scholarly journals The cardiac amyloidosis clinical pathway implementation: a real world experience

2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
M Brons ◽  
F.H Rutten ◽  
M.C Minnema ◽  
A.F Baas ◽  
F.W Asselbergs ◽  
...  

Abstract Background/Introduction Prognosis for patients with cardiac amyloidosis (CA) is determined by disease stage at diagnosis, underlining the importance of timely referral. In 2018, a CA clinical pathway was implemented in an Amyloidosis Expert Center in The Netherlands to increase regional awareness. Purpose To evaluate the impact of CA clinical pathway implementation. Methods Patients diagnosed before implementation (2007–2018; T1) were retrospectively compared to the period after clinical pathway implementation (2019–2020; T2). Demographics and disease characteristics were collected from electronic health records. Results In total, 113 patients were diagnosed, mean age 67.8±8.5 years, 26% female. AL-CA was diagnosed in 62%, ATTR-CA in 38%. The number of CA diagnoses/year increased over time (Figure 1). NYHA class III (45% vs. 24%,p=0.04), CA stage (MAYO/Gillmore stage III or IV; 59% vs. 33%, p=0.03) and delay between symptoms and diagnosis (14.5 vs. 8.4 months, p<0.01) improved considerably between T1 and T2. Reasons for referral changed over time, with increasing awareness for right ventricular hypertrophy (9% vs. 36%) and unexplained HFpEF (22% vs 38%). Conclusion CA patients were diagnosed earlier with less severe symptoms. Clinical pathway implementation facilitated regional increased awareness and improved timely referral. FUNDunding Acknowledgement Type of funding sources: None.

2021 ◽  
Vol 28 (Supplement_1) ◽  
Author(s):  
S Pallikadavath ◽  
R Patel ◽  
CL Kemp ◽  
M Hafejee ◽  
N Peckham ◽  
...  

Abstract Funding Acknowledgements Type of funding sources: None. Introduction Cardiovascular adaptations as a result of exercise conducted at high-intensity and high-volume are often termed the ‘Athlete’s heart’. Studies have shown that these cardiovascular adaptations vary between sexes. It is important that both sexes are well represented in this literature. However, many studies assessing the impact of high-dose exercise on cardiovascular outcomes under-recruit female participants. Purpose This scoping review aimed to evaluate the representation of females in studies assessing the impact of high-dose exercise on cardiovascular outcomes and demonstrate how this has changed over time. Methods The scoping review protocol as outlined by Arksey and O’Malley was used. OVID and EMBASE databases were searched and studies independently reviewed by two reviewers. Studies must have investigated the effects of high-dose exercise on cardiovascular outcomes. To assess how the recruitment of females has changed over time, two methods were used. One, the median study date was used to categorise studies into two groups. Two, studies were divided into deciles to form ten equal groups over the study period. Mean percentage of female recruitment and percentage of studies that failed to include females were calculated. Results Overall, 250 studies were included. Over half the studies (50.8%, n = 127) did not include female participants. Only 3.2% (n = 8) did not include male participants. Overall, mean percentage recruitment was 18.2%. The mean percentage of recruitment was 14.5% before 2011 and 21.8% after 2011. The most recent decile of studies demonstrated the highest mean percentage of female recruitment (29.3%) and lowest number of studies that did not include female participants (26.9%). Conclusion Female participants are significantly underrepresented in studies assessing cardiovascular outcomes caused by high-dose exercise. The most recent studies show that female recruitment may be improving, however, this still falls significantly short for equal representation. Risk factors, progression and management of cardiovascular diseases vary between sexes, hence, translating findings from male dominated data is not appropriate. Future investigators should aim to establish barriers and strategies to optimise fair recruitment. Mean percentage females recruited per study (%) Percentage studies that do not include women (%) Overall (n = 250) 18.2 50.8 (n = 127) Studies before 2011 (n = 121) 14.5 59.5 (n = 72) Studies after 2011 (n = 129) 21.8 42.6 (n = 55) Table 1: Female recruitment characteristics. The year 2011 (median study year) was chosen as this divides all included studies into two equal groups.


Circulation ◽  
2007 ◽  
Vol 116 (suppl_16) ◽  
Author(s):  
Lynne W Stevenson ◽  
Yong K Cho ◽  
J. T Heywood ◽  
Robert C Bourge ◽  
William T Abraham ◽  
...  

