Farwati, Medhat

Introduction: Severe hypertriglyceridemia (SHTG), defined as plasma triglyceride (TG) level ≥ 500 mg/dl, is a risk factor for cardiovascular disease. In the NHANES dataset (2001 - 2006), the prevalence of SHTG was 1.7%. However, the prevalence of primary SHTG in the community is unknown. We conducted a community-based study to estimate the prevalence of primary SHTG, in Olmsted County, Minnesota. Methods: We used the Rochester Epidemiology Project (REP) records linkage system to identify individuals with TG ≥ 500 mg/dl between 1998-2015. Index date was defined as the first documentation of TG ≥500 mg/dl during the study period. We excluded individuals with mixed hyperlipidemia (TG ≥500 mg/dl and non-high density lipoprotein (HDL) cholesterol ≥190 mg/dl). HTG was defined as secondary in the presence of hypothyroidism, biliary obstruction, nephrotic syndrome, uncontrolled diabetes, pregnancy, liver disease, renal failure, BMI ≥35 and medication use (isotretinoin, tetracyclines, anti-retrovirals). Primary HTG was defined as absence of an identifiable secondary etiology. Characteristics of the primary SHTG individuals were ascertained from the electronic health record. Results: SHTG was identified in 3802 individuals of whom 2511 had mixed hyperlipidemia, 743 had a secondary cause for SHTG and the remaining 548 had primary SHTG. Using the 2015 population census of 151,436 for Olmsted County, the prevalence of SHTG was 2.5%, of secondary SHTG was 0.49% and of primary SHTG was 0.36%. Only half of the primary SHTG group had a diagnosis of dyslipidemia in the electronic health record and 44.3% were on lipid-lowering therapy on index date. Characteristics of individuals with primary SHTG are summarized in Table 1. Conclusions: These data highlight that primary SHTG is relatively common (≈1 in 300, similar to the prevalence of familial hypercholesterolemia), underdiagnosed and undertreated.

Introduction: Familial Hypercholesterolemia (FH) is a common inherited disorder of lipid metabolism. FH patients are at an increased risk of atherosclerotic cardiovascular disease (ASCVD) due to elevated low-density lipoprotein cholesterol (LDL-C) levels. Patients with FH may not reach target LDL-C levels despite maximally tolerated statin (±ezetimibe) therapy (MTT). The AHA scientific statement on FH highlighted the need for developing decision aids (DAs) to facilitate shared decision making (SDM) regarding additional therapy including PCSK9 inhibitors. Methods: A user-centered design process was initiated to understand FH treatment decisions and to develop prototype interventions. Patients with FH had their diagnosis confirmed by genetic testing. Encounters between FH patients and health care providers were video recorded and analyzed to assess how decisions are made regarding lipid lowering therapy. Nine encounters were recorded: 3 with a genetic counselor and 6 with an FH specialist. Informed by clinical encounters and literature review, we developed content for a DA related to available therapeutic options for FH patients on MTT. Results: A prototype online DA was created to support in-encounter discussion of a patient’s risk of adverse cardiovascular events and which therapies may be best suited to the patient (Figure). 5 and 10-year ASCVD risk is estimated using the SAFEHEART Risk Score (SRS). Risk is communicated in numbers using a 100-person icon array and the language “Out of 100 persons like you...”. Therapeutic options include watchful waiting, bile acid sequestrants, and PCSK9 inhibitors. For each option, the degree of risk reduction, costs, and side effects are presented. The tool is currently being refined by analyzing additional encounters with FH patients. Conclusion: We developed an interactive prototype DA to facilitate SDM for FH patients on MTT. After further iterative testing the tool will be deployed in the electronic health record.

