Diagnostic score for the detection of cardiac amyloidosis in patients with left ventricular hypertrophy and impact on prognosis

Background: Among diagnosis associated with left ventricular hypertrophy (LVH), cardiac amyloidosis (CA) is a progressive disease with poor prognosis. Early noninvasive identification is of growing clinical importance. The objective of our study was to integrate clinical, biologic, electrocardiographic and echocardiographic parameters to build a diagnostic score in patients with LVH.

Methods and results: One hundred and fourteen patients with LVH underwent a cardiac magnetic resonance (CMR) and a ^99mTc-hydroxymethylene-diphosphonate scintigraphy (^99mTc-HMDP) allowing to discriminate three groups of diagnoses: CA (n?=?50 including 31, 18 and 1 ATTR, AL and AA amyloidosis), hypertrophic cardiomyopathy (n?=?19) and unspecific cardiomyopathy (n?=?45). Seven continuous variables associated with CA (systolic arterial pressure <130?mmHg; PR duration >200?ms; Sokolow index <12?mV; diastolic left ventricular posterior thickness >13?mm; E/Ea ratio >10; global longitudinal strain?>??12% and sum of basal longitudinal strain?>??47%) were selected and dichotomized according to the best cutoff value to build the diagnostic score, which was validated in an independent cohort of 34 patients with LVH from aortic stenosis. The area under the ROC curve for the diagnosis of CA using the score was 0.933 (95%CI 0.889–0.978). The best cut off value for the score was 3 leading to a sensitivity of 90% and specificity of 81%. Area under the ROC curve for the score was 0.932 in the validation cohort. A diagnostic score >3 was associated with a poorest prognosis.

Conclusion: An integrated evaluation of 6 diagnostic factors including arterial blood pressure, ECG and echocardiographic parameters to build a diagnostic score is a simple and easily method to discriminate the 3 main CA in patients with LVH.     

Regional amyloid distribution and impact on mortality in light-chain amyloidosis: a T1 mapping cardiac magnetic resonance study

Background: T1 mapping allows quantitative assessment of “diffuse” deposition of amyloid protein in the myocardium. Early detection of cardiac involvement and potential prognostic improvement could benefit patients with AL amyloidosis.

Objectives: This study aims to evaluate the regional variation of amyloid infiltration in the left ventricle and the prognostic value of T1 mapping in patients with AL amyloidosis.

Methods: We prospectively enrolled 77 patients with AL amyloidosis who underwent cardiac magnetic resonance on a 3.0-T scanner. Native T1 and extracellular volume (ECV) were quantitated on the basal, mid, and apical levels of the left ventricle. Late gadolinium enhancement (LGE) pattern (no or non-specific LGE, sub-endocardial LGE, and transmural LGE) was also assessed. Forty healthy subjects served as controls. The primary end point was all-cause mortality.

Results: Basal ECV (26.9?±?2.8% versus 31.1?±?4.9%, p?<?.001) were lower than apical ECV in the healthy controls; however, basal ECV (60.6?±?11.5% versus 53.0?±?9.6%, p?=?.003) were significantly higher than apical ECV in patients with transmural LGE. During the follow-up period (median duration, 28?months; 25th–75th percentile, 13.5–38.0?months), 46 patients died. Basal ECV has the largest area under the curve of 0.845 (95% CI, 0.747–0.917) to predict all-cause mortality. Multivariable Cox analysis indicated that basal ECV was an independent prognostic factor and showed incremental prognostic value beyond NYHA class, Mayo stage, and LGE pattern.

Conclusion: We demonstrated that T1 mapping may have the potential to detect a characteristic amyloid deposition with a decreasing gradient from base to apex. Furthermore, myocardial ECV indicated that basal amyloid infiltration provided robust and incremental prognostic value in patients with AL amyloidosis.     

Right ventricular strain and strain rate properties in patients with right ventricular myocardial infarction