Introduction : Elevated filling pressures are a hallmark of chronic heart failure. They can be reduced acutely during HF hospitalization but the hemodynamic impact of ongoing therapy to maintain optivolemia has not been established. Methods and Results : After recent HF hospitalization, 274 NYHA Class III or IV HF patients were enrolled in the COMPASS-HF study at 28 experienced HF centers and received intense HF management (average 24.7 staff contacts/ 6 months) ± access to filling pressure information to adjust diuretics to maintain optivolemia, usually defined as estimated pulmonary artery diastolic (PAD) pressure of 12±4 mmHg. Filling pressure information was available for half the patients during the first 6 months (the Chronicle group, <Access), and for all patients during the next 6 months. Diuretics were adjusted 12.7 times per patient in the Chronicle group and 8.2 times per patient in the Control (-Access) group during the first 6 months (p = 0.0001). Compared to baseline, decreases in RV systolic pressure (RVSP) and ePAD were significant for the +Access patients by one year (p=0.0012 and p =.04, respectively). The Control patients exhibited a similar trend 6 months after crossing to +Access (figure ). Conclusions: Targeted therapeutic adjustments, based on continuous filling pressures along with intensification of HF management contacts, are associated with a reduction in chronic left-sided filling pressures and right ventricular load.


Circulation ◽  
2007 ◽  
Vol 116 (suppl_16) ◽  
Author(s):  
Michel White ◽  
Peter Carson ◽  
Inder S Anand ◽  
Stephen S Gottlieb ◽  
JoAnn Lindenfeld ◽  
...  

Introduction: Bucindolol is a nonselective beta-adrenergic blocker with potent sympatholytic properties. The Beta-blocker Evaluation of Survival Trial (BEST) reported that the administration of bucindolol resulted in a nonsignificant decrease in total mortality (HR = 0.89 (0.78, 1.02), unadjusted p=0.10) in patients with advanced, NYHA Class III-IV heart failure (HF). Recent observations from that trial also reported that the amino acid arginine (Arg/Arg) or glycine (any Gly) in position 389 of the beta-1 receptor plays a significant role on the clinical response to bucindolol. The impact of bucindolol on cardiovascular mortality and morbidity (cardiovascular hospitalizations) has been incompletely investigated, because hospitalizations had been evaluated from case report forms (CRFs) only, and never adjudicated by the endpoints committee (EPC). Methods: The BEST data base consists of 2708 patients with a mean follow-up of 2.0 years. Cardiovascular (CV) mortality and hospitalizations have now been evaluated by EPC, which further subclassified total hospitalizations into cardiovascular (CV) and those due to worsening heart failure (HF). The impacts of Arg or Gly encoded at amino acid position 389 on endpoints were further investigated in the 1040 patient substudy. Results: Time to event results for adjudicated CV endpoints are presented below. Conclusions: Chronic administration of bucindolol results in a significant reduction in cardiovascular hospitalizations and mortality. Effects on either are strikingly beta-1 389 Arg/Gly specific, with the higher functioning, Arg/Arg version of the receptor associated with large treatment effects and Gly carriers exhibiting little or no evidence of efficacy. Genetic targeting of the β 1 -ΑR 389 polymorphism may improve the clinical responses to bucindolol for CV mortality and morbidity.


2021 ◽  
Vol 10 (24) ◽  
pp. 5962
Author(s):  
JinShil Kim ◽  
Seongkum Heo ◽  
Bong Roung Kim ◽  
Soon Yong Suh ◽  
Jae Lan Shim ◽  
...  

Evidence for non-modifiable and modifiable factors associated with the utilization of advance directives (ADs) in heart failure (HF) is lacking. The purpose of this study was to examine baseline-to-3-month changes in knowledge, attitudes, and benefits/barriers regarding ADs and their impact on the completion of life-sustaining treatment (LST) decisions at 3-month follow-up among patients with HF. Prospective, descriptive data on AD knowledge, attitudes, and benefits/barriers and LSTs were obtained at baseline and 3-month follow-up after outpatient visits. Of 64 patients (age, 68.6 years; male, 60.9%; New York Heart Association (NYHA) classes I/II, 70.3%), 53.1% at baseline and 43.8% at 3-month follow-up completed LST decisions. Advanced age (odds ratio (OR) = 0.91, p = 0.012) was associated with less likelihood of the completion of LST decisions at 3-month follow-up, while higher education (OR = 1.19, p = 0.025) and NYHA class III/IV (OR = 4.81, p = 0.049) were associated with more likelihood. In conclusion, advanced age predicted less likelihood of LST decisions at 3 months, while higher education and more functional impairment predicted more likelihood. These results imply that early AD discussion seems feasible in mild symptomatic HF patients with poor knowledge about ADs, considering the non-modifiable and modifiable factors.