Introduction: Multiple arrhythmias including ventricular tachycardia (VT) and atrial fibrillation (AF) have been reported in patients with left ventricular noncompaction (LVNC). We sought to determine the frequency of rhythm abnormalities on Holter monitoring, and their association with all-cause mortality, in patients with LVNC. Methods: Imaging databases from 2000-2016 were reviewed to identify patients with LVNC. On transthoracic echocardiography (TTE), LVNC was defined as: end systolic noncompacted: compacted (NC:C) ratio>2 and end diastolic epicardium to trough: epicardium to peak trabeculation (X:Y) ratio<0.5. Holter monitor reports from the medical record were evaluated. VT was defined as ≥3 consecutive beats of ventricular origin. All-cause mortality was obtained from a web-based research and location service (Accurint). We used a proportional hazards model to determine associations of Holter variables with all-cause mortality. Results: Among 340 LVNC patients, Holter reports were available for 177 patients (median age 45 years, interquartile range [IQR] 33-61; 45% females). On TTE, the median NC:C ratio was 2.7 (IQR 2.4-3.2) and X:Y ratio was 0.25 (IQR 0.20-0.28). The median ejection fraction (EF) was 48% (IQR 33-58). 48 patients (27%) had at least one episode of VT (median 4 episodes, IQR 2-4). The median longest duration of VT was 5 beats (IQR 4-9) and maximum rate was 148 beats per minute (IQR: 125-169). PVC burden >5% and 20% was present in 13% and 5% of patients respectively. Patients with EF<50% were more likely to have VT (43% vs 16%, p=0.0003) and PVC burden >5% (19% vs 7%, p=0.03). AF was infrequent (N=5, all persistent). All-cause mortality was 12% over median 6.9 years of follow up (IQR: 3.6-11.3 years). Presence of VT and PVC burden on Holter monitoring were not associated with all-cause mortality (p>0.05). Among patients with VT, the number, longest duration, and fastest rate of VT episodes were not associated with all-cause mortality. Conclusions: Ventricular tachycardia on Holter monitoring is common among patients with LVNC, especially those with a reduced EF. However, the clinical significance of this finding is uncertain, and presence of VT was not associated with long term all-cause mortality.

Introduction: Increasingly, catheter ablation for atrial fibrillation (AF) has been utilized for rhythm control and to improve quality of life (QoL). Data are limited on patient-centered ablation outcomes in individuals with chronic kidney disease (CKD). Objective: To examine clinical outcomes and QoL following AF ablation in CKD patients from a large patient-reported outcomes (PRO) database. Methods: All patients undergoing AF ablation (2013-2016) at our center were enrolled and assessed for outcomes using automated PRO surveys (baseline, 3 and 6 months after ablation, every 6 months thereafter). This analysis captured data at 1- and 3-year follow-up. CKD was defined as estimated glomerular filtration rate (eGFR) <60 ml/min using the 2021 CKD-EPI equation. The atrial fibrillation symptom severity scale (AFSSS) was used as the main measure for QoL assessment. In addition, data were collected on clinical improvement by patient report, AF burden, and healthcare utilization. Results: A total of 488 patients were identified (66% male, mean age 68.5, 56.2% persistent AF). QoL was significantly improved from baseline median (IQR) AFSSS of 14 (6-19.5) to 3 (0-9) at 1-year and 4 (0-9.5) at 3-year; P≤0.0001 for both. About 90% of patients reported improvement in their overall AF-related symptoms both at 1- and 3-year follow up. The proportion of patients in AF at the time of completion of the baseline survey was 51.2%, and this decreased to 6.2% at 1-year and 9.7% at 3-year follow up (P<0.0001 for both). AF burden was significantly reduced at 1-year and 3-year including frequency and duration of episodes (P<0.05 for both), with associated decrease in ER visits and hospitalizations; P<0.0001 for all comparisons. The proportion of patients on antiarrhythmics decreased from 61.8% at baseline to 35.6% and 22.7% at 1-year and 3-year, respectively (P<0.0001 for both). Conclusions: In a large AF-specific PRO system, catheter ablation for AF was associated with a significant improvement of QoL in most patients with CKD; with a decrease in AF burden and healthcare utilization.

