Relative Apical Sparing Pattern Research Articles

Echocardiographic assessment of left ventricular mechanics in individuals with mitral valve prolapse: a systematic review and meta-analysis.

During the last decade, a number of echocardiographic studies have employed speckle tracking echocardiography (STE) for assessing myocardial deformation properties in individuals with mitral valve prolapse (MVP), reporting not univocal results. Accordingly, we performed a systematic review and meta-analysis to summarize the main findings of these studies and to examine the overall influence of MVP on left ventricular (LV) global longitudinal strain (GLS). All echocardiographic studies assessing conventional echoDoppler parameters and myocardial strain indices in MVP individuals vs. controls without MVP, selected from PubMed and EMBASE databases, were included. The risk of bias was assessed by using the National Institutes of Health (NIH) Quality Assessment of Case-Control Studies. Continuous data (LV-GLS) were pooled as a standardized mean difference (SMD) comparing MVP group with healthy controls. The overall SMD of LV-GLS was calculated using the random-effect model. The full-texts of 15 studies with 1088 individuals with MVP and 591 healthy controls were analyzed. Average LV-GLS magnitude was significantly, even though modestly, reduced in MVP individuals in comparison to controls (19.4 ± 3.4% vs. 21.1 ± 2.8%, P < 0.001). The overall effect of MVP on LV-GLS was small-to-medium (SMD - 0.54, 95%CI -0.76,-0.32, P < 0.001). Substantial heterogeneity was detected for the included studies, with an overall I2 statistic value of 75.9% (P < 0.001). Egger's test for a regression intercept gave a P-value of 0.58, indicating no publication bias. On meta-regression analysis, none of the moderators (the age, the percentage of females among MVP individuals, body mass index, heart rate and systolic blood pressure of MVP individuals, the degree of mitral regurgitation, the type of ultrasound machine employed for strain echocardiographic imaging and finally the beta blocker treatment) was significantly associated with effect modification (all P < 0.05). Regional strain analysis, performed by two-third of the studies, highlighted a more enhanced reduction in myocardial strain parameters at level of the LV basal infero-lateral segments in all directions (longitudinal, circumferential and radial), with apical sparing. The longitudinal strain impairment detected in MVP individuals is more regional than global, with peculiar involvement of the LV basal infero-lateral segments and relative apical sparing pattern.

Prevalence and significance of relative apical sparing in aortic stenosis: insights from an echo and cardiovascular magnetic resonance study of patients referred for surgical aortic valve replacement.

This study aims to assess the prevalence of relative apical sparing pattern (RASP) in patients with severe symptomatic aortic stenosis (AS), referred for surgical aortic valve replacement (AVR), to evaluate its significance, possible relation to amyloid deposition, and persistence after surgery. Prospective study of 150 consecutive patients [age 73 (interquartile range: 68-77), 51% women], with severe symptomatic AS referred to surgical AVR. All patients underwent cardiac magnetic resonance (CMR) before surgery. RASP was defined by [average apical longitudinal strain (LS)/(average basal LS + average mid LS)] > 1 by echocardiography. AVR was performed in 119 (79.3%) patients. Both Congo red and sodium sulphate-Alcian blue (SAB) stain were used to exclude amyloid on septal myocardial biopsy. LV remodelling and tissue characterization parameters were compared in patients with and without RASP. Deformation pattern was re-assessed at 3-6 months after AVR.RASP was present in 23 patients (15.3%). There was no suspicion of amyloid at pre-operative CMR [native T1 value 1053 ms (1025-1076 ms); extracellular volume (ECV) 28% (25-30%)]. None of the patients had amyloid deposition at histopathology. Patients with RASP had significantly higher pre-operative LV mass and increased septal wall thickness. They also had higher N-terminal pro b-type natriuretic peptide (NT-proBNP) levels [1564 (766-3318) vs. 548 (221-1440) pg/mL, P = 0.010], lower LV ejection fraction (53.7 ± 10.5 vs. 60.5 ± 10.2%, P = 0.005), and higher absolute late gadolinium enhancement (LGE) mass [9.7 (5.4-14.1) vs. 4.8 (1.9-8.6) g, P = 0.016] at CMR. Follow-up evaluation after AVR revealed RASP disappearance in all except two of the patients. RASP is not specific of cardiac amyloidosis. It may also be found in severe symptomatic AS without amyloidosis, reflecting advanced LV disease, being mostly reversible after surgery.

