospective studies on appropriate patient selection, indications, and / or alternatives, as well as refining HP strategies for managing newborns with HLHS are needed to improve outcomes.

Disparities by Sex, Race, and Ethnicity in Use of Left Ventricular Assist Devices and Heart Transplants Among Patients With Heart Failure With Reduced Ejection Fraction.

The extent to which sex, racial, and ethnic groups receive advanced heart therapies equitably is unclear. We estimated the population rate of left ventricular assist device (LVAD) and heart transplant (HT) use among (non-Hispanic) White, Hispanic, and (non-Hispanic) Black men and women who have heart failure with reduced ejection fraction (HFrEF). We used a retrospective cohort design combining counts of LVAD and HT procedures from 19 state inpatient discharge databases from 2010 to 2018 with counts of adults with HFrEF. Our primary outcome measures were the number of LVAD and HT procedures per 1000 adults with HFrEF. The main exposures were sex, race, ethnicity, and age. We used Poisson regression models to estimate procedure rates adjusted for differences in age, sex, race, and ethnicity. In 2018, the estimated population of adults aged 35 to 84 years with HFrEF was 69 736, of whom 44% were women. Among men, the LVAD rate was 45.6, and the HT rate was 26.9. Relative to men, LVAD and HT rates were 72% and 62% lower among women (P<0.001). Relative to White men, LVAD and HT rates were 25% and 46% lower (P<0.001) among Black men. Among Hispanic men and women and Black women, LVAD and HT rates were similar (P>0.05) or higher (P<0.01) than among their White counterparts. Among adults with HFrEF, the use of LVAD and HT is lower among women and Black men. Health systems and policymakers should identify and ameliorate sources of sex and racial inequities.

Coronary Angiography in Patients With Left Ventricular Hypertrabeculation/Noncompaction.

Left ventricular hypertrabeculation/noncompaction (LVHT) is a cardiac abnormality of unknown pathogenesis, frequently associated with neuromuscular disorders. The relevance of coronary artery disease (CAD) in LVHT is largely unknown. This study aimed to assess the role of CAD as a prognostic marker in LVHT. Data from patients with LVHT were collected from an echocardiographic laboratory. The hospital information system was retrospectively screened for coronary angiography. The association of CAD with clinical, echocardiographic, and neurologic baseline parameters was assessed. End points were all-cause death and heart transplantation. A total of 154 patients (mean [SD] age, 57 [13.7] years; 31% female) who had undergone coronary angiography between 1995 and 2020 were included in the study. Coronary angiography disclosed CAD in 53 of 154 patients. Patients with CAD were older (mean [SD] age of, 64.2 [12.9] years vs 52.7 [12.4] years; P < .001); more frequently had angina pectoris (P = .05), diabetes (P = .002), and hypertension (P = .03); and more frequently had 3 or more electrocardiographic abnormalities (P = .04) than patients without CAD. During a median (IQR) follow-up period of 6.48 (2.44-11.20) years, 39% of patients reached an end point (death, n = 56; heart transplantation, n = 4). Mortality was 4.5% per year, and the rate of death or heart transplantation did not differ between patients with and without CAD (P = .26). Patients with 3-vessel disease had a worse prognosis than patients with 1- or 2-vessel disease (P = .046). In patients with LVHT, CAD does not appear to be associated with an increased rate of death or heart transplantation.

Ventricular Remodeling Following Metabolic and Bariatric Surgery Decreases Need for Heart Transplantation: A Predictive Model.

For patients with obesity and congestive heart failure (CHF) who require heart transplantation (HT), aggressive weight loss has been associated with ventricular remodeling, or subclinical alterations in left and right ventricular structure that affect systolic function. Many have suggested offering metabolic and bariatric surgery (MBS) for these patients. As such, we evaluated the role of MBS in HT for patients with obesity and CHF using predictive modelling techniques. Markov decision analysis was performed to simulate the life expectancy of 30,000 patients with concomitant obesity, CHF, and 30% ejection fraction (EF) who were deemed ineligible to be waitlisted for HT unless they achieved a BMI < 35kg/m2. Life expectancy following diet and exercise (DE), Roux-en-Y gastric bypass (RYGB), and sleeve gastrectomy (SG) was estimated. Base case patients were defined as having a pre-intervention BMI of 45kg/m2. Sensitivity analysis of initial BMI was performed. RYGB patients had lower rates of HT and received HT quicker when needed. Base case patients who underwent RYGB gained 2.2 additional mean years survival compared with patients who underwent SG and 10.3 additional mean years survival compared with DE. SG patients gained 6.2 mean years of life compared with DE. In this simulation of 30,000 patients with obesity, CHF, and reduced EF, MBS was associated with improved survival by not only decreasing the need for transplantation due to improvements in EF, but also increasing access to HT when needed due to lower average BMI.

