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Perform literature review to analyze current practices in imaging patient with peripheral arterial disease (PAD) and examine patterns in our practice in order to assess whether a lower extremity computed tomography angiography (CTA) in addition to digital subtraction angiography enhanced the assessment of vessel calcification, percentage of stenosis, and affected outcomes in patients with PAD.

•Propylene glycol-free melphalan was evaluated for transplantation in patients with AL amyloidosis.•The patients who received propylene glycol-free melphalan had low rates of renal dysfunction.•There was no treatment-related mortality.•The hematologic overall response rate was 81% at 6 months post-transplantation.•Renal response occurred in 52% of patients, and cardiac response occurred in 27% at 12 months.

OBJECTIVE--To determine the association of weight-based insulin dose with hypoglycemia in noncritically ill inpatients with diabetes. RESEARCH DESIGN AND METHODS--We performed a retrospective, case-control study of 1,990 diabetic patients admitted [...]

To authenticate to the full-text of this article, please visit this link: http://dx.doi.org/10.1111/j.1532-5415.2010.02808.x Byline: Lisa Fredman (*), Jane A. Cauley ([dagger]), Marc Hochberg ([double dagger]), Kristine E. Ensrud (s.), Gheorghe Doros ([parallel]) Keywords: caregiving; mortality; perceived stress Abstract: OBJECTIVES: To investigate the separate and combined effects of caregiver status and high stress on mortality risk over 8 years in elderly women. DESIGN: Prospective cohort study conducted in four U.S. communities followed from 1999/01 (baseline) to December 31, 2007. SETTING: Home-based interviews. PARTICIPANTS: Three hundred seventy-five caregiver and 694 noncaregiver participants from the Caregiver-Study of Osteoporotic Fractures (Caregiver-SOF) who participated in the baseline Caregiver-SOF interview. MEASUREMENTS: Caregiver status was based on SOF respondents' self-report of performing one or more instrumental or basic activities of daily living for a relative or friend with impairments. Two measures of stress were used: Perceived Stress Scale and stress related to caregiving tasks. All-cause mortality was the outcome. RESULTS: Caregivers were more stressed than noncaregivers; 19.7% of caregivers and 27.4% of noncaregivers died. Mortality was lower in caregivers than noncaregivers (adjusted hazard ratio, (AHR)=0.74, 95% confidence interval (CI)=0.56-0.89). High-stress respondents had greater mortality risk than low-stress respondents over the first 3 years of follow-up (AHR=1.81, 95% CI=1.16-2.82) but not in later years. Likewise, high-stress caregivers and noncaregivers had higher mortality risk than low-stress noncaregivers, although low-stress caregivers had significantly lower mortality than did noncaregivers, whether perceived stress or caregiving-related stress was measured (AHR=0.67 and 0.57). Similar results were observed in analyses comparing spouse caregivers with married noncaregivers. CONCLUSION: Short-term effects of stress, not caregiving per se, may increase the risk of health decline in older caregivers. Author Affiliation: (*)Epidemiology Department ([parallel])Biostatistics Department, School of Public Health, Boston University, Boston, Massachusetts ([dagger])Department of Epidemiology, University of Pittsburgh, Pittsburgh, Pennsylvania ([double dagger])Departments of Medicine and Epidemiology and Preventive Medicine, School of Medicine, University of Maryland, Baltimore, Maryland (s.)Department of Epidemiology, University of Minnesota, Minneapolis, Minnesota. Article note: Address correspondence to Lisa Fredman, Epidemiology Department, Boston University School of Public Health, 715 Albany Street, Boston, MA 02118. E-mail: lfredman@bu.edu

There are limited data on access type when treating ruptured abdominal aortic aneurysms (AAAs) with endovascular aneurysm repair (EVAR). Our study's objective was to evaluate if the type of access in ruptured AAAs affected outcomes.

Cardiovascular disease remains the leading cause of death worldwide [1].