Circulation ◽  
2008 ◽  
Vol 118 (suppl_18) ◽  
Author(s):  
Victoria V Dickson ◽  
Barbara Riegel

Background : Heart failure (HF) self-care requires both knowledge and skill, but little attention has been given to identifying how to improve skill in HF self-care. Methods: We assessed what self-care skills are needed and how patients develop these skills using qualitative descriptive meta-analysis techniques. Transcripts from three mixed methods studies (n=85) were re-examined and translated to yield themes about the process of developing skill in HF self-care, defined as behaviors that maintain physiologic stability (maintenance) and the response to symptoms when they occur (management). Results: The sample was 63.5% Caucasian and 58.8% male, mean age of 55.69 years (± 13.42). Most (52.9%) were NYHA class III. Narrative accounts of HF self-care revealed that the most challenging self-care maintenance skills were diet, and exercise. The management skill that was most challenging was how to titrate diuretics in response to a weight gain. Skills developed over time through coaching by trusted resources (mostly friends or family with HF). Tactical skills (“how to” prepare low salt meals; “how to” start an exercise plan) were helped by role-playing. Situational skills (“what to do when”) most essential for self-care management were evident in descriptions of how they planned for special occasions. For example, significant others helped patients think through how to manage symptoms of fluid retention while traveling. Few patients saw traditional healthcare providers as resources they could call upon to aid them in developing tactical and situational skills. Patient education was not identified as a factor associated with the development of HF self-care skill. Rather, they struggled to operationalize the self-care knowledge they attained. Conclusion: Skill in HF self-care evolves over time, with experience and practice, as patients learned how to make self-care practices fit into their daily lives, but coaching by nurses may help patients develop the skill needed to master self-care skills. Research testing coaching interventions that target skill building tactics such as role-playing in specific situations is needed.


2019 ◽  
Vol 8 (12) ◽  
pp. 2165 ◽  
Author(s):  
Giuseppe Romano ◽  
Giuseppe Vitale ◽  
Laura Ajello ◽  
Valentina Agnese ◽  
Diego Bellavia ◽  
...  

Background: Sacubitril/valsartan has been shown to be superior to enalapril in reducing the risks of death and hospitalization for heart failure (HF). However, knowledge of the impact on cardiac performance remains limited. We sought to evaluate the effects of sacubitril/valsartan on clinical, biochemical and echocardiographic parameters in patients with heart failure and reduced ejection fraction (HFrEF). Methods: Sacubitril/valsartan was administered to 205 HFrEF patients. Results: Among 230 patients (mean age 59 ± 10 years, 46% with ischemic heart disease) 205 (89%) completed the study. After a follow-up of 10.49 (2.93 ± 18.44) months, the percentage of patients in New York Heart Association (NYHA) class III changed from 40% to 17% (p < 0.001). Median N–Type natriuretic peptide (Nt-proBNP) decreased from 1865 ± 2318 to 1514 ± 2205 pg/mL, (p = 0.01). Furosemide dose reduced from 131.3 ± 154.5 to 120 ± 142.5 (p = 0.047). Ejection fraction (from 27± 5.9% to 30 ± 7.7% (p < 0.001) and E/A ratio (from 1.67 ± 1.21 to 1.42 ± 1.12 (p = 0.002)) improved. Moderate to severe mitral regurgitation (from 30.1% to 17.4%; p = 0.002) and tricuspid velocity decreased from 2.8 ± 0.55 m/s to 2.64 ± 0.59 m/s (p < 0.014). Conclusions: Sacubitril/valsartan induce “hemodynamic recovery” and, consistently with reduction in Nt-proBNP concentrations, improve NYHA class despite diuretic dose reduction.


