Abstract 15607: Right Atrial Reservoir Strain Predicts Survival in Patients With Cardiac Amyloidosis

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Peter Huntjens ◽  
Kathleen Zhang ◽  
Yuko Soyama ◽  
Maria Karmpalioti ◽  
Daniel Lenihan ◽  
...  

Introduction: Myofibril deposition in amyloidosis diffusely may affect cardiac structure and function. Right ventricular involvement has been associated with adverse clinical outcome. However, the utility of right atrial (RA) function assessment by echocardiographic strain imaging is unclear. Hypothesis: We hypothesize that right atrial stain has prognostic value in cardiac amyloidosis. Methods: We studied 121 consecutive patients with cardiac amyloidosis: 18% had transthyretin and 79% had light chain amyloidosis. Cardiac amyloidosis was either confirmed by endocardial biopsy (36%) or by a combination of non-cardiac tissue biopsy and proof of left ventricular hypertrophy (64%). Speckle tracking peak RA reservoir strain was assessed based on 6 segments from the apical 4-chamber view. All-cause mortality was tracked over a median of 5 years. Results: Echocardiographic peak longitudinal RA strain was feasible in 109 patients (90%). 60 CA patients died during follow-up period. Peak longitudinal RA strain was reduced in cardiac amyloidosis non-survivors (8.1%) in comparison to survivors (18.3%, p<0.01), showing RA involvement in cardiac amyloidosis. Peak RA strain was significantly associated with survival (using median 12.5%) (p<0.001). Low peak longitudinal RA strain was associated with a 3.3-fold increase in mortality risk (95% confidence interval: 1.83 - 5.96). Conclusions: Reduced peak longitudinal RA strain was significantly associated with survival in patients with cardiac amyloidosis. RA reservoir function assessed by strain appears to be useful as a new means to predict prognosis in cardiac amyloidosis patients and has promise for clinical application.

Medicina ◽  
2021 ◽  
Vol 57 (7) ◽  
pp. 660
Author(s):  
Csilla-Andrea Eötvös ◽  
Roxana-Daiana Lazar ◽  
Iulia-Georgiana Zehan ◽  
Erna-Brigitta Lévay-Hail ◽  
Giorgia Pastiu ◽  
...  

Among the different types, immunoglobulin light chain (AL) cardiac amyloidosis is associated with the highest morbidity and mortality. The outcome, however, is significantly better when an early diagnosis is made and treatment initiated promptly. We present a case of cardiac amyloidosis with left ventricular hypertrophy criteria on the electrocardiogram. After 9 months of follow-up, the patient developed low voltage in the limb leads, while still maintaining the Cornell criteria for left ventricular hypertrophy as well. The relative apical sparing by the disease process, as well as decreased cancellation of the opposing left ventricular walls could be responsible for this phenomenon. The discordance between the voltage in the frontal leads and precordial leads, when present in conjunction with other findings, may be helpful in raising the clinical suspicion of cardiac amyloidosis.


Circulation ◽  
2008 ◽  
Vol 118 (suppl_18) ◽  
Author(s):  
Tudor Vagaonescu ◽  
Alan C Wilson ◽  
John B Kostis

Background: To assess if diuretic-based antihypertensive treatment improves long term fatal (cardiovascular) outcomes in the elderly with isolated systolic hypertension (ISH) and ECG documented left ventricular hypertrophy (LVH). Methods: Retrospective analysis of the SHEP database of 4,736 patients age ≥60 years and ISH and subsequent vital status ascertainment by matching to the National Death Index. Results: 348 subjects (7.35%) of SHEP participants had ECG documented LVH at baseline. Subjects with LVH had at baseline: higher SBP and pulse pressure (p<0.0001), carotid bruits (13% vs. 7%, p<0.0001) and previous history of myocardial infarction (8% vs. 4%, p=.0008) when compared with participants without LVH. There were no significant differences with regard to age, sex, heart rate, body mass index, smoking and alcohol use, previous history of stroke, diabetes, angina, and assignment to treatment or placebo group. Over 14.3 years (mean) of follow up subjects with baseline LVH experienced significantly more all cause mortality (51% vs. 40%, p<0.0001) and cardiovascular death (24% vs. 19%, p=0.002) than participants without baseline LVH. In the group of participants with LVH at baseline active treatment of hypertension did not decrease all cause mortality (51% vs. 50%, NS) or cardiovascular death (26% vs. 24%, NS). There was no statistically significant interaction between LVH and the assignment to treatment (antihypertensive medication vs. placebo). In a multivariable analysis, the adjusted Cox hazard ratio of developing any fatal outcome in the LVH group was 1.181 (95% CI 1.005–1.387, p=0.043) after adjusting for age, sex, race, history of myocardial infarction, diabetes, alcohol smoking status, education, blood pressure, and assignment to treatment or to placebo group. Conclusion: In the elderly with ISH the presence of LVH documented by ECG increased the risk for long term fatal outcomes despite treatment with diuretic-based antihypertensive therapy. Although active treatment lowered risk in the SHEP study, treated participants with LVH had a higher risk for fatal outcomes than treated subjects without LVH.