Treatment Strategies and Outcomes of Right Ventricular Failure Post Left Ventricular Assist Device Implantation: An INTERMACS Analysis.

Right heart failure (RHF) management after left ventricular assist device (LVAD) implantation includes inotropes, right ventricular mechanical support, and heart transplantation. The purpose of this study is to compare different RHF treatment strategies in patients with a magnetically levitated centrifugal LVAD. A total of 6,632 Interagency Registry for Mechanically Assisted Circulatory Support (INTERMACS) patients from 2013 to 2020 were included. Of which, 769 (69.6%) patients (group 1) were supported with inotropes (≥14 days post-LVAD implantation), 233 (21.1%) patients (group 2) were supported with temporary right ventricular assist device (RVAD) that was implanted during LVAD implant, 77 (7.0%) patients (group 3) with durable centrifugal RVAD implanted during the LVAD implant, and 26 (2.4%) patients (group 4) were supported with RVAD (temporary or permanent), which was implanted at a later stage. Groups 1 and 4 had higher survival rates in comparison with group 2 (hazard ratio [HR] = 0.513, 95% confidence intervals [CIs] = 0.402-0.655, p < 0.001, versus group 1) and group 3 (HR = 0.461, 95% CIs = 0.320-0.666, p < 0.001, versus group 1). Patients in group 3 showed higher heart transplantation rates at 12 and 36 months as compared with group 1 (40.4% and 46.6% vs. 21.9% and 37.4%, respectively), group 2 (40.4% and 46.6% vs. 25.8% and 39.3%, respectively), and group 4 (40.4% and 46.6% vs. 3.8% and 12.0%, respectively). Severe RHF post-LVAD is associated with poor survival. Patients with LVAD who during the perioperative period are in need of right ventricular temporary or durable mechanical circulatory support constitute a group at particular risk. Improvement of devices tailored for right ventricular support is mandatory for further evolution of the field.

Acute changes of right ventricular longitudinal strain after transcatheter edge-to-edge repair in patients with functional mitral regurgitation

Abstract Background Transcatheter edge-to-edge repair (TEER) is a valid therapeutic option in symptomatic patients with severe secondary mitral valve regurgitation (SMR) at high surgical risk. While right ventricular (RV) dysfunction is a predictor of poor outcomes in this context, TEER may lead to an improvement of RV function which portends a better outcome. Purpose The aim of this study was to investigate acute change in RV function and its prognostic implication after successful TEER in patients with severe SMR. Methods Patients with severe SMR who underwent TEER from 2013 to 2021 at our center were retrospectively screened. Only patients with successful procedure (MR ≤ grade 2) and adequate echocardiographic images for longitudinal strain assessment were enrolled. RV function was estimated at baseline and pre-discharge by echocardiographic evaluation; RV global longitudinal strain (RVGLS) and RV free wall longitudinal strain (RVFWLS) were assessed before and after TEER. Longitudinal strain improvement was defined as an increase of post procedural strain greater than median value variation. Population was then divided in two groups based on RVGLS significant improvement. The primary outcome was a composite of all-cause death, heart transplantation (HT) and left ventricular assist device (LVAD) implantation during follow-up, assessed with Kaplan-Maier curves and compared by log-rank test. Results A total of 61 patients were included. Mean age was 70±10 years, 89% patients were symptomatic with NYHA class 3-4, mean furosemide daily dose was 200±146 mg. Pre-TEER median left ventricle ejection fraction (LVEF) was 29% [IQR, 26; 35%], RV fractional area change (FAC) was 32% [IQR 26; 37%] and tricuspid anulus plan systolic excursion (TAPSE) was 17 mm [IQR 14; 20 mm]. Median RVGLS and RVFWLS at baseline were respectively -11% [IQR -13; -9%] and -14% [IQR -17; -11%]. After TEER a significant variation of RVGLS and RVFWLS was observed (RVGLS: -13% [IQR -15; -10%] p 0.012; RVFWLS: -16% [-19; - 13%] p 0.001). Moreover, a significant reduction in estimated systolic pulmonary artery pressure (sPAP) between baseline (45 mmHg [IQR 35; 55mmHg]) and post TEER (40 mmHg [IQR 30;45 mmHg] p &amp;lt;0.001) was observed. Median improvement of RVGLS was 1.3%. After mean follow up time of 55±7 months event rate for the combined primary endpoint was numerically lower in the improved RVGLS group, although the difference was not statistically significant (p 0.243). Interestingly, rate of HT was significantly lower in RVGLS-improved group (p 0.032). Conclusions This study showed significant pre-discharge improvement of RV longitudinal strain in patient with severe SMR after successful TEER. At follow-up this improvement did not affect the outcome in terms of freedom from composite endpoint of all-cause death, HT or LVAD implantation but events were numerically lower in patients with strain improvement. Further data on larger population are needed to confirm our observations.Table 1,2Figure 1,2