BACKGROUND: This study was planned to assess strain and strain rate properties of right ventricle in patients with RV myocardial infarction. MATERIAL AND METHOD: Thirty patients with acute inferior myocardial infarction were included in this study. The presence of right ventricular infarction in association with an inferior myocardial infarction was defined by an ST-segment elevation 0.1 mV in lead V4 R. According to this definition, 15 patients had electrocardiographic signs of inferior myocardial infarction without right ventricular infarction (group I), and 15 patients had electrocardiographic signs of inferior myocardial infarction with right ventricular infarction (group II). Echocardiography was performed using a Vivid 5 System (GE Ultrasound; Horten, Norway) and a 2.5-MHz transducer. 2-dimensional color doppler myocardial imaging (CDMI) data for longitudinal function were recorded from the RV free wall using standard apical view. Offline analysis of the myocardial color Doppler data for regional velocity (V), strain rate (Sr), and strain (S) curves was performed using a special software program (EchoPac 6.4 Vingmed, Horten, Norway). They were assessed in basal, middle and apical segments of the RV. The differences between different groups were assessed with the Mann-Whitney U-test. A value of P < 0.05 was considered statistically significant. RESULTS: Systolic tissue velocity, strain, strain rate of basal (4.8 +/- 0.8 cm/s vs 6.5 +/- 1.2 cm/s, -12 +/- 3% vs -24 +/- 5%, 1.28 +/- 0.3/s vs -1.9 +/- 0.4/s; P < 0.001, <0.001, <0.001, respectively) and mid (4.2 +/- 0.5 cm/s vs 5.4 +/- 0.5 cm/s, -16 +/-3% vs -26 +/- 4%, -1.2 +/- 0.3/s vs -2.1 +/- 0.3/s; P < 0.001, <0.001, <0.001, respectively) segments of right ventricle were significantly lower in patients with RV infarction than in patients without RV infarction. There were no differences between groups for apical strain, strain rate, and systolic tissue velocity. CONCLUSION: This study demonstrates that right ventricular strain and strain rate were lower in patients with left ventricular inferior wall myocardial infarction with, compared to without, right ventricular infarction.     

Prognostic significance of semiautomatic quantification of left ventricular long axis shortening in systemic light-chain amyloidosis

Aims: To assess left ventricular long axis shortening (LAS) in patients with AL amyloidosis as a potential predictor for outcome.

Methods and results: We performed a de novo echocardiographic analysis of LAS in 120 patients with biopsy-proven AL amyloidosis evaluated at first presentation before specific treatment. Additionally, 47 control subjects were analyzed retrospectivly. LAS was measured using a semiautomatic tissue motion annular displacement software algorithm (TMAD). LAS was significantly better than ejection fraction (EF) (p?<?0.0001) and M-mode-derived mitral annular plane systolic excursion (MAPSE) (p?<?0.05) discriminating AL patients from control subjects, while being non-inferior compared to tissue Doppler-derived peak systolic mitral annular velocity. One year outcome analysis in patients with AL amyloidosis showed that LAS remained the only significant echocardiographic parameter (HR:0.76; p?<?0.005) in a multivariable Cox regression model of echocardiographic values. In a comprehensive clinical model, LAS (HR:0.72, p?<?0.0001), cardiac troponin-T (HR:2.86, p?<?0.01) and free light chain difference (HR:1.00; p?<?0.05) were independently associated with the outcome. Assessment of LAS led to a significant integrated discrimination improvement and offered incremental information compared to EF and biomarkers. The cut-off value for LAS discriminating the endpoint was 5.8%.

Conclusion: LAS was an independent predictor of survival within the first year and offers incremental information in patients with AL amyloidosis evaluated prior to specific treatment.     

Comparison of left ventricular diastolic filling with myocyte bulk modulus using Doppler echocardiography and acoustic microscopy in pressure-overload left ventricular hypertrophy and cardiac amyloidosis

BACKGROUND: The myocardial bulk modulus has been described as the constitutive properties of the left ventricular (LV) wall and is measured as rho V2 (rho = density, V = sound speed) using acoustic microscopy. HYPOTHESIS: The study was undertaken to assess the relationship between the myocyte bulk modulus and transmitral inflow patterns in patients with pressure-overload LV hypertrophy (LVH) and cardiac amyloidosis (AMD). METHODS: In 8 patients with LVH, 8 with AMD, and 10 controls without heart disease, the transmitral inflow pattern was recorded by Doppler echocardiography before death, and myocardial tissue specimens were obtained at autopsy. The tissue density and sound speed in the myocytes were measured by microgravimetry and acoustic microscopy, respectively. The diameters of the myocytes were measured on histopathologic specimens stained by the elastica Van Gieson method. RESULTS: In the subendocardium, the myocyte bulk modulus was larger in LVH (2.98 x 10(9) N/m2, p < 0.001) and smaller in AMD (2.61 x 10(9) N/m2, p < 0.001) than in the controls (2.87 x 10(9) N/m2). The myocyte diameter in LVH (26 +/- 1 microns) was larger than that in the control (21 +/- 1 microns, p < 0.001) and AMD (20 +/- 1 microns, p < 0.001). The bulk modulus in the subendocardial myocyte significantly correlated with the deceleration time (DT) of the early transmitral inflow (r = 0.689, p = 0.028 in control, r = 0.774, p = 0.024 in LVH, and r = 0.786, p = 0.021 in AMD). CONCLUSION: The changes in the myocyte elasticity as represented by the bulk modulus were limited to the subendocardial layers and may be related to relaxation abnormalities in LVH and a reduction in LV compliance in AMD.     

