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Background and ObjectivesHereditary transthyretin amyloidosis (hATTR) is a rare autosomal dominant systemic disease with variable penetrance and heterogeneous clinical presentation. Several effective treatments can reduce mortality and disability, though diagnosis remains challenging, especially in the United States where disease is nonendemic. Our aim is to describe the neurologic and cardiac characteristics of common US ATTR variants V122I, L58H, and late-onset V30M at presentation.MethodsWe conducted a retrospective case series of patients with a new diagnosis of ATTRv between January 2008 and January 2020 to characterize features of prominent US variants. The neurologic (examination, EMG, and skin biopsy), cardiac (echo), and laboratory assessments (pro b-type natriuretic peptide [proBNP] and reversible neuropathy screens) are described.ResultsA total of 56 patients with treatment-naïve ATTRv with symptoms/signs of peripheral neuropathy (PN) or cardiomyopathy and confirmatory genetic testing presenting with Val122Ile (N = 31), late-onset Val30Met (N = 12), and Leu58His ATTRv (N = 13) were included. The age at onset and sex distributions were similar (V122I: 71.5 ± 8.0, V30M: 64.8 ± 2.6, and L58H: 62.4 ± 9.8 years; 26, 25, 31% female). Only 10% of patients with V122I and 17% of patients with V30M were aware of an ATTRv family history, while 69% of patients with L58H were aware. PN was present in all 3 variants at diagnosis (90%, 100%, and 100%), though neurologic impairment scores differed: V122I: 22 ± 16, V30M: 61 ± 31, and L58H: 57 ± 25. Most points (deficits) were attributed to loss of strength. Carpal tunnel syndrome (CTS) and a positive Romberg sign were common across all groups (V122I: 97%, 39%; V30M: 58%, 58%; and L58H: 77%, 77%). ProBNP levels and interventricular septum thickness were highest among patients with V122I (5,939 ± 962 pg/mL, 1.70 ± 0.29 cm), followed by V30M (796 ± 970 pg/mL, 1.42 ± 0.38 cm) and L58H (404 ± 677 pg/mL, 1.23 ± 0.36 cm). Atrial fibrillation was present among 39% of cases with V122I and only 8% of cases with V30M and L58H. Gastrointestinal symptoms were rare (6%) among patients with V122I and common in patients with V30M (42%) and L58H (54%).DiscussionImportant clinical differences exist between ATTRv genotypes. While V122I is perceived to be a cardiac disease, PN is common and clinically relevant. Most patients with V30M and V122I were diagnosed de novo and therefore require clinical suspicion for diagnosis. A history of CTS and a positive Romberg sign are helpful diagnostic clues.

Advanced heart failure (HF) therapies improve survival in patients with stage D HF. We sought to evaluate differences by race/ethnicity and sex in advanced HF therapy referrals and decision-making across a multicenter survey. We performed a retrospective analysis of patients referred for evaluation for advanced HF therapies at 9 centers (n = 515) across the United States. The median age was 58 years, and 73% were male. White patients comprised 55.7% of referrals, whereas non-White patients comprised 44.3%. Non-ischemic etiology was more common in non-White patients (66.6% vs 47.4% p = 0.0005), and ischemic etiology was more common in men (37.8% vs 20.4% p = 0.0005). The primary reason for referral differed by race/ethnicity but not sex, with ventricular arrhythmias (7.6% vs 3%, p = 0.024) and pulmonary hypertension (3.4% vs 0.4% p = 0.018) being more common in White patients, whereas worsening HF was less common (25.4% vs 35.9%; p = 0.009). White patients were offered left ventricular assist devices (LVADs) (60.3% vs 54.7 p = 0.039) and heart transplants (51.8% vs 33.1% p = 0.0007) more often than non-White patients. The preference not to pursue LVAD therapy was more common in non-White patients (17.6% vs 9.6%; p = 0.049). Men were more often declined for a heart transplant because of psychosocial contraindications (34% vs 15%, p = 0.005). In conclusion, in this multicenter analysis of referrals for advanced HF therapies, we observed significant differences by race, ethnicity, and sex in both referral characteristics and evaluation outcomes. Further investigation is warranted to better understand why rates of LVAD and transplantation may be lower in non-White patients who are referred for advanced therapies.