Abstract 15768: Relative Longitudinal Strain Apical Sparing Pattern as a Detrimental Factor of Cardiovascular Outcome After Transcatheter Aortic Valve Replacement

Introduction: Several risk scores have been developed for outcome after heart surgery. However, risk factors associated with clinical outcome in patients undergoing transcatheter aortic valve replacement (TAVR) are still not well defined. This study aimed to determine laboratory and echocardiography determinants of short- and long-term outcome after TAVR. Methods: This retrospective study comprised 598 consecutive patients with sole aortic stenoseis (AS) who received balloon-expandable TAVR in our hospital (mean age 81.7±5.7 years, 48.7% male, median EuroSCORE II 5.0%). Pre-operative laboratory and imaging data including speckle-tracking echocardiography were analyzed. Clinical outcomes included cardiovascular (CV) death, CV hospitalization, and procedure related complications at 30 days, 1 year, and at 2 years after TAVR. Results: Semi-quantitative assessment defined as ≥3 LV wall segments with the apico-basal LS ratio &gt;3.0 was used to define relative apical sparing pattern (RASP). The prevalence of RASP was 19.2%. RASP patients had higher prevalence of acute kidney injury (AKI, 10.4% vs. 3.3%, P=0.001) and new-onset left bundle branch block (LBBB) after TAVR (17.4% vs. 6.8%, P&lt;0.001). CV mortality at 30 days (7.0% vs. 3.1%, P=0.062), 1 year (15.7% vs. 7.9%, P=0.010), and 2 years (22.6% vs. 9.9%, P&lt;0.001) was also higher after TAVR. RASP remained the strongest determinant for AKI (OR=3.11, P=0.007), new-onset LBBB (OR=2.89, P&lt;0.001), increased 30-day CV related hospitalization or death risk (OR=2.20, P=0.033). Lower albumin level and RASP were identified as independent determinants of increased 1-year and 2-year CV mortality risk (RASP: HR=1.83 and 2.18, P=0.039 and 0.002; albumin&lt;4.0g/dl: HR=3.18 and 2.55, both P&lt;0.001), regardless of TAVR approach and EuroSCORE II. After adjustment, the addition of RASP significantly improved prognostic power of a model including clinical variables and low albumin level for predicting CV death at 2-year follow-up ( χ 2 from 28.18 to 40.70, P=0.001). Conclusions: RASP represents a novel clinically relevant determinant of outcome after TAVR. Low albumin and RASP were identified as the strongest laboratory and echocardiography determinants for increased short- and long-term outcome after TAVR.

Exercise-induced myocardial edema in master triathletes: Insights from cardiovascular magnetic resonance imaging

BackgroundStrenuous exercise has been associated with functional and structural cardiac changes due to local and systemic inflammatory responses, reflecting oxidative, metabolic, hormonal, and thermal stress, even in healthy individuals. We aimed to assess changes in myocardial structure and function using cardiovascular magnetic resonance (CMR) imaging in master triathletes early after a full-distance Ironman Triathlon race.Materials and methodsTen master triathletes (age 45 ± 8 years) underwent CMR within 3 h after a full-distance Ironman Triathlon race (3.8 km swimming, 180 km cycling, and 42.2 km running) completed with a mean time of 12 ± 1 h. All the triathletes had a 30-day follow-up CMR. Cine balanced steady-state free precession, T2-short tau inversion recovery (STIR), tagging, and late gadolinium enhancement (LGE) imaging sequences were performed on a 1.5-T MR scanner. Myocardial edema was defined as a region with increased T2 signal intensity (SI) of at least two SDs above the mean of the normal myocardium. The extent of myocardial edema was expressed as the percentage of left ventricular (LV) mass. Analysis of LV strain and torsion by tissue tagging included the assessment of radial, longitudinal, and circumferential peak systolic strain, rotation, and twist.ResultsCompared with postrace, biventricular volumes, ejection fraction, and LV mass index remained unchanged at 30-day follow-up. Global T2 SI was significantly higher in the postrace CMR (postrace 10.5 ± 6% vs. follow-up 3.9 ± 3.8%, P = 0.004) and presented with a relative apical sparing distribution (P < 0.001) matched by reduction of radial peak systolic strain of basal segments (P = 0.003). Apical rotation and twist were significantly higher immediately after the competition compared with follow-up (P < 0.05).ConclusionStrenuous exercise in master triathletes is associated with a reversible regional increase in myocardial edema and reduction of radial peak systolic strain, both presenting with a relative apical sparing pattern.

