Value of Longitudinal Strain to Identify Wild-Type Transthyretin Amyloidosis in Patients With Aortic Stenosis.

BACKGROUND: Wild-type transthyretin-related amyloidosis (ATTRwt) and degenerative aortic stenosis (AS) are both age-related. Diagnosis of cardiac amyloidosis (CA) among patients with AS may be difficult due to overlapping morphological and functional criteria. The aim of this study was to describe an echocardiographic longitudinal strain (LS) pattern among patients with AS with and without ATTRwt.Methods and Results:Patients who have AS with ATTRwt (n=30), AS without ATTRwt (n=50) and ATTRwt without AS (n=31) underwent two-dimensional speckle-tracking echocardiography. Transthyretin CA was based on positive bone scintigraphy without monoclonal gammopathy. All patients showed a gradual decrease in LS from the base to the apex resulting in a decrease of the global LS. A cut-off value of 1.0 for relative apical LS (average apical LS/[average basal LS+mid-LS]) was sensitive (88%) but less specific (68%) in differentiating ATTRwt among patients with severe AS. The best cut-off value for relative apical LS for identifying patients with ATTRwt among the whole population was 0.9 (sensitivity 74%, specificity 66%); however, 35%, 25% and 11% of patients who have ATTRwt without AS, with moderate AS and with severe AS, respectively, did not reach this threshold. CONCLUSIONS: A decrease of global and relative apical LS is common in patients with AS, even in the absence of ATTRwt. ATTRwt CA can be present even in the absence of relative apical sparing of LS.

Feasibility and accuracy of gated blood pool SPECT equilibrium radionuclide ventriculography for the assessment of left and right ventricular volumes and function in patients with left ventricular assist devices.

BACKGROUND: Left ventricular assist devices (LVADs) require serial assessment of right and left ventricular (RV & LV) volumes and function. Because the RV is not assisted, its function is a critical determinant of the hemodynamic and contributes significantly to postoperative morbidity and mortality. We evaluated the feasibility and the accuracy of tomographic-equilibrium radionuclide ventriculography (t-ERV) for the assessment of patients with LVADs. METHODS: Twenty-four patients with LVAD underwent t-ERV. Because of the limited acoustic window, transthoracic echocardiography (TTE) was only feasible in 19 patients. Functional evaluation including six-minute walk test (6MWT) and peak oxygen consumption (POC) was performed in 18 patients. Nine patients underwent a cardiac multidetector computed tomography (MDCT). Eight patients underwent a second evaluation by ERV 4.3 ± 1.4 months later. RESULTS: Reliability between t-ERV and MDCT for LV end-diastolic volume, LV end-systolic volume, LV ejection fraction, RV end-diastolic volume, RV end-systolic volume, and RV ejection fraction (RVEF) was 0.900 (P = .001), 0.911 (P = .001), 0.765 (P = .021), 0.728 (P = .042), 0.875 (P = .004), and 0.781 (P = .023), respectively. There was no correlation between t-ERV and RV systolic parameters assessed by TTE. RVEF was correlated with POC (R = 0.521; P = .027). A cut-off value of 40% for RVEF measured by t-ERV could discriminate patients with poor functional status (P = .048 for NYHA stage; P = .016 for 6MWT and P = .007 for POC). CONCLUSION: t-ERV is a simple, reproducible, and an accurate technique for the assessment of RV function in patients with LVADs and warrants consideration in the evaluation and monitoring of symptomatic patients.

Mitral Annular Calcium and Mitral Stenosis Determined by Multidetector Computed Tomography in Patients Referred for Aortic Stenosis.

