Klarich, Kyle W.

Abstract Background Sarcoidosis, amyloidosis, hemochromatosis and scleroderma are the most common systemic disorders leading to infiltrative cardiomyopathy (InCM). Each of them has been associated with conduction abnormalities and sudden cardiac death. In patients who undergo in-hospital cardiac arrest, a high index of suspicion is required to rule out InCM as an underlying contributor. Purpose We aimed to analyse the temporal trend in the prevalence of InCM among patients with in-hospital cardiac arrest and identify factors associated with increased mortality in this population. Methods We analysed data from the National Inpatient Sample (NIS), which is the largest publicly available database of in-hospital patients in the Unites States. Using ICD-10 coding, we identified patients who were hospitalized across 10 years from 2010–2019. Patients with diagnosis codes for both cardiac arrest and a subtype of InCM were included. Demographic characteristics and co-morbidity data were collected. Statistical analysis was performed using STATA. Results The total number of patients with a diagnosis of in-hospital cardiac arrest was 19,34,260. Of these, the total number with InCM was 14,803 (0.77%), which comprised our study population. Mean age was 63 years. The overall prevalence of InCM across the years ranged between 0.75 to 0.9%, with a significant temporal increase (p<0.01). Prevalence of females ranged between 51% to 62% and significantly outnumbered males across the time period. (p=0.011). Incidence of in-hospital mortality ranged between 61% to 76% for females and 30 to 38% for males. Race based analysis showed that prevalence of Caucasians ranged from 51% to 62% while that of African Americans (AA) ranged from 57% to 73%. The incidence of mortality was similar in both populations. Sarcoidosis was the most prevalent form of InCM, accounting for nearly two-fifths of all cases while amyloidosis and scleroderma both accounted for nearly 20% each. Haemochromatosis was present in 10% of the population. Overall mortality rate in our study population was 60.6%. The following co-morbidities were more prevalent in patients with InCM than those without: history of heart failure, chronic obstructive pulmonary disease (COPD), chronic kidney disease, anaemia, malignancy, coagulopathy, ventricular tachycardia, acute kidney injury and stroke. The following factors were independent predictors of in-hospital mortality-age (p=0.001), female gender (p=0.016), Hispanic race (p=0.039), history of COPD (p<0.001) and presence of malignancy (p=0.042). Conclusion The prevalence of infiltrative cardiomyopathy in patients with in-hospital cardiac arrest is increasing. Females, older patients and Hispanic population are at an increased risk of mortality. Sex and race based disparities in the prevalence of InCM in patients with in-hospital cardiac arrest is an area of further research. Funding Acknowledgement Type of funding sources: None.

Introduction and Hypothesis: In patients with hypertrophic cardiomyopathy (HCM), ventricular tachycardia (VT) is associated with adverse prognosis. Cardiopulmonary exercise testing (CPET) is part of the standard clinical evaluation in HCM. However, limited data are available on the incidence of VT and other adverse events during CPET in HCM. Methods: We reviewed the Mayo Clinic’s Integrated Stress Center Database for CPETs performed on HCM patients between January 2011 and July 2016. Patients were referred for clinical CPET by a physician, and medications were continued for the test. Electronic medical records were reviewed for clinical information and CPET data. Results: A total of 1,208 CPETs were performed on 976 HCM patients during the study period. Most tests were symptom-limited (94.6%), most common symptom was dyspnea (55%). Despite the relatively high number of patients with a history of VT (10.4%) and/or cardiac arrest (1.6%), there was a low rate of adverse events. Non-sustained VT occurred in 6 patients, most frequently during active recovery (3/6), and 2 of these 6 patients had prior history of VT. A 34 year-old female patient experienced sustained VT degenerating into ventricular fibrillation terminated by ICD shock. One patient had syncope during the test but no arrhythmia. Importantly, none of the patients died within 48 hours of the exercise test. Conclusions: In a large cohort of HCM patients, the incidence of VT or other adverse events was a rare finding during symptom-limited CPET.

