Novel electrocardiographic criteria may render possible the more accurate recognition of cardiac amyloidosis.

AIMS: 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. METHODS: 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 < 0.55 mV (I < 0.55); QRS amplitude in lead aVR < 0.5 mV (aVR < 0.5); average QRS amplitude of leads I + aVR < 0.575 mV [(I + aVR) < 0.575]; average QRS amplitude of leads I + aVR/average QRS amplitude of leads V1-4 < 0.375 [(I + aVR)/(V1-4) < 0.375]; average QRS amplitude of leads I + aVR/longest intrinsicoid deflection in leads I,aVL,V1-6 < 0.0115 [(I + aVR)/I,aVL,V1-6ID < 0.0115]. RESULTS: The I < 0.55, aVR < 0.5, (I + aVR) < 0.575, (I + aVR)/(V1-4) < 0.375, (I + aVR)/I,aVL,V1-6ID < 0.0115 test accuracy (TA) were 81%, 84.8%, 82.3%, 84.8%, and 83.3%, respectively; the sensitivity (SE): 76.5%, 82.4%, 85.3%, 82.4%, and 76.9%; specificity (SP): 84.4%, 86.7%, 80%, 86.7%, and 87.5%; positive predictive values (PPV): 78.8%, 82.4%, 76.3%, 82.4%, and 80%; negative predictive values (NPV): 82.6%, 86.7%, 87.8%, 86.7%, and 85.4%; area under curve (AUC) values: 0.8922, 0.8794, 09016, 0.8824, and 0.8462 were respectively. These parameters of the novel ECG criteria were at least as good as those reported by other authors in the literature of the qualitative (TA: 67%, SE: 80%, SP: 34%, PPV: 75%, NPV: 42%, AUC: 0.57) and quantitative apical sparing (TA: 64-80%, SE: 66-81.3%, SP: 55-78.3%, PPV: 33-83.9%, NPV: 41-75%, AUC: 0.62-0.68) and left ventricular ejection fraction/global longitudinal strain >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%). CONCLUSIONS: 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.

INTRODUCTION: Systemic amyloidosis is an uncommon disorder in which amyloid fibrils deposit extracellularly. Three-dimensional speckle-tracking echocardiography (3DSTE) is a novel method able to assess left ventricular (LV) global longitudinal strain (GLS). Our aim was to evaluate the prognostic impact of 3DSTE-derived LV-GLS in patients with cardiac amyloidosis (CA). MATERIALS AND METHODS: A total of 35 patients suffering from light-chain (AL) CA or transthyretin (TTR) CA were selected, but 7 patients had to be excluded due to insufficient image quality or were lost for follow-up. With AL-CA 23 cases, while for TTR-CA 5 patients were diagnosed. Complete two-dimensional Doppler and 3DSTE were performed in all subjects. RESULTS: The median follow-up was 201 days (ranging from 36 to 632 days) during which cardiovascular event was detected in 17 CA patients, including 8 cardiac deaths. Six patients were diagnosed with acute heart failure, two patients needed invasive interventions (percutaneous coronary intervention with stent-implantation, implantable cardioverter defibrillator implantation) and in one patient new higher grade atrioventricular block was registered. Using ROC analysis, 3DSTE-derived LV-GLS ≥11.8% (absolute value) was found to be a significant predictor for cardiovascular event-free survival (sensitivity 65%, specificity 64%, area under the curve 0.71, p = .05). Lower LV ejection fraction was confirmed in patients with LV-GLS <11.8% as compared to cases with LV-GLS ≥11.8%. In case of a cardiovascular event, LV-GLS was lower as compared to that of subjects with no events. Multivariable regression analysis confirmed that LV-GLS and LV end-diastolic diameter were independent predictors of cardiovascular survival. CONCLUSION: 3DSTE-derived LV-GLS is an independent predictor for future cardiovascular events in CA patients.