IMPORTANCE: One-third of Medicare beneficiaries are enrolled in Medicare Advantage (MA), Medicare’s private plan option. Medicare Advantage incentivizes performance on evidence-based care, but limited information exists using reliable clinical data to determine whether this translates into better quality for patients with coronary artery disease (CAD) enrolled in MA compared with those enrolled in traditional fee-for-service (FFS) Medicare. OBJECTIVE: To determine differences in evidence-based secondary prevention treatments and intermediate outcomes among patients with CAD enrolled in MA vs FFS Medicare. DESIGN, SETTING, AND PARTICIPANTS: In this observational, retrospective, cohort study, deidentified data from patients 18 years or older diagnosed as having CAD between January 1, 2013, and May 1, 2014, at cardiology practices participating in the Practice Innovation and Clinical Excellence (PINNACLE) registry were studied, including 35 563 patients enrolled in MA and 172 732 enrolled in FFS Medicare. Data were analyzed from March to July 2018. EXPOSURES: Medicare Advantage enrollment. MAIN OUTCOMES AND MEASURES: Medication prescription patterns among eligible patients and intermediate outcomes, including blood pressure and low-density lipoprotein cholesterol. RESULTS: Of the 35 563 patients with CAD enrolled in MA, 20 193 (56.8%) were male, and the mean (SD) age was 76.7 (7.6) years; of the 172 732 patients with CAD enrolled in FFS Medicare, 100 025 (57.9%) were male, and the mean (SD) age was 77.5 (8.0) years. Patients enrolled in MA were younger, less likely to be white, and more likely to be female and to have heart failure, diabetes, and chronic kidney disease compared with those enrolled in FFS Medicare. Compared with FFS Medicare beneficiaries, MA beneficiaries were more likely to receive secondary prevention treatments, including β-blockers (80.6% vs 78.8%; P < .001), angiotensin-converting enzyme inhibitors or angiotensin II receptor blockers (70.7% vs 65.1%; P < .001), and statins (68.4% vs 64.5%; P < .001). Patients enrolled in MA were also more likely to receive all 3 medications when eligible (48.9% vs 40.4%; P < .001). After adjustment, MA beneficiaries had higher odds of receiving guideline-recommended therapy compared with FFS Medicare beneficiaries for β-blockers (odds ratio, 1.10; 95% CI, 1.04-1.17; P = .002), angiotensin-converting enzyme inhibitors or angiotensin II receptor blockers (odds ratio, 1.13; 95% CI, 1.08-1.19; P < .001), and all 3 medications (odds ratio, 1.23; 95% CI, 1.001-1.50; P = .047). There were no significant differences in intermediate outcomes between those enrolled in MA and FFS Medicare, including systolic and diastolic blood pressure and low-density lipoprotein cholesterol levels. CONCLUSIONS AND RELEVANCE: Among patients with CAD in the PINNACLE registry, MA beneficiaries had more comorbidities than FFS Medicare beneficiaries and were more likely to receive secondary prevention treatments. However, this did not translate into differences in intermediate outcomes. These findings suggest that MA plans may drive improvements in process-based quality measures for Medicare beneficiaries, although this may have a limited effect on improving patient outcomes over FFS Medicare.

Therapies for AL amyloidosis have dramatically improved, leading to longer patient survival; however, more AL amyloidosis patients are reaching end-stage renal disease (ESRD). There are no clear guidelines regarding eligibility for kidney transplantation in patients with AL amyloidosis, and data on outcomes are limited. We evaluated the clinical and laboratory data of 49 patients who were followed in the Amyloidosis Center at Boston University and underwent kidney transplantation at a center in the United States between 1987-2017. During a median follow-up of 7.2 years (range 0-19), the median patient survival from diagnosis was 15.4 years, and from kidney transplantation was 10.5 years. One, three, and five-year graft survival were 94%, 89%, and 81%, respectively. Patients with hematologic complete response or very good partial response prior to kidney transplantation had significantly better patient survival than patients with partial response or no response, and the median time to graft loss was 10.4 years versus 5.5 years, respectively. This is the largest published series of kidney transplantation in patients with AL amyloidosis, suggesting that kidney transplantation can have a good outcome in carefully selected patients, particularly in those who have achieved a complete response or very good partial response at the time of kidney transplantation.