Abstract 395: Outcomes Of Mechanical Circulatory Support for Cardiogenic Shock in the United States: An Analysis of the 2017-2020 National Inpatient Sample Database

Introduction: Mechanical circulatory support devices, such as Extracorporeal membrane oxygenation(ECMO), Impella, and intra-aortic balloon pump(IABP), play a crucial role in managing patients with cardiogenic shock(CS). This study aimed to compare the outcomes and patient characteristics of different mechanical circulatory support modalities. Methods: We utilized the National Inpatient Sample Database(NIS) data from 2017 to 2020 to identify patients with the diagnosis of CS who received mechanical circulatory support. The study population was categorized into six groups: ECMO, Impella, IABP, ECMO + Impella, and ECMO + IABP. The groups were compared based on patient characteristics, length of stay(LOS), complications, need for a heart transplant, mortality, and total charges. Results: A total of 715,470 patients were identified with CS, 17%(122,100) received mechanical circulatory support, 5,675 had ECMO therapy, 33,360 Impella, 79,540 IABP, 1,890 cases ECMO+Impella, and 1,635 cases ECMO+IABP. Age significantly differed, with ECMO patients being the youngest 54[36-65] years. There were higher proportions of females in the ECMO-only(37.4%) and males in the ECMO+Impella(76.7%). LOS was prolonged in the ECMO group(14 [5-28] days) compared to other groups(p&lt;0.001). Mortality rates were higher in the ECMO group(47.1%), followed by ECMO+Impella(45.8%) and ECMO+IABP (40.1%) (p&lt;0.001). Higher rates of stroke, disseminated intravascular coagulation, and continuous renal replacement therapy requirements were observed in the ECMO group. Bleeding requiring transfusion was more frequent with ECMO+IABP, and limb ischemia was more common in the ECMO+Impella group (p&lt; 0.001). Heart transplant rates were highest after ECMO+IABP therapy. Over the years, ECMO utilization increased, while Impella and IABP utilization remained stable. Notably, the ECMO+IABP had the highest total charges among all the therapy modalities evaluated (p &lt; 0.001). Conclusions: The rising utilization of ECMO and ECMO-IABP therapies indicates a changing treatment landscape. However, these approaches exhibit lower survival rates, potentially due to the severity of CS, heightened complications, and increased costs compared to standalone IABP or Impella therapies.