The relative apical sparing pattern is common in elderly patients with type 2 diabetes mellitus

Abstract Background Type 2 diabetes mellitus (T2DM) impairs cardiac function and is one of the risk factors for heart failure. However, the mechanism of cardiac impairment is not elucidated. Despa et al. reported that amylin has aggregation properties similar to amyloidogenic proteins and impairs cardiac function in T2DM patients with hyperamylinemia. In this hypothesis, T2DM-induced myocardial impairment is thought of as amylin-induced cardiac amyloidosis. The purpose of this study is to clarify whether a relative apical sparing pattern (RASP), which is well known as a typical pattern of cardiac amyloidosis, is common in T2DM. Methods We studied patients aged 75 years or over who underwent echocardiography from January 2018 to December 2020 in our clinic. We calculated the quantitative relative apical sparing (qRASP) as average apical-longitudinal strain (LS)/(average basal-LS + average mid-LS) in each patient. According to the validated threshold, a qRASP ≥1.0 was defined as an obvious RASP (oRASP). We compared the ratio of oRASP between patients with and without T2DM. Results We researched 506 patients, mean 81.8 years, 290 females, 133 with T2DM. The average age, atrial fibrillation rate, heart failure rate, and hypertension rate were similar in both groups. The ratio of ischemic heart disease was higher in the T2DM group. Echocardiography showed that the left atrial dimension (LAD), E/e', and left ventricular hypertrophy (LVH) ratio was higher in the T2DM group. Speckle tracking echocardiography revealed that global longitudinal strain (GLS) and qRASP of the T2DM group were higher than the non-DM group (GLS;-18.0% vs. −19.2%, p&amp;lt;0.001. qRASP; 0.809 vs. 0.699, P&amp;lt;0.001). Furthermore, the ratio of oRASP was significantly higher in the T2DM group (19.0% vs. 1.34%, p&amp;lt;0.001). Multivariable logistic regression analysis showed T2DM was an independent predictor for oRASP. Conclusion This study revealed that the qRASP and the ratio of oRASP in the T2DM group were higher than non-DM group. This finding supports the hypothesis that T2DM related cardiomyopathy is a kind of cardiac amyloidosis caused by amylin. Funding Acknowledgement Type of funding sources: None.

Exploration of electrocardiographic and echocardiographic findings to screen transthyretin amyloid cardiomyopathy in patients with mild left ventricular hypertrophy

Abstract Background The transthyretin amyloid cardiomyopathy (ATTR-CM), a common condition in the elderly, is a life-threatening disease; however, it is treatable. The early accurate diagnosis is, therefore, an important key to improve the patient's outcomes. Electrocardiography and echocardiography are instant diagnostic tools with red flags for the screening of the ATTR-CM. In fact, ATTR-CM often mimics left ventricular hypertrophy (LVH), and the differential diagnosis is difficult to establish. Thus, the characteristics of cardiac amyloidosis may be obscured in ATTR-CM patients with mild LVH, possibly making the disease difficult to diagnose. Therefore, in this study, the effect of LVH on the occurrence of electrocardiographic and echocardiographic parameters for ATTR-CM was investigated, and their incremental value over the age for the screening of ATTR-CM in the patients with mild LVH was recorded. Methods This study retrospectively studied 319 consecutive nonischemic LVH patients with a sinus rhythm who underwent detailed diagnostic tests. The light chain-associated amyloidosis was an exclusion criterion. The mean left ventricular wall thickness (MWT) &amp;lt;12 mm was defined as mild LVH, while MWT ≥12 mm was defined as moderate to severe LVH. The ATTR-CM was diagnosed with biopsy or 99 mTc-PYP scintigraphy. The elderly were defined as people aged ≥65 years in males and ≥70 years in females, according to the literature. Each electrocardiographic and echocardiographic parameter was binarized with an external cut-off point to increase the external validity, being the incremental benefit of each parameter over age for identifying ATTR-CM assessed using a receiver operating characteristic curve analysis and comparisons of the area under the curve (AUC). Results Fourteen patients (8%) among the 170 patients with mild LVH had ATTR-CM, while 27 patients (18%) among the 149 patients with moderate to severe LVH had ATTR-CM. The patients with mild LVH had fewer electrocardiographic and echocardiographic parameters, showing a significant difference between ATTR-CM and non-ATTR-CM patients than those with moderate to severe LVH (Table 1). Among several binarized parameters, the voltage-to-mass ratio, E/A ratio, the global longitudinal strain, and also the relative apical sparing pattern demonstrated additive value for identifying the ATTR-CM over aging (Table 2). In addition, the discriminative ability of the propensity score calculated from these four variables and age was considered excellent for the screening of ATTR-CM (AUC = 0.98). Conclusion The patients with mild LVH appear to have fewer electrocardiographic and echocardiographic specific findings of the ATTR-CM compared to patients with moderate to severe LVH. However, several red flags may be useful for screening ATTR-CM even in patients with a mild LVH. Funding Acknowledgement Type of funding sources: None. Table 1Table 2