Background:

Immunoglobulin light chain amyloidosis (AL amyloidosis) is caused by misfolded light chains that form soluble toxic aggregates that deposit in tissues and organs, leading to organ dysfunction. The leading determinant of survival is cardiac involvement. Current staging systems use N-terminal pro-brain natriuretic peptide (NT-proBNP) and cardiac troponins T and I (TnT and TnI) for prognostication, but many centers do not offer NT-proBNP. We sought to derive a new staging system using brain natriuretic peptide (BNP) that would correlate with the Mayo 2004 staging system and be predictive for survival in AL amyloidosis. Two cohorts of patients were created: a derivation cohort of 249 consecutive patients who had BNP, NT-proBNP, and TnI drawn simultaneously to create the staging system and a complementary cohort of 592 patients with 10 years of follow-up to determine survival. In the derivation cohort, we found that a BNP threshold of more than 81 pg/mL best associated with Mayo 2004 stage and also best identified cardiac involvement. Three stages were developed based on a BNP higher than 81 pg/mL and a TnI higher than 0.1 ng/mL and compared with Mayo 2004 with high concordance (κ = 0.854). In the complementary cohort, 25% of patients had stage I, 44% had stage II, 15% had stage III, and 16% had stage IIIb disease with a median survival not reached in stage I, 9.4 years in stage II, 4.3 years in stage III, and 1 year in stage IIIb. This new Boston University biomarker scoring system will allow centers without access to NT-proBNP the ability to appropriately stage patients with AL amyloidosis. This trial was registered at www.clinicaltrials.gov as #NCT00898235.

Many studies have reported that 5α-reductase inhibitors (finasteride and dutasteride) raise serum testosterone (T) levels, yet there is lack of consistency among studies on this point.

High resting heart rate (HR ≥70 b.p.m.) is associated with worse clinical outcomes in heart failure with reduced ejection fraction (HFrEF). Heart rate, guideline-directed medical therapy (GDMT) with beta-blocker (BB), and cardiovascular outcomes were evaluated in a large integrated health network.

There is a documented learning curve when adopting ultrasound guidance (UG) to aid vascular access. In the Vascular Study Group of New England (VSGNE), routine UG during percutaneous femoral artery access was shown to protect against the complication of groin hematoma. We sought to confirm this finding in the Vascular Quality Initiative (VQI), a data set with a larger sample size and broader geographic distribution, and to evaluate the effects of ultrasound usage and interventionalist volume on hematoma rates following peripheral vascular interventions (PVIs).

IMPORTANCE: The prevalence of autism spectrum disorder (ASD) has been increasing rapidly, with current estimates of 1 in 68 children affected. Simultaneously, use of prenatal ultrasonography has increased substantially, with limited investigation into its safety and effects on brain development. Animal studies have demonstrated that prenatal ultrasonography can adversely affect neuronal migration. OBJECTIVE: To quantify prenatal ultrasound exposure by the frequency, timing, duration, and strength of ultrasonographic scans in children with later ASD, developmental delay, and typical development. DESIGN, SETTING, AND PARTICIPANTS: This case-control study included 107 patients with ASD, 104 control individuals with developmental delay, and 209 controls with typical development. Participants were identified from medical records based on prenatal care and delivery at Boston Medical Center, a diverse, academic, safety-net medical center, from July 1, 2006, through December 31, 2014, with a gestational age at birth of at least 37 weeks. Data were analyzed from May 1, 2015, through November 30, 2017. EXPOSURES: Ultrasonographic exposure was quantified by the number and timing of scans, duration of exposure, mean strength (depth, frame rate, mechanical index, and thermal index), and time of Doppler and 3- and 4-dimensional imaging. MAIN OUTCOMES AND MEASURES: Among participants with ASD and controls with developmental delay and typical development, ultrasound exposure was quantified and compared per trimester and for the entire pregnancy, with adjustment for infant sex, gestational age at birth, and maternal age. RESULTS: A total of 420 participants were included in the study (328 boys [78.1%] and 92 girls [21.9%]; mean age as of January 1, 2016, 6.6 years; 95% CI, 6.5-6.8 years). The ASD group received a mean of 5.9 scans (95% CI, 5.2-6.6), which was not significantly different from the 6.1 scans (95% CI, 5.4-6.8) in the developmental delay group or the 6.3 scans (95% CI, 5.8-6.8) in the typical development group. Compared with the typical development group, the ASD group had shorter duration of ultrasound exposure during the first (290.4 seconds [95% CI, 212.8-368.0 seconds] vs 406.4 seconds [95% CI, 349.5-463.3 seconds]) and second (1687.6 seconds [95% CI, 1493.8-1881.4 seconds] vs 2011.0 seconds [95% CI, 1868.9-2153.1 seconds]) trimesters but no difference in the number of scans. The ASD group had greater mean depth of ultrasonographic penetration than the developmental delay group in the first trimester (12.5 cm [95% CI, 12.0-13.0 cm] vs 11.6 cm [95% CI, 11.1-12.1 cm]). The ASD group had greater mean depth than the typical development group during the first (12.5 cm [95% CI, 12.0-13.0 cm] vs 11.6 cm [95% CI, 11.3-12.0 cm]) and the second (12.9 cm [95% CI, 12.6-13.3 cm] vs 12.5 cm [95% CI, 12.2-12.7 cm]) trimesters. CONCLUSIONS AND RELEVANCE: This study found significantly greater mean depth of ultrasonographic penetration in the ASD group compared with the developmental delay group in the first trimester and compared with the typical development group in the first and second trimesters. Further research is needed to determine whether other variables of ultrasound exposure also have adverse effects on the developing fetus.

