Risultati per: Diagnosi, trattamento e follow-up del cancro alla vescica

Circulation, Volume 150, Issue Suppl_1, Page A4135852-A4135852, November 12, 2024. Background:Vertebrobasilar artery stenosis (VBAS) can cause posterior circulation strokes (PCS). Optimal management is controversial, with options including medical therapy (MT), endovascular stenting (ES), and surgical revascularization (SR). This study compares outcomes of these treatments and evaluates the correlation between clinical features and medical history with 30-day outcome.Methods:Patients with VBAS were identified from the 2017-2018 National Inpatient Sample (NIS). Propensity scores adjusted for baseline differences. Outcomes included mortality, neurological complications (NC), discharge destination (DD), length of stay (LOS), total charges (TC), and procedural complications (PC). Predictive ability of clinical variables was assessed using logistic regression (LR) and machine learning techniques (MLT).Results:Of 1,343 patients, 1,061 (79.0%) received NI, 234 (17.4%) underwent ES, and 24 (1.8%) had SR. Mean age was 69.45 years, with 64.1% male. Demographics: 69.8% White, 14.9% Black, 10.0% Hispanic, and 5.3% other races. Hypertension (HTN, 85.4%) and diabetes (DM, 18.9%) were prevalent. After propensity weighting, ES was associated with higher odds of mortality, surgical/medical complications (SMC), and device/graft complications (DGC) compared to NI. SR showed a non-significant trend toward higher non-home discharges (NHD). ES and SR groups had higher resource utilization with longer LOS and greater TC. Clinical variables alone were weak predictors, with AUC values ranging from 0.454 to 0.71 across different outcomes and models.Conclusion:ES of VBAS was associated with higher mortality and complication rates compared to MT alone, with inconsistent benefits for NC. SR also carried elevated risks without clear advantages over MT. These results support that current clinical independent variables from the NIS are weak predictors. This highlights the limitation of the database in relying solely on clinical and medical history, and suggests that future use of radiological and anatomical features can improve predictions of outcomes and determination of subgroups that can benefit from certain treatment. More studies should be conducted, including post hoc analyses based on radiological and anatomical features, to better inform treatment decisions and determine subgroups that can benefit from intervention or surgery. These findings suggest a need for judicious patient selection and reinforce the role of optimal MT.

Circulation, Volume 150, Issue Suppl_1, Page A4146242-A4146242, November 12, 2024. Background:In 2021, the Food and Drug Administration approved the first Photon Counting Computed Tomography (PCCT) system, marking an extraordinary milestone in medical imaging. This advanced technology offers significant advantages in cardiac imaging, particularly in detecting severe calcification lesions and in-stent restenosis (ISR). Subsequently, in October 2023, our hospital became the first to implement a PCCT system. Leveraging the benefits of PCCT, we conducted a study to investigate ISR in patients who had undergone stenting over the course of a year.Methods:From October 2023 to January 2024, all high-risk stented patients, including those with multiple stents, bifurcation lesions, chronic total occlusions, and severe calcification lesions, were included in the study. The PCCT system (tube voltage 120 kV, collimation 120 x 0.2 mm, 50-70 ml Iohexol 300 mg/ml) was utilized to detect ISR. Intra-stent lesions with more than 50% narrowing were classified as ISR. Additionally, the quality of the PCCT images was assessed by two radiology experts using a five-point scale, where 1 indicated excellent quality (absence of artifacts) and 5 indicated non-diagnostic quality (severe artifacts).Results:Eighty patients (77.5% male) met the inclusion criteria, with a mean age of 64.7 ± 10.9 years. Among these patients, 56 had stents in the Left Anterior Descending (LAD) artery, while 27 and 34 had stents in the Left Circumflex Artery (LCx) and Right Coronary Artery (RCA), respectively. In total, 25 patients (31.2%) were identified with ISR. Specifically, ISR was most prevalent in the LCx at 25.9% (7/27), followed by the LAD at 21.4% (12/56), and the RCA at 17.7% (6/34). Nine patients underwent repeat percutaneous coronary intervention (PCI). The overall image quality was rated as excellent, with a median score of 1.5 [IQR, 1-2]. Additionally, 40 patients (50%) had a calcium score over 400.Conclusion:These findings represent the first results obtained using PCCT at our hospital. The results indicated a relatively high rate of ISR, particularly among high-risk patients. Given the excellent image quality, PCCT is a promising technique for the follow-up of patients post-PCI.