Cardiac amyloidosis screening using a relative apical sparing pattern in patients with left ventricular hypertrophy

BackgroundCardiac amyloidosis (CA) mimics left ventricular hypertrophy (LVH). It is treatable, but its prognosis is poor. A simple screening tool for CA would be valuable. CA is more precisely diagnosed with echocardiographic deformation parameters (e.g., relative apical sparing pattern [RASP]) than with conventional parameters. We aimed to 1) investigate incremental benefits of echocardiographic deformation parameters over established parameters for CA screening; 2) determine the resultant risk score for CA screening; and 3) externally validate the score in LVH patients.MethodsWe retrospectively studied 295 consecutive non-ischemic LVH patients who underwent detailed diagnostic tests. CA was diagnosed with biopsy or 99mTc-PYP scintigraphy. The base model comprised age (≥65 years [men], ≥70 years [women]), low voltage on the electrocardiogram, and posterior wall thickness ≥ 14 mm in reference to the literature. The incremental benefit of each binarized echocardiographic parameter over the base model was assessed using receiver operating characteristic curve analysis and comparisons of the area under the curve (AUC).ResultsFifty-four (18%) patients had CA. RASP showed the most incremental benefit for CA screening over the base model. After conducting multiple logistic regression analysis for CA screening using four variables (RASP and base model components), a score was determined (range, 0–4 points). The score demonstrated adequate discrimination ability for CA (AUC = 0.86). This result was confirmed in another validation cohort (178 patients, AUC = 0.88).ConclusionsWe developed a score incorporating RASP for CA screening. This score is potentially useful in the risk stratification and management of LVH patients.

Clinical significance of the relative apical sparing pattern of longitudinal strain in patients with cardiac amyloidosis

Abstract Background The relative apical sparing pattern (RASP) of left ventricular (LV) longitudinal strain (LS) is frequently associated with cardiac amyloidosis (CA). However, some patients with CA do not show the RASP, and their clinical characteristics have not been fully clarified. We sought to investigate the clinical significance of RASP in patients with CA. Methods One hundred consecutive CA patients who were diagnosed by biopsy or myocardial pyrophosphate scintigraphy and evaluated for RASP (mean age: 76 years, male: 77%, LV mean wall thickness: 13.5 mm, light-chain [AL] type: 33 cases, transthyretin [TTR] type: 67 cases) were retrospectively enrolled. The RASP was semi-quantitatively and quantitatively assessed. Semi-quantitative RASP was defined as reduction of LS (≥−10%) in ≥5 (of 6) basal segments relative to preserved LS (&amp;lt;−15%) in ≥1 apical segment. Quantitative RASP was calculated according to the following formula: Quantitative RASP = [Average apical LS] / [Average basal LS + Average mid LS]. We adapted three validated thresholds (&amp;gt;1.00, &amp;gt;0.90, and &amp;gt;0.87) according to the literature. Results Semi-quantitative and binalized quantitative RASP (&amp;gt;1.00, &amp;gt;0.90, and &amp;gt;0.87) were observed in 55, 55, 63, and 65 patients, respectively. RASP in each definition was more prevalent in the TTR group than in the AL group. Additionally, RASP was significantly associated with higher LV wall thickness even after adjustment for the CA subtypes (all, p&amp;lt;0.05, Figure). After the RASP assessment, 35 all-cause deaths and 26 cardiac deaths were observed during the follow-up period (median, 1.1 years). Although these events were significantly associated with poor nutrition, lower blood pressure, higher New York Heart Association class, and the AL group, no association was found with RASP and LV wall thickness. Conclusions The incidence of RASP is low in the case of thin LV wall thickness in CA patients, which may indicate the difficulty of early diagnosis of CA using RASP in patients with mild LV hypertrophy. The prognostic prediction using RASP may be challenging in this cohort. Figure 1 Funding Acknowledgement Type of funding source: None