Author(s):  
Tor Skibsted Clemmensen ◽  
Hans Eiskjær ◽  
Bertil Ladefoged ◽  
Fabian Mikkelsen ◽  
Jens Sørensen ◽  
...  

Abstract Aims Left ventricular (LV) myocardial work index (LVMWI) derived from pressure–strain analysis resembles a novel non-invasive method for LV function evaluation. LV global longitudinal strain (LVGLS) has proven beneficial for risk stratification in cardiac amyloidosis (CA) patients. This study aimed to evaluate the potential additive value of LVMWI for outcome prediction in CA patients. Methods and results We enrolled 100 CA patients in the period 2014–19 from Aarhus University Hospital, Denmark and Uppsala University Hospital, Sweden. All patients underwent comprehensive echocardiographic evaluation and were prospectively followed until censuring date on 31 March 2019 or death. During follow-up, we registered major adverse cardiac events (MACE) comprising heart failure requiring hospitalization and all-cause mortality. The median follow-up was 490 (228–895) days. During follow-up, a total of 42% of patients experienced MACE and 29% died. Patients with LVMWI &lt;1043 mmHg% had higher MACE risk than patients with LVMWI &gt;1043 mmHg% [hazard ratio (HR) 2.3, 95% confidence interval (CI) 1.2–4.3; P = 0.01]. Furthermore, patients with LVMWI &lt;1039 mmHg% also had higher all-cause mortality risk than patients with LVMWI &gt;1039 mmHg% (HR 2.6, 95% CI 1.2–5.5; P &lt; 0.05). Moreover, the apical-to-basal segmental work ratio was a significant MACE and all-cause mortality predictor. By combining LVMWI and apical-to-basal segmental work ratio, we obtained an independent model for all-cause mortality prediction (high vs. low risk: HR 6.4, 95% CI 2.4–17.1; P &lt; 0.0001). In contrast, LVGLS did not predict all-cause mortality. Conclusion LV myocardial work may be of prognostic value in CA patients by predicting both MACE and all-cause mortality.


2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
S Haxha ◽  
U Pedersen-Bjergaard ◽  
J.B Nielsen ◽  
J Pallisgaard ◽  
R.B Devereux ◽  
...  

Abstract Background Cornell voltage criteria (CL) and Sokolow-Lyon criteria (SL) for electrocardiographic left ventricular hypertrophy (ECG-LVH) are well known predictors of cardiovascular outcome. However, their predictive value may differ according to patient type and remains to be further tested in diabetic mellitus (DM) patients. Purpose The present study aims to determine the prevalence of each ECG-LVH criteria and their respective predictive value in DM patients. Method A retrospective cohort study of individuals age &gt;40 years with digital ECGs from primary care were collected during 2001 to 2011. Data on medication, comorbidity, and outcomes were collected from Danish nationwide registries. DM was defined if individuals were prescribed oral antidiabetics or insulin, if they were diagnosed with DM type I or II, or had a HbA1c&gt;48 mmol/l. Cox multivariable analysis was used for estimating hazard ratio (HR) and 95% confidence intervals (95% CI) for all-cause mortality during follow-up of up to 17 years. Results Included were 183,749 individuals with a digital ECG collected in primary care. A total of 13,003 (7.1%) individuals had DM, they were older (65.8 vs. 61.3 years), had more myocardial infarction (16.1% vs. 5.2%), stroke (14.4% vs. 6.2%), hypertension (35.1% vs. 13.2%), CL LVH (8.0% vs. 5.6%) and more were males (53.3% vs. 45.3%) compared to the non-DM individuals (all p&lt;0.001). CL identified a larger percentage of LVH in DM compared to non-DM individuals (8.0% vs. 5.6%, p&lt;0.001), whereas SL identified similar percentage LVH in DM and non-DM individuals (8.5% vs. 8.1%, p=0.068). In multivariable adjusted analysis CL LVH remained strongly associated with all-cause mortality [HR 1.45 (95% CI: 1.42–1.48)] compared to SL LVH which found only a modest association [HR 1.06 (95% CI: 1.03–1.10)] (Figure 1). Of note, the association of CL LVH and all-cause mortality was even stronger than DM per se. There was no interaction with DM and either ECG LVH criteria (p&gt;0.45). Conclusion Cornell Voltage Left Ventricular Hypertrophy is a strong predictor of mortality in patients with and without diabetes and an independent risk factor compared to hypertension and diabetes. The predictive value was substantially stronger than Sokolow-Lyon Voltage criteria for hypertrophy. Figure 1. LVH and all-cause mortality Funding Acknowledgement Type of funding source: None


2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
P Philabout ◽  
L Soulat-Dufour ◽  
I Benhamou-Tarallo ◽  
S Lang ◽  
S Ederhy ◽  
...  