Percutaneous mechanical circulatory support (pMCS) devices are increasingly used in patients with cardiogenic shock as a bridge to recovery or bridge to decision to advanced heart failure therapies. Gastrointestinal bleeding (GIB) is a common complication that can be catastrophic. Because of the paucity of data describing the association of GIB with pMCS, we analyzed this population using the United States National Inpatient Sample database. We performed a retrospective study in patients with pMCS devices who had GIB during the index hospitalization using the National Inpatient Sample. Multivariate logistic regression analysis was performed to determine independent predictors of GIB in these patients. A total of 466,627 patients were included. We observed an overall increase in the incidence of adjusted GIB from 2.9% to 3.5% (p = 0.0025) from 2005 to 2014. In comparison to patients without GIB, those with GIB had significantly higher in-hospital mortality, length of stay, and hospitalization cost. In addition to the usual co-morbid conditions, the presence of small bowel and colonic ischemia, colon cancer, diverticulosis, chronic liver disease, and peptic ulcer disease were noted to be significant predictors of GIB for all (p0.001). In conclusion, patients with pMCS and GIB have higher in-hospital mortality, longer length of stay, and higher cost of hospitalization. Awareness of patient risk factors for bleeding and gastrointestinal disorders are important before the use of mechanical circulatory support devices because they are associated with a substantially higher risk for bleeding.

National heart failure (HF) guidelines recommend that in patients with HF with preserved ejection fraction (EF;HFpEF) and hypertension, systolic blood pressure (SBP) should be maintained below 130 mmHg. The objective of the study is to examine the association between initiation of anti-hypertensive drugs and outcomes in patients with HFpEF with persistent hypertension.Of the 8873 hospitalized patients with HFpEF (EF ≥50%) with a history of hypertension without renal failure in Medicare-linked OPTIMIZE-HF, 3315 had a discharge SBP ≥130 mmHg, of whom 1971 were not receiving anti-hypertensive drugs, thiazides and calcium channel blockers, before hospitalization. Of these, 366 received discharge prescriptions for those drugs. We assembled a propensity score-matched cohort of 365 pairs of patients initiated and not initiated on anti-hypertensive drugs, balanced on 37 baseline characteristics. Hazard ratios (HR) and 95% confidence intervals (CI) for outcomes associated with anti-hypertensive drug initiation were estimated in the matched cohort.Matched patients (n = 730) had a mean age of 78 years; 67% were women and 17% African Americans. During 6 (median 2.5) years of follow-up, 66% of the patients died and 45% had HF readmission. HRs (95% CIs) for all-cause mortality at 30 days, 12 months and 6 years associated with anti-hypertensive drug initiation were 0.64 (0.30-1.36), 0.70 (0.51-0.97), and 0.95 (0.79-1.13), respectively. Respective HRs (95% CIs) for HF readmission were 1.65 (0.97-2.80), 1.18 (0.90-1.56) and 1.09 (0.88-1.35).Among hospitalized older patients with HFpEF with uncontrolled hypertension, the initiation of therapy with anti-hypertensive drugs was not associated with all-cause mortality or hospital readmission.

Background In patients with heart failure with reduced ejection fraction (HFrEF) and hypertension, systolic blood pressure is recommended to be maintained below 130 mmHg, although this has not been shown to be associated with improved outcomes. We examined the association of anti-hypertensive drug initiation and outcomes in patients with HFrEF. Methods In the Medicare-linked OPTIMIZE-HF, 7966 patients with HFrEF (ejection fraction ≤40%) without renal failure were not receiving anti-hypertensive drugs before hospitalization, of whom 692 received discharge prescriptions for those drugs (thiazides and calcium channel blockers). We assembled a propensity score-matched cohort of 687 pairs of patients initiated and not initiated on anti-hypertensive drugs, balanced on 38 baseline characteristics. Hazard ratios (HR) and 95% confidence intervals (CI) for outcomes associated with anti-hypertensive drug initiation were estimated in matched cohort. Results Matched patients (n=1374) had a mean age of 74 years, 41% were women, 17% were African American, 66% were discharged on renin-angiotensin system inhibitors and beta blockers, and 10% on aldosterone antagonists. During 6 (median 2.5) years of follow-up, 70% of the patients died and 53% had heart failure readmission. Anti-hypertensive drug initiation was not significantly associated with all-cause mortality (HR, 0.95; 95% CI, 0.83–1.07) or heart failure readmission (HR, 0.93; 95% CI, 0.80–1.07). Similar associations were observed during 30 days and 12 months of follow-up. Conclusions Among hospitalized older patients with HFrEF receiving contemporary treatments for heart failure, initiation of an anti-hypertensive drug was not associated with a lower risk of all-cause mortality or hospital readmission.