Incremental benefits of echocardiographic indices over clinical parameters for screening cardiac amyloidosis in patients with left ventricular hypertrophy

Abstract Background Cardiac amyloidosis (CA), characterized by amyloid protein deposition in the heart, is a treatable disease. Although left ventricular (LV) wall thickness is the most established imaging predictor for CA, several echocardiographic indices including deformation parameters also contribute to the screening of CA. However, it is unclear whether additive values of echocardiographic indices have greater benefit over the conventional clinical predictors for the screening of CA. Therefore, we sought to compare the incremental benefits of echocardiographic indices over the clinical parameters for the screening of CA and externally validate their incremental benefits. Methods We retrospectively studied 295 consecutive patients (median age, 67 years; male, 65%; mean LV wall thickness (MWT), 12 mm) with LV hypertrophy who underwent echocardiography as well as the detailed work-up for myocardium (Biopsy, technetium pyrophosphate scintigraphy (99mTc-PYP) or cardiac magnetic resonance imaging). CA was diagnosed through biopsy or 99mTc-PYP. The clinical model considers patients' age and the low-voltage in electrocardiography in reference to previous studies. Continuous echocardiographic variables were represented in binary through generally accepted external cutoff points. The incremental benefits of the echocardiography findings over the clinical model were assessed using with the help of both receiver-operated characteristic curve analysis and comparison of area under the curves. Furthermore, these incremental benefits were validated in the external validation sample (median age, 70 years; male, 69%; MWT, 12 mm). Results Among the enrolled patients, CA was observed in 18% of cases. Table presents the results of this study. Of the echocardiographic parameters, relative apical sparing pattern (RASP) was the greatest contributor for improvement of diagnostic accuracy of the clinical model. The next greatest contributor was LV wall thickness, followed by left atrial reservoir strain (LAS), E/e', left atrial volume index, ejection fraction strain ratio, and pericardial effusion, respectively. Similarly, RASP, LV wall thickness, global longitudinal strain, ejection fraction, LAS, and granular sparkling showed significant incremental benefit in the validation cohort. Only mean wall thickness, LV wall thickness, LAS, E/e' and RASP consistently improved the diagnostic accuracy of the clinical model. Conclusion During the screening process, adding LV wall thickness, LAS, and RASP to the clinical parameters may be useful for the accurate diagnosis of CA in patients with LV hypertrophy. Figure 1 Funding Acknowledgement Type of funding source: None

Utility of scoring system including relative apical sparing pattern for screening cardiac amyloidosis in patients with left ventricular hypertrophy

Abstract Background Cardiac amyloidosis (CA) is an infiltrative disease mimicking left ventricular hypertrophy (LVH), although its prognosis is poorer than other diseases with LVH. Moreover, because CA is treatable, appropriate screening for CA is an important area of study for clinicians to prevent and treat the disease. Several imaging predictors of CA have been reported so far;. in particular, deformation parameters such as relative apical sparing patterns of longitudinal strain (RASP) may diagnose CA with better precision than conventional parameters. Accordingly, we hypothesized that the inclusion of deformation parameters into the established diagnostic parameters would permit derivation of a risk score for CA screening in patients with LVH. Thus, we aimed to 1) investigate the incremental benefits of deformation parameters over established diagnostic parameters for CA screening in patients with LVH; 2) determine the risk score to screen CA patients with LVH using all of these variables; and 3) externally validate the score. Methods We retrospectively studied 295 consecutive non-ischemic patients with LVH who underwent echocardiography as well as the detailed work-up for LVH (biopsy, technetium pyrophosphate scintigraphy (99mTc-PYP) or cardiac magnetic resonance imaging) (median age, 67 years; MWT, 12 mm). CA was diagnosed by biopsy or 99mTc-PYP. The base model consisted of age (≥65 [male], ≥70 [female]), low voltage in electrocardiography, and posterior wall thickness ≥14 mm in reference to previous studies. Continuous echocardiographic variables were binarized by the use of generally accepted external cutoff points to avoid best clinical scenario. Incremental benefits were assessed using receiver operating characteristic curve analysis and area under the curve (AUC) comparison. Multiple logistic regression analysis was performed to determine the risk score. The score was then validated in the external validation sample (N=178, median age, 70 years; MWT, 12 mm). Results CA was observed in 54 patients (18%) and of the several echocardiographic parameters studied, only RASP demonstrated a significant incremental benefit for the screening of CA over the base model (Figure A). After multiple logistic regression analysis in the prediction of CA with 4 variables (RASP and basal model components), each was assigned a numeric value based on its relative effect (Figure B). The incidence rate of CA clearly increased as the sum of the risk score increased (Figure C). The score had good discrimination ability, with an AUC of 0.87, a total score of ≥2 with 70% sensitivity and 90% specificity. Similarly, the discrimination ability of the score in the validation cohort was sufficient (AUC = 0.87). Conclusion Overall, we determined a simple risk score including RASP to screen CA. This score takes into account 4 common parameters used in daily practice, and therefore, has potential utility in risk stratification and management of patients with LVH. Figure 1 Funding Acknowledgement Type of funding source: Private hospital(s). Main funding source(s): Kitaishikai

