18 research outputs found
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
Mit kell tudnunk a cardialis amyloidosisról? A tünettantól a kezelésig | What should we know about cardiac amyloidosis? From clinical signs to treatment
Absztrakt:
A szisztémás amyloidosis ritka betegség, amelyben a szívérintettség viszonylag
gyakran fordul elő és a túlélést jelentősen befolyásolja. Az alapbetegség és a
szervi érintettség szempontjából új diagnosztikus eljárások segítenek a korai
diagnózis felállításában és a mihamarabbi kezelés megkezdésében. Szívérintettség
inkább a monoklonális immunglobulin-könnyűlánc (AL-amyloidosis) és a
transthyretin formában fordul elő. AL-amyloidosis esetén a szívérintettség
súlyos következményekhez vezet. A kezdeti súlyosság megítélésében és a kezelésre
adott válasz mértékének mérésében a szívfunkcióval kapcsolatos
biomarker-vizsgálatok segítenek. Amyloidosis esetén az életkorral nő a
szívérintettség, a prevalencia nem ismert pontosan, de feltételezhető, hogy több
eset van valójában, mint amennyi felismerésre kerül. A szerzők a klinikai
tünetek, diagnosztikus eljárások, kiemelten a kardiológiai vizsgálatok
jelentőségét ismertetik. Orv Hetil. 2017; 158(46): 1811–1818.
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Abstract:
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
Bal kamrai deformáció cardialis könnyűlánc-amyloidosisban és hypereosinophilia-szindrómában. Eredmények a MAGYAR-Path Tanulmányból = Left ventricular deformation in cardiac light-chain amyloidosis and hypereosinophilic syndrome Results from the MAGYAR-Path Study
Absztrakt:
Bevezetés: A hypereosinophilia-szindróma (HES) és az
immunglobulinkönnyűlánc-amyloidosis (ALA) két ritka hematológiai betegség,
melyek cardialis eltérésekkel járnak együtt. Célkitűzés: A
jelen vizsgálat célja a HES- és ALA-betegek bal kamrai (BK-i) deformációs
paramétereinek összehasonlító vizsgálata volt háromdimenziós speckle-tracking
echokardiográfia (3DSTE) segítségével. Módszer: A vizsgálatok
során 10 HES-beteg (átlagos életkor: 60,9 ± 14,7 év) és 19 ALA-ban szenvedő
páciens (átlagos életkor: 63,4 ± 7,8 év, 13 férfi) eredményeit elemeztük.
Kontrollcsoportként 13, korban és nemben egyeztetett, egészséges felnőtt
szolgált (átlagos életkor: 59,2 ± 4,3 év, 5 férfi). Valamennyi esetben teljes
körű kétdimenziós Doppler-echokardiográfiás vizsgálat készült 3DSTE-vel
kiegészítve. Eredmények: A kontrollcsoporthoz képest az
ALA-betegcsoportban mért valamennyi basalis szegmentális BK-i strain
szignifikánsan alacsonyabbnak mutatkozott. Az ALA-betegek globális és átlagolt
szegmentális BK-i longitudinális strain (LS) értékei az egészséges
kontrollcsoporthoz hasonlítva szignifikánsan alacsonyabbnak bizonyultak. A
HES-betegcsoport és az egészséges kontrollok összehasonlítása során szignifikáns
különbséget tapasztaltunk a globális BK-LS tekintetében, míg a szegmentális
basalis BK-LS is szignifikánsan alacsonyabbnak bizonyult a HES-betegekben. A
HES- és az ALA-betegcsoport értékeit összehasonlítva a basalis BK-i radiális és
3D strain mutatott szignifikáns eltérést. Következtetések: A
3DSTE alkalmas módszer a HES- és az ALA-betegcsoportban a BK-i deformációs
mechanika részletes vizsgálatára. Mindkét betegcsoportban jelentős deformációs
eltérések tapasztalhatók, ALA fennállása esetén az eltérések kifejezettebbek.
Orv Hetil. 2020; 161(5): 169–176.
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Abstract:
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
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