To examine the association between income and cardiovascular disease (CVD) in community-dwelling older adults.Of the 5795 Medicare-eligible community-dwelling older Americans aged 65-100 years in the Cardiovascular Health Study (CHS), 4518 (78%) were free of baseline CVD, defined as heart failure, acute myocardial infarction, stroke, or peripheral arterial disease. Of them, 1846 (41%) had lower income, defined as a total annual household income$16,000. Using propensity scores for lower income, estimated for each of the 4518 participants, we assembled a matched cohort of 1078 pairs balanced on 42 baseline characteristics. Outcomes included centrally adjudicated incident CVD and mortality.Matched participants (n = 2156) had a mean age of 73 years, 63% were women, and 13% African American. During an overall follow-up of 23 years, incident CVD, all-cause mortality and the combined endpoint of incident CVD or mortality occurred in 1094 (51%), 1726 (80%) and 1867 (87%) individuals, respectively. Compared with the higher income group, hazard ratio (HR) for time to the first occurrence of incident CVD in the lower income group was 1.16 with a 95% confidence interval (CI) of 1.03 to 1.31. A lower income was also associated with a significantly higher risk of all-cause mortality (HR, 1.19; 95% CI, 1.08-1.30), and consequently a higher risk of the combined endpoint of incident CVD or death (HR, 1.20; 95% CI, 1.09-1.31).Among community-dwelling older Americans free of baseline CVD, an annual household income$16,000 is independently associated with significantly higher risks of new-onset CVD and death.

Best practices for left ventricular assist devices (LVADs) deactivation at end-of-life (EOL) have yet to be elucidated. We conducted a single-institution retrospective review of patients who died following LVAD deactivation between January 2017 and March 2020. Data were obtained from institutional databases and electronic health record and were analyzed using descriptive statistics. Fifty-eight patients (70% male, 70% African American, median age 62 years) were categorized by implant strategy: bridge therapy (BT, N = 22, 38%) or destination therapy (DT, N = 36, 62%). Clinical events leading to deactivation were categorized either acute ( e.g. , stroke [ N = 31, 53%]), gradual decline ( N = 12, 21%), or complications during index hospitalization ( N = 15, 26%). Implant strategy was not associated with clinical trajectory leading to EOL ( p = 0.67), hospital unit of death ( p = 0.13), or use of mechanical ventilation ( p = 0.69) or renal replacement therapy ( p = 0.81) during terminal hospitalization. Overall time from admission to code status change was mean 27.0 days (SD 30.3 days). Compared with BT patients, DT experienced earlier do-not-resuscitate (DNR) orders ( p ≤ 0.01) and shorter survival post-deactivation ( p ≤ 0.01). Deactivations after gradual decline tended to occur outside ICUs, compared with acute events or index implant-related complications ( p = 0.04). Implant strategy was not associated with differences in EOL experience except regarding timing of DNR order and survival post-deactivation.

The prevalence of sarcoidosis-related cardiomyopathy is increasing. Sarcoidosis impacts cardiac function through granulomatous infiltration of the heart, resulting in conduction disease, arrhythmia and/or heart failure. Diagnosis of cardiac sarcoidosis can be challenging and requires clinician awareness as well as differentiation from overlapping diagnostic phenotypes such as other forms of myocarditis and arrhythmogenic cardiomyopathy. Clinical manifestations, extracardiac involvement, histopathology, and advanced cardiac imaging can all lend support to a diagnosis of cardiac sarcoidosis. Mainstay therapy for cardiac sarcoidosis is immunosuppression, however no prospective clinical trials exist to guide management. Patients may progress to developing advanced heart failure or ventricular arrhythmia, for which ventricular assist device therapies or heart transplantation may be considered. The existing knowledge gaps in cardiac sarcoidosis call for an interdisciplinary approach to both patient care and future investigation to improve mechanistic understanding and therapeutic strategies.

Background Therapies for advanced heart failure (AHF) improve the likelihood of survival in a growing population of patients with stage D heart failure (HF). Successful implementation of these therapies is dependent upon timely and appropriate referrals to AHF centers. Methods We performed a retrospective analysis of patients referred to 9 AHF centers for evaluation for AHF therapies. Patients’ demographics, referring providers’ characteristics, referral circumstances, and evaluation outcomes were collected. Results The majority of referrals (n = 515) were male (73.4%), and a majority of those were in the advanced state of the disease: very low left ventricular ejection fraction ( Conclusions In this multicenter review of AHF referrals, HF cardiologists referred the most patients despite being a relatively small proportion of the overall clinician population. Late referral was prevalent in this high-risk patient population and correlates with worsened outcomes, suggesting a significant need for broad clinician education regarding the benefits, triggers and appropriate timing of referral to AHF centers for optimal patient outcomes.