Laparoscopic splenectomy in our practice at the University of Szeged Department of Surgery / Laparoszkópos lépsebészet a szegedi Sebészeti Klinikán

Since its introduction in 1991, laparoscopic splenectomy has been considered the gold standard in spleen surgery, and the advantages of this technique over open surgery are indisputable. The technique was initiated in the Department of Surgery, University of Szeged in 1994 and since then our working group has gained one of the greatest experiences in this field in Hungary. Based on our results, similarly to literature data, it may be established that laparoscopic splenectomy can be considered a surgical procedure with low morbidity after gaining the necessary experience. In our study, the bowel motility recovered earlier, and hospital stay was significantly shorter after laparoscopic procedures. We proved laparoscopic splenectomy is a safe method in cases of extremely large spleens, and the Pfannenstiel incision is a cosmetically acceptable alternative for the retrieval of the spleen. Although several medications are available for second-line ITP therapy, laparoscopic splenectomy provides the longest-lasting results. In our study, young age and a preoperative response to steroids (steroid-dependent cases) were positive predictors for the success of splenectomy. Based on our experiences in the case of an immediate complete response to splenectomy, relapse occurred significantly less often.

The tricuspid annulus in amyloidosis with cardiac involvement: Detailed analysis from the three-dimensional speckle tracking echocardiographic MAGYAR-Path Study.

Introduction: Amyloidosis is a rare condition due to extracellular deposition of excessive amount of protein in parenchymal tissues including the heart. The present study aimed to test whether cardiac amyloidosis (CA) is associated with morphological and functional abnormalities of the tricuspid annulus (TA). For this aim, the results of patients having CA were compared to age- and gender-matched healthy controls by three-dimensional speckle-tracking echocardiography (3DSTE). Moreover, differences in TA parameters between light-chain CA (AL-CA) and transthyretin CA (TTR-CA) were studies as well. Materials and Methods: The study comprised 27 CA patients (mean age: 62.7 ±â€¯9.1 years, 21 males), their results were compared to those of 20 age- and gender-matched healthy volunteers (59.3 ±â€¯3.8 years, 13 males). Complete two-dimensional Doppler echocardiography and 3DSTE were performed in all CA patients and controls. Results: Dilated end-diastolic and end-systolic TA diameter, area and perimeter could be detected in all CA patients and in the AL-CA and TTR-CA subgroups, as well. Although only a few TTR-CA patients were involved, morphologic TA parameters proved to be tendentiously higher as compared to those of AL-CA patients. Functional parameters of TA were found to be reduced in CA patients, which were more deteriorated in AL-CA patients. Conclusions: Dilated TA is associated with its functional deterioration in CA.

Real-world efficacy of brentuximab vedotin plus bendamustine as a bridge to autologous hematopoietic stem cell transplantation in primary refractory or relapsed classical Hodgkin lymphoma.

Up to 30% of patients with classical Hodgkin lymphoma (cHL) are not responsive to frontline therapy or relapse after primary treatment. In these cases, autologous hematopoietic stem cell transplantation (AHSCT) is the standard of care. The combination of brentuximab vedotin and bendamustine (BV + B) is an effective salvage regimen in this challenging subpopulation. This nationwide multicenter study investigated the real-world efficacy and safety of the BV + B regimen as a bridge to AHSCT in patients with primary refractory or relapsed cHL. A total of 41 cHL patients underwent AHSCT after receiving at least 1 cycle of BV + B (with brentuximab vedotin given at 1.8 mg/kg on day 1 and bendamustine at 90 mg/m2 on days 1-2 every 4 weeks). After a median of 3 (1-6) cycles of BV + B, the objective response rate was 78%, with 29 (70.7%) patients achieving complete remission. Twelve (29.3%) patients relapsed after AHSCT, 2 (4.9%) of them died, while 2 (4.9%) patients are lost to follow-up. After a median of 17 months of follow-up, the estimated 2-year overall- and progression-free survival after AHSCT was 93 and 62%, respectively. Features of advanced disease at recurrence (p = 0.038) and the presence of stage IV cHL at relapse (p = 0.024) are strong predictor markers of unfavorable outcomes. Twenty-four (58.5%) patients experienced adverse events of any grade, while no grade IV toxicities were reported. BV + B is an effective salvage option with a manageable toxicity profile in cHL. The real-world safety and efficacy of this combination are similar to the observations made on the study population.

[Left ventricular deformation in cardiac light-chain amyloidosis and hypereosinophilic syndrome. Results from the MAGYAR-Path Study]. / Bal kamrai deformáció cardialis könnyulánc-amyloidosisban és hypereosinophilia-szindrómában. Eredmények a MAGYAR-Path Tanulmányból.

