Faculty Bibliography

Background Arrhythmogenic cardiomyopathy (ACM) can mimic inflammatory processes. We present a complex patient with scleroderma (Sc)-dermatomyositis overlap syndrome (Sc-DM) and cardiac disease. Case A 57-year-old woman with family history of Sc presented with progressive weakness, dyspnea, edema, and Raynaud's (1A). Troponin was 1.6 ng/mL and CRP was 13.2 mg/L. EKGs revealed sinus rhythm with RBBB and AV sequential pacing with multifocal PVCs (1B-C). CT chest showed bibasilar fibrosis (1D). Echocardiography revealed biventricular dysfunction. Cardiac catheterization showed non-obstructive coronaries and a cardiac index of 1.8 L/min/m2. Cardiac MRI had diffuse biventricular subendocardial late gadolinium enhancement (1E). Electromyography revealed proximal myopathy. Rheumatologic workup was consistent with seronegative Sc-DM. Decision-making She was treated with steroids, mycophenolate, IV immunoglobulins, diuretics, and inotropes. Her course was complicated by recurrent VT cardiac arrests, prompting escalation to VA-ECMO. She underwent cardiac transplant on day 9 of ECMO. Pathology revealed biventricular fibrofatty replacement consistent with ACM (1F-G), patchy fibrosis of the pericardium, and mitral valve with thickened and fused chordae suggestive of inflammatory changes from Sc (1H-I). Conclusion This case highlights an atypical presentation of ACM in a patient with Sc-DM and the multidisciplinary approach necessary for proper diagnosis and management. [Figure presented]
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OBJECTIVE:Several researchers have argued that racism-related stressors play an important role in adverse cardiovascular outcomes among African-American women. However, studies have primarily focused on experiences of racism; thus, the role of expectations of racism is insufficiently understood. The current proof-of-concept study was designed to examine associations among expectations of racism, self-reported experiences of racism, and carotid intima-media thickness (IMT), a marker of cardiovascular risk, in African-American women. METHODS:Participants were 52 healthy African-American women, aged 30-50 (Mean=40.8, sd=4.3). Expectations of racism were assessed with a modified version of the Race-Based Rejection Sensitivity Questionnaire, experiences of racism were assessed with the Schedule of Racist Events, and carotid IMT was measured using B-mode ultrasound. RESULTS:In linear regression analyses adjusted for age, expectations of racism were associated with higher levels of carotid IMT (b=.04, s.e.=.014, p=.013), after adjusting for experiences of racism. Findings remained significant after additional adjustments for cardiovascular risk factors (b=.03, s.e.=.014, p=.032). Associations were not confounded by additional stressors, hostility, or negative affect (depressive symptoms). CONCLUSIONS:Independent of actual reports of racism, "expectations" of racism may be associated with increased cardiovascular risk in African-American women. Additionally, although experiences of discrimination were associated with depressive symptoms, expectations of racism were not, suggesting that other negative emotions likely play a role. Future studies are needed to replicate these results in larger samples, and to explore the psychological and physiological pathways through which expectations of racism might affect CVD risk across a range of populations.

BACKGROUND:Evidence-based therapy for heart failure remains underutilized at hospital discharge, particularly for patients with heart failure who are hospitalized for another cause. We developed clinical decision support (CDS) to recommend an angiotensin converting enzyme (ACE) inhibitor during hospitalization to promote its continuation at discharge. The CDS was designed to be implemented in both interruptive and non-interruptive versions. OBJECTIVES/OBJECTIVE:To compare the effectiveness and implementation of interruptive and non-interruptive versions of a CDS to improve care for heart failure. METHODS:Hospitalizations of patients with reduced ejection fraction were pseudo-randomized to deliver interruptive or non-interruptive CDS alerts to providers based on even or odd medical record number. We compared discharge utilization of an ACE inhibitor or angiotensin receptor blocker (ARB) for these two implementation approaches. We also assessed adoption and implementation fidelity of the CDS. RESULTS:percentile) of 14 (5,32) alerts were triggered per hospitalization. CONCLUSIONS:A CDS implemented as an interruptive alert was associated with improved quality of care for heart failure. Whether the potential benefits of CDS in improving cardiovascular care were worth the high burden of interruptive alerts deserves further consideration. CLINICALTRIALS. GOV IDENTIFIER/UNASSIGNED:NCT02858674.