Abstract Background Few studies have assessed the evolution of cardiac chambers deformation imaging in patients with atrial fibrillation (AF) according to cardiac rhythm outcome. Purpose To evaluate cardiac chamber deformation imaging in patients admitted for AF and the evolution at 6-month follow-up (M6). Methods In forty-one consecutive patients hospitalised for AF two-dimensional transthoracic echocardiography was performed at admission (M0) and after six months (M6) of follow up. In addition to the usual parameters of chamber size and function, chamber deformation imaging was obtained including global left atrium (LA) and right atrium (RA) reservoir strain, global left ventricular (LV) and right ventricular (RV) free wall longitudinal strain. Patients were divided into three groups according to their cardiac rhythm at M0 and M6: AF at M0 and sinus rhythm (SR) at M6 (AF-SR) (n=23), AF at M0 and AF at M6 (AF-AF) (n=11), SR at M0 (spontaneous conversion before the first echocardiography exam) and SR in M6 (SR-SR) (n=7) Results In comparison with SR patients (n=7), at M0, AF patients (n=34)) had lower global LA reservoir strain (+5.2 (+0.4 to 12.8) versus +33.2 (+27.0 to +51.5)%; p&lt;0.001), lower global RA reservoir strain (+8.6 (−5.4 to 11.6) versus +24.3 (+12.3 to +44.9)%; p&lt;0.001), lower global LV longitudinal strain (respectively −12.8 (−15.2 to −10.4) versus −19.1 (−21.8 to −18.3)%; p&lt;0.001) and lower global RV longitudinal strain (respectively −14.2 (−17.3 to −10.7) versus −23.8 (−31.1 to −16.2)%; p=0.001). When compared with the AF-SR group at M0 the AF-AF group had no significant differences with regard to global LA and RA reservoir strain, global LV and RV longitudinal strain (Table). Between M0 and M6 there was a significant improvement in global longitudinal strain of the four chambers in the AF-SR group whereas no improvements were noted in the AF-AF and SR-SR group (Figure). Conclusion Initial atrial and ventricular deformations were not associated with rhythm outcome at six-month follow up in AF. The improvement in strain in all four chambers strain suggests global reverse remodelling all cardiac cavities with the restoration of sinus rhythm. Evolution of strain between M0 and M6 Funding Acknowledgement Type of funding source: None


2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Pankaj Garg ◽  
Hosamadin Assadi ◽  
Rachel Jones ◽  
Wei Bin Chan ◽  
Peter Metherall ◽  
...  

AbstractCardiac magnetic resonance (CMR) is emerging as an important tool in the assessment of heart failure with preserved ejection fraction (HFpEF). This study sought to investigate the prognostic value of multiparametric CMR, including left and right heart volumetric assessment, native T1-mapping and LGE in HFpEF. In this retrospective study, we identified patients with HFpEF who have undergone CMR. CMR protocol included: cines, native T1-mapping and late gadolinium enhancement (LGE). The mean follow-up period was 3.2 ± 2.4 years. We identified 86 patients with HFpEF who had CMR. Of the 86 patients (85% hypertensive; 61% males; 14% cardiac amyloidosis), 27 (31%) patients died during the follow up period. From all the CMR metrics, LV mass (area under curve [AUC] 0.66, SE 0.07, 95% CI 0.54–0.76, p = 0.02), LGE fibrosis (AUC 0.59, SE 0.15, 95% CI 0.41–0.75, p = 0.03) and native T1-values (AUC 0.76, SE 0.09, 95% CI 0.58–0.88, p < 0.01) were the strongest predictors of all-cause mortality. The optimum thresholds for these were: LV mass > 133.24 g (hazard ratio [HR] 1.58, 95% CI 1.1–2.2, p < 0.01); LGE-fibrosis > 34.86% (HR 1.77, 95% CI 1.1–2.8, p = 0.01) and native T1 > 1056.42 ms (HR 2.36, 95% CI 0.9–6.4, p = 0.07). In multivariate cox regression, CMR score model comprising these three variables independently predicted mortality in HFpEF when compared to NTproBNP (HR 4 vs HR 1.65). In non-amyloid HFpEF cases, only native T1 > 1056.42 ms demonstrated higher mortality (AUC 0.833, p < 0.01). In patients with HFpEF, multiparametric CMR aids prognostication. Our results show that left ventricular fibrosis and hypertrophy quantified by CMR are associated with all-cause mortality in patients with HFpEF.