Introduction: Hypereosinophilic syndrome (HES) and immunoglobulin light-chain amyloidosis (ALA) are two, rare haematological disorders associated with cardiac alterations. Aim: The goal of the present study was a comparative assessment of left ventricular (LV) deformational parameters in HES and ALA patients using three-dimensional speckle-tracking echocardiography (3DSTE). Method: In the present study, results of 10 HES patients (mean age: 60.9 ± 14.7 years) and 19 ALA patients (mean age: 63.4 ± 7.8 years, 13 males) were analysed. The control group contained 13 age- and gender-matched healthy adults (mean age: 59.2 ± 4.3 years, 5 males). All patients underwent a complete two-dimensional Doppler echocardiography followed by 3DSTE. Results: All basal segmental LV strains were significantly reduced in ALA patients as compared to the control group. Global and mean segmental LV longitudinal strain (LS) values of ALA patients proved to be significantly decreased as compared to those of the healthy control group. During comparison of HES patients and healthy controls, significant difference could be detected in global LV-LS, while segmental basal LV-LS was also significantly reduced in HES patients. Basal LV radial and 3D strains showed significant differences when parameters of HES and ALA patient groups were compared. Conclusion: 3DSTE is a feasible tool for the detailed assessment of LV deformation in HES and ALA patients. Significant LV deformational abnormalities could be detected in both groups. In the case of ALA, these abnormalities are more prominent. Orv Hetil. 2020; 161(5): 169-176.

Predictive Factors for Success of Laparoscopic Splenectomy for ITP.

BACKGROUND AND OBJECTIVES: Therapy-resistant immune thrombocytopenia (ITP) is the most frequent indication of laparoscopic splenectomy (LS). It ensures the best results for this disease compared with possible second-line pharmacologic therapies. Therefore, learning about the safety of the surgical method and its long-term efficacy is important, as is selecting patients who respond to surgical treatment. Our purpose was to analyze the safety of LS and the short-and long-term prognostic significance of known perioperative parameters. METHODS: We performed 40 LSs for ITP from January 1, 2000, to January 1, 2015. We analyzed the roles of the perioperative parameters by using evidence-based guidelines. RESULTS: Complete response (CR; platelet count over 100 × 109/L) occurred in 28 cases (70%) and partial response (PR; platelet count between 30 and 100 × 109/L) in 5 cases (12.5%). Below the age of 50, 9% (2/22) of the patients had no response (NR; platelet count not increasing over 30 × 109/L), 28% (5/18) over the age of 50 (P = .023) had no response. In the steroid-refractory group, 30% did not respond, whereas 100% of the steroid-dependent patients had a CR (NR: 7/23 steroid refractory vs 0/17 steroid dependent; P = .027). The patients were followed up for a mean of 10.9 ± 6.9 years, and a long-term response (LTR) was detected in 21 of the responders (n = 33). Of the patients who originally had a CR, 71% also achieved LTR, whereas only 20% of the PR patients did. CONCLUSION: LS is safe and remains the most effective second-line treatment for ITP. In our study, younger age and response to preoperative steroids were predictive factors for the long-term success of splenectomy.

Right Atrial Deformation Analysis in Cardiac Amyloidosis - Results from the Three-Dimensional Speckle-Tracking Echocardiographic MAGYAR-Path Study / Análise da Deformação Atrial Direita na Amiloidose Cardíaca - Resultados de Ecocardiografia Tridimensional de Speckle-Tracking do Estudo MAGYAR-Path