Circulation, Volume 150, Issue Suppl_1, Page A4140097-A4140097, November 12, 2024. Introduction:The MI-GENES randomized clinical trial (NCT01936675) assessed the effect of disclosing a polygenic risk score (PRS) for coronary heart disease (CHD), in addition to a clinical risk based on Framingham risk score (FRS), on LDL-C levels. The trial enrolled participants from Olmsted County, Minnesota, without cardiovascular disease, at intermediate CHD risk (10-y risk: 5-20%), and not on statins. There was a significant LDL-C reduction in the integrated risk score group (IRSg; received PRS information in addition to the FRS) compared to the FRS group (FRSg; received their risk based on FRS), due to more frequent statin initiation.Research Question:Does disclosure of an IRS for CHD lead to a lower rate of major adverse cardiovascular events (MACE)?Methods:Participants were followed from randomization beginning in October 2013 until September 2023 to ascertain cardiovascular events, testing for CHD, and risk factor changes, by blinded review of electronic health records. The primary outcome was time from randomization to the first MACE— defined as cardiovascular death, nonfatal MI, coronary revascularization, and nonfatal stroke. Analyses were done using Cox and linear mixed-effects models.Results:We followed all 203 participants, who completed the MI-GENES trial, 100 in FRSgand 103 in IRSg(mean age at the end of follow-up 68.2±5.2 years, 48% male). During a median follow-up of 9.5 years, 9 MACE occurred in FRSgand 2 in IRSg(HR, 0.20; 95% CI, 0.04 to 0.94;P=0.042, Figure 1A). In FRSg, 47 (47%) underwent at least one test for CHD, compared to 30 (29%) in IRSg(HR, 0.51; 95% CI, 0.32 to 0.81;P=0.004, Figure 1B). IRSgparticipants had a longer duration of statin therapy during the first four years post-randomization (Figure 1C) and a greater reduction in LDL-C for up to 3 years post-randomization (Figure 1D). No significant differences between the two groups were observed for hemoglobin A1C, blood pressure, weight, and smoking cessation rate during follow-up.Conclusion:The disclosure of 10-year CHD risk that included a PRS to those at intermediate risk was associated with lower incidence of MACE after a decade, likely due to more frequent and prolonged statin use, leading to lower LDL-C levels.

Circulation, Volume 150, Issue Suppl_1, Page A4143142-A4143142, November 12, 2024. Introduction:Malonyl-CoA decarboxylase deficiency (MLYCDD) is a rare inherited metabolic disorder with multi-organ involvement, causing cognitive impairment, cardiomyopathy, seizures, hypotonia, and acidosis. Variants in MLYCCD, the gene for malonyl-CoA decarboxylase, disrupt long chain fatty acid synthesis in cardiac tissue. Prevalence is estimated to be less than 1 in 1,000,000, with cardiomyopathy being the leading cause of morbidity and mortality. Less than 40 cases have been documented, the majority of which detail newborns. No guidelines exist for treatment. A high-carbohydrate, low-fat diet, and levocarnitine reportedly improve cardiac function.Presentation:A 30-year-old Spanish-speaking male with MLYCDD, with resultant heart failure with reduced ejection fraction (EF) and cognitive delay, presented to a community hospital with malaise. He was diagnosed at age 9 and followed with an interdisciplinary team, receiving care in Spanish and English since diagnosis. His treatment consisted of levocarnitine and dietary restrictions that had led to cardiac recovery. At 24, his doctor retired, his insurance changed, and he was lost to follow-up, leading to dietary liberalization. On presentation, he was diagnosed with pneumonia complicated by atrial fibrillation (AF). AF was treated with a diltiazem infusion, resulting in cardiogenic shock with an EF of 15% from 39%. He was transferred to an academic center where diltiazem was stopped, inotropes started, and impella placed. He started enteral nutrition with liquid protein, dextrose infusion, medium-chain triglyceride (MCT) oil, and levocarnitine, along with supportive care for pneumonia. Given persistent shock he was transferred to our center for advanced therapies evaluation. Notable changes included replacing MCT oil with triheptanoin and stopping the dextrose infusion. A repeat echocardiogram showed recovery of EF to 40%Conclusions:MLYCDD exhibits a range of phenotypes, with limited data on long-term outcomes. This presentation is atypical: an adult with cardiomyopathy diagnosed in adolescence, successfully treated, who decompensated after stopping a modified diet in the setting of pneumonia and diltiazem. This case offers several clinical insights: (1) Restrictive diets with levocarnitine supplementation are instrumental in treating MLYCDD. (2) Avoid diltiazem in heart failure to prevent iatrogenic shock. (3) Language-concordant care ensures patients understanding and supports seamless care transitions.