Mitral annular calcium (MAC) is a common finding in older patients referred for transcatheter aortic valve implantation (TAVI). Multidetector computed tomography (MDCT) allows fine quantification of the calcific deposits. Our objective was to estimate the prevalence of MAC and associated mitral stenosis (MS) in patients referred for TAVI using MDCT. A cohort of 346 consecutive patients referred for TAVI evaluation was screened by MDCT for MAC: 174 had MAC (50%). Of these patients, 165 patients (95%) had mitral valve area (MVA) assessable by MDCT planimetry (age 83.8 ± 5.9 years). Median mitral calcium volume and MVA were 545 mm3 (193 to 1,253 mm3) and 234 mm2 (187 to 297 mm2), respectively. The MS was very severe, severe, and moderate in 2%, 22%, and 10% patients, respectively. By multivariate analysis, MVA was independently correlated to mitral calcium volume, aortic annular area, and some specific patterns of mitral leaflet calcium. Based on these findings, a formula was elaborated to predict the presence of a significant MS. In conclusion, MDCT allows detailed assessment of MAC in TAVI populations, demonstrating a high prevalence. Mitral analysis should become routine during MDCT screening before TAVI as it may alter therapeutic strategy.

Pilot study for left ventricular imaging phenotype of patients over 65 years old with heart failure and preserved ejection fraction: the high prevalence of amyloid cardiomyopathy.

This study sought to phenotype patients over 65 years old with heart failure and preserved ejection fraction (HFpEF) using clinical available comprehensive cardiovascular imaging modalities. Forty-nine patients with HFpEF and without coronary artery disease underwent clinical evaluation, electrocardiography, echocardiography, cardiac magnetic resonance (CMR) and 99mTc-3,3-diphosphono-1,2-propanodicarboxylic acid scintigraphy (99mTc-DPD). The mean population age was 76 ± 8 years. Most of the patients (53 %) were NYHA class II. Mean NT-Pro-NBNP level was 1961 ± 2372 pg/ml. CMR exhibited a hypertrophic cardiomyopathy or infiltrative pattern in 3 (6 %) and 15 (31 %) patients, respectively. In the latter subgroup, 99mTc-DPD was suggestive of transthyretin-related cardiac amyloidosis for nine (18 %) patients, while AL amyloidosis was proven in five patients (10 %) by extracardiac (n = 3, 6 %) or endomyocardial (n = 2, 4 %) biopsies-one patient declined tissue biopsy. Compared to patients with unspecified cardiomyopathy (n = 31), patients with amyloid cardiomyopathy (n = 15 or n = 14/proven) had less hypertension, lower systolic blood pressure and higher NT-pro BNP level. Their electrocardiogram showed lowest QRS voltage and longer QRS duration. Left ventricular (LV) pattern was characterized by a more pronounced LV hypertrophy, a smaller ejection fraction and a decrease of global longitudinal strain associated with an increase of longitudinal strain apical-to-basal ratio. In patients over 65 years, HFpEF is a heterogeneous syndrome with at least a 29 % prevalence of amyloid cardiomyopathy. Combined CMR and 99mTc-DPD are helpful imaging tools for accurate phenotyping of patients amenable to histopathological diagnosis or genetic testing, and should be considered for proper management of this population. Further longitudinal investigations are needed to better clarify these preliminary results.

Lack of Correlations between Electrophysiological and Anatomical-Mechanical Atrial Remodeling in Patients with Atrial Fibrillation.

BACKGROUND: Atrial fibrillation (AF) progressively leads to electrical remodeling (ER) and anatomical-mechanical remodeling (AR), whose relationships in humans remain poorly known. METHODS: ER and AR were compared in patients undergoing percutaneous radiofrequency (RF) ablation for AF. ER was defined by right and left appendage activation rates as a surrogate for atrial refractory periods. AR was approached by left atrial (LA) diameters and area and left atrial appendage (LAA) area and contractile function (mean emptying flow velocity) (LAAFV) before RF ablation. Mean duration between successive LAA contractions was considered as LAA mechanical rate. RESULTS: Forty-one patients (31 men, age: 64 ± 9 years) with paroxysmal (27%), persistent (61%), or long-persistent AF (12%) were prospectively included (ejection fraction: 44 ± 16%). Parameters exploring AR were highly correlated to each other: LA area (28 ± 7 cm(2) ), LAA area (5.7 ± 2.25 cm(2) ), LA transverse (49 ± 7 mm), and anteroposterior diameter (59 ± 13 mm) or LAAFV (29 ± 13 cm/s; P < 0.05 for each comparison). Parameters exploring ER were also highly correlated: right atrial appendage (RAA; 181 ± 39 ms) and LAA (176 ± 33 ms) activation rates (P < 0.0001). There was no significant correlation between any ER and AR parameter. Only LAA mechanical rate (174 ± 36 ms) was correlated to LAA or RAA activations rates (P ≤ 0.01). CONCLUSION: ER and AR are not mutually related, atrial activation rate being not correlated to LA or LAA size or function. Thus, the mechanisms leading to AF-induced atrial remodeling may differ for anatomical and electrophysiological aspects.

