scholarly journals Comparative analysis of phasic left atrial strain and left ventricular posterolateral strain pattern to discriminate Fabry cardiomyopathy from other forms of left ventricular hypertrophy

Author(s):  
David Frumkin ◽  
Isabel Mattig ◽  
Maamoun Al-Daas ◽  
Nina-Maria Laule ◽  
Sima Canaan-kühl ◽  
...  

Background ‘Classical’ echocardiographic signs of Fabry cardiomyopathy (FC), such as left ventricular hypertrophy (LVH), posterolateral strain deficiency (PLSD) and papillary muscle hypertrophy may have a limited diagnostic accuracy in clinical practice. Our aim was to evaluate the diagnostic value of left atrial (LA) strain impairment compared to ‘classical’ echocardiographic findings to discriminate FC. Methods In standard echocardiographic assessments, we retrospectively analyzed the diagnostic value of the “classical” red flags of FC as well as LA strain in 20 FC patients and in 20 subjects with other causes of LVH. Receiver operating characteristic (ROC) curve analysis was performed to assess the respective diagnostic accuracy. Results FC was confirmed in 20 patients by genetic testing. In the LVH group, 12 patients were classified by biopsy to have hypertrophic cardiomyopathy, two had hypertensive heart disease, and six LVH combined with borderline myocarditis. Global and regional left ventricular (LV) strain was not significantly different between groups while LA strain was significantly impaired in FC (Left atrial reservoir strain (LASr) 19.1%±8.4 in FC and 25.6%±8.9 in LVH, p=0.009; left atrial conduction strain (LAScd) -8.4%±4.9 in FC and -15.9%±8.4 in LVH, p<0.01). LAScd, with an area under the curve (AUC) of 0.81 [95% confidence interval (CI) 0.66-0.96] showed the highest diagnostic accuracy to discriminate FC. The PLSD pattern showed an AUC of 0.49, quantification of papillary muscle hypertrophy an AUC of 0.47. Conclusion Adding LA strain analysis to a comprehensive echocardiographic work-up of unclear LVH may be helpful to identify FC as a possible cause.

2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
D Frumkin ◽  
F Knebel ◽  
K Stangl ◽  
I Mattig ◽  
N Laule ◽  
...  

Abstract Background “Classic” echocardiographic signs of Fabry cardiomyopathy (FC), such as left ventricular hypertrophy (LVH) and posterolateral strain deficiency (PLSD) have a low diagnostic accuracy in clinical practice. Purpose Our aim was to evaluate the diagnostic accuracy of phasic left atrial strain impairment compared to PLSD to discriminate FC from other forms of LVH. Methods 40 patients with LVH due to bioptically and genetically confirmed FC or with LVH due to other causes, defined by exclusion of storage diseases, such as Amyloidosis or FC, by myocardial biopsy, were retrospectively analysed. Standard echocardiographic views (Vivid E9, GE, Vingmed, Horton) were used to analyse left atrial (LA) reservoir, conduit, and contraction strain using 2D speckle tracking echocardiography (2DSTE; EchoPAC software, GE) as well as the PLSD, obtained by the mean of deformation values in basal posterior and lateral segments in a 17-segment model. Receiver operating characteristic (ROC) curve analysis and a logistic regression model were performed to assess the diagnostic accuracy of LA and LV strain impairment. Results FC was confirmed in 20 patients by genetic testing and myocardial biopsy. In the LVH group, 12 patients were classified to have hypertrophic cardiomyopathy, two had hypertensive heart disease, and six expressed the pattern of LV hypertrophy combined with borderline myocarditis. LV septum thickness (15.8mm±3.4 in FC; 17.9mm±4.3 in LVH) and left atrial volume index (LAVI) (36.7ml/m2±11.3 in FC; 45.7ml/m2±16.3 in LVH) as well as LVEF (54.2%± 9.8 in FC; 52.5%±7.7 in LVH,) were not statistically different between groups. LV filling parameters such as E/A (1.2±0.5 in FC; 1.2±0.7 in LVH) and E/e' (11.0±4.9 in FC; 13.2±5.3 in LVH) showed a slightly more advanced impairment in the LVH group. Global and regional LV function was not different between groups (LVGLS −13.8±3.7% in FC and −12.8±3.7% in LVH; PLSD −10.7±5.2% in FC and −8.85±3.9% in LVH; p-value?). LA reservoir strain (LASr) and LA conduit strain (LAScd) were significantly impaired in FC compared to the LVH group (LASr 14,6±2.5% in FC and 26.3±8.5% in LVH, p&lt;0.01; LAScd −5.9±2.6% in FC and −15.8±4.7% in LVH, p&lt;0.01). In ROC analysis, LASr, with an area under the curve (AUC) of 0.81 (95% CI 0.64–0.97) and LAScd with an AUC of 0.85 (95% CI 0.71–0.99), respectively, showed the highest diagnostic accuracy to discriminate FC. PLSD, in contrast, held a low diagnostic accuracy with an AUC of only 0.47 (95% CI 0.27–0.68). Conclusion A substantially higher diagnostic accuracy could be shown for LASr and LAScd impairment in discriminating FD and other forms of LVH compared to PLSD. The echocardiographic assessment of phasic LA strain may help to identify FC in patients with unclear LVH. FUNDunding Acknowledgement Type of funding sources: None. ROC analysis Representative examples


2016 ◽  
Vol 117 ◽  
pp. S65
Author(s):  
Hilal Olgun Kucuk ◽  
Ugur Kucuk ◽  
Sevket Balta ◽  
Zafer Isilak ◽  
Mustafa Aparci ◽  
...  

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
C.A.M Tavares ◽  
N Samesima ◽  
L.A Hajjar ◽  
L.C Godoy ◽  
E.M.P Hirano ◽  
...  