Circulation, Volume 150, Issue Suppl_1, Page ASa303-ASa303, November 12, 2024. Background:Active compression-decompression cardiopulmonary resuscitation (ACD-CPR) combined with an impedance threshold device (ITD) and controlled head-up positioning, collectively termed AHUP-CPR, is associated with improved outcomes compared with conventional CPR (C-CPR). Active decompression (AD) forcibly lifts the anterior chest wall with suction after each compression, while the ITD simultaneously impedes airflow into the lungs. AD and ITD synergistically lower intrathoracic pressure during decompression, improving venous return, ventricular refilling and cardiac output. Concurrently, head-up positioning lowers intracranial pressures and improves cardiac preload. This pig study focused on the physiological role of AD during AHUP-CPR, simulating real clinical scenarios.Methods:Ten male and female farm pigs (~40 kg) were anesthetized, intubated and ventilated. Hemodynamic parameters, end-tidal CO2 (ETCO2), and biventricular pressure-volume loops were recorded continuously. Ventricular fibrillation was induced and left untreated for 10 mins. Then, after 2 min of automated C-CPR in the flat position, AHUP-CPR with a 3 cm lift above the neutral position of the chest was performed for 13 min. AD was then abruptly discontinued, restarted 1 min later, and increased incrementally every min to up to 4 cm of active lift. Data were analyzed with a linear mixed-effects model, using random intercepts for individual pigs.Results:Upon cessation of AD, coronary and cerebral perfusion pressures, compression and decompression aortic pressures, compression right atrial pressure, ETCO2, right and left ventricular preload, compliance, stroke volume and cardiac output all decreased immediately and significantly (p3 cm of AD, was needed to maintain and optimize hemodynamics during AHUP-CPR in pigs. After pausing AD, incremental restoration of AD resulted in nearly complete restoration of peripheral and central hemodynamic measures. These observations support the benefit of and need for ≥3 cm of AD to optimize AHUP-CPR. Effective means to generate ≥3 cm of AD should be considered when implementing and developing AHUP-CPR devices to optimize outcomes.

Circulation, Volume 150, Issue Suppl_1, Page A4143150-A4143150, November 12, 2024. Background:The FACTOR-64 study was a randomized controlled trial designed to assess whether routine screening for CAD by coronary computed tomography angiography (CCTA) in high-risk patients with diabetes followed by CCTA-directed therapy would reduce the risk of death and nonfatal coronary outcomes. Results at four years showed a lower revascularization rate (3.1% (14) vs. 8.9% (40), p

Circulation, Volume 150, Issue Suppl_1, Page A4143109-A4143109, November 12, 2024. Background:The use of cardiac resynchronization therapy (CRT) devices has significantly increased in usage in recent years. Identifying predictors of mortality in CRT patients remains an area of investigation.Objective:To establish a relationship between timed up-and-go test time (TUGT) and mortality in heart failure patients (HF) with CRT devices.Methods:This retrospective study included 506 patients with heart failure with reduced ejection fraction (HFrEF) who underwent CRT implantation at our institution between 2017-2022. All patients were followed up with a multidisciplinary team, including electrophysiology and HF physicians about 6 months after CRT implantation, where frailty was assessed. We used TUGT as a measure of frailty and divided patients into 2 groups: TUGT: >15 seconds (n=73) and ≤15 seconds (n=433). The primary endpoint was a composite of left ventricular assist device implantation, transplant, or death at 2 years post-CRT. Data was collected retrospectively from electronic medical records.Results:The study population was 65.6% male, with a mean age of 69.1 years, and 79.4% of devices being CRT-D.Response was defined as an improvement in LVEF >5% with reduction in LVESV >10%; anybody not meeting this definition was classified as a non-responder. Responder and non-responder rates among TUGT >15 and TUGT15s have worse outcomes (Figure 1).Conclusion:Frailty testing using TUGT post-CRT implantation is a strong predictor of mortality in HFrEF patients after CRT implantation.

