Dataset Information

Prevalence and Outcomes of Concomitant Aortic Stenosis and Cardiac Amyloidosis.

ABSTRACT:

Background

Older patients with severe aortic stenosis (AS) are increasingly identified as having cardiac amyloidosis (CA). It is unknown whether concomitant AS-CA has worse outcomes or results in futility of transcatheter aortic valve replacement (TAVR).

Objectives

This study identified clinical characteristics and outcomes of AS-CA compared with lone AS.

Methods

Patients who were referred for TAVR at 3 international sites underwent blinded research core laboratory ^99mtechnetium-3,3-diphosphono-1,2-propanodicarboxylic acid (DPD) bone scintigraphy (Perugini grade 0: negative; grades 1 to 3: increasingly positive) before intervention. Transthyretin-CA (ATTR) was diagnosed by DPD and absence of a clonal immunoglobulin, and light-chain CA (AL) was diagnosed via tissue biopsy. National registries captured all-cause mortality.

Results

A total of 407 patients (age 83.4 ± 6.5 years; 49.8% men) were recruited. DPD was positive in 48 patients (11.8%; grade 1: 3.9% [n = 16]; grade 2/3: 7.9% [n = 32]). AL was diagnosed in 1 patient with grade 1. Patients with grade 2/3 had worse functional capacity, biomarkers (N-terminal pro-brain natriuretic peptide and/or high-sensitivity troponin T), and biventricular remodeling. A clinical score (RAISE) that used left ventricular remodeling (hypertrophy/diastolic dysfunction), age, injury (high-sensitivity troponin T), systemic involvement, and electrical abnormalities (right bundle branch block/low voltages) was developed to predict the presence of AS-CA (area under the curve: 0.86; 95% confidence interval: 0.78 to 0.94; p < 0.001). Decisions by the heart team (DPD-blinded) resulted in TAVR (333 [81.6%]), surgical AVR (10 [2.5%]), or medical management (65 [15.9%]). After a median of 1.7 years, 23% of patients died. One-year mortality was worse in all patients with AS-CA (grade: 1 to 3) than those with lone AS (24.5% vs. 13.9%; p = 0.05). TAVR improved survival versus medical management; AS-CA survival post-TAVR did not differ from lone AS (p = 0.36).

Conclusions

Concomitant pathology of AS-CA is common in older patients with AS and can be predicted clinically. AS-CA has worse clinical presentation and a trend toward worse prognosis, unless treated. Therefore, TAVR should not be withheld in AS-CA.

SUBMITTER: Nitsche C

PROVIDER: S-EPMC7805267 | biostudies-literature | 2021 Jan

REPOSITORIES: biostudies-literature

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Publications

Prevalence and Outcomes of Concomitant Aortic Stenosis and Cardiac Amyloidosis.

Nitsche Christian C Scully Paul R PR Patel Kush P KP Kammerlander Andreas A AA Koschutnik Matthias M Dona Carolina C Wollenweber Tim T Ahmed Nida N Thornton George D GD Kelion Andrew D AD Sabharwal Nikant N Newton James D JD Ozkor Muhiddin M Kennon Simon S Mullen Michael M Lloyd Guy G Fontana Marianna M Hawkins Philip N PN Pugliese Francesca F Menezes Leon J LJ Moon James C JC Mascherbauer Julia J Treibel Thomas A TA

Journal of the American College of Cardiology 20201109 2

<h4>Background</h4>Older patients with severe aortic stenosis (AS) are increasingly identified as having cardiac amyloidosis (CA). It is unknown whether concomitant AS-CA has worse outcomes or results in futility of transcatheter aortic valve replacement (TAVR).<h4>Objectives</h4>This study identified clinical characteristics and outcomes of AS-CA compared with lone AS.<h4>Methods</h4>Patients who were referred for TAVR at 3 international sites underwent blinded research core laboratory <sup>99m ...[more]