Clinical usefulness of relative apical sparing pattern for predicting functional recovery after transcatheter aortic valve implantation in patients with severe aortic stenosis

Abstract Background Relative apical sparing pattern (RASP) is thought to be associated with prognosis in patients with cardiac amyloidosis or left ventricular hypertrophy (LVH). Although almost all patients with severe aortic stenosis (AS) have LVH, little is known about the effect of transcatheter aortic valve implantation (TAVI) in patients with severe AS exhibiting a RASP. Purpose This study aimed to elucidate the effect of TAVI on left ventricular global longitudinal strain (LS; LVGLS) in patients with severe AS exhibiting a RASP. Methods Eighty-four patients who underwent transfemoral or subclavian TAVI were evaluated. They were divided into the RASP and non-RASP groups. The average apical LS divided by the sum of the average mid and basal LS values of &amp;gt;1.0 was defined as the RASP. We analyzed the difference between pre- and post-TAVI LVGLS (ΔGLS = post-TAVI LVGLS − pre-TAVI LVGLS). Results Of the 84 patients (mean age, 84.5±3.9 years; 24 men), 15 (17.9%) exhibited a RASP. No significant difference in mean pre-TAVI LVGLS was found between the RASP and non-RASP groups (−16.6% ± 3.8% vs. −15.8% ± 3.9%). The ΔGLS in the RASP group was significantly higher than that in the non-RASP group (−0.97% ± 2.5% vs. −2.6% ± 3.0%; P&amp;lt;0.05). Multivariate analysis revealed that relative apical longitudinal strain was an independent predictor of ΔGLS (β = 0.35, p=0.002). Conclusion Relative apical longitudinal strain was associated with LVGLS recovery. The effect of TAVI on LVGLS in patients with a RASP is inferior to that in patients without a RASP. Funding Acknowledgement Type of funding source: None

Semiquantitative assessment of the relative apical sparing pattern of longitudinal strain for cardiac amyloidosis identification.

The relative apical sparing pattern (RASP) of left ventricular (LV) longitudinal strain (LS) is frequently associated with cardiac amyloidosis (CA). However, the visual assessment of RASP is inconsistent, and the quantitative assessment of RASP is time-consuming. This study aimed to compare assessments of RASP for the identification of CA with conventional assessments and investigate their reproducibility and relevance on the assessments. Forty patients with biopsy-proven CA were compared with 80 hypertrophied patients matched for mean LV wall thickness. We compared the discriminative abilities of three assessments of RASP to identify CA (visual, quantitative, and semiquantitative). Nine patterns of semiquantitative RASP were investigated; finally, it was defined as "reduction of LS" (≥ -10%) in ≥5 (of 6) basal segments, relative to "preserved LS" (< -15%) in at least one apical segment. The concordance between the two observers for visual RASP was modest (κ=0.65). On the other hand, the consistency for semiquantitative RASP was perfect (κ=1.00). The discriminative ability of semiquantitative RASP (area under the curve [AUC] =0.74) was significantly better than that of visual RASP (AUC=0.65) and equivalent to that of binary quantitative RASP. Semiquantitative RASP assessment is reproducible and accurately discriminates CA. This simple assessment may help readily refine the risk stratification of patients with diffuse LV hypertrophy.