Circulation, Volume 150, Issue Suppl_1, Page A4136210-A4136210, November 12, 2024. Background:In the management of acute myocardial infarction (AMI) following percutaneous coronary intervention (PCI), dual antiplatelet therapy (DAPT) plays a crucial role in preventing recurrent ischemic events. Recent studies have explored the feasibility and safety of de-escalating DAPT from ticagrelor to clopidogrel.Methods:We conducted a systematic review and meta-analysis by searching several databases, including Cochrane Central Register of Controlled Trials (CENTRAL), PubMed, MEDLINE (including MEDLINE InProcess) (OvidSP), Web of Science, Embase (OvidSP), and Scopus. We assessed the risk of bias using the ROB2 Cochrane tools for randomized controlled trials (RCTs). The analysis was performed using RevMan Cochrane software.Results:A total of ten studies including observational and clinical studies involving N=18,001patients (11,458 de-escalated from ticagrelor to clopidogrel after 12 months and 6,543 remained on ticagrelor after 12 months post-PCI) were included. There was no difference in the risk of all-cause death (RR 0.98; 95% CI 0.69 to 1.38; p=0.90), cardiovascular death (RR 1.09; 95% CI 0.68 to 1.74; p=0.73), myocardial infarction (RR 0.90; 95% CI 0.71 to 1.14; p=0.37) and stroke (RR 0.81; 95% CI 0.50 to 1.32; p=0.41) between the two groups.Conclusion:In conclusion, transitioning from ticagrelor to clopidogrel in acute myocardial infarction following percutaneous coronary intervention appears to be a feasible strategy for de-escalating dual antiplatelet therapy (DAPT). While maintaining efficacy in preventing adverse cardiovascular events, such as stent thrombosis, this approach may mitigate bleeding risks associated with prolonged ticagrelor use.

Circulation, Volume 150, Issue Suppl_1, Page ASa308-ASa308, November 12, 2024. Background:Active compression-decompression (ACD) cardiopulmonary resuscitation (CPR), an impedance threshold device (ITD) and controlled, gradual, automated head and thorax elevation, collectively termed automated Head Up Position (AHUP) CPR, increases cerebral perfusion pressure (CerPP), brain blood flow, coronary perfusion pressure (CorPP), end tidal CO2 (ETCO2) and cerebral oximetry (rSO2) in animal models when compared with conventional (C) CPR. AHUP-CPR in patients is associated with increased neurologically favorable survival versus C-CPR. This study tested the hypothesis that AHUP CPR will increase cardiac stroke volume (SV) and other hemodynamics compared with C-CPR in a porcine model of cardiac arrest.Methods:Farm pigs (n=15) were sedated, anesthetized, and ventilated. Hemodynamics, including intracardiac conductance catheter based biventricular (BiV) pressure-volume (PV) loops, were continuously measured and recorded. After 10 minutes of untreated ventricular fibrillation, C-CPR was performed for 2 minutes in the supine position using an automated CPR device designed for pigs at a rate of 100 compressions/minute, depth of 21% of the chest antero-postero diameter, a 50% duty cycle, and no active decompression. ACD+ITD was then performed with 3 cm of active decompression for 2 minutes, followed by AHUP-CPR, where the head and thorax were initially raised to 10 cm and 8 cm for a 2-minute priming phase, followed by elevation over the next 2 minutes to 24 cm and 9 cm. A linear mixed-effects model with a random intercept for individual pigs was used for statistical analysis.Results:CerPP, CorPP, ETCO2, and rSO2, as well as BiV SV and cardiac output, increased progressively and significantly with implementation of AHUP-CPR (p