PMID: 33181246

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Project description:AimsCardiac amyloidosis is common in elderly patients with aortic stenosis (AS) referred for transcatheter aortic valve implantation (TAVI). We hypothesized that patients with dual aortic stenosis and cardiac amyloid pathology (AS-amyloid) would have different baseline characteristics, periprocedural and mortality outcomes.Methods and resultsPatients aged ≥75 with severe AS referred for TAVI at two sites underwent blinded bone scintigraphy prior to intervention (Perugini Grade 0 negative, 1-3 increasingly positive). Baseline assessment included echocardiography, electrocardiogram (ECG), blood tests, 6-min walk test, and health questionnaire, with periprocedural complications and mortality follow-up. Two hundred patients were recruited (aged 85 ± 5 years, 50% male). AS-amyloid was found in 26 (13%): 8 Grade 1, 18 Grade 2. AS-amyloid patients were older (88 ± 5 vs. 85 ± 5 years, P = 0.001), with reduced quality of life (EQ-5D-5L 50 vs. 65, P = 0.04). Left ventricular wall thickness was higher (14 mm vs. 13 mm, P = 0.02), ECG voltages lower (Sokolow-Lyon 1.9 ± 0.7 vs. 2.5 ± 0.9 mV, P = 0.03) with lower voltage/mass ratio (0.017 vs. 0.025 mV/g/m2, P = 0.03). High-sensitivity troponin T and N-terminal pro-brain natriuretic peptide were higher (41 vs. 21 ng/L, P < 0.001; 3702 vs. 1254 ng/L, P = 0.001). Gender, comorbidities, 6-min walk distance, AS severity, prevalence of disproportionate hypertrophy, and post-TAVI complication rates (38% vs. 35%, P = 0.82) were the same. At a median follow-up of 19 (10-27) months, there was no mortality difference (P = 0.71). Transcatheter aortic valve implantation significantly improved outcome in the overall population (P < 0.001) and in those with AS-amyloid (P = 0.03).ConclusionsAS-amyloid is common and differs from lone AS. Transcatheter aortic valve implantation significantly improved outcome in AS-amyloid, while periprocedural complications and mortality were similar to lone AS, suggesting that TAVI should not be denied to patients with AS-amyloid.

Project description:Importance:Severe aortic stenosis causes pressure overload of the left ventricle, resulting in progressive cardiac dysfunction that can extend beyond the left ventricle. A staging system for aortic stenosis has been recently proposed that quantifies the extent of structural and functional cardiac changes in aortic stenosis. Objectives:To confirm the reproducibility of a proposed staging system and expand the study findings by performing a survival analysis and to evaluate the association of aortic stenosis staging with both cardiac and noncardiac post-transcatheter aortic valve replacement (TAVR) readmissions. Design, Setting, and Participants:A cohort analysis was conducted involving patients with severe aortic stenosis who underwent TAVR at the University of Pittsburgh Medical Center between July 1, 2011, and January 31, 2017. Patients who had undergone TAVR for valve-in-valve procedures and had an incomplete or unavailable baseline echocardiogram study for review were excluded. Clinical, laboratorial, and procedural data were collected from the Society of Thoracic Surgeons database and augmented by electronic medical record review. Exposures:The aortic stenosis staging system is based on echocardiographic markers of abnormal cardiac function. The stages are as follows: stage 1 (left ventricle changes - increased left ventricular mass index; early mitral inflow to early diastolic mitral annulus velocity (E/e') >14; and left ventricular ejection fraction <50%), stage 2 (left atrial or mitral changes - left atrial volume index >34 mL/m2; moderate to severe mitral regurgitation; and atrial fibrillation), stage 3 (pulmonary artery or tricuspid changes - pulmonary artery systolic pressure ≥60 mm Hg; moderate to severe tricuspid regurgitation), and stage 4 (right ventricle changes - moderate to severe right ventricle dysfunction). Main Outcomes and Measures:Primary outcome was post-TAVR all-cause mortality. Secondary outcomes were composite outcomes of all-cause mortality and post-TAVR all-cause and cardiac-cause readmissions. Results:A total of 689 consecutive patients (351 [50.9%] were male, with a mean [SD] age of 82.4 [7.6] years) were included. The prevalence of stage 1 was 13%; stage 2, 62%; stage 3, 21%; and stage 4, 4%. Patients with higher staging had a greater burden of comorbidities as captured by the Society of Thoracic Surgeons Predicted Risk of Mortality (STS-PROM). Despite adjustment for STS-PROM, a graded association was found between aortic stenosis staging and all-cause mortality (hazard ratio [HR] stage 2 vs stage 1: 1.37 [95% CI, 0.81-2.31; P = .25]; stage 3 vs stage 1: 2.24 [95% CI, 1.28-3.92; P = .005]; and stage 4 vs stage 1: 2.83 [95% CI, 1.39-5.76; P = .004]). Stage 3 patients had higher post-TAVR readmission rates for both cardiac (HR, 1.84; 95% CI, 1.13-3.00; P = .01) and noncardiac causes. Conclusions and Relevance:Aortic stenosis staging appears to show a strong graded association between the extent of cardiac changes and post-TAVR all-cause mortality; such staging may improve patient care, risk stratification, assessment of prognosis, and shared decision making for patients undergoing TAVR.