Abstract Background: Light-chain (AL) cardiac amyloidosis (CA) is characterized by fibril deposits, which are composed of monoclonal immunoglobulin light chains. The right ventricle is mostly involved in AL-CA and impairment of its function is a predictor of worse prognosis. Objectives: To characterize the volumetric and functional properties of the right atrium (RA) in AL-CA by three-dimensional speckle-tracking echocardiography (3DSTE). Methods: A total of 16 patients (mean age: 64.5 ± 10.1 years, 11 males) with AL-CA were examined. Their results were compared to that of 15 age- and gender-matched healthy controls (mean age: 58.9 ± 6.9 years, 8 males). All cases have undergone complete two-dimensional Doppler and 3DSTE. A two-tailed p value of less than 0.05 was considered statistically significant. Results: Significant differences could be demonstrated in RA volumes respecting cardiac cycle. Total (19.2 ± 9.3% vs. 27.9 ± 10.7%, p = 0.02) and active atrial emptying fractions (12.1 ± 8.1 vs. 18.6 ± 9.8%, p = 0.05) were significantly decreased in AL-CA patients. Peak global (16.7 ± 10.3% vs. 31.2 ± 19.4%, p = 0.01) and mean segmental (24.3 ± 11.1% vs. 38.6 ± 17.6%, p =0.01) RA area strains, together with some circumferential, longitudinal and segmental area strain parameters, proved to be reduced in patients with AL-CA. Global longitudinal (4.0 ± 5.2% vs. 8.2 ± 5.5%, p = 0.02) and area (7.8 ± 8.1% vs. 15.9 ± 10.3%, p = 0.03) strains at atrial contraction and some circumferential and area strain parameters at atrial contraction were reduced in AL-CA patients. Conclusion: Significantly increased RA volumes and deteriorated RA functions could be demonstrated in AL-CA.

Resumo Fundamento: A amiloidose cardíaca (AC) de cadeias leves (AL) é caracterizada por depósitos fibrilares, que são compostos por cadeias leves de imunoglobulina monoclonal. O ventrículo direito é mais afetado pela AC-AL, e o comprometimento da sua função é preditor de um prognóstico pior. Objetivos: Caracterizar as propriedades volumétricas e funcionais do átrio direito (AD) na AC-AL por ecocardiografia tridimensional de speckle-tracking (3DSTE). Métodos: Um total de 16 pacientes (idade média: 64,5 ± 10,1 anos, 11 homens) com AC-AL foram examinados. Seus resultados foram comparados aos de 15 controles saudáveis pareados por idade e gênero (média de idade: 58,9 ± 6,9 anos, 8 homens). Todos os casos foram submetidos a Doppler bidimensional completo e 3DSTE. Um valor p bicaudal inferior a 0,05 foi considerado estatisticamente significativo. Resultados: Diferenças significativas foram demonstradas em volumes do AD com respeito ao ciclo cardíaco. O total (19,2 ± 9,3% vs. 27,9 ± 10,7%, p = 0,02) e as frações ativas de esvaziamento atrial (12,1 ± 8,1 vs. 18,6 ± 9,8%, p = 0,05) foram significativamente menores nos pacientes com AC-AL. Picos de strain no AD em áreas globais (16,7 ± 10,3% vs. 31,2 ± 19,4%, p = 0,01) e segmentares médias (24,3 ± 11,1% vs. 38,6 ± 17,6%, p = 0,01), juntamente com alguns parâmetros de strain por áreas circunferenciais, longitudinais e segmentares, mostraram-se menores em pacientes com AC-AL. Strains globais longitudinais (4,0 ± 5,2% vs. 8,2 ± 5,5%, p = 0,02) e por área (7,8 ± 8,1 vs. 15,9 ± 10,3%, p = 0,03) na contração atrial e alguns parâmetros de circunferência e de strain por área na contração atrial foram menores em pacientes com AC-AL. Conclusão: Foi possível demonstrar o aumento significativo dos volumes do AD e a deterioração de suas funções na AC-AL.

Right Atrial Deformation Analysis in Cardiac Amyloidosis - Results from the Three-Dimensional Speckle-Tracking Echocardiographic MAGYAR-Path Study.

BACKGROUND: Light-chain (AL) cardiac amyloidosis (CA) is characterized by fibril deposits, which are composed of monoclonal immunoglobulin light chains. The right ventricle is mostly involved in AL-CA and impairment of its function is a predictor of worse prognosis. OBJECTIVES: To characterize the volumetric and functional properties of the right atrium (RA) in AL-CA by three-dimensional speckle-tracking echocardiography (3DSTE). METHODS: A total of 16 patients (mean age: 64.5 ± 10.1 years, 11 males) with AL-CA were examined. Their results were compared to that of 15 age- and gender-matched healthy controls (mean age: 58.9 ± 6.9 years, 8 males). All cases have undergone complete two-dimensional Doppler and 3DSTE. A two-tailed p value of less than 0.05 was considered statistically significant. RESULTS: Significant differences could be demonstrated in RA volumes respecting cardiac cycle. Total (19.2 ± 9.3% vs. 27.9 ± 10.7%, p = 0.02) and active atrial emptying fractions (12.1 ± 8.1 vs. 18.6 ± 9.8%, p = 0.05) were significantly decreased in AL-CA patients. Peak global (16.7 ± 10.3% vs. 31.2 ± 19.4%, p = 0.01) and mean segmental (24.3 ± 11.1% vs. 38.6 ± 17.6%, p =0.01) RA area strains, together with some circumferential, longitudinal and segmental area strain parameters, proved to be reduced in patients with AL-CA. Global longitudinal (4.0 ± 5.2% vs. 8.2 ± 5.5%, p = 0.02) and area (7.8 ± 8.1% vs. 15.9 ± 10.3%, p = 0.03) strains at atrial contraction and some circumferential and area strain parameters at atrial contraction were reduced in AL-CA patients. CONCLUSION: Significantly increased RA volumes and deteriorated RA functions could be demonstrated in AL-CA.