Circulation, Volume 150, Issue Suppl_1, Page A4137805-A4137805, November 12, 2024. Background:Many of the 1.4 million adults with congenital heart disease (CHD) are lost to follow up (LTF). We aimed to identify factors associated with LTF and compare to those who remained in care.Methods:In the Congenital Heart Disease Project to Understand Lifelong Survivor Experience (CHD PULSE), we performed a cross-sectional survey in 2021-2023 of CHD survivors with a history of intervention at 11 centers in the Pediatric Cardiac Care Consortium, a large US-based registry of CHD procedures. Participants reported their cardiac history, general health, education, income, health insurance, healthcare utilization, and marital status. LTF was defined as having seen a cardiologist three or more years ago.Results:Among the 3109 respondents with CHD (median age 33) there were 1157 (37%) LTF and 1952 (63%) in care. Age, sex, marital status, and household income were not risk factors for LTF; but lack of health insurance, lower severity of CHD, and increasing time since last heart surgery were. Notably, respondents who reported being told in adolescence about the lifelong need for a cardiologist were almost three times as likely to report being in care (79% vs 28%, p

Circulation, Volume 150, Issue Suppl_1, Page A4140642-A4140642, November 12, 2024. Background:The diagnosis of VVS largely relies on clinical history and simple diagnostic tools (e.g., electrocardiogram) to rule out dangerous differential diagnoses. However, using the head-up tilt test (HUTT) has become controversial among clinicians. This retrospective study aims to evaluate whether the prodromal symptoms experienced during HUTT are consistent with those experienced during spontaneous syncope.Methods:This study utilized data from the HUTT registry at the Syncope Unit of the tertiary Heart Center, focusing on adults aged 18 and older diagnosed with VVS. Diagnoses were based on clinical histories, physical examinations, and the latest syncope guidelines. Out of 1914 patients with HUTT results, 764 patients with positive tests were analyzed for mutual prodromal symptoms during HUTT and spontaneous syncope.Results:The McNemar test revealed significant differences for several symptoms, including palpitation (X2 = 30.59, P < 0.001), nausea (X2 = 16.13, P < 0.001), chest pain (X2 = 24.32, P < 0.001), abdominal discomfort (X2 = 22.33, P < 0.001), flushing (X2 = 10.87, P < 0.001), and aura (X2 = 19.86, P < 0.001), indicating discrepancies in the occurrence of these symptoms. Cohen's Kappa values ranged from 0.06 to 0.32, signifying slight to fair agreement. Specifically, diaphoresis (k = 0.32), palpitation (k = 0.27), and vertigo (k = 0.25) demonstrated fair agreement, whereas nausea, aura, chest pain, abdominal discomfort, and flushing exhibited slight agreement. Among the 640 patients who experienced prodrome during spontaneous syncope, 110 (17.19%) had no symptoms. Conversely, among the 123 patients who did not experience prodrome, 96 (78.05%) experienced at least one symptom during the tilt test (Figure).Conclusion:The assessment of prodromal symptoms during HUTT compared to spontaneous syncope showed significant differences for several symptoms and overall low levels of agreement. Also, tilt cannot differentiate patients with or without prodrome during their spontaneous spells.

Circulation, Volume 150, Issue Suppl_1, Page A4138374-A4138374, November 12, 2024. Introduction:Although coronary endothelial dysfunction is thought to affect coronary atherothrombogenic processes, there has been little practical evidence for the relationship between clinical evolution of fatal or non-fatal acute coronary syndrome and coronary endothelial dysfunction.Hypothesis:We assessed hypothesis that coronary endothelial dysfunction has clinical impacts on development of acute coronary syndrome and fatal cardiovascular events.Methods:Coronary endothelial dysfunction was practically graded by the flow-mediated endothelium-dependent reactive changes in coronary artery diameter (CFMD) to infusion of adenosine triphosphate (ATP ; 50μg) into the normal left coronary artery using quantitative coronary arteriography in 150 patients with stable coronary artery disease. The enrolled patients were categorized into tertile groups according to the values of CFMD, and we prospectively followed-up major adverse clinical cardiac events including acute coronary syndrome and cardiovascular death.Results:For a mean follow-up period of 132 months (range; 120 to 144) with complete follow-up, the patients in the lower third with severe coronary endothelial dysfunction (Group-L) more frequently developed acute coronary syndrome than those in the middle third with mild coronary endothelial dysfunction (Group-M) plus those in the higher third without coronary endothelial dysfunction (Group-H) [Group-L versus Group-M plus Group-H: 15(30%) versus 5(10%) plus 0(0%), p