Project description:OBJECTIVES:Cardiac magnetic resonance (CMR) was used to investigate the extracellular compartment and myocardial fibrosis in patients with aortic stenosis, as well as their association with other measures of left ventricular decompensation and mortality. BACKGROUND:Progressive myocardial fibrosis drives the transition from hypertrophy to heart failure in aortic stenosis. Diffuse fibrosis is associated with extracellular volume expansion that is detectable by T1 mapping, whereas late gadolinium enhancement (LGE) detects replacement fibrosis. METHODS:In a prospective observational cohort study, 203 subjects (166 with aortic stenosis [69 years; 69% male]; 37 healthy volunteers [68 years; 65% male]) underwent comprehensive phenotypic characterization with clinical imaging and biomarker evaluation. On CMR, we quantified the total extracellular volume of the myocardium indexed to body surface area (iECV). The iECV upper limit of normal from the control group (22.5 ml/m2) was used to define extracellular compartment expansion. Areas of replacement mid-wall LGE were also identified. All-cause mortality was determined during 2.9 ± 0.8 years of follow up. RESULTS:iECV demonstrated a good correlation with diffuse histological fibrosis on myocardial biopsies (r = 0.87; p < 0.001; n = 11) and was increased in patients with aortic stenosis (23.6 ± 7.2 ml/m2 vs. 16.1 ± 3.2 ml/m2 in control subjects; p < 0.001). iECV was used together with LGE to categorize patients with normal myocardium (iECV <22.5 ml/m2; 51% of patients), extracellular expansion (iECV ?22.5 ml/m2; 22%), and replacement fibrosis (presence of mid-wall LGE, 27%). There was evidence of increasing hypertrophy, myocardial injury, diastolic dysfunction, and longitudinal systolic dysfunction consistent with progressive left ventricular decompensation (all p < 0.05) across these groups. Moreover, this categorization was of prognostic value with stepwise increases in unadjusted all-cause mortality (8 deaths/1,000 patient-years vs. 36 deaths/1,000 patient-years vs. 71 deaths/1,000 patient-years, respectively; p = 0.009). CONCLUSIONS:CMR detects ventricular decompensation in aortic stenosis through the identification of myocardial extracellular expansion and replacement fibrosis. This holds major promise in tracking myocardial health in valve disease and for optimizing the timing of valve replacement. (The Role of Myocardial Fibrosis in Patients With Aortic Stenosis; NCT01755936).

Project description:Background The prevalence and outcomes of the different subtypes of severe low-gradient aortic stenosis (AS) in routine clinical cardiology practice have not been well characterized. Methods and Results Data were derived from the National Echocardiography Database of Australia. Of 192 060 adults (aged 62.8±17.8 [mean±SD] years) with native aortic valve profiling between 2000 and 2019, 12 013 (6.3%) had severe AS. Of these, 5601 patients (47%) had high-gradient and 6412 patients (53%) had low-gradient severe AS. The stroke volume index was documented in 2741 (42.7%) patients with low gradient; 1750 patients (64%) with low flow, low gradient (LFLG); and 991 patients with normal flow, low gradient. Of the patients with LFLG, 1570 (89.7%) had left ventricular ejection fraction recorded; 959 (61%) had paradoxical LFLG (preserved left ventricular ejection fraction), and 611 (39%) had classical LFLG (reduced left ventricular ejection fraction). All-cause and cardiovascular-related mortality were assessed in the 8162 patients with classifiable severe AS subtype during a mean±SD follow-up of 88±45 months. Actual 1-year and 5-year all-cause mortality rates varied across these groups and were 15.8% and 49.2% among patients with high-gradient severe AS, 11.6% and 53.6% in patients with normal-flow, low-gradient severe AS, 16.9% and 58.8% in patients with paradoxical LFLG severe AS, and 30.5% and 72.9% in patients with classical LFLG severe AS. Compared with patients with high-gradient severe AS, the 5-year age-adjusted and sex-adjusted mortality risk hazard ratios were 0.94 (95% CI, 0.85-1.03) in patients with normal-flow, low-gradient severe AS; 1.01 (95% CI, 0.92-1.12) in patients with paradoxical LFLG severe AS; and 1.65 (95% CI, 1.48-1.84) in patients with classical LFLG severe AS. Conclusions Approximately half of those patients with echocardiographic features of severe AS in routine clinical practice have low-gradient hemodynamics, which is associated with long-term mortality comparable with or worse than high-gradient severe AS. The poorest survival was associated with classical LFLG severe AS.

Dataset Information

Prevalence and Outcomes of Concomitant Aortic Stenosis and Cardiac Amyloidosis.

Background

Objectives

Methods

Results

Conclusions

Publications

Prevalence and Outcomes of Concomitant Aortic Stenosis and Cardiac Amyloidosis.

Similar Datasets

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