The coronavirus disease 2019 (COVID-19) pandemic continues to pose a significant threat to patients receiving advanced heart failure therapies. The current study was undertaken to better understand the relationship between obesity and outcomes of SARS-CoV-2 infection in patients with a left ventricular assist device (LVAD) or heart transplant. We performed a retrospective review of patients with a heart transplant or LVAD who presented to one of the participating 11 institutions between April 1 and November 30, 2020. Patients were grouped by body mass index (BMI) into obese (BMI ≥ 30 k/m2) and nonobese cohorts (BMI30 kg/m2). Multivariable logistic regression models were used to estimate effects of obesity on outcomes of interest. Across all centers, 162 heart transplant and 81 LVAD patients were identified; 54 (33%) and 38 (47%) were obese, respectively. Obese patients tended to have more symptoms at presentation. No differences in rates of hospitalization or ICU admission were noted. Obese patients with LVADs were more likely to require mechanical ventilation (39% vs. 8%, p0.05). No differences in renal failure or secondary infection were noted. Mortality was similar among heart transplant patients (11% [obese] vs. 16% [nonobese], p = 0.628) and LVAD patients (12% vs. 15%, p = 1.0). BMI was not associated with increased adjusted odds of mortality, ICU admission, or mechanical ventilation (all p0.10). In summary, acute presentations of SARS-CoV-2 among heart transplant and LVAD recipients carry a significantly higher mortality than the general population, although BMI does not appear to impact this. Further studies on the longer-term effects of COVID-19 on this population are warranted.

BACKGROUND Due to anatomic and physiologic concerns, prior generations of the left ventricular assist devices (LVAD) have frequently been denied to patients with small body size. However, outcomes in patients with small body surface area (BSA) following HeartMate 3 (HM3) LVAD implantation remain relatively unknown. METHODS A cohort of 220 patients implanted at a single center was divided into two groups: BSA ≤1.8 m2 (small BSA, n = 37) and BSA >1.8 m2 (large BSA, n = 183). We investigated baseline characteristics and clinical outcomes including survival and incidence of adverse events. RESULTS Small BSA patients were older (60 vs. 57 years), more likely female (60% vs. 20%), had a lower body mass index (24 vs. 32 kg/m2 ), lower incidence of diabetes (32% vs. 51%), history of stroke (5% vs. 19%), and left ventricular thrombus (0% vs. 11%). They had smaller left ventricular end diastolic diameter (64.8 vs. 69.3 mm). Pump speed and pump flows at discharge were lower in the small BSA group. Survival at 1 year and 2 years was 86% versus 87% and 86% versus 79% for small versus large BSA groups (p = 0.408), respectively. The rates of adverse events were similar between groups and there were no cases of confirmed pump thrombosis. The incidence of readmissions for low flow alarms was higher in the small BSA group (0.55 vs. 0.24 EPPY). CONCLUSIONS These findings demonstrate comparable outcomes in patients with small body size and suggest that this parameter should not be an exclusion criterion on patients who are otherwise candidates for HM3 LVAD implantation.

Background Many patients with American College of Cardiology/American Heart Association Stage D (advanced) heart failure are discharged home on chronic intravenous inotropic support (CIIS) as bridge to surgical therapy or as palliative therapy. This study analyzed the clinical trajectory of patients with advanced heart failure who were on home CIIS. Methods We conducted a single-institution, retrospective cohort study of patients on CIIS between 2010 and 2016 (n = 373), stratified by indication for initiation of inotropic support. Study outcomes were time from initiation of CIIS to cessation of therapy, time to death for patients who did not receive surgical therapy and rates of involvement with palliative care. Results Overall, patients received CIIS therapy for an average of 5.9 months (standard deviation [SD] 7.3). Patients on CIIS as palliative therapy died in an average of 6.2 months (SD 6.6) from the time of initiation of CIIS, and those on CIIS as bridge therapy who did not ultimately receive surgical therapy died after an average of 8.6 months (SD 9.3). Patients who received CIIS as bridge therapy were significantly less likely to receive palliative-care consultation than those on inotropes as palliative therapy, whether or not they underwent surgery. Conclusions In this large cohort of patients with advanced HF, patients who on CIIS as palliative therapy survived for 6.2 months, on average, with wide variation among patients. Patients who were on CIIS as bridge therapy but did not ultimately receive surgical therapy received less palliative care despite the high mortality rate in this subgroup.