Prediction of all-cause mortality from gated-SPECT global myocardial wall thickening: comparison with ejection fraction and global longitudinal 2D-strain.

BACKGROUND: The aim of this study was to assess the correlation between global wall thickening (GWT) obtained by gated-single photon emission computed tomography (SPECT) and echocardiographic measures [ejection fraction (EF), global longitudinal strain (GLS), and strain rate (GLSR)] and to compare their prognostic value for all-cause mortality. METHODS AND RESULTS: Seventy-four patients with referral for dipyridamole myocardial perfusion SPECT were prospectively included and underwent transthoracic echocardiography to measure left ventricular EF, GLS, and GLSR. The strongest correlation with GWT was for EF (R = 0.63, P < .001), followed by GLSR (R = -0.57, P < .001) and GLS (R = -0.53, P < .001). There were ten deaths over a period of 14.6 ± 5.7 months. Using the multivariate Cox analysis, summed stress score (HR 1.108; P = .023), EF (HR 1.01, P = .031), GLS (HR 1.593, P = .001), and GWT (HR 0.898, P = .034) remained independent predictors of mortality. Mean survival rate evaluated by Kaplan-Meier analysis was longer in patients with GWT ≥ 24% (21.9 ± 0.6 months) than those with GWT < 24% (13.6 ± 2.7 months; P < .001). CONCLUSIONS: GWT assessed is a highly sensitive tool to detect early myocardial systolic dysfunction and may bring additional prognostic information.

Comparison of two- and three-dimensional transthoracic echocardiography for measurement of aortic annulus diameter in children.

BACKGROUND: Accurate evaluation of aortic root geometry is necessary in congenital aortic valve lesions in children, to guide surgical or angiographical intervention. AIM: To compare aortic annulus diameters measured by two- and three-dimensional transthoracic echocardiography (2D- and 3D-TTE), to determine the feasibility and reproducibility of 3D imaging and assess the dynamic changes during the cardiac cycle. METHODS: Thirty children without heart disease were prospectively included. Two orthogonal aortic annulus diameters were measured offline using multiplanar reconstruction in diastole and in systole and were compared with the measurement of the aortic annulus diameter by 2D-TTE. RESULTS: Mean age was 11±3.6 years. Feasibility of 3D imaging was 100%. The coefficients of intra- and interobserver variability were 3.5% and 6%, respectively. The 2D mean diameter was significantly smaller than the 3D maximum diameter in systole (1.94 vs. 2.01mm; p=0.005). 2D and 3D measurements were well correlated (p<0.0001). The maximum and minimum diameters in 3D were significantly different both in systole and in diastole (p<0.001) underlining an aortic annulus eccentricity. The mean aortic annulus diameters were not significantly different between systole and diastole, with important individual variability during the cardiac cycle. CONCLUSION: This study demonstrated the feasibility and reproducibility of 3D-TTE for the assessment of the aortic annulus diameter in a normal paediatric population. Because of an underestimation of the maximum diameter by 2D-TTE and the asymmetry of the aortic annulus, 3D measurements could be important before percutaneous aortic valvuloplasty or surgical replacement.

Relation between left ventricular outflow tract obstruction and left ventricular shape in patients with hypertrophic cardiomyopathy: a cardiac magnetic resonance imaging study.