The use of statin and antiplatelet medications has been advocated in patients with cerebrovascular disease as primary medical therapy and as an adjunct to carotid endarterectomy (CEA). Our goal was to assess the prevalence of preoperative statin and antiplatelet use and its effect on perioperative outcomes after CEA.

The development of coronary drug-eluting stents has included use of new metal alloys, changes in stent architecture, and use of bioresorbable polymers. Whether these advancements improve clinical safety and efficacy has not been shown in previous randomised trials. We aimed to examine the clinical outcomes of a bioresorbable polymer sirolimus-eluting stent compared with a durable polymer everolimus-eluting stent in a broad patient population undergoing percutaneous coronary intervention.

One of the main advantages of endovascular abdominal aortic aneurysm repair (EVAR) is shorter postoperative hospital length of stay (LOS) compared to open repair. However, patients with preexisting conditions may be predisposed to a protracted LOS. Our aim was to identify preoperative risk factors for prolonged postoperative LOS after elective EVAR.

Dose finding Phase I oncology designs can be broadly categorized as rule based, such as the 3 + 3 and the accelerated titration designs, or model based, such as the CRM and Eff-Tox designs. This paper systematically reviews and compares through simulations several statistical operating characteristics, including the accuracy of maximum tolerated dose (MTD) selection, the percentage of patients assigned to the MTD, over-dosing, under-dosing, and the trial dose-limiting toxicity (DLT) rate, of eleven rule-based and model-based Phase I oncology designs that target or pre-specify a DLT rate of ∼0.2, for three sets of true DLT probabilities. These DLT probabilities are generated at common dosages from specific linear, logistic, and log-logistic dose-toxicity curves. We find that all the designs examined select the MTD much more accurately when there is a clear separation between the true DLT rate at the MTD and the rates at the dose level immediately above and below it, such as for the DLT rates generated using the chosen logistic dose-toxicity curve; the separations in these true DLT rates depend, in turn, not only on the functional form of the dose-toxicity curve but also on the investigated dose levels and the parameter set-up. The model based mTPI, TEQR, BOIN, CRM and EWOC designs perform well and assign the greatest percentages of patients to the MTD, and also have a reasonably high probability of picking the true MTD across the three dose-toxicity curves examined. Among the rule-based designs studied, the 5 + 5 a design picks the MTD as accurately as the model based designs for the true DLT rates generated using the chosen log-logistic and linear dose-toxicity curves, but requires enrolling a higher number of patients than the other designs. We also find that it is critical to pick a design that is aligned with the true DLT rate of interest. Further, we note that Phase I trials are very small in general and hence may not provide accurate estimates of the MTD. Thus our work provides a map for planning Phase I oncology trials or developing new ones.

Native arteriovenous fistulas (AVFs) have a high 1 year failure rate leading to a need for secondary procedures. We set out to create a predictive model of early failure in patients undergoing first-time AVF creation, to identify failure-associated factors and stratify initial failure risk.

Group sequential designs are used to potentially shorten randomized clinical trials and thereby reduce subject burden, improve safety, and save time and resources. Clinical trials comparing treatments for systemic lupus erythematosus (SLE) might adopt such designs if the ordinal outcome scales for SLE, such as the Systemic Lupus Activity Measure and Systemic Lupus Erythematosus Disease Activity Index, were more like continuous outcome scales with interval properties. After describing the basic features of sequential trials and highlighting some major issues in their design, we propose approaches that mitigate these issues. In particular, high-speed computing has accelerated advances in sequential design, making available a variety of designs that can be implemented with minimal technical support. The challenge now is to understand the concepts behind such flexible designs and then to apply them to improve studies of SLE.