AbstractBackgroundWhile ventricular tachycardia (VT) in the setting of postmyocardial infarction left ventricular aneurysms (LVA) is not uncommonly encountered, there is a scarcity of data regarding the safety, efficacy, and outcomes of ablation of VT in this subset of patients.MethodsOur study included consecutive patients aged 18 years or older with postmyocardial infarction LVA who presented to Mayo Clinic for catheter ablation of VT between 2002 and 2018.ResultsOf 34 patients, the mean age was 70.4 ± 9.1 years; 91% were male. Mean LVEF was 29 ± 9.7% and left ventricular end‐diastolic dimension was 64.9 ± 6.6 mm. The site of the LVA was apical in 21 patients (62%). Fifteen patients (44%) presented with electrical storm or incessant VT. Nine patients (26%) had a history of intracardiac thrombus. All except for one patient had at least one VT originating from the aneurysm. The mean number of VTs was 2.9 ± 1.7. All patients underwent ablation at the site of the aneurysm. Ablation outside the aneurysm was performed in 13 patients (38%). Low‐voltage fractionated potentials and/or late potentials at the aneurysmal site were present in all cases. Complete elimination of all VTs was achieved in 18 (53%), while the elimination of the clinical VT with continued inducibility of nonclinical VTs was achieved in a further 11 patients (32%). Two patients developed cardiac tamponade requiring pericardiocentesis. During a mean follow‐up period of 2.3 ± 2.4 years, 11 patients (32%) experienced VT recurrence. Freedom from all‐cause mortality at 1‐year follow‐up was 94%.ConclusionRadiofrequency catheter ablation targeting the aneurysmal site is a feasible and reasonably effective management strategy for clinical VTs in patients with postinfarction LVA.

Background: Atrial fibrillation (AF) ablation targets improvement in quality of life (QoL). Data is scarce on predictors of QoL improvement following ablation. We aimed to investigate the clinical characteristics underlying differential response in QoL after AF ablation (with or without arrhythmia recurrence). Methods: All patients undergoing AF ablation (2013–2016) at our center were prospectively enrolled in a fully automated patient-reported outcomes registry. A large number of variables were collected including AF symptom severity scale and AF burden (as indicated by AF frequency and duration scores). Patients were divided into 3 groups based on self-report of QoL improvement: remarkable (super responders), mild/moderate, and unchanged or worse (nonresponders). Univariable and multivariable logistic regression models assessed clinical characteristics and QoL outcomes. Results: A total of 956 patients were included (25% females, mean age 63.9). Most patients (≈80%) were super responders (n=761), 138 (14.4%) had mild/moderate improvement, and 57 (5.9%) were nonresponders. The strongest predictors of remarkable QoL improvement were freedom of arrhythmia recurrence (OR, 2.42 [95% CI, 1.27–4.59], P =0.007) and lower AF burden at 12 months. Similarly, higher AF burden was significantly associated with clinical nonresponse. In patients with observed clinical recurrence-QoL mismatch, changes in AF burden at 12 months were the main predictors of QoL outcome, with lower burden predicting higher improvement in QoL and vice versa. Conclusions: Most patients derive significant QoL benefit from AF ablation. Freedom from arrhythmia recurrence and lower AF burden predict remarkable QoL improvement following ablation.

Background Hypertriglyceridemia is associated with increased risk of coronary heart disease but the association is often attributed to concomitant metabolic abnormalities. We investigated the epidemiology of primary isolated hypertriglyceridemia (PIH) and associated cardiovascular risk in a population‐based setting. Methods and Results We identified adults with at least one triglyceride level ≥500 mg/dL between 1998 and 2015 in Olmsted County, Minnesota. We also identified age‐ and sex‐matched controls with triglyceride levels <150 mg/dL. There were 3329 individuals with elevated triglyceride levels; after excluding those with concomitant hypercholesterolemia, a secondary cause of high triglycerides, age <18 years or an incomplete record, 517 patients (49.4±14.0 years, 72.0% men) had PIH (triglyceride 627.6±183.6 mg/dL). The age‐ and sex‐adjusted prevalence of PIH in adults was 0.80% (0.72–0.87); the diagnosis was recorded in 60%, 46% were on a lipid‐lowering medication for primary prevention and a triglyceride level <150 mg/dL was achieved in 24.1%. The association of PIH with coronary heart disease was attenuated but remained significant after adjustment for demographic, socioeconomic, and conventional cardiovascular risk factors (hazard ratio [HR], 1.53; 95% CI, 1.06‐2.20; P = 0.022). There was no statistically significant association between PIH and cerebrovascular disease (HR, 1.06; 95% CI, 0.65‐1.73, P = 0.813), peripheral artery disease (HR, 1.27; 95% CI, 0.43‐3.75; P = 0.668), or the composite end point of all 3 (HR, 1.28; 95% CI, 0.92‐1.80; P =0.148) in adjusted models. Conclusions PIH was associated with incident coronary heart disease events (although there was attenuation after adjustment for conventional risk factors), supporting a causal role for triglycerides in coronary heart disease. The condition is relatively prevalent but awareness and control are low.