BACKGROUND: Hypertrophic cardiomyopathies (HCM) are often associated with left ventricular (LV) outflow tract obstruction, which can explain symptoms and impact prognosis. AIMS: To better understand the mechanisms that link obstruction and LV shape in HCM. METHODS: Patients with HCM who underwent cardiac magnetic resonance (CMR) imaging were included retrospectively. Obstructive HCM was defined as LV outflow gradient more than 30 mm Hg at rest by transthoracic echocardiography. The LV shape and mitral angle were assessed by CMR. Results were compared with control subjects. RESULTS: Mean LV-mitral angle was smaller in patients with obstructive HCM (n=29) than in patients with non-obstructive HCM (n=15) or control subjects (n=15) (80 ± 5° vs 87 ± 7° [P=0.0002] and 89 ± 2° [P<0.0001]). Mean mitral papillary muscles angle was greater in patients with non-obstructive HCM than in patients with obstructive HCM or control subjects (136 ± 17° vs 123 ± 16° [P=0.007] and 118 ± 10° [P=0.002]). Patients with non-obstructive HCM had a greater mean LV-aortic root angle than patients with obstructive HCM or control subjects (139 ± 6° vs 135 ± 7° [P=0.04] and 133 ± 7° [P=0.03]). CONCLUSION: There is a relation between morphological and functional parameters in HCM within which the mitral valve is probably part of pathophysiogenesis.

Relationship between left ventricular sphericity and trabeculation indexes in patients with dilated cardiomyopathy: a cardiac magnetic resonance study.

AIMS: To study the relationship between left ventricular (LV) trabeculations, volume, and sphericity in patients with dilated cardiomyopathy (DCM) by cardiac magnetic resonance imaging (CMR). METHODS AND RESULTS: Eighty-two patients with DCM were prospectively explored by CMR. The segmental trabeculation index (STI) was defined by the ratio of trabeculated layer thickness on compacted layer thickness per segment. The global trabeculation index (GTI) was defined by the ratio of the sum of the total trabeculated layer thickness to the sum of the total compacted layer thickness. The apex was excluded from the analysis. The mean number of segments with trabeculation per patient was 10 ± 2 with a mean GTI of 0.68 ± 0.32. The LV sphericity index was inversely correlated with LV ejection fraction (R = -0.42, P = 0.0002) and positively with the brain natriuretic peptide (BNP) level (R = 0.34, P = 0.003). The maximal STI was positively correlated with the indexed LV end-diastolic volume (R = 0.32, P = 0.004) and the LV sphericity index (R = 0.25, P = 0.02), but not with the BNP level or LV ejection fraction. The GTI was positively correlated with the LV sphericity index (R = 0.27, P = 0.016) but not with indexed LV end-diastolic volume, BNP levels, or LV ejection fraction. CONCLUSION: Trabeculation indexes depend on LV shape and are positively correlated with LV sphericity. These results encourage interpreting LV trabeculation with caution in patients with DCM, considering additional morphologic criteria such as LV geometry.

Patent foramen ovale appearance with association of left ventricular assist device and mechanical ventilation.

We report on a 62-year-old man referred to the cardiac intensive care unit less than 24 hours after anterior ST-segment elevated myocardial infarction. The patient developed cardiogenic shock requiring elective intubation, vasopressor, and inotropic support. Emergency coronary angiography, revascularization, and insertion of an intraaortic balloon pump were performed. Nevertheless, the hemodynamic situation remained unstable, and a left ventricular assist device (LVAD) was inserted. Severe hypoxemia occurred several minutes after initiating the Impella Recover LD/LP 5.0 (Abiomed, Danvers, MA). Transesophageal echocardiography allowed diagnosis of patent foramen ovale. The shunt resulted from abnormal left-to-right pressure gradient due to left ventricular unloading and increased right ventricular afterload induced by mechanical ventilation. Transesophageal echocardiography was used to regulate both LVAD output and positive end-expiratory pressure settings to optimize oxygen transport. Because patent foramen ovale is common in humans, transesophageal echocardiography should be performed systematically after LVAD insertion.