BMJ Open ◽  
2017 ◽  
Vol 7 (12) ◽  
pp. e018719 ◽  
Author(s):  
Nuria Farré ◽  
Josep Lupon ◽  
Eulàlia Roig ◽  
Jose Gonzalez-Costello ◽  
Joan Vila ◽  
...  

ObjectivesThe aim of this study was to analyse baseline characteristics and outcome of patients with heart failure and mid-range left ventricular ejection fraction (HFmrEF, left ventricular ejection fraction (LVEF) 40%–49%) and the effect of 1-year change in LVEF in this group.SettingMulticentre prospective observational study of ambulatory patients with HF followed up at four university hospitals with dedicated HF units.ParticipantsFourteen per cent (n=504) of the 3580 patients included had HFmrEF.InterventionsBaseline characteristics, 1-year LVEF and outcomes were collected. All-cause death, HF hospitalisation and the composite end-point were the primary outcomes.ResultsMedian follow-up was 3.66 (1.69–6.04) years. All-cause death, HF hospitalisation and the composite end-point were 47%, 35% and 59%, respectively. Outcomes were worse in HF with preserved ejection fraction (HFpEF) (LVEF>50%), without differences between HF with reduced ejection fraction (HFrEF) (LVEF<40%) and HFmrEF (all-cause mortality 52.6% vs 45.8% and 43.8%, respectively, P=0.001). After multivariable Cox regression analyses, no differences in all-cause death and the composite end-point were seen between the three groups. HF hospitalisation and cardiovascular death were not statistically different between patients with HFmrEF and HFrEF. At 1-year follow-up, 62% of patients with HFmrEF had LVEF measured: 24% had LVEF<40%, 43% maintained LVEF 40%–49% and 33% had LVEF>50%. While change in LVEF as continuous variable was not associated with better outcomes, those patients who evolved from HFmrEF to HFpEF did have a better outcome. Those who remained in the HFmrEF and HFrEF groups had higher all-cause mortality after adjustment for age, sex and baseline LVEF (HR 1.96 (95% CI 1.08 to 3.54, P=0.027) and HR 2.01 (95% CI 1.04 to 3.86, P=0.037), respectively).ConclusionsPatients with HFmrEF have a clinical profile in-between HFpEF and HFrEF, without differences in all-cause mortality and the composite end-point between the three groups. At 1 year, patients with HFmrEF exhibited the greatest variability in LVEF and this change was associated with survival.


2021 ◽  
Vol 129 (Suppl_1) ◽  
Author(s):  
Cristine J Reitz ◽  
Marjan Tavassoli ◽  
Da Hye Kim ◽  
Sina Hadipour-Lakmehsari ◽  
Saumya Shah ◽  
...  

Dilated cardiomyopathy (DCM) is one of the most common causes of heart failure, yet the majority of the underlying signaling mechanisms remain poorly characterized. Protein phosphorylation is a key regulatory element with profound effects on the activity and function of signaling networks; however, there is a lack of comprehensive phosphoproteomic studies in human DCM patients. We assessed the hypothesis that an integrative phosphoproteomics analysis of human DCM would reveal novel phosphoprotein candidates involved in disease pathophysiology. Combined proteomic and phosphoproteomic analysis of explanted left ventricular tissue samples from DCM patients ( n =4) and non-failing controls ( n =4) identified 5,570 unique proteins with 13,624 corresponding phosphorylation sites. From these analyses, we identified αT-catenin as a unique candidate protein with a cluster of 4 significantly hyperphosphorylated sites in DCM hearts ( P <0.0001), with no change in total αT-catenin expression at the protein level. Bioinformatics analyses of human datasets and confocal imaging of human and mouse cardiac tissue show highly cardiac-enriched expression of αT-catenin, localized to the cardiomyocyte intercalated disc. High resolution 3-dimensional reconstruction shows elongated intercalated disc morphology in DCM hearts (10.07±0.76 μm in controls vs. 17.20±1.87 μm in DCM, P <0.05, n =3/group), with significantly increased colocalization of αT-catenin with the intercalated disc membrane protein N-cadherin (Pearson’s coefficient 0.55±0.04 in controls vs. 0.71±0.02 in DCM, P <0.05, n =3/group). To investigate the functional role of cardiac αT-catenin phosphorylation, we overexpressed WT protein vs. non-phosphorylatable forms based on the loci identified in DCM hearts, in adult mouse cardiomyocytes using lentiviral transduction. Confocal imaging revealed significant internalization of the phospho-null form, as compared to the prominent intercalated disc staining of the WT protein (17.78±0.79% of WT vs. 9.25±0.49% of 4A mutant, P <0.0001, n =50 cells/group). Together, these findings suggest a critical role for αT-catenin phosphorylation in maintaining cardiac intercalated disc organization in human DCM.


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