Iliac conduit or direct iliac access (ICDA) can be used when anatomy is unfavorable for femoral access during abdominal endovascular aortic aneurysm repair (EVAR). The impact of this approach has not been adequately addressed. The objective of this study was to analyze perioperative outcomes of patients requiring use of ICDAs for EVAR.

Since the 2004 approval of carotid artery angioplasty and stenting (CAS), there have been 2 seminal publications about CAS reimbursement (Center for Medicaid and Medicare Several guidelines [CMSG]; 2008) and clinical outcomes (Carotid Revascularization Endarterectomy versus Stent Trial [CREST]; 2010). We explored trends in CAS utilization after these publications nationally.

Major depressive disorder is often accompanied by elevated levels of anger, hostility, and irritability, which may contribute to worse outcomes. The present study is a secondary analysis examining the role of anger/hostility in the treatment response to low-dose aripiprazole added to antidepressant therapy in 225 patients with major depressive disorder and inadequate response to antidepressant treatment. Repeated-measures model demonstrated no drug-placebo difference in treatment response across levels of anger/hostility. However, within-group analyses showed significantly lower placebo response rates in patients with high anger/hostility and a trend for lower drug response rates in patients with high anger/hostility. Pooled response rates across phases and treatments revealed a lower response rate among patients with high anger/hostility. Depressed patients with high anger/hostility demonstrate greater illness severity and lower depressive treatment response rates than patients with low anger/hostility, suggesting that patients with high anger/hostility may have poorer outcomes in response to adjunctive treatment.

Treatment with aspirin and a P2Y12 inhibitor is commonly used in patients with cardiovascular disorders. The overall effect of such treatment on all-cause mortality is unknown. In the Dual Antiplatelet Therapy (DAPT) Study, continuation of dual antiplatelet therapy beyond 12 months after coronary stenting was associated with an unexpected increase in non-cardiovascular death. In view of the potential public health importance of these findings, we aimed to assess the effect of extended duration dual antiplatelet therapy on mortality by doing a meta-analysis of all randomised, controlled trials of treatment duration in various cardiovascular disorders.

We describe the organization of a prospective, randomized, multicenter trial comparing the effectiveness of open popliteal artery aneurysm repair (OPAR) and endovascular popliteal artery aneurysm repair (EPAR) of asymptomatic popliteal artery aneurysms (PAAs) as an example for how to use the Vascular Quality Initiative (VQI) framework. Given that many centers participate in the VQI, this model can be used to perform multicenters' prospective trials on very modest budget.

To assess the safety and tolerability of high-dose creatine, the feasibility of enrolling premanifest and 50 at-risk subjects in a prevention trial, and the potential of cognitive, imaging, and blood markers.

BACKGROUND Aberrant accumulation of transition metals in the brain may have an early and important role in the pathogenesis of several neurodegenerative disorders, including Huntington disease (HD). OBJECTIVE To comprehensively evaluate and validate the distribution of metal deposition in the brain using advanced magnetic resonance imaging methods from the premanifest through symptomatic stages of HD. DESIGN Observational study. SETTING University imaging center. PARTICIPANTS Twenty-eight HD expanded gene carriers, 34 patients with symptomatic HD, and 56 age- and sex-matched healthy control subjects were included in the study. INTERVENTIONS Participants underwent magnetic resonance imaging for the quantification of the phase evolution of susceptibility-weighted images. MAIN OUTCOME MEASURES To verify the identity of the metals responsible for the changes in the phase evolution of the susceptibility signal in the brain and to assess correlations with systemic levels. Inductively coupled plasma mass spectrometry was used to measure transition metal concentrations in postmortem brains. RESULTS In the basal ganglia, progressive increases in the phase evolution were found in HD, beginning in premanifest individuals who were far from expected onset and increasing with proximity to expected onset and thereafter. Increases in the cerebral cortex were regionally selective and present only in symptomatic HD. Increases were verified by excessive deposition of brain iron, but a complex alteration in other transition metals was found. CONCLUSION An important and early role of altered metal homeostasis is suggested in the pathogenesis of HD.