Background There are limited data to guide oncology and cardiology decision-making in patients with a left ventricular assist device (LVAD) and concurrent active malignancy. Objectives The goal of this study was to describe cancer treatment approaches, complications, and survival among patients with active cancer on LVAD support in 2 tertiary heart failure and oncology programs. Methods In this retrospective cohort study, LVAD databases were reviewed to identify patients with a cancer diagnosis at the time of or after LVAD implantation. We created a 3:1 matched cohort based on age, sex, etiology of cardiomyopathy, LVAD implant strategy, and INTERMACS profile stratified by site. Kaplan-Meier analysis and Cox proportional hazards models were used to compare survival between patients with cancer and non-cancer comparators. Results Among 1,123 patients who underwent LVAD implantation between 2005 and 2019, 22 patients with LVADs with active cancer and 66 matched non-cancer comparators were identified. Median age was 62 years (range 41 to 73 years); 50% of patients with cancer were African-American, and 27% were women. Prostate cancer, followed by renal cell cancer and hematologic malignancies were the most common diagnoses. There was no significant difference in unadjusted Kaplan-Meier median survival estimates from the time of LVAD placement between patients with cancer (3.53 years; 95% confidence interval [CI]: 1.41 to 5.33) and non-cancer comparators (3.03 years; 95% CI: 1.83 to 5.26; log-rank P = 0.99). In Cox proportional hazard models, cancer diagnosis as a time-varying variable was associated with a statistically significant increase in death (hazard ratio: 2.05; 95% CI: 1.03 to 4.12; P = 0.04). Patients with cancer had less gastrointestinal bleeding compared with matched non-cancer comparators (P = 0.016). Other complications were not significantly different. Conclusions Our study provides initial feasibility and safety data and set a framework for multidisciplinary team management of patients with cancer and LVADs., Central Illustration

Background/purpose The aim of this study is to characterize the pattern and the severity of coronary artery lesions in cardiac amyloidosis . Methods We retrospectively compared patients with heart failure who tested positive (i.e., biopsy or gene tests – HF/CA+) against those who tested negative (HF/CA−) for cardiac amyloidosis. Groups were compared demographically and angiographically for qualitative and quantitative variables to determine patterns of involvement in the major epicardial coronary vessels . Results The study included 110 heart failure patients, of whom 55 patients (88 lesions) were in the HF/CA+ group, and 55 patients (66 lesions) were HF/CA−. Despite the advanced age of HF/CA+ patients (74.5 ± 11.0 years vs. 54.1 ± 15.0 years; p = 0.05), no severe calcification was found in the HF/CA+ group (0.0% vs. 4.5%; p = 0.018). The HF/CA+ group also had fewer ostial lesions (3.4% vs. 15.1%; p = 0.0095) and a higher, albeit not significant, Thrombolysis in Myocardial Infarction frame count (30.4 ± 12.6 vs. 26.6 ± 11 frames; p = 0.06). In the HF/CA+ group, men had a significant number of tandem lesions compared to women (14.5% vs 0.0%, p = 0.02). Conclusions Overall, heart failure patients with cardiac amyloidosis were older but were found to have less calcified lesions, less ostial involvement, and a reduced anterograde coronary blood flow . This is the first report examining coronary lesions in heart failure patients with cardiac amyloidosis.

Objectives The objective of this study was to describe the risk of cardiac implantable electronic devices (CIEDs) complications in patients with left ventricular assist devices (LVADs). Background Patients with LVADs are predisposed to ventricular arrhythmias and frequently have CIEDs before receiving their LVAD. However, the role of CIED procedures such as generator changes (GC) are unclear in this population, given the potential complications of bleeding and infection. Methods This was a retrospective, multicenter study from January 1, 2012, to September 30, 2018. All patients with LVADs were screened and those who had a CIED GC, implantation, or revision were included in the study and followed until December 31, 2018. Results A total of 179 patients across 6 centers had a CIED procedure after LVAD implantation. The mean age was 59.5 ± 13.4, with the cohort comprising mostly men (78%), destination LVAD therapy (53.8%), and GC (66%). The 30-day primary composite endpoint of hematoma or device infection occurred in 34 (19%) patients. The secondary endpoints of rehospitalization within 30 days and appropriate device therapy during follow-up occurred in 40 (22%) and 42 (24%) patients respectively. Of the 126 patients without previous device therapy, 14.3% received appropriate therapy during follow-up. Conclusions In this large, multicenter cohort, we report the incidence of complications for CIED procedures in the LVAD population; specifically, LVAD patients are at increased risk of pocket hematomas, without downstream risk of infection, and do experience a high rate of appropriate device therapies.

Preoperative cardiopulmonary exercise testing (CPET) is well validated for prognostication before advanced surgical heart failure therapies, but its role in prognostication after LVAD surgery has never been studied. VE/VCO2 slope is an important component of CPET which has direct pathophysiologic links to right ventricular (RV) performance. We hypothesized that VE/VCO2 slope would prognosticate RV dysfunction after LVAD. All CPET studies from a single institution were collected between September 2009 and February 2019. Patients who ultimately underwent LVAD implantation were selectively analyzed. Peak VO2 and VE/VCO2 slope were measured for all patients. We evaluated their association with hemodynamic, echocardiographic and clinical markers of RV dysfunction as well mortality. Patients were stratified into those with a ventilatory class of III or greater. (VE/VCO2 slope of ≥ 36, n = 43) and those with a VE/VCO2 slope