Prognostic assessment of relative apical sparing pattern of longitudinal strain for severe aortic valve stenosis

BackgroundsThe relative apical sparing pattern (RASP) of left ventricular (LV) longitudinal strain (LS) is frequently associated with cardiac amyloidosis. Elderly patients with aortic valve stenosis (AS) complicated by transthyretin amyloid cardiomyopathy have poor prognosis. Furthermore, deteriorated basal LS in AS patients has been reported to be associated with adverse outcome. We investigated the association between RASP and outcomes in patients with severe AS. MethodsWe retrospectively studied 156 consecutive patients with severe AS and preserved LV ejection fraction. RASP was assessed by both of semi-quantitative (sRASP) and quantitative (qRASP) methods. sRASP was defined as a deterioration of LS (≥-10%) in ≥ 5 (of 6) basal segments, relative to preserved LS (<-15%) in at least 1 apical segment. qRASP was calculated using the following formula: average apical LS/(average basal LS + average mid-ventricle LS); qRASP ≥ 1 was defined as positive. Patients were followed up to determine outcomes, which included sudden cardiac death or unexpected admission due to heart failure, over a median of 1.9 years. ResultssRASP and qRASP were assessed in all patients, but 24 and 42 patients fulfilled the criteria for sRASP and qRASP, respectively. Both assessments were significantly associated with outcomes (n = 44; 28%). Furthermore, sRASP was significantly associated with outcome after adjusting for EuroSCORE, NYHA ≥ II, or global longitudinal strain. A model based on these covariates for predicting outcomes significantly improved by adding sRASP. ConclusionRASP is observed in some patients with severe AS and provides additive prognostic information over conventional parameters.

3078Usefulness of a semi-quantitative and layer-specific assessment of the relative apical sparing pattern of longitudinal strain for the identification of cardiac amyloidosis

Abstract Background The relative apical sparing pattern (RASP) of left ventricular longitudinal strain (LS) is determined using a strain polar map, while global longitudinal strain is measured using speckle-tracking echocardiography, and it is frequently associated with cardiac amyloidosis (CA). However, the definition of visual RASP is ambiguous, and this leads to insufficient reproducibility, whereas quantitative RASP takes time and leads to difficulty in the clinical application. Generally, amyloid predominantly accumulates in the endo-myocardial layer. As such, layer-specific analysis of RASP may more accurately identify CA. Therefore, the aims of this study were to explore the reproducible and easy definition of RASP for identifying CA and investigate the effect of layer-specific analysis on the assessment. Methods A total of 40 patients with CA diagnosed by biopsy and technetium pyrophosphate scintigraphy were compared with 120 control patients matched for mean left ventricular wall thickness (40 aortic stenosis, 40 hypertrophic cardiomyopathy, and 40 hypertensive heart disease). We compared the discriminative abilities of three definitions of RASP (visual, quantitative, and semi-quantitative). According to a previous paper, visual RASP was defined as visual reduction of LS in the basal and middle LS segments (light red or blue) relative to the apical LS (red). Quantitative RASP was calculated using the following formula: average apical LS/(average basal LS + average mid-ventricle LS), then binarized by the optimal cut-off value for predicting CA. Semi-quantitative RASP was defined as reduction of LS (≥-10%) in five or more segments out of the basal six segments, relative to apical LS (≤-15%). Sample cases are shown in Figure (left). Visual and semi-quantitative RASP were independently assessed by two blinded sonographers. The RASP at the endo-myocardial and all layers was evaluated using customized software. The concordance was assessed using the kappa statistic, whereas the discriminative ability was assessed using receiver operating characteristic curve analysis. Results The concordance of visual RASP was modest but its semi-quantitative RASP was perfect (Table right). The discriminative ability of semi-quantitative RASP at each layer was significantly better than that of visual RASP and close to that of the binary quantitative RASP. Additionally, the discriminative abilities of visual (p=0.10) and semi-quantitative (p=0.11) RASP at the endo-myocardial layer appeared to be better than those at all layers. Conclusions The assessment method of semi-quantitative RASP is easy and highly reproducible. Furthermore, it accurately discriminates CA. In addition, assessment at the endo-myocardial layer potentially improves the discriminative ability.

P305Prognostic value of relative apical sparing pattern of longitudinal strain in patients with severe aortic stenosis