Previous studies have suggested that, in patients with AL amyloidosis treated with high-dose melphalan and autologous stem-cell transplantation (HDM/SCT), the greatest benefit is seen in those patients achieving a hematologic complete response (CR). We analyzed a series of 421 consecutive patients treated with HDM/SCT at a single referral center and compared outcomes for patients with and without CR. Treatment-related mortality was 11.4% overall (5.6% in the last 5 years). By intention-to-treat analysis, the CR rate was 34% and the median event-free survival (EFS) and overall survival (OS) were 2.6 and 6.3 years, respectively. Eighty-one patients died within the first year after HDM/SCT and were not evaluable for hematologic and organ response. Of 340 evaluable patients, 43% achieved CR and 78% of them experienced an organ response. For CR patients, median EFS and OS were 8.3 and 13.2 years, respectively. Among the 195 patients who did not obtain CR, 52% achieved an organ response, and their median EFS and OS were 2 and 5.9 years, respectively. Thus, treatment of selected AL patients with HDM/SCT resulted in a high organ response rate and long OS, even for those patients who did not achieve CR.

Follistatin-like 1 (FSTL1) is an extracellular glycoprotein found in human serum. Recent work suggests that FSTL1 is secreted in response to ischemic injuries and that its overexpression is protective in the heart and vasculature.

Black Americans have higher mortality from breast cancer than white Americans. This study explores the influence of socioeconomic factors and black race on treatment and mortality for early-stage breast cancer.

Objective: To determine whether type and timing of surfactant preparation affect cerebral blood flow (CBF) hemodynamics differently. Study design: Two sites monitoring cerebral hemodynamics during surfactant administration. Beractant (4.0 ml/kg) and poractant (2.5 ml/kg first dose, 1.25 ml/kg subsequent doses) were administered according to the manufacturer. CBF velocity (CBFv) was assessed before and after the first (Site A) and second (Site B) doses of beractant and poractant. Results: At Site A, 10 infants (birth weight 690 ± 133 g) were studied. CBFv decreased 28.9 ± 5.1% following the first dose of surfactant; there were no differences between beractant and poractant. At Site B, 16 infants (birth weight 896 ± 311 g) were studied. CBFv increased 47.4 ± 35.7% after the second dose of beractant and did not change after the second dose of poractant. Conclusion: This is a preliminary report of two related trials examining CBFv responses to beractant and poractant. CBFv decreased similarly after the first dose of beractant and poractant. CBFv increased after the second dose of beractant, but not after poractant. Cerebral hemodynamic responses between the first and second doses may be due to differences in quantities of airway fluid, while second dose differences between beractant and poractant may be due to differences in dose-volume.

To compare the changes induced by oral versus transdermal estrogen therapy on the total and free serum concentrations of testosterone (T), thyroxine (T4), and cortisol (C) and the concentrations of their serum binding globulins sex hormone-binding globulin, thyroxine-binding globulin, and cortisol-binding globulin in naturally menopausal women.

There is urgent need for safe, effective, and easily implementable treatments that reduce the progression of respiratory failure in COVID-19. Despite increased adoption during the pandemic, the effectiveness of prone positioning on respiratory failure progression among non-intubated patients is unclear.

Background:

Supplemental Digital Content is available in the text.

The day of the week (DOW) for performing procedures and operations has been shown to affect clinical and resource utilization outcomes. Limited published data are available on vascular surgery operations. Our primary objective was to assess outcomes by DOW for infrainguinal lower extremity bypass (LEB) performed for claudication or rest pain. The secondary objective was to assess outcomes by DOW for LEBs performed for tissue loss.

Long-term survival and outcome were determined for 80 patients with immunoglobulin light chain (AL) amyloidosis treated with high-dose melphalan and stem cell transplantation (HDM/SCT) more than 10 years ago. Seventeen (21%) patients died within the first year of treatment, of treatment-related complications (14%) or progressive disease (8%). Of the 63 surviving evaluable patients at one year, 32 (51%) achieved a complete hematologic response (CR). For all 80 patients, the median survival was 57 months (4.75 yrs). The median survival exceeds 10 years for patients achieving a CR after HDM/SCT, compared with 50 months for those not achieving a CR (P < .001). In conclusion, HDM/SCT leads to durable remissions and prolonged survival, particularly for those patients who achieve a hematologic CR.

Background:Racial/ethnic minorities have been underrepresented in most population-based studies of AL amyloidosis published to date. This observation stands in marked contrast to multiple myeloma, a closely related disorder with a twofold higher incidence among blacks vs. whites. Given the scarcity of information about health disparities in AL amyloidosis, we aimed to characterize the clinical presentation and outcomes of this unique plasma cell disorder according to race/ethnicity.

Supplemental Digital Content is available in the text.