Durable left ventricular assist devices (LVADs) provide circulatory support in patients with end-stage heart failure; however, complications include infection of the driveline exit site. Nontuberculous mycobacterial infections are rare in patients with LVADs, but they should be considered in those who have undergone device exchanges and have bacterial infections with driveline exit-site discharge but no fever or leukocytosis. We reviewed the charts of patients who had an LVAD implanted at our institution from January 2009 through December 2019, to identify those with a device-related nontuberculous mycobacterial infection. Collected data included patient demographics, premorbid conditions, infection type, previous device complications, treatment, and outcomes. We identified infections in 3 patients (mean age, 41 yr): Mycobacterium abscessus in 2 and M. chimaera in 1. All had a HeartMate II device and had undergone device exchanges for pump thrombosis or for driveline fault or infections. All presented with driveline exit-site discharge without fever or leukocytosis. The mean time between initial device implantation and diagnosis of a nontuberculous mycobacterial infection was 55 months. All 3 patients were treated with antibiotics and underwent localized surgical débridement; one underwent an additional device exchange. The M. abscessus infections disseminated, and both patients died; the patient with M. chimaera infection continued to take suppressive antibiotics. Nontuberculous mycobacterial infections are associated with high morbidity and mortality rates, warranting prompt diagnosis and treatment.

Sustained ventricular tachycardia and ventricular fibrillation (VF) are life-threatening arrhythmias which remain highly prevalent in patients with advanced heart failure. These ventricular arrhythmias may impair the support provided by continuous-flow left ventricular assist devices (CF-LVADs) and lead to frequent hospitalizations, antiarrhythmic medication use, external defibrillations, and need for heart transplantation. We report a case in which a patient with a CF-LVAD and an implantable cardioverter defibrillator at end of life presented with asymptomatic low-flow alarms and was found to have VF of unknown duration. Unique in our case was the presence of apparent organized contractility and rhythmic opening of the mitral valve on echocardiogram despite VF on electrocardiogram.

In this document, we outline the challenges faced by patients and clinicians in heart failure, specifically centered around the needed coordination of care among the various subspecialties within cardiovascular medicine. We call for a more organized and collaborative effort among clinicians in primary care, general cardiology, electrophysiology, interventional cardiology, cardiothoracic surgery, cardiac imaging, and heart failure—all caring for mutual patients. Care is contextualized within the framework of two phases: a cardiomyopathy phase and an advanced heart failure phase, each of which lends to different considerations in therapy. Ultimately multidisciplinary coordinated care within cardiovascular medicine may lead to greater patient and clinician satisfaction as well as improved outcomes, but this remains to be investigated.

Background Women differ from men in their left ventricular (LV) structure, function and remodeling with age and diseases. The LV assist device (LVAD) unloads the LV and reversely remodels the heart. We sought to define the effects of sex on longitudinal reverse remodeling after LVAD implantation. Methods and Results Cardiac structure and function were assessed by serial echocardiograms. Mixed effect regression models were constructed to assess the independent contribution of sex to longitudinal changes in cardiac structure and function. A total of 355 consecutive patients with advanced heart failure received continuous flow LVADs between 2006 and 2016. The average age was 56 ± 13 years, 73% were men, and 67% were black. Early (within 3 months) after LVAD implantation, women had a greater reduction in LV dimensions and a greater increase in LV ejection fraction compared with men. These differences were independent of age, body surface area, device type, or ischemic etiology of heart failure. At long-term follow-up, LV dimensions increased slightly over time in women compared with men, but overall, earlier changes were maintained. Conclusion Women had significantly more favorable longitudinal changes in cardiac structure and function in response to LV unloading compared with men. Understanding the cause of sex difference in reverse remodeling after LVAD may help to devise novel therapeutic strategies for women with advanced heart failure.

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare, heritable myocardial disease associated with the development of ventricular arrhythmias, heart failure, and sudden cardiac death in early adulthood. Multimodality imaging is a central component in the diagnosis and evaluation of ARVC. Diagnostic criteria established by an international task force in 2010 include noninvasive parameters from echocardiography and cardiac magnetic resonance imaging. These criteria identify right ventricular structural abnormalities, chamber and outflow tract dilation, and reduced right ventricular function as features of ARVC. Echocardiography is a widely available and cost-effective technique, and it is often selected for initial evaluation. Beyond fulfillment of diagnostic criteria, features such as abnormal tricuspid annular plane excursion, increased right ventricular basal diameter, and abnormal strain patterns have been described. 3-dimensional echocardiography may also expand opportunities for structural and functional assessment of ARVC. Cardiac magnetic resonance has the ability to assess morphological and functional cardiac features of ARVC and is also a core modality in evaluation, however, tissue characterization of the right ventricle is limited by spatial resolution and low specificity for detection of pathological changes. Nonetheless, the ability of cardiac magnetic resonance to identify left ventricular involvement, offer high negative predictive value, and provide a reproducible structural evaluation of the right ventricle enhance the ability and scope of the modality. In this review, the prognostic significance of multimodality imaging is outlined, including the supplemental value of multidetector computed tomography and nuclear imaging. Strengths and weaknesses of imaging techniques, as well as future direction of multimodality assessment, are also described.