Abstract Background The relative apical sparing pattern (RASP) of left ventricular longitudinal strain (LS) is determined on the strain polar map, while global longitudinal strain (GLS) is measured using speckle-tracking echocardiography and is frequently associated with cardiac amyloidosis (CA). According to recent reports, some elderly patients with aortic stenosis (AS) suffer from transthyretin CA and have a poor prognosis. Accordingly, we aimed to investigate the association of RASP and outcome of patients with severe AS. Methods We retrospectively studied 157 consecutive patients (age: 81±10 years, 33% men) with severe AS (mean transaortic pressure gradient: 49 mmHg) and preserved ejection fraction (&gt;50%). After measuring GLS, RASP was semi-quantitatively and quantitatively assessed. Semi-quantitative RASP (sRASP) was defined as reduction of LS (more than −10%), showing light red or blue in ≥5 segments out of the basal six segments, relative to apical LS (less than −15%) showing red. This analysis was independently performed in a blinded manner by two observers. Quantitative RASP (qRASP) was calculated using the following formula: average apical LS/(average basal LS + average mid-ventricle LS), then qRASP ≥1 was determined as positive according to the previous paper. Patients were followed up to determine their outcomes, i.e., sudden cardiac death or unexpected admission due to heart failure over a median duration of 1.9 years. Concordance of sRASP was assessed using the kappa statistic, and a Cox proportional hazards model was used to assess the association between the parameters and primary outcome. Results The consistency in the observations of the two sonographers in identifying sRASP was found to be excellent (κ = 1.00). sRASP and qRASP were observed in 24 (15%) and 42 (27%) patients, respectively, and were significantly associated with the primary outcome (n=44; 28%). The representative case is shown in figure (left panel). Further, positive sRASP was associated with the outcome after adjusting for the Euro score, NYHA ≥II, and GLS (hazard ratio = 2.69, p=0.01). The model based on these covariates significantly improved following the addition of sRASP (Figure; right panel). In addition, sRASP was observed in four patients out of 50 patients who underwent aortic valve replacement. Of these, one patient had the primary outcome (25%). On the other hand, in the remaining 46 patients without sRASP, four patients had the outcome (9%). Figure 1 Conclusions RASP was observed in some patients with severe AS and has been shown to have increasing importance in predicting adverse cardiac events in such patients.

Cardiac manifestations and prognostic implications of hereditary transthyretin amyloidosis associated with transthyretin Ala97Ser

The cardiac manifestations of late-onset hereditary transthyretin amyloidosis with p.A97S variant have not been extensively studied, and the prognostic factors remain unclear. The clinical profile, echocardiography, and ECG of patients diagnosed with ATTR p.A97S polyneuropathy between 2000 and 2016 were retrospectively collected. 67 patients with ATTR p.A97S were collected. A total of 82% of patients met the criteria for left ventricular (LV) hypertrophy. Reduced global longitudinal strain (GLS) was noted in 42.1% of patients, and 14% of patients had a relative apical sparing pattern. A low voltage pattern in the ECG was observed in 31.3% of patients, while 64.2% presented with a pseudoinfarction pattern. End-systolic LV inner dimension (HR: 2.25 (95% CI: 1.01-5.01), p=0.048), reduced GLS (HR: 5.26 (1.08-25.0), p=0.039), relative apical longitudinal strain (RALS>1, HR: 8.57 (1.69-43.3), p=0.009), increased E/A ratio (HR: 6.51 (1.17-36.4), p=0.033), and increased QRS duration (HR: 1.02 (1.00-1.04), p=0.05) were correlated with reduced survival in univariate analysis. Multivariate analysis revealed reduced RALS was significantly correlated with reduced survival (HR: 13.00 (1.81-93.45), p=0.011). Our findings reveal that ATTR p.A97S is a cardiomyopathy as well as a polyneuropathic syndrome. Routine use of more contemporary echocardiographic techniques are recommended to identify cardiac amyloidosis and provide prognostic information.

O28-4 - Apical Thickening Score Using Quantitative Gated SPECT for the Diagnosis of Cardiac Amyloidosis

Introduction: Cardiac amyloidosis (CA) is characterized by regional variations from base to apex in longitudinal strain (LS) using echocardiography. A relative apical sparing pattern of LS is reproductive method of differentiating CA from other causes of left ventricular hypertrophy. Methods: 40 consecutive patients suspected CA by echocardiography underwent thallium-201-chloride and 99mTc-PYP SPECT. We evaluated apical sparing on nuclear cardiology using the each percentage of left ventricular thickenings (17 segment) that were accumulated by quantitative gated SPECT. We calculated the relative apical thickening score (average apical thickening/average basal thickening + mid thickening). Results: Mean age of study population was 70 ± 12 years, and 70% were males. Six patients had biopsy proven systemic amyloidosis. The average score in the group of CA was 1.07 ± 0.25 and the other was 0.78 ± 0.44 (P = .034). Cutoff value of the apical thickening score was 0.82 and the sensitivity and specificity for diagnosing CA were 83% and 68%. Conclusions: In CA, the relative apical thickening score accumulated by SPECT expressed in a similar pattern to that of echocardiography. We may predict likelihood of CA by adding the score when we check SPECT image.