Abstract 16193: Differences in Mortality Between Patients With Left Ventricular Assist Devices and End-Stage Renal Disease on Peritoneal Dialysis versus Hemodialysis

Introduction: End-stage renal disease (ESRD) patients with concomitant heart failure often require left ventricular assist devices (LVADs), but the impact on mortality in peritoneal dialysis (PD) versus hemodialysis (HD) is unclear. This study aims to compare mortality rates in prevalent and incident ESRD patients with LVADs on PD versus HD. Hypothesis: We hypothesize differences in mortality rates and heart transplantation rates between LVAD patients on PD versus HD. Methods: A retrospective cohort analysis used data from the TriNetX Research Network database. Propensity score matching compared two cohorts: LVAD with HD (Cohort 1) and LVAD with PD (Cohort 2). Demographic data, comorbidities, and baseline laboratory values were collected. Mortality rates were assessed using risk analyses over a 3-year period following LVAD placement. Results: The study cohorts included 252 patients each after propensity score matching, with mean ages of 63.8 ± 14.3 years (Cohort 1) and 62.87 ± 15.4 years (Cohort 2). No significant differences were observed in demographic data including gender, race, or ethnicity between the two cohorts. The mortality rate did not significantly differ between Cohort 1 (HD) and Cohort 2 (PD) (0.563 vs. 0.603), with a risk difference of -0.04 (95% CI: -0.126, 0.046, p = 0.366). The risk ratio and odds ratio for mortality also showed no significant differences. Conclusion: ESRD patients with LVADs receiving PD demonstrated similar mortality rates compared to those receiving HD. These findings suggest that dialysis modality does not seem to influence mortality outcomes in patients with LVAD. Further research is needed to better understand the differences between these complex patient populations.

Abstract 17067: Long-Term Transplant-Free Survival is Improved in Hypoplastic Left Heart Syndrome With Cell-Based Therapy

Hypoplastic left heart syndrome (HLHS) is a univentricular congenital heart defect that has high morbidity and mortality and requires three-stage surgical reconstruction so that the right ventricle (RV) delivers systemic circulation. Poor outcomes result from RV failure in the systemic position. Accordingly, we conducted a phase I clinical trial (called ELPIS) of Lomecel-B™, an allogeneic bone-marrow derived cell-based therapy designed to improve cardiovascular performance, delivered as a one-time treatment during the Stage II (Glenn) surgery at approximately 4 months after birth. This trial met its primary endpoint (safety through 1-year post-treatment). To assess whether Lomecel-B™ has survival benefits, all ELPIS patients ( n=10, 7 males, and 3 females) were enrolled in a multi-year follow-on study and compared to a retrospective control group which was identified by a relevant clinical HLHS database. Patients in both studies were assessed for up to 5 years (range 3.5 - 5.0 years post-treatment for ELPIS) for mortality, heart transplants, and stage III (Fontan) surgery. Outcomes were compared with long-term historical data from patients in the Single Ventricle Reconstruction (SVR) Trial receiving the same shunt type at Stage I (Norwood) operation. 5-year Kaplan-Meier survival was 100% in ELPIS patients with none requiring heart transplant. This compared to 81.6% (95% CI= [76.5, 87.0]) transplant-free survival in the SVR trial through 5 years post-Glenn surgery, and a 5.2% (95% CI= [2.0, 8.3]) heart transplantation rate (Figure 1). No stem cell related safety issues were reported. These findings support Lomecel-B™ as a potential adjunct to HLHS reconstruction surgery to improve clinical benefits and reduce the need for subsequent heart transplantation. Further long-term follow-up and controlled trials are both warranted and underway.

Abstract 17038: Mineralocorticoid Antagonist Use for Transthyretin Amyloid Cardiomyopathy and Associated Outcomes