Use of continuous intravenous inotropic support (CIIS) strictly as palliative therapy for patients with ACC/AHA Stage D (end-stage) Heart Failure (HF) has increased significantly. The harms of CIIS therapy may detract from its benefits. To describe benefits (improvement in NYHA functional class) and harms (infection, hospitalization, days-spent-in-hospital) of CIIS as palliative therapy. Methods: Retrospective analysis of patients with end-stage HF initiated on CIIS as palliative therapy at an urban, academic center in the United States between 2014-2016. Clinical outcomes were extracted, and data were analyzed using descriptive statistics. Seventy-five patients, 72% male, 69% African American/Black, with a mean age 64.5 years ( SD = 14.5) met study criteria. Mean duration of CIIS was 6.5 months ( SD = 7.7). Most patients (69.3%) experienced improvement in NYHA functional class from class IV to class III. Sixty-seven patients (89.3%) were hospitalized during their time on CIIS, with a mean of 2.7 hospitalizations per patient ( SD = 3.3). One-third of patients (n = 25) required at least one intensive care unit (ICU) admission while on CIIS therapy. Eleven patients (14.7%) experienced catheter-related blood stream infection. Patients spent an average of 20.6% ( SD = 22.8), approximately 40 days, of their time on CIIS admitted to the study institution. Patients on CIIS as palliative therapy report improvement in functional class, survive 6.5 months following initiation, but spend a significant number of days in the hospital. Prospective studies quantifying the symptomatic benefit and the direct and indirect harms of CIIS as palliative therapy are warranted.

Background At the recent 6th World Symposium on Pulmonary Hypertension (PH), the definition of PH was redefined to include lower pulmonary artery pressures in the setting of elevated pulmonary vascular resistance (PVR). However, the relevance of this change to subjects with PH due to left-heart disease as well as the preoperative assessment of heart transplant (HT) recipients is unknown. Methods The United Network for Organ Sharing database was queried to identify adult recipients who underwent primary HT from 1996 to 2015. Recipients were subdivided into those with mean pulmonary artery pressure (mPAP) Results Over the study period, 32,465 patients underwent HT, including 12,257 (38%) with mPAP Conclusions Elevated PVR remains associated with a significant increase in the hazard for 30-day mortality after cardiac transplantation, even in the setting of lower pulmonary artery pressures. These data support the validity of the new definition of pulmonary hypertension.

HeartMate 3 left ventricular assist device implantation is an important treatment modality in patients with advanced heart failure. Polycythemia may predispose this population to adverse events. We present a case of a thrombotic event in a HeartMate 3 patient.

Background: Heart failure disproportionately affects Black patients. Whether differences among race influence outcomes in advanced heart failure with use of a fully magnetically levitated continuous-flow left ventricular assist device remains uncertain. Methods: We included 515 IDE (Investigational Device Exemption) clinical trial patients and 500 Continued Access Protocol patients implanted with the HeartMate 3 left ventricular assist device in the MOMENTUM 3 study (Multicenter Study of MagLev Technology in Patients Undergoing Mechanical Circulatory Support Therapy With HeartMate 3). Outcomes were compared between Black and White left ventricular assist device recipients for the primary end point of survival free of disabling stroke or reoperation to replace or remove a malfunctioning device at 2 years, overall survival, adverse events, 6-minute walk distance, and quality of life scores. Results: Of 1015 HeartMate 3 patients, 675 were self-identified as White and 285 as Black individuals. The Black patient cohort was younger, more obese and with a history of hypertension, and more nonischemic cause of heart failure, relative to the White patient group. Black and White patients did not experience a difference in the primary end point (81.1% versus 77.9%; hazard ratio, 1.08 [95% CI, 0.76–1.54], P =0.6568). Black patients were at higher risk of adverse events (calculated as events per 100 patient-years), including bleeding (75.4 versus 63.5; P P =0.0183), and hypertension (10.1 versus 3.2; P P =0.01). The absolute quality of life measurement (EuroQoL group, 5-dimension, 5-level instrument visual analog scale) at baseline and 6 months was better in the Black patient group, but relative improvement from baseline to 6 months was greater in White patients (median: +20.0 [interquartile range, 5.0–40.0] versus +25.0 [interquartile range, 10.0–45.0]; P =0.0298). Conclusions: Although the survival free of disabling stroke or reoperation to replace/remove a malfunctioning device at 2 years with the HM 3 left ventricular assist device did not differ by race, Black HeartMate 3 patients experienced a higher morbidity burden and smaller gains in functional capacity and quality of life when compared with White patients. These findings require efforts designed to better understand and overcome these gaps through systematic identification and tackling of putative factors. Registration: URL: https://www.clinicaltrials.gov ; Unique identifiers: NCT02224755 and NCT02892955.