2021 ◽  
Vol 10 (15) ◽  
pp. 3415
Author(s):  
Amaury Broussier ◽  
Jean Philippe David ◽  
Mounira Kharoubi ◽  
Silvia Oghina ◽  
Lauriane Segaux ◽  
...  

ATTRwt-CA occurs in elderly patients and leads to severe heart failure. The disease mechanism involves cardiac and extracardiac infiltration by amyloid fibrils. The objectives of this study are to describe the frailty phenotype in patients with ATTRwt-CA and to assess the associations between frailty parameters, the severity of cardiac involvement, and the course of amyloid disease. We used multidimensional geriatric tools to prospectively assess frailty in patients with ATTRwt-CA consulting (in 2018–2019) in the French National Reference Center for Cardiac Amyloidosis. We included 36 patients (35 males; median age: 82 years (76–86). A third of the patients were categorized as NYHA class III or IV, and 39% had an LVEF below 45%. The median serum NTproBNP was 3188 (1341–8883) pg/mL. The median duration of amyloidosis was 146 months (73–216). The frequency of frailty was 50% and 33% according to the physical frailty phenotype and the Short Emergency Geriatric Assessment questionnaire, respectively. Frailty affected a large number of domains, namely autonomy (69%), balance (58%), muscle weakness (74%), malnutrition (39%), dysexecutive syndrome (72%), and depression (49%). The severity of CA was significantly associated with many frailty parameters independently of age. Balance disorders and poor mobility were also significantly associated with a longer course of amyloid disease. Frailty is frequent in patients with ATTRwt-CA. Some frailty parameters were significantly associated with a longer course of amyloid disease and CA severity. Taking into account frailty in the assessment and management of ATTRwt should improve patients’ quality of life.


Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Pranav Chandrashekar ◽  
Zack Dale ◽  
Lana Rashdan ◽  
Miriam Elman ◽  
Babak Nazer ◽  
...  

Introduction: Tafamidis has been shown to improve survival and decrease heart failure (CHF) hospitalizations in transthyretin cardiac amyloidosis (ATTR-CM). In the ATTR-ACT trial, survival benefit emerged after approximately 18 months of treatment, with less clear benefit in those with NYHA class III symptoms. We sought to evaluate the short-term changes in NYHA class, biomarkers, and clinical outcomes in a real-world cohort of patients treated with tafamidis. Methods: A single center retrospective observational study of patients with ATTR-CM who were prescribed tafamidis since its FDA approval in May 2019. We collected data on prospectively assessed NYHA class, NTproBNP, Troponin I, and outcomes of CHF hospitalizations and death. Results: Fifty-one patients with ATTR-CM (mean age 73.3±7 years, 100.0% male) were prescribed tafamidis. Forty-five patients had wild-type and 6 had hereditary ATTR-CM. At the visit when tafamidis was initiated 6 (11.8%) patients were NYHA Class I, 24 (47.1%) Class II, 20 (39.2%) Class III, and 1 (2%) Class IV. Over a median follow up of 8 (IQR 6.0-11) months after starting tafamidis, there were no significant differences before and after tafamidis treatment in time-averaged median NYHA functional class (paired Wilcoxon test, p=0.1610; Figure 1A), time-averaged median NTproBNP (paired exact Wilcoxon test, p > 0.9999; Figure 1B), and time-averaged median troponin I (paired exact Wilcoxon test, p=0.9400; Figure 1C). Following initiation of tafamidis, 13 patients (25.5%) were admitted for CHF hospitalization. A total of 8 patients (15.7%) died a median of 9.5 (IQR 2.5-12.5) months after initiation of tafamidis. Conclusions: ATTR-CM patients taking tafamidis were observed to have stable functional status and biomarkers, including those with NYHA class III symptoms. Despite a higher proportion of worse NYHA functional class, the rate of death at 10 months appears comparable to the ATTR-ACT trial in this real-world population.