Abstract Background Left Ventricular Hypertrophy (LVH) is an independent predictor of mortality and cardiovascular morbidity and the 12-lead ECG is recommended as a universal screening for patients with hypertension. However, the ECG has low sensitivity and there is limited data in patients 70 years or older. The recently published Peguero-Lo Presti (PLP) criteria had improved accuracy compared with other ECG criteria but with very few patients with age ≥70 years included. Purpose To compare the accuracy of the PLP criteria versus the traditional ECG criteria for detecting LVH in patients ≥70 years. Methods Retrospective single-center study. Patients were included if they were 70 years or older and underwent an ECG and echocardiogram (gold standard) less than 180 days apart from jan/2017 to mar/2018. Patients with left or right bundle branch block, non-sinus rhythm or ventricular paced rhythm were excluded. All tracings were independently reviewed by two cardiologists, blinded to the echocardiogram. The PLP criteria was compared against Cornell voltage (CV), Sokolow-Lyon voltage (SL), and Romhilt-Estes 4 and 5 (RE) criteria. LVH was defined as a left ventricular mass index &gt;115 g/m2 in males and &gt;95 g/m2 in females, according to the echocardiogram. McNemar's test, F1 score, and the area under the Receiver Operating Characteristic curves (AUC) were used to compare the diagnostic performance of the tests Results A total of 592 patients were included (mean age 77.5 years, SD: 5.9; 50.8% were women). The PLP criteria had increased sensitivity compared with both the SL and CV criteria (p&lt;0.0001 for both comparisons) and RE5 (p=0.042). PLP also had better specificity than the RE4 criteria (p&lt;0.0001) and the highest F1 accuracy score (Table 1). The AUC of the PLP was significantly higher than the AUC of the CV and RE criteria (0.70 vs 0.66 vs 0.64, respectively, p&lt;0.05) and numerically higher than the SL criteria (AUC=0.67, p=0.311, Figure 1). Conclusion Compared to the traditional ECG criteria for LVH, the PLP criteria had the highest diagnostic accuracy in elderly patients. Figure 1. AUC of the ECG criteira Funding Acknowledgement Type of funding source: None


1993 ◽  
Vol 14 (suppl D) ◽  
pp. 8-15 ◽  
Author(s):  
R. B. Devereux ◽  
M. J. Koren ◽  
G. de Simone ◽  
P. M. Okin ◽  
P. Kligfield

2008 ◽  
Vol 26 (7) ◽  
pp. 1472-1476 ◽  
Author(s):  
Marcello Chinali ◽  
Giovanni de Simone ◽  
Kristian Wachtell ◽  
Eva Gerdts ◽  
Julius M Gardin ◽  
...  

2003 ◽  
Vol 23 (6) ◽  
pp. 563-567 ◽  
Author(s):  
Ali Ihsan Günal ◽  
Erdogan Ilkay ◽  
Ercan Kirciman ◽  
Ilgin Karaca ◽  
Ayhan Dogukan ◽  
...  

Background It is still not clear whether hypertension and left ventricular hypertrophy (LVH) are more common in continuous ambulatory peritoneal dialysis (CAPD) than in hemodialysis (HD) patients. Methods To examine this subject, the indices of cardiac performance were compared between 50 HD and 34 CAPD patients. Patients were further divided into two subgroups [long-term (L) CAPD and L-HD] according to dialysis modality and duration of dialysis (more than 60 months’ duration). Results The blood pressure and cardiothoracic index of CAPD patients did not differ from HD patients. On average, the left atrial index was 2 mm/m2 higher in HD patients than in CAPD patients. Left ventricular chamber sizes, wall thickness, and left ventricular mass index (LVMI) in patients on CAPD were similar to those of HD patients. Isovolumic relaxation time (IVRT) of CAPD patients was insignificantly less than that of HD patients (101 ± 22 and 115 ± 27 msec respectively). There was no significant difference between the two subgroups (L-HD and L-CAPD) in blood pressure, left atrial diameter, left ventricular chamber size, wall thickness, LVMI, ejection fraction, or IVRT. Conclusion If normovolemia and normotension are obtained by strict volume control without using antihypertensive drugs, the effects of the two modalities of chronic dialysis treatment (HD and CAPD) on cardiac structure and function are not different from each other.


2002 ◽  
Vol 282 (3) ◽  
pp. H1127-H1134 ◽  
Author(s):  
Liliana G. Bianciotti ◽  
Adolfo J. de Bold

We investigated the effect of long-term in vivo blockade of the ET-1 receptor subtype B (ETB) with A-192621, a selective ETBantagonist, on atrial and ventricular natriuretic peptide (NP) gene expression in deoxycorticosterone acetate (DOCA)-salt hypertension. In this model, stimulation of the cardiac natriuretic peptide (NP) and the endothelin system and suppression of the renin-angiotensin system is observed. DOCA-salt induced significant hypertension, cardiac hypertrophy and increased NP plasma and left atrial and right and left ventricular NP gene expression. ETB blockade per se produced hypertension and left ventricular hypertrophy but induced little change on the levels of ventricular NP and only increased left atrial natriuretic factor (ANF) mRNA levels. Combined ETBblockade/DOCA-salt treatment worsened hypertension, increased left ventricular hypertrophy and induced right ventricular hypertrophy. All animals so treated had increased ventricular NP gene expression. Collagen III and β-myosin heavy chain gene expression were enhanced in both the right and the left ventricle of DOCA-salt hypertensive rats. The results of this study suggest that the ETBreceptor does not participate directly in the modulation of atrial or ventricular NP gene expression and that this receptor mediates a protective cardiovascular function. ETB blockade can induce significant ventricular hypertrophy without an increase in ANF or brain NP gene expression.


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