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.

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.

[What should we know about cardiac amyloidosis? From clinical signs to treatment]. / Mit kell tudnunk a cardialis amyloidosisról? A tünettantól a kezelésig.

Systemic amyloidosis is a rare disease, in which the heart involvement is rather frequent and determines survival remarkably. Regarding the disease and organ involvement, new diagnostic procedures help to establish the diagnosis and to start the adequate treatment as soon as possible. Cardiac involvement is more likely to be characterised by monoclonal immunglobulin free light chain (AL amyloidosis) type and transthyretin type. In case of AL amyloidosis, heart involvement can lead to serious consequences. Biomarker assessments for cardiac function are important to determine disease severity at the beginning and to measure response to the treatment. In case of amyloidosis, the incidence of the heart involvement grows with age. The prevalence is not known exactly, but probably there are more cases than recognised. The authors present the clinical signs and diagnostic methods, emphasizing the importance of the cardiac examination methods. Orv Hetil. 2017; 158(46): 1811-1818.

Cardiac Amyloidosis Associated with Enlargement and Functional Impairment of the Mitral Annulus: Insights from the Three-Dimensional Speckle Tracking Echocardiographic MAGYAR-Path Study.

BACKGROUND AND AIM OF THE STUDY: 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.

Secondary myocardial involvement by diffuse large B-cell lymphoma is a rare occurrence. Left ventricular (LV) twist is considered an essential part of LV function. In normal circumstances LV twist results from the movement of two orthogonally oriented muscular bands of a helical myocardial structure with consequent clockwise rotation of the base and counterclockwise rotation of the apex. Three-dimensional (3D) speckle-tracking echocardiography (3DSTE) has been found to be feasible for non-invasive 3D quantification of LV wall motion and rotational mechanics. The present report aimed to assess LV twisting motion in a patient with diffuse large B-cell lymphoma with positron emission tomography/computer tomography-proven cardiac involvement by 3DSTE. During 3DSTE, reduction in some segmental radial, longitudinal, circumferential, area and 3D LV strains were found. Apical and basal LV rotations were found to be in the same counterclockwise direction, confirming near absence of LV twist - so-called rigid body rotation.

Different patterns of left ventricular rotational mechanics in cardiac amyloidosis-results from the three-dimensional speckle-tracking echocardiographic MAGYAR-Path Study.

Cardiac amyloidosis (CA) is an infiltrative disease primarily caused by extracellular tissue deposition of amyloid fibrils in the myocardial interstitium. The aim of the present study was to examine left ventricular (LV) rotational mechanics in biopsy-proven CA by three-dimensional (3D) speckle-tracking echocardiography (STE). Ten patients (65.3±11.5 years, 6 males) with CA entered the study. The mean basal LV rotations were 0.3±3.8°, while mean apical LV rotations proved to be 7.0±3.3°. LV basal and apical rotations were in the same counterclockwise direction in 6 out of 10 CA patients demonstrating near absence of LV twist [LV rigid body rotation (RBR)]. Apico-basal difference was near 3 or less degrees in three patients with LV-RBR, and 6-10 degrees in the other three subjects with LV-RBR. One another patient showed normal rotational mechanics, while two patients had significant hyporotations and one had significant hyperrotations in normal directions. To conclude with, different patterns of LV rotational mechanics could be demonstrated in CA. LV RBR, the near absence of LV twist seems to be a frequent phenomenon in CA.