2021 ◽  
Vol 39 (15_suppl) ◽  
pp. e18572-e18572
Author(s):  
Jianrong Zhang ◽  
Jasmeen Oberoi ◽  
Napin Karnchanachari ◽  
Paige Druce ◽  
Maarten J. IJzerman ◽  
...  

e18572 Background: Lung cancer is characterized by the aggressive nature and lack of early noticeable symptoms, highlighting the importance of timely care for lung cancer. This systematic review of systematic and scoping reviews provides the summary of review-level results related to the length of time intervals and the impact of these intervals on oncological outcomes. Methods: Eligible articles included published systematic or scoping reviews and meta-analyses that used systematic searching to investigate the length of time intervals and their impact on patient survival and disease stage at diagnosis in original studies on lung cancer, based on at least one interval involving the date of diagnosis as the start/endpoint. Articles were searched via PubMed, Embase, Web of Science, and the Cochrane Library (date range: from database inception to 6 August 2020) (PROSPERO identifier: CRD42020203530). Results: From 1395 identified publications, we included a total of 8 systematic/scoping reviews published in 2002-2018 (7 on length of intervals, 5 on survival/stage impact). For the length of intervals, these reviews found that intervals across countries/medical facilities are heterogeneous and often longer than recommended by guidelines. For the survival/stage impact, the results from these reviews indicated mixed (positive, negative, or no) associations between time intervals and patient survival/disease stage at diagnosis. We further summarized study-level results and confirmed the finding of mixed associations based on 36 eligible original studies from over 100 studies included in these reviews. Methodologically, review authors pointed out large variations in time intervals (96 unique variations) and methods used to measure intervals. Based on the reviews, we found that the intervals in studies were normally investigated cross-sectionally, rather than temporarily (to demonstrate the trend of intervals’ length over time). Conclusions: Current evidence indicates that patients with lung cancer may experience diagnosis and treatment delays, however, the association results between the length of intervals and lung cancer survival and stage are paradoxical. To minimize avoidable delays, future studies should keep updating the length of intervals and provide the trend analysis of the intervals over time, as part of cancer surveillance to guide future policy making and clinical practice. To improve the quality of future original studies and systematic reviews, specific guidelines and corresponding studies related to study design and methodology (e.g. types of time intervals, measures of time intervals, statistical analysis strategy) are warranted, especially underscoring the effect of “waiting time paradox” when investigating the impact of time intervals on patient outcomes.


Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Aadhavi Sridharan ◽  
Martin S Maron ◽  
Dou Huang ◽  
Craig Cooper ◽  
Jennifer Drummond ◽  
...  

Background: The impact of comorbid disease states and lifestyle on the natural history of patients with hypertrophic cardiomyopathy (HCM) remains unknown. Objective: Evaluate the association of non-HCM comorbidities including obesity, hypertension, diabetes, obstructive sleep apnea, kidney disease, tobacco use, alcohol use, and lung disease with disease progression in a large cohort of HCM patients. Methods: 2269 patients evaluated at the Tufts HCM Institute between 2004 to 2019, ≥ 18 years of age (54 ± 15 years; 1392 male), and followed for an average of 4 ± 3.4 years for disease progression including progressive heart failure (HF) symptoms (from NYHA class I/II to NYHA class III/IV), new-onset atrial fibrillation (AF), or sudden death (SD) event (including appropriate defibrillation for ventricular arrhythmias, resuscitated cardiac arrest, or SD). Results: Of 1376 patients with NYHA class I/II symptoms at initial clinical evaluation, 252 (18%) developed progressive HF symptoms to NYHA class III/IV over follow-up (5%/year). Obesity (BMI ≥ 30) was significantly more prevalent in patients who had progressive HF during follow-up (43%) compared to those who remained without HF (34%, p = 0.014). In contrast, other comorbidities were not significantly associated with progressive HF symptoms (p > 0.10 for all other comorbidities). Of the 1823 patients without AF history at initial clinical visit, 198 (11%) developed new-onset AF over follow-up (3%/year). No comorbidities were significantly associated with new-onset AF in HCM (p > 0.10), although obesity was more common in patients who developed new-onset AF (48%) compared to those who had no AF (41%, p = 0.08). Notably, SD events were not associated with non-HCM comorbidities (p > 0.10 for all comorbidities), and patients with SD events were less likely to have comorbidities than patients without SD events. Conclusions: In adult HCM patients, obesity is associated with progressive symptoms and outcomes supporting weight loss as an important modifier in obese HCM patients to potentially help prevent HCM complications. In contrast, other non-HCM comorbidities do not appear to impact disease course, and SD events are not associated with comorbidities in HCM.


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