AbstractObjectivesThe aim of this study was to determine the safety of eliminating the pre‐discharge transthoracic echocardiogram (TTE) on 30‐day outcomes in patients undergoing transcatheter aortic valve replacement (TAVR).BackgroundTTE is utilized before, during, and after TAVR. Post‐procedural, pre‐discharge TTE assists in assessment of prosthesis function and detection of clinically significant paravalvular leak (PVL) after TAVR.MethodsPatients who underwent TAVR at Mayo Clinic from July 2018 to July 2019 were included in a prospective institutional registry. Patients undergoing TAVR prior to February 2019 received a pre‐discharge TTE, while those undergoing TAVR after February 2019 did not. Both cohorts were evaluated with TTE at 30 days post‐TAVR.ResultsA total of 330 consecutive patients were included. Of these, 160 patients (age 81.1 ± 7.6) had routine pre‐discharge TTE, while 170 patients (age 78.9 ± 7.5) were dismissed without routine pre‐discharge TTE. Mortality at 30 days was similar between the two groups (0% and 1.2%, respectively). One episode of PVL requiring intervention (0.6%) occurred in the pre‐discharge TTE group and none in the group without pre‐discharge TTE at 30‐day follow‐up. There was a similar incidence of total composite primary and secondary adverse events between the cohort receiving a pre‐discharge TTE and those without (28.1% vs. 25.3%, P = 0.56) at 30 days. The most common event was need for permanent pacemaker or ICD implantation in both groups (13.1% vs. 11.8%, P = 0.71).ConclusionsElimination of the pre‐discharge TTE is safe and associated with comparable 30‐day outcomes to routine pre‐discharge TTE. These findings have implication for TAVR practice cost‐efficiency and health care utilization.

BACKGROUND: Idiopathic recurrent pericarditis (IRP) is a debilitating condition that can be recalcitrant to conventional therapy. Some patients develop steroid dependency with the attendant risks of systemic side effects and increased future recurrence. Anakinra is a recombinant human interleukin-1 receptor antagonist that reduces systemic inflammatory responses. The aim of this study was to evaluate the therapeutic role of anakinra in IRP. Methods: We retrospectively studied consenting patients with treatment refractory IRP who received anakinra, between January 2009 and November 2013. The primary end points were symptom resolution and steroid discontinuation. Results: Nine patients were followed for a median of 16.8 (IQR 1.3-24) months. Study subjects were predominantly female (7 [78%]) with a median age of 53 (IQR 38 - 58) years. All 9 patients had failed maximum tolerated doses of NSAIDs, colchicine and prednisone (median dose 20 mg [IQR 15 - 22.5]). Primary symptom was chest pain in 9 (100%), with concurrent dyspnea in 5 (56%) patients. Symptom duration prior to anakinra initiation was 24 (IQR 13 - 71) months. Indication for anakinra was steroid sparing agent in 7 (78%) and symptom control in 2 (22%) patients. The dosage was 100 mg once daily via subcutaneous injection. Baseline LVEF was 62% (IQR 59% - 67%). Echocardiographic findings include pericardial effusion in 3 (33%), pericardial thickening in 6 (67%), and constrictive physiology in 4 (44%). Pericardial enhancement on cardiac MRI was seen in 7 (78%) patients. All 9 patients (100%) had symptom improvement with complete resolution in 8 (89%) and partial resolution in 1 (11%) patient. At last follow up, all 9 (100%) patients had discontinued NSAIDs and colchicine, 5 (56%) patients discontinued and 4 (44%) had reduced prednisone dosage. All patients remained on anakinra at the end of follow up. The only reported side effect was transient injection site reaction in 4 (44%) patients. In 2 (22%) patients, attempted anakinra weaning was unsuccessful due to symptomatic flare after 6 weeks. Conclusion: Anakinra is an effective alternative agent for the management of steroid dependent IRP. It provides remarkable symptomatic amelioration, limits steroid dependency, and is associated with minimal side effects.