Introduction:Advance care planning (ACP) and prognosis education are key components of high-quality, patient-centered care in heart failure (HF) and associated with better patient-reported outcomes. While research suggests that these occur infrequently in HF, studies have been limited to small and regional samples. We examined the prevalence of documented ACP and prognosis/end-of-life education as well as their associations with healthcare utilization in a large national sample of outpatients with HF.Method:Among patients with HF in the NCDR PINNACLE Registry enrolled between January 2013 and June 2018, we determined the proportion receiving ACP and prognosis education. T-tests and chi-squares were used to examine characteristics of ACP and prognosis education. In those over 65 who could be linked with Medicare claims data, adjusted multivariable linear and two-level hierarchical logistic regression modelss were conducted to examine the associations of ACP and prognosis education with inpatient and outpatient utilization. The first heart failure encounter for each patient was analyzed for presence of ACP and prognosis education.Results:Among 1.6 million patients with HF (Mage= 68.6+14.3), approximately 34% received ACP, while only 2% received prognosis education. Rates of ACP and prognosis education were lower in women, patients in the northeast and rural areas, and those in NYHA classes III-IV. Both ACP and prognosis education were low overall among patients with cardiac devices. Among 276,916 patients linked to Medicare claims data (Mage= 77.2+7.8), ACP was associated with fewer outpatient visits (p< 0.001), and both a lower likelihood of 30-day all-cause (OR = 0.86; 95% CI = 0.82-0.90) and HF related admissions (OR = 0.78, 95% CI = 0.73-0.84). A similar pattern was observed for prognosis education.Conclusions:Less than half of patients with HF had documented ACP and almost no patients had documented prognosis education, despite being quality metrics in PINNACLE. Further, these quality metrics were associated with lower healthcare utilization rates. Research is needed to better understand the factors that promote ACP and prognosis education, and the effects of these quality metrics on clinical and patient-reported outcomes.

Background:Wild-type transthyretin (ATTRwt) cardiac amyloidosis is a progressive restrictive cardiomyopathy, characterized by the extracellular deposition of amyloid protein fibrils derived from genetically normal transthyretin (TTR) protein. Diflunisal is a TTR stabilizer that can reduce neurologic deterioration in patients with familial amyloid polyneuropathy due to hereditary transthyretin amyloidosis. We hypothesized that diflunisal would also improve clinical outcomes in ATTRwt cardiac amyloidosis.Methods:We conducted a retrospective case-control cohort study of patients with ATTRwt cardiac amyloidosis seen between 2009 and 2016 who were treated with diflunisal for at least one year, or untreated. Diflunisal administration was per standard clinical practice at our center with treatment decisions based on renal function and heart failure severity. Baseline and one year follow up characteristics, including serum markers and echocardiography, were assessed. Cox proportional hazards analysis was used to assess primary outcome of all-cause mortality.Results:There were 105 patients evaluated (97% male, 81% white), with 35 receiving diflunisal and 70 in the control group. Patients in the diflunisal group were younger (76.7 years vs 79.3 years, p = 0.07), with lower BNP levels (340.9 ? 365.0 vs 520.9 ? 297.4 pg/mL, p = 0.008), and troponin I levels (0.098 ? 0.1 vs 0.19 ? 0.34 ng/mL, p = 0.03). There was a trend towards worse renal function in the control group (eGFR 53.35 ? 19.45 vs 61.53 ? 24.29 mL/min, p = 0.14). Diflunisal administration was associated with improved survival in unadjusted survival analysis (HR 0.20, 95% CI 0.08 - 0.49, p = 0.0001), which persisted after adjustment for BNP, creatinine, troponin I, interventricular septal thickness, and LVEF (HR 0.23, 95% CI 0.09 - 0.60, p = 0.003). Diflunisal use was also associated with non-significant improvement in BNP, stability in troponin I and echocardiographic parameters, and a non-significant increase in serum creatinine as compared to controls.Conclusion:Diflunisal use in ATTRwt cardiac amyloidosis was well tolerated in selected patients and was associated with improved survival, as well as stability in echocardiographic and biochemical markers of disease.

Sloan: Merck: Other: endpoint review commitee; Stemline: Consultancy; Abbvie: Other: Endpoint Review Committee. Sarosiek:Acrotech: Research Funding. Sanchorawala:Proclara: Consultancy, Honoraria; Caelum: Consultancy, Honoraria, Membership on an entity's Board of Directors or advisory committees; Janssen: Research Funding; Prothena: Research Funding; Celgene: Research Funding; Takeda: Research Funding.