Amyloidosis describes a family of related disease states associated with the extracellular tissue deposition of fibrils composed of low-molecular-weight subunits of a variety of proteins circulating as constituents of plasma. Depending on the disease subtype, fibrillar deposits in a several organs including the heart, kidney, liver, and peripheral nerves cause organ dysfunction and associated morbidity and mortality. The most common amyloid fibril deposits associated with cardiac manifestations are of monoclonal light-chain or transthyretin (ATTR) types. This review will focus on the ATTR types of cardiac amyloidosis. ATTR amyloidosis may be associated with abnormal metabolism of wild-type transthyretin (previously called senile systemic amyloidosis) or with hereditary variants in the transthyretin gene. Cardiac amyloidosis is often under-recognized in its early stages, and when a diagnosis of cardiac amyloidosis is made, patients are often at the advanced stages of the disease. Treatments now available appear to exert their benefit predominantly in individuals with the early stages of disease. Increased awareness and early diagnosis of cardiac amyloidosis and continued discovery of effective therapies will increase opportunities to improve clinical outcomes in this patient population.

BACKGROUND:Mineralocorticoid receptor antagonists (MRA) are an underutilized therapy for heart failure with a reduced ejection fraction (HFrEF), but the current impact of hospitalization on MRA use is not well characterized. The objective of this study was to describe contemporary MRA prescription for heart failure patients before and after the full scope of hospitalizations and the association between MRA discharge prescription and post-hospitalization outcomes. METHODS:We conducted a retrospective cohort study at an academic hospital system in 2013-2016. Among 1500 included hospitalizations of 1009 unique patients with HFrEF and without MRA contraindication, the mean age was 71.9 ± 13.6 years and 443 (29.5%) were female. We compared MRA prescription before and after hospitalizations with McNemar's test and between patients with principal and secondary diagnoses of HFrEF with the chi-square test, and association of MRA discharge prescription with 30-day and 180-day mortality and readmissions using generalized estimating equations. RESULTS:MRA prescriptions increased from 303 (20.2%) to 375 (25.0%) at discharge (+4.8%, p < 0.0001). More patients with principal diagnosis of HFrEF compared to those hospitalized for other reasons received MRA (34.9% versus 21.3%, p < 0.0001) and had them initiated (21.8% versus 9.7%, p < 0.0001). MRA prescription at discharge was not associated with mortality or readmission at 30 and 180 days, and there was no interaction with principal/secondary diagnosis. CONCLUSIONS:Among hospitalized HFrEF patients, 75% did not receive MRA before or after hospitalization, and nearly 90% of eligible patients did not have MRA initiated. As we found no signal for short-term harm after discharge, hospitalization may represent an opportunity to initiate guideline-directed heart failure therapy.

