12 research outputs found
The tricuspid annulus in amyloidosis with cardiac involvement: Detailed analysis from the three-dimensional speckle tracking echocardiographic MAGYAR-Path Study
Novel electrocardiographic criteria may render possible the more accurate recognition of cardiac amyloidosis
The early diagnosis of cardiac amyloidosis (CA) is paramount, since there are effective therapies that improve patient survival. The diagnostic accuracy of classical electrocardiographic (ECG) signs, such as low voltage, pseudoinfarct pattern, and conduction disturbances in the diagnosis of CA, is inferior to that of the echocardiographic myocardial deformation criteria; therefore, our aim was to find more accurate novel ECG criteria for this purpose.We tested the diagnostic value of five novel ECG criteria, two of them devised by us, in 34 patients with confirmed CA (20 transthyretin amyloidosis and 14 AL amyloidosis) and 45 control patients with left ventricular hypertrophy on echocardiography due to hypertension, valvular aortic stenosis and hypertrophic cardiomyopathy. The following novel ECG criteria, that suggested CA, were tested: QRS amplitude in lead I 4.1 (TA: 77%, SE: 93%, SP: 38%, PPV: 79%, NPV: 69%, AUC: 0.65) echocardiographic criteria. Among the classical criteria, the low voltage in limb leads criterion was present most frequently (in 73.5%) in patients with CA, with slightly worse diagnostic value than the novel ECG criteria (TA: 78.5%, SE: 73.5%, SP: 82.2%, PPV: 75.8%, NPV: 80.4%).The novel ECG criteria [mostly the aVR < 0.5, (I + aVR)/(V1-4 ) < 0.375] seem at least as reliable in the diagnosis of CA as the best echocardiographic myocardial deformation criteria and might be used either together with the echocardiographic criteria or as stand-alone criteria to diagnose CA in the future
Improved survival of autologous stem cell transplantation in primary refractory and relapsed Hodgkin lymphoma in the brentuximab vedotin era - real-world data from Hungary
Autologous stem cell transplantation (ASCT) is the standard treatment of primary refractory or relapsed Hodgkin-lymphoma, which can provide a cure rate of about 50%. The aim of our study was to analyze the data of 126 HL patients undergoing AHSCT in Hungary between 01/01/2016 and 31/12/2020. We assessed the progression-free and overall survival, the prognostic role of PET/CT performed before transplantation and effect of brentuximab vedotin (BV) treatment on survival outcomes. The median follow-up time from AHSCT was 39 (1-76) months. The 5-year OS comparing PET- and PET + patients was 90% v. 74% (p = 0.039), and 5-year PFS was 74% v. 40% (p = 0.001). There was no difference in either OS or PFS compared to those who did not receive BV before AHSCT. We compared BV treatments based on their indication (BV only after AHSCT as maintenance therapy, BV before and after AHSCT as maintenance treatment, BV only before AHSCT, no BV treatment). There was statistically significant difference in the 5-year PFS based on the inication of BV therapy. Recovery rates of our R/R HL patient population, who underwent AHSCT, improved significantly. Our positive results can be attributed to the PET/CT directed, response-adapted treatment approach, and the widespread use of BV
Prognostic role of three‐dimensional speckle‐tracking echocardiography‐derived left ventricular global longitudinal strain in cardiac amyloidosis: Insights from the MAGYAR‐Path Study
Different patterns of left ventricular rotational mechanics in cardiac amyloidosis—results from the three-dimensional speckle-tracking echocardiographic MAGYAR-Path Study
Cardiac amyloidosis is associated with increased aortic stiffness
OBJECTIVE: Cardiac amyloidosis (CA) is as an infiltrative disorder primarily caused by extracellular tissue deposition of amyloid fibrils in the myocardial interstitium. The current study was designed to test whether alterations in ascending aortic elastic properties could be detected by echocardiography in CA patients, and to compare their results to controls. PATIENTS AND METHODS: We included 19 CA patients from which CA proved to be AL amyloidosis in 17 cases and transthyretin (TTR) amyloidosis in 2 cases. Their results were compared to 20 age-, gender-, and risk factor-matched controls. RESULTS: There was significantly greater interventricular septum and left ventricular (LV) posterior wall thickness, lower LV ejection fraction and greater E/A in CA patients than in controls, suggesting systolic, and diastolic dysfunction. CA patients also showed significantly reduced aortic strain and pulsatile change in aortic diameter, and increased aortic stiffness index. CONCLUSION: These results suggest increased aortic stiffness in CA patients