Circulation, Volume 150, Issue Suppl_1, Page A4133654-A4133654, November 12, 2024. Introduction:Conventional cardiovascular disease (CVD) risk scores are inaccurate for prostate cancer patients (PC); the Guha-Stabellini machine learning (GS-ML) score shows promise but lacks external validation.Hypothesis:The GS-ML score is superior to conventional CVD scores in patients with PC.Aim(s):To conduct external validation of the GS-ML score in patients diagnosed with PC.Methods:The validation used holdout data from Seidman Cancer Center’s CAISIS platform (internal validation cohort) and RADICAL PC1 (external validation cohort; a prospective study of men diagnosed with PC within 1 year or starting ADT within 1 month of enrollment), matching covariates with the GS-ML score. With limited follow-up, short-term CVD (2,000 days from PC diagnosis) was the outcome. Performance was assessed via area under the receiver operating characteristic curve (AUC) with assistance from Youden statistic cutoff, comparing metrics with ACC/AHA pooled cohort equations (PCE), SCORE-2, and AHA-PREVENT scores. Atherosclerotic cardiovascular disease (ASCVD) included non-fatal ischemic stroke and myocardial infarction. CVD included ASCVD and heart failure.Results:We included 2,495 patients from RADICAL PC1 and 1,506 from the internal validation cohort (Table 1). In the internal validation cohort, the CVD AUCs were 0.58, 0.49, 0.65, 0,73, and 0.75 for PCE, SCORE2, PREVENT simple, PREVENT enhanced, and GS-ML score, respectively. In RADICAL PC1, the AUCs for CVD were 0.60, 0.43, 0.67, and 0.64 for PCE, SCORE2, PREVENT simple and GS-ML score, respectively. For ASCVD, internal validation cohorts’ AUCs were 0.63, 0.62, 0.66, 0.73, and 0.67 for PCE, SCORE2, PREVENT simple, PREVENT enhanced, and Guha-Stabellini, respectively. The RADICAL PC1 ASCVD AUCs were 0.64, 0.46, 0.65, and 0.61, for PCE, SCORE2, PREVENT simple and GS-ML scores, respectively,Conclusion(s):The GS-ML score was validated for PC patients, showing superior performance to PCE and SCORE2, and similar performance to AHA-PREVENT in predicting CVD. The European Cardio-Oncology guidelines should reconsider using SCORE2 for PC patients. Further improvement and validation with nationally representative datasets are needed to corroborate these findings and enhance the generalizability of the GS-ML score.

Circulation, Volume 150, Issue Suppl_1, Page A4144360-A4144360, November 12, 2024. Background:Transthyretin cardiac amyloidosis (ATTR-CA) typically manifests with heart failure (HF). Discontinuing beta-blockers and avoiding angiotensin converting enzyme inhibitors/angiotensin receptor blockers (ACEi/ARB) in patients with ATTR-CA has been recommended.Methods:We investigated the prescription of neurohormonal therapies and their relationship with all-cause mortality in a multicenter cohort.Results:Patients (n=926) had a median age of 79 years (interquartile range 74-83), 90% were men, 17% had a left ventricular ejection fraction (LVEF) ≤40%, and 27% were in New York Heart Association (NYHA) class III/IV. At diagnosis, 60% of patients were on beta-blockers, 58% on ACEi/ARB/ARNI, and 35% on MRA. Patients on beta-blockers had more often NYHA class III/IV, a greater burden of comorbidities, and lower LVEF, and those on ACEi/ARB/ARNI had more comorbidities. Nonetheless, the survival of patients on beta-blockers or ACEi/ARB/ARNI was not significantly shorter over a 2.5-year follow-up (1.6-3.8) (p=0.577 and p=0.977, respectively), and patients on both drugs had not a worse outcome than those not receiving any neurohormonal drug (p=0.575). During the entire follow-up, the number of neurohormonal drugs remained unchanged in 54%, decreased in 27%, and increased in 19%. Patients with a number of neurohormonal drugs either unchanged or increased had a lower risk of mortality (odds ratio 0.71, 95% confidence interval 0.53-0.95, p=0.023).Conclusions:ATTRwt-CA patients on beta-blockers or ACEi/ARB/ARNI at diagnosis did not have a shorter survival. Beta-blockers were discontinued less often than ACEi/ARB/ARNI. There was no sign of better outcomes in patients discontinuing these therapies, or worse outcomes in those starting them.