Introduction: Cognitive impairment (CI) is prevalent in heart failure and is associated with higher mortality rates. The mechanism behind CI in heart failure with preserved ejection fraction (HFpEF) has not been established. The purpose of this study was to evaluate associations between abnormal cardiac hemodynamics and CI in individuals with HFpEF. Hypothesis: Diastolic dysfunction, systolic dysfunction, and impaired ventricular vascular coupling will be associated with CI in HFpEF.
Method(s): This was a secondary analysis of data from the Atherosclerosis Risk in Communities Study. Individuals who completed in-person neurocognitive assessments at visit 5 were included. Individuals with stroke or dementia were excluded. Participants were classified as having HFpEF, heart failure with reduced ejection fraction (HFrEF), or no heart failure. Independent variables included echocardiographic measures of cardiac function and factors hypothesized to influence CI in HFpEF based on an extensive literature review. Dependent variables included scores on neurocognitive tests. Descriptive statistics were used to describe sample characteristics and identify significant differences among those with HFpEF, HFrEF, and no heart failure. Bivariate analysis identified predictors for multivariate models and evaluated collinearity. Multiple imputation by chained equations was conducted to account for missing values. Multiple linear regression identified independent predictors of CI.
Result(s): Scores on tests of attention, language, executive function, and global cognitive function were worse among individuals with HFpEF than those with no heart failure. The effect of HFpEF on CI was small to moderate. Worse diastolic function was weakly associated with worse performance in memory, attention, and language. Higher cardiac index was associated with worse performance on one test of attention. No association between ventricular-vascular coupling and CI was identified. Older age, history of hypertension, and high numbers of depressive symptoms also were associated with CI.
Conclusion(s): Cognitive impairment is prevalent in HFpEF and affects several cognitive domains. The current study supports the need to screen individuals with HFpEF for CI. As CI is associated with worse outcomes, early identification and appropriate intervention has the potential to mitigate the effect of CI on outcomes, including mortality rates. The current study demonstrated an association between abnormal cardiac hemodynamics and CI. Although abnormal hemodynamics may contribute to CI in HFpEF, other factors may be involved. Future research should explore other mechanisms that contribute to CI in HFpEF.
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Purpose: In October 2018, a new US adult heart allocation scheme was enacted in which the etiology of cardiomyopathy can play a significant role in the prioritization of patients listed for transplantation. Given this, we embarked on a review of the diagnoses of patients who underwent therapy for advanced heart failure at our center.
Method(s): We retrospectively reviewed the etiology of cardiomyopathy of patients receiving either durable ventricular assist device (VAD) or orthotopic heart transplantation (OHT) at NYU Langone Medical Center in New York, NY between January 2011 and October 2018. We evaluated for discrepancies between the primary HF diagnosis at time of operation with the ultimate diagnosis, combining both clinical follow-up data and cardiac pathology.
Result(s): During the study period, a total of 110 patients were treated with advanced therapies, of which the majority (74.5%) were male. 40.9% were African American, 35.4% Caucasian, 4.5% Asian, and 23.6% Hispanic. 86.3% underwent VAD and 22.0% underwent OHT. The average age of those undergoing OHT and VAD were 58 and 61 respectively. The most common reported etiology of HF was dilated cardiomyopathy (57.3%), followed by ischemic (36.3%), familial DCM (1.8%), amyloidosis (1.8%), restrictive cardiomyopathy (1.8%), and sarcoidosis (0.9%). On final review of the diagnoses in these patients, 14 (12.7%) had a final diagnosis that was inconsistent with the prior reported one. 5 were clerical errors, but 9 were significant deviations from the prior diagnosis. The most common diagnoses that were misidentified prior to VAD or OHT were cardiac sarcoidosis (2), cardiac amyloidosis (2), and hypertrophic cardiomyopathy (2). Among those 9 patients, 7 patients received VAD with 5 eventually requiring OHT (median days to OHT = 248); 2 patients directly received OHT. All of those are alive except one patient who was lost to follow-up (transferred care to another center). Patients in whom the diagnosis was misidentified prior to VAD or OHT had smaller LV dimensions on transthoracic echocardiography on average than other LVAD or OHT patients with non-ischemic cardiomyopathy.
Conclusion(s): In this single-center review, we found that the majority of HF patients undergoing VAD and OHT had a correct diagnosis for their heart failure prior to treatment, although notably 8.1% had a missed diagnosis at time of intervention (VAD or OHT). Appropriately identifying the subtype of cardiomyopathy remains challenging especially in advanced HF patients but can significantly impact waiting list time in the current organ allocation scheme. A normal or minimally increased LV dimension on echocardiogram in a patient with advanced non-ischemic cardiomyopathy may warrant further workup for another diagnosis.
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BACKGROUND:Infection has been associated with stroke in patients with left ventricular assist devices (LVAD); however, little data exist on the timing, type and mortality impact of infection-related stroke. METHODS:Prospectively collected data of HeartMate II (N = 332) and HeartWare (N = 70) LVAD patients from a single center were reviewed. Only strokes (ischemic or hemorrhagic) that occurred within 6 weeks following a LVAD infection were considered in analyses. The association between LVAD infections (wound, pump pocket, driveline and/or bloodstream infection [BSI]), specific pathogens and ischemic and hemorrhagic strokes was evaluated using multivariable logistic regression analysis. The impact of infection-related stroke on cumulative survival was assessed using Kaplan-Meier analysis. RESULTS:Of 402 patients, LVAD infection occurred in 158 (39%) including BSI in 107 (27%), driveline infection in 67 (17%), wound infection in 31 (8%) and pump pocket infection in 24 (6%). LVAD infection-related stroke occurred in 20/158 (13%) patients in a median of 4 days (0-36 days) from documented infection. In multivariable analysis, ischemic stroke was associated with wound infection (aOR 9.0, 95% CI 2.4-34.0, P = 0.001) and BSI (aOR 7.7, 95% CI 0.9-66.0, P = 0.064), and hemorrhagic stroke was associated with BSI in 100% of cases (P = 0.01). There was no association with driveline or pump pocket infection. The cumulative survival rate among patients with infection-related stroke was significantly lower compared to those with LVAD infection but no stroke (log-rank P < 0.001). There was a trend toward shorter stroke-free survival among patients with LVAD infection. CONCLUSIONS:LVAD infections, particularly BSI, are significantly associated with stroke, and infection-related stroke conferred significantly lower cumulative survival.