It has been suggested that maintaining low mean arterial pressure (MAP) in left ventricular assist device (LVAD) recipients is associated with a reduced risk of stroke/death. However, the lower limit of the optimal MAP range has not been established. We aimed to identify this lower limit in a contemporary cohort of LVAD recipients with frequent longitudinal MAP measurements. We analyzed 86,651 MAP measurements in 309 patients with an LVAD (32% LVADs with full magnetic levitation of the impeller) at a tertiary medical center during a mean follow-up of 1.7 ± 1.1 years. Cox proportional hazards regression modeling was used to study the association of serial MAP measurements with stroke/death within 3 years after index discharge. Multivariate analysis identified MAP ≤75 mm Hg, compared with MAP75 mm Hg, as the low MAP threshold associated with increased risk of death (hazard ratio [HR] 4.74, 95% confidence interval [CI] 2.85 to 7.87, p 0.001), stroke (HR 2.72;, 95% CI 1.39 to 5.33, p = 0.01), and stroke/death (HR 4.45, 95% CI 2.83 to 6.99, p 0.001). The risk associated with MAP ≤75 mm Hg was consistent in subgroups categorized by age, gender, race, device type, renal function, right-sided heart failure, and blood pressure medications. In conclusion, our findings suggest that maintaining MAP ≤75 mm Hg during long-term follow-up in LVAD recipients is associated with increased risk of stroke/death regardless of risk factors or medical management.

Continuous infusion of ambulatory inotropic therapy (AIT) is increasingly used in patients with end-stage heart failure (HF). There is a paucity of data concerning the concomitant use of beta-blockers (BB) in these patients.We retrospectively reviewed all patients discharged from our institution on AIT. The cohort was stratified into 2 groups based on BB use. The 2 groups were compared for differences in hospitalizations due to HF, ventricular arrhythmias and ICD therapies (shock or antitachycardia pacing).Between 2010 and 2017, 349 patients were discharged on AIT (95% on milrinone); 74% were males with a mean age of 61 ± 14 years. BB were used in 195 (56%) patients, whereas 154 (44%) did not receive these medications. Patients in the BB group had longer duration of AIT support compared to those in the non-BB group (141 [1-2114] vs 68 [1-690] days). After adjusting for differences in baseline characteristics and indication for AIT, patients in the BB group had significantly lower rates of hospitalizations due to HF (hazard ratio [HR] 0.61 (0.43-0.86); P = 0.005), ventricular arrhythmias (HR 0.34 [0.15-0.74]; P = 0.007) and ICD therapies (HR 0.24 [0.07-0.79]; P = 0.02).In patients with end-stage HF on AIT, the use of BB with inotropes was associated with fewer hospitalizations due to HF and fewer ventricular arrhythmias.

OBJECTIVES The influence of preoperative left ventricular size in outcomes following centrifugal-flow left ventricular assist device (LVAD) implantation has not been well characterized. METHODS A cohort of 313 patients who received a centrifugal-flow LVAD at a single institution was analysed. Using a maximally selected log-rank statistic, we investigated whether a left ventricular end-diastolic dimension (LVEDD) cut-off point was associated with worse outcomes. The cohort was then divided in 2 groups based on the LVEDD cut-off point. RESULTS An LVEDD cut-off point of 59 mm was found to predict worse survival. Smaller LVEDD patients (≤59 mm, N = 52) were older and more likely to have a history of coronary artery disease compared those with a larger LVEDD (>59 mm, N = 261). Smaller LVEDD patients had lower survival compared to larger LVEDD patients (71% vs 85% at 1 year and 58% vs 80% at 2 years, P = 0.003). The need for temporary right ventricular mechanical support was significantly higher in the smaller LVEDD cohort (11.5% vs 1.9%, P = 0.002). Pump flows at time of discharge were lower in the smaller LVEDD group (3.8 vs 4.2 l/min, P = 0.005), who also had a higher incidence of late right ventricular failure (23% vs 12%, P = 0.02), higher rates of gastrointestinal bleeding (0.416 vs 0.256 events per patient-year, P = 0.025) and higher readmissions secondary to low flow alarms (0.429 vs 0.240 events per patient-year, P = 0.007). Multivariable analysis demonstrated that smaller LVEDD, older age, high BUN and high bilirubin levels were independent predictors of worse survival. CONCLUSIONS In patients receiving a centrifugal-flow LVAD, smaller preoperative LVEDD (≤59 mm) was associated with lower survival and higher incidence of adverse outcomes.