Background: Apical hypertrophic cardiomyopathy (apical HCM) is a less common subtype of HCM characterized by a focal thickening in the left ventricular apex. “Classic” ECG features have been described, however, apical HCM can persist for many years without detection. We investigated the relationship between ECG findings and echocardiographic morphometry in a large referral series of patients with apical HCM. Methods: We enumerated all patients diagnosed with apical HCM prior to Sept. 30, 2006 using the Mayo Clinic HCM database. We compared echocardiographic measures separately for patients with positive status for two ECG indices of left ventricular hypertrophy (LVH); the Sokolow-Lyon index and the Romhilt-Estes (RE) point-score. We also compared echocardiographic measurements in patients with and without negative T-waves in the precordial leads. Results: Apical HCM was detected in 177 patients (111 men and 68 women). Only 51% had positive Sokolow criteria and 51% had positive RE criteria. The agreement between Sokolow and RE status was high (agreement = 75.0%; kappa = 0.50; 95% CI = 0.38 – 0.62). In particular, Sokolow positive patients had increased LV ejection fraction (P = 0.02), and decreased LV end-systolic diameter (P = 0.03) compared with Sokolow negative patients. The prevalence of right atrial enlargement (47 vs. 28%; P = 0.02) and intracavity obstruction (22 vs. 8%; P = 0.01) were more common in Sokolow positive patients. Positive RE criteria was associated with a greater thickness of the basal septal and basal posterior walls (P = 0.001 and 0.02, respectively), and with a higher frequency of intracavity obstruction (21 vs. 9%; P = 0.04). Most patients (89%) exhibited at least one negative T-wave in the precordial leads; however, only 10% of patients had a negative T-wave of greater than 1.0 mV. We found that patients with an inverted T-wave larger than 0.4 mV (median) had a significantly increased LV ejection fraction (P = 0.03) compared with patients who had smaller or no negative T-waves. Conclusions: Among patients with apical HCM, nearly half do not have ECG evidence of LVH based on classic criteria and most do not have marked T-wave inversions. However, the majority did have at least a mild expression of negative T-waves.

Introduction: Papillary fibroelastomas (PFEs) are the 3rd most common primary cardiac tumors with a prevalence of around 0.01% in patients with echocardiograms. The pathogenesis is controversial but endocardial trauma has been implicated. We hypothesized that increased turbulence in hypertrophic cardiomyopathy (HCM) would be a risk factor for PFE development and characterized PFEs in HCM. Around 5% of HCM patients had neurologic events. We postulated that patients with both PFE and HCM had a higher risk of neurologic events. Methods: We reviewed pathology records from 1990 –2006 and identified 129 subjects with PFEs and 2479 patients with HCM. We then identified the subjects who had both HCM and PFE (n = 19). All subjects signed release for use of their medical records for research. Results: 19 subjects (68.4% female) had both HCM and PFE. Mean age at diagnosis of HCM was 49 (range 21– 81) years. All had outflow tract obstruction with resting gradient >/=30 mm Hg except 2 who had resting gradients <30 mm Hg but elevated gradients on provocation. PFE diagnosis was after a mean of 9.7 years (SD 8.3) following HCM diagnosis. 5 subjects were diagnosed pre-operatively by transthoracic echocardiography, 2 subjects by transesophageal echocardiography and 12 subjects at surgery only. Most subjects had only 1 PFE (57.9%) but 1 subject had as many as 40 tumors. Most PFEs were on the aortic valve (47.4%) and the left ventricular side of the septum and left ventricular outflow tract (42.1%). Only 2 patients had right heart lesions that were presumably unrelated to HCM. 3 patients had previous septal myectomies and all 3 had PFEs near the previous resection. 5 of 19 subjects (26.3%) with PFE and HCM had neurologic events (either strokes or transient ischemic attacks) compared to 199 of 2261 subjects (8.8%) with HCM alone (OR = 4.1; 95% CI 1.4 –11.4, p < 0.05). Conclusion: Nearly 90% of PFEs in HCM are located in the septum, left ventricular outflow tract and aortic valve. This may be supportive of endocardial trauma as a risk factor for PFE development in HCM. About a quarter of patients with PFEs and HCM had neurologic events. This may indicate that these lesions are not innocuous and should perhaps be actively searched for in patients with HCM and unexplained neurologic events.