Background The prognosis of left ventricular noncompaction (LVNC) remains elusive despite its recognition as a clinical entity for >30 years. We sought to identify clinical and imaging characteristics and risk factors for mortality in patients with LVNC. Methods and Results 339 adults with LVNC seen between 2000 and 2016 were identified. LVNC was defined as end‐systolic noncompacted to compacted myocardial ratio >2 (Jenni criteria) and end‐diastolic trough of trabeculation‐to‐epicardium (X):peak of trabeculation‐to‐epicardium (Y) ratio <0.5 (Chin criteria) by echocardiography; and end‐diastolic noncompacted:compacted ratio >2.3 (Petersen criteria) by magnetic resonance imaging. Median age was 47.4 years, and 46% of patients were female. Left ventricular ejection fraction <50% was present in 57% of patients and isolated apical noncompaction in 48%. During a median follow‐up of 6.3 years, 59 patients died. On multivariable Cox regression analysis, age (hazard ratio [HR] 1.04; 95% CI, 1.02–1.06), left ventricular ejection fraction <50% (HR, 2.37; 95% CI, 1.17–4.80), and noncompaction extending from the apex to the mid or basal segments (HR, 2.11; 95% CI, 1.21–3.68) were associated with all‐cause mortality. Compared with the expected survival for age‐ and sex‐matched US population, patients with LVNC had reduced overall survival ( P <0.001). However, patients with LVNC with preserved left ventricular ejection fraction and patients with isolated apical noncompaction had similar survival to the general population. Conclusions Overall survival is reduced in patients with LVNC compared with the expected survival of age‐ and sex‐matched US population. However, survival rate in those with preserved left ventricular ejection fraction and isolated apical noncompaction was comparable with that of the general population.

Abstract Recent guideline statements approve the performance of magnetic resonance imaging (MRI) in patients with non-conditional cardiac implantable electronic devices (CIEDs) under certain closely monitored conditions. Data from current registries may have lower power to discern changes that may occur in the CIED after the MRI. Objective We aimed to systematically review the literature to identify the adverse events and significance of changes in device function associated with performing MRI in patients with CIEDs Methods A comprehensive literature search of the databases was performed between 1980- 2017. Two independent reviewers selected studies and extracted data. A random-effects model was used for meta-analysis. Results A total of 7,422 patients underwent 8,865 MRI studies. No death occurred post MRI. Clinical adverse events were extremely rare (mostly less than 1%) and are summarized in Table 1. No significant changes in the pooled mean effect size estimate was noted for the changes in the lead parameters (pacing threshold, sensing and impedance) Binary outcomes post magnetic resonance imaging in patients with non-conditional cardiac implantable electronic devices Outcome Sample size Number of patients Rate SE LL of CI UL of CI Death 7401 0 0 0 0 0 Atrial arrhythmia 7173 10 0.001 0.0004 0.0007 0.0024 Ventricular arrhythmia 7371 9 0.0012 0.0004 0.0006 0.0022 Oversensing 4981 17 0.0034 0.0008 0.0020 0.0053 Inhibition of pacing 7371 6 0.0008 0.0003 0.0003 0.0016 Lead Failure/Generator Failure 7475/7475 2/8 0.0002/0.0011 0.0001/0.0004 0.0001/0.0005 0.0009/0.002 Power on Reset 1388 105 0.0131 0.0014 0.0107 0.0161 Did not complete scan 6851 13 0.0019 0.0005 0.0011 0.0031 Chest pain 7080 11 0.0021 0.0004 0.0008 0.0027. Lead threshold rise (>0.5v/>50%-A/V) 5076/6246 12/16 0.0024/0.0026 0.0007/0.0006 0.0013/0.0015 0.0040/0.0041 Change in Battery voltage >0.04V 7132 42 0.0061 0.0009 0.0043 0.0079 Sensing decrease >50%-A/V 5087/5834 17/9 0.0033/0.0015 0.0008/0.0005 0.0020/0.0008 0.0052/0.0028 Impedance changes >50ohms/>50% 5810 22 0.0038 0.0008 0.0024 0.0057 Rise in cardiac enzymes 1703 26 0.0152 0.0030 0.0102 0.0219 A, atrial; V, ventricular; SE, standard Error; LL, lower limit; UL, upper limit; CI, Confidence intervals. Conclusions MRI in patients with non-conditional CIEDs can be performed with high degree of safety and low rate of clinical events when performed under standardized protocols Acknowledgement/Funding None