A haemophagocytás lymphohistiocytosis jellemzőinek, diagnosztikájának és kezelési lehetőségeinek áttekintése egy T-sejtes lymphomás beteg bemutatása kapcsán
Cardiac Amyloidosis Associated with Enlargement and Functional Impairment of the Mitral Annulus: Insights from the Three-Dimensional Speckle Tracking Echocardiographic MAGYAR-Path Study
BACKGROUND: Cardiac amyloidosis (CA) is a rare condition characterized by the extracellular deposition of amyloidogenic proteins in the heart. The aim of the present study was to compare the size and function of the mitral annulus (MA) between CA patients and age- and gender-matched controls, using three-dimensional speckle-tracking echocardiography (3-D STE). METHODS: The study included 17 patients (mean age 64.2 +/- 9.8 years) with CA, whose results were compared to 26 age- and gender-matched healthy controls (mean age 59.0 +/- 8.2 years). Complete two-dimensional (2-D) Doppler echocardiography and 3-D STE were performed in all cases. RESULTS: Significantly enlarged end-diastolic and end-systolic MA diameters (3.09 +/- 0.56 cm versus 2.70 +/- 0.37 cm, p = 0.01 and 2.71 +/- 0.68 cm versus 1.87 +/- 0.31 cm, p <0.001) and MA area (11.22 +/- 3.56 cm2 versus 8.60 +/- 1.92 cm2, p = 0.004 and 8.57 +/- 3.35 cm2 versus 4.55 +/- 1.05 cm2, p <0.001) were demonstrated in CA. MA fractional area change (24.10 +/- 13.97% versus 46.06 +/- 14.37%, p <0.001) and MA fractional shortening (12.92 +/- 9.55% versus 30.98 +/- 11.65%, p <0.001) were also impaired in CA patients as compared to matched controls. CONCLUSIONS: CA is associated with MA enlargement and functional impairment represented by MA fractional shortening and MA fractional area change, as assessed using 3-D STE
Left ventricular rigid body rotation in a diffuse large B-cell lymphoma patient with cardiac involvement: a case from the three-dimensional speckle-tracking echocardiographic MAGYAR-Path Study
Left atrial dysfunction in light-chain cardiac amyloidosis and hypertrophic cardiomyopathy - a comparative three-dimensional speckle-tracking echocardiographic analysis from the MAGYAR-Path Study
INTRODUCTION: While cardiac amyloidosis (CA) is a rare systemic disease characterized by extracellular deposition of protein-derived fibrils, hypertrophic cardiomyopathy (HCM) is histopathologically characterized by myocyte hypertrophy and disarray, interstitial fibrosis, and small intramural coronary arteriole dysplasia. The aim of the present study was to compare left atrial (LA) volumetric and functional characteristics between light-chain (AL) CA and HCM by three-dimensional (3D) speckle-tracking echocardiography (STE). METHODS: The AL-CA group initially consisted of 17 patients with AL-CA, but one patient was excluded due to inadequate image quality, and so the study population consisted of 16 patients (mean age: 64.0+/-9.6 years, five men). Their results were compared with data on 20 age-matched HCM patients (mean age: 59.8+/-5.2 years, 10 men) and on 16 age-matched healthy controls (mean age: 58.2+/-7.2 years, six men). Complete two-dimensional Doppler echocardiography and 3D-STE were performed in all cases. RESULTS: Significantly increased LA volumes were observed in both AL-CA and HCM compared with the control group. Only active atrial emptying fraction was found to be significantly reduced in AL-CA patients compared to controls. Peak global and mean segmental circumferential, longitudinal and area strains showed significant reductions in AL-CA patients compared with controls, but only peak mean segmental longitudinal strain differed significantly between HCM patients and controls. While no differences were demonstrated in global and mean segmental strain at atrial contraction between HCM patients and controls, AL-CA patients showed reductions in certain strain parameters compared to controls and HCM patients. CONCLUSIONS: Different patterns of LA functional characteristics were demonstrated in AL-CA and HCM patients by 3D-STE