Decreased plasma adiponectin is associated with impaired left ventricular longitudinal systolic function in hypertensive patients: a two-dimensional speckle tracking study

The influence of plasma adiponectin levels on myocardial contractile function has not been fully examined. We aimed to investigate the relationship between three-directional systolic function and plasma adiponectin levels in asymptomatic hypertensive patients using two- dimensional speckle-tracking echocardiography. The study population consisted of 78 patients with hypertension and 40 healthy controls. Longitudinal strain was significantly reduced in all patients, including those without LV hypertrophy (p?=?0.009). In multiple-regression analysis, plasma adiponectin levels (β?=??0.273, p?=?0.008) and LV mass index (β?=?0.458, p?相似文献   

血浆hsa-miR-30a与高血压左室肥厚患者预后的关系

目的 探讨血浆hsa-miR-30a与高血压左室肥厚(LVH)患者预后的关系。方法 随机入选118例高血压LVH患者,包括预后良好组(n=82)和预后不良组(n=36)。用real-time PCR法检测血浆hsa-miR-30a的含量。研究预后不良的高血压LVH患者血浆hsa-miR-30a的变化。用Logistic回归研究血浆hsa-miR-30a是否是高血压LVH患者预后不良的独立预测因子。结果 与预后良好组比较,预后不良组高血压LVH患者血浆hsa-miR-30a升高[11.24±2.15(2_-Δct*10₄)vs20.87±4.36(2_-Δct*10₄),P <0.05]。血浆hsa-miR-30a升高是高血压LVH患者预后不良的独立预测因子,OR为2.57,P<0.05。结论 血浆hsa-miR-30a升高对高血压LVH患者预后不良有预测价值。     

Impaired biventricular deformation in Marfan syndrome: a strain and strain rate study in adult unoperated patients

Objective: To investigate the presence of any regional myocardial deformation abnormalities in Marfan syndrome (MFS) and determine the benefits of using advanced echocardiography compared to conventional techniques. Background: Myocardial dysfunction in MFS may be caused by extracellular matrix remodeling thus, resulting in uniform reduced functionality. However, increased aortic stiffness may cause segmental ventricular abnormalities. Strain rate imaging (SRI) constitutes a validated technique to assess regional deformation in various clinical conditions. With this in mind, we aimed to investigate biventricular function in MFS using SRI. Methods: Forty‐four MFS patients (mean age 30 ± 12 years, 26 men) and 49 controls without valvular disease were examined using SRI. Ejection fraction (EF) was calculated by the Simpson's biplane method. Biventricular deformation was assessed by measuring strain/strain rate. Strain values were divided by left ventricular (LV) end‐diastolic volume to adjust LV deformation for geometry changes providing a strain index (SI). Aortic stiffness was evaluated using the β‐stiffness index. Results: EF (%) was reduced in MFS patients (59 ± 5 vs 72 ± 4, P < 0.001), whereas β‐stiffness was increased (P < 0.001). LV radial and LV and right ventricular (RV) long‐axis strain values (%) were reduced in the patient group (70 ± 17 vs 93 ± 10; 19 ± 2 vs 25 ± 2; 30 ± 9 vs 36 ± 8, respectively, P < 0.001). Strain rate measurements were also reduced (P < 0.001). In a multiple regression analysis, MFS diagnosis was negatively associated with LV SI (?0.262 [?0.306, ?0.219], P < 0.001). β‐Stiffness was negatively associated with SI obtained from the septum, inferior and anterior walls. ROC analyses demonstrated that SRI, when compared with conventional echocardiography, had higher sensitivity and specificity in predicting biventricular dysfunction in MFS. Conclusions: Our study showed a uniform reduction in biventricular deformation in MFS. These findings suggest that assessment of myocardial function using advanced echocardiographic techniques could be more accurate in MFS patient evaluation than conventional echocardiography alone. (Echocardiography 2011;28:416‐430)