Background: Diuretics have been the mainstay of symptomatic treatment for patients with amyloid cardiomyopathy however, there remains no established guideline directed medical therapy for this population. The anti-fibrotic properties of mineralocorticoid antagonists (MRA) are intriguing for potential use in transthyretin amyloid cardiomyopathy (ATTR CM); while they have shown promise for treatment of patients with heart failure with preserved ejection fraction, their use has never been studied specifically in the ATTR CM population. The purpose of our study is to evaluate the real-world use of MRA and associated outcomes in patients with ATTR CM at a large cardiac amyloid treatment center. Methods: Between 2009 and 2023, 168 patients with ATTR CM at our center were assessed for history of MRA use. Biomarkers, including baseline levels of troponin and natriuretic peptides, as well as kidney function were assessed. We also assessed the outcomes of 5-year survival, need for heart transplant, hospitalization, and mechanical circulatory support (MCS) use. Results: Of the 168 patients, 49 (29%) had a history of MRA use and 119 (71%) had no prior use. Between the two groups, there was no significant difference in age, frequency of ATTR CM subtype, kidney disease, or biomarker level at the time of diagnosis. While 5-year survival and need for MCS were similar between the two groups, there was a higher rate of transplant and hospitalization in the MRA use group. Conclusion: In a cohort of patients with ATTR CM, MRA use is uncommon and was not associated with improved outcomes. It was associated with higher rates of hospitalizations and heart transplantation; however, this may represent confounding by indication. Future investigations with larger registry cohorts and randomized control trials are warranted for further evaluation of biological and clinical effect of MRA use.

DMD-Associated Dilated Cardiomyopathy: Genotypes, Phenotypes, and Phenocopies.

Variants in the DMD gene, that encodes the cytoskeletal protein, dystrophin, cause a severe form of dilated cardiomyopathy (DCM) associated with high rates of heart failure, heart transplantation, and ventricular arrhythmias. Improved early detection of individuals at risk is needed. Genetic testing of 40 male probands with a potential X-linked genetic cause of primary DCM was undertaken using multi-gene panel sequencing, multiplex polymerase chain reaction, and array comparative genomic hybridization. Variant location was assessed with respect to dystrophin isoform patterns and exon usage. Telomere length was evaluated as a marker of myocardial dysfunction in left ventricular tissue and blood. Four pathogenic/likely pathogenic DMD variants were found in 5 probands (5/40: 12.5%). Only one rare variant was identified by gene panel testing with 3 additional multi-exon deletion/duplications found following targeted assays for structural variants. All of the pathogenic/likely pathogenic DMD variants involved dystrophin exons that had percent spliced-in scores >90, indicating high levels of constitutive expression in the human adult heart. Fifteen DMD variant-negative probands (15/40: 37.5%) had variants in autosomal genes including TTN, BAG3, LMNA, and RBM20. Myocardial telomere length was reduced in patients with DCM irrespective of genotype. No differences in blood telomere length were observed between genotype-positive family members with/without DCM and controls. Primary genetic testing using multi-gene panels has a low yield and specific assays for structural variants are required if DMD-associated cardiomyopathy is suspected. Distinguishing X-linked causes of DCM from autosomal genes that show sex differences in clinical presentation is crucial for informed family management.

Paediatric heart transplantation during the COVID-19 pandemic in Iran: single-centre experience.

Transplant services were imposed to the multiple challenges of insufficient facilities and resources during the COVID-19 pandemic. Thus, each centre modified and altered its routine practice to maintain the service. We report our experience in Iran's single-centre paediatric heart transplantation unit during the 2year pandemic in transplant rate and early transplant outcomes. We retrospectively reviewed all paediatric heart transplants performed at our centre between 19 February 2020 and February 2022. We studied the number and percentage of paediatric transplanted patients who developed COVID-19, in-hospital outcome, 30day survival and left ventricular function during the first month of the follow-up visit. From February 2020 to February 2022, 59 transplants were performed compared with 62 patients from February 2018 to 2020. Compared with the year before the pandemic, the heart transplant rate was reduced by 34% in the first year of the pandemic; however, it bounced back in the second year with a 19% increase. The in-hospital mortality rate during the 2year pandemic was (11.8%). One death occurred related to COVID-19 acute respiratory distress syndrome. The in-hospital COVID-19 infection rate was 11 patients (18%). In the 30day follow-up, the overall 30day survival rate was 88%, and the 30day rejection rate was 10%. The mean left ventricular ejection fraction was (53.6±4.9), the mean left ventricular global longitudinal strain was -16.9±3.3, and the mean right ventricular global longitudinal strain was -17.0±3.8 with no significant difference between patients with in-hospital COVID and other recipients (P=0.1, P=0.2 and P=0.2). Throughout the pandemic, although we experienced a reduction in the transplant rate in the first year, with the increasing vaccination rate in health care and later in the general population, we continued to maintain our transplant rate by incorporating safety protocols and more resources.