Circulation, Volume 150, Issue Suppl_1, Page A4143931-A4143931, November 12, 2024. Background:According to the 2020 ACC/AHA guidelines, patients with severe aortic stenosis (AS) should either undergo rapid AVR or be followed by an echocardiogram (echo) every 6-12 months to assess disease progression. Discrepancies have been reported in the care of severe AS.Goal:This study aims to evaluate adherence to guidelines, and identify factors associated with guidelines recommended care in patients with severe AS.Methods:All patients with a first echo report of severe AS diagnosis from January 2019 to December 2022 in a tertiary care academic health center were included. Adherence to guidelines was defined as undergoing AVR in 6 months or having a follow-up echo within 6-12 months of the index echo. Univariable and multivariable Cox proportional hazards models were performed to identify factors associated with guidelines recommended care. Patients who did not meet the endpoint and died before one year were censored at the time of death.Results:Of the 1655 patients included in the study (mean ± SD age: 77±12 years, 56% male, 80% white, 10% African-American, and 10% other race), 727 (43%) had AVR in 6 months, 184 (11%) had no AVR in 6 months but a follow-up echo within 6-12 months, and 744 (45%) did not achieve the guidelines recommended care. Two hundred and thirty-eight (14%) patients died within one year (178 patients did not meet guidelines). The multivariable Cox proportional hazards model (Table) revealed that younger age, lower LVEF, higher aortic valve mean gradient, cardiovascular history or risk factors, and having a cardiac specialty provider increased the rate of adherence to guidelines, while African-Americans and patients with renal failure were less likely to receive the guidelines recommended care.Conclusions:A substantial proportion of patients with severe AS does not receive the guidelines recommended care and follow-up. Factors including age, race, comorbidities such as renal failure, cardiovascular history and risk factors, provider specialty, and characteristics of the AS are associated with these discrepancies. These results call for urgent action to guide patient and provider behavior towards equitably implementing guideline-recommended valvular heart disease therapies.

Circulation, Volume 150, Issue Suppl_1, Page A4146105-A4146105, November 12, 2024. Introduction:International guidelines recommend cardiogenetic screening of relatives to sudden cardiac death (SCD) patients suspected of having an inherited cardiac disease. However, little is known about the long-term diagnostic yield and risk of cardiac events among relatives. This information is crucial for designing appropriate follow-up strategies for relatives.Aims:To determine the 10-year diagnostic yield and frequency of clinical outcomes in referred families to patients with all cause-SCD.Methods:In this retrospective single-center study, we included consecutively referred families to patients with all cause-SCD from 1 January 2005 until 1 October 2018. Upon referral, the cause of death in the SCD proband was assessed through autopsy reports, genetic testing, and premortem medical records. First-degree relatives were screened following a standard protocol, and diagnostic yields were assessed after five and 10 years of follow-up. Furthermore, cardiac events (i.e. death due to cardiac cause, SCD, aborted SCD, sustained ventricular tachycardia, appropriate implantable cardioverter defibrillator therapy) and relevant clinical outcomes in relatives were monitored.Results:A total of 686 relatives (304 families) were included, and mean age at follow-up was 45 years (47% males). The relatives were followed for a mean of 10.9 years during which 22 patients were lost to follow up. Screening over five years led to 73 relatives (49 families) being diagnosed, resulting in a diagnostic yield of 11%. After 10-years of follow-up, 82 relatives (56 families) were diagnosed, increasing the overall diagnostic yield to 12% with arrhythmogenic right ventricular cardiomyopathy being the most prevalent diagnosis. A total of 11 relatives experienced a cardiac event during follow-up, of whom 10 had a definite diagnosis.Conclusion:Over 10 years of follow-up, 82 (12%) relatives to patients with all cause-SCD were diagnosed with an inherited cardiac disease, primarily within the first five years. The majority of relatives who experienced a cardiac event during follow-up had a definite diagnosis. This underscores the importance of early detection and management of inherited cardiac diseases in the screening of SCD relatives.