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A chloroquint napjainkban szisztémás autoimmun kórképek kezelésére használják, autoimmun szöveti károsodást gátló és immunomoduláns hatása miatt. Ritka, ám súlyos mellékhatása a szernek, hogy restriktív cardiomyopathia-szerű képet okozhat, ami fenotípus és hisztológiai kép tekintetében egyaránt a Fabry-kór kardiális manifesztációjára emlékeztet. Munkánkban egy 73 éves nőbeteg esetét mutatjuk be, akinek fulmináns lefolyású szívelégtelensége hátterében chloroquin-cardiomyopathia állt. A beteg anamnézisében tartós chloroquinszedés szerepelt, rheumatoid arthritise miatt. 71 éves, korában harmadfokú atrioventricularis blokk miatt igényelt végleges pacemaker-implantációt. 2 évvel később kardiális dekompenzáció miatt került intézeti felvételre. Ekkor transthoracalis echokardiográfia során masszív koncentrikus bal kamrai hipertrófia, közepes fokban csökkent szisztolés balkamra-funkció és restriktív típusú mitrális beáramlási görbe volt észlelhető. A beteg a kéthetes hospitalizáció során kompenzálható volt, azonban 1 hónapon belül súlyos állapotban rehospitalizációra került sor, ami fatális kimenetelű volt. A klinikai kép és az autopsziás lelet alapján felmerült Fabry-betegség gyanúja. Tekintettel azonban a beteg tartós chloroquin szedésére, chloroquin indukálta cardiomyopathiát valószínűsítettünk. A chloroquin kardiális jellegű mellékhatásai – bár az alkalmazási előírásban említésre kerülnek – kevésbé ismertek. Ajánlásokra lenne szükség azzal kapcsolatban, hogy milyen gyakran, milyen módszerrel javasolt a tartósan chloroquint szedő betegek kardiológiai szűrése.

Background Progressive cardiac fibrosis is the central aspect of the myocardial involvement in systemic sclerosis (SSc). We hypothesized that circulating biomarkers of the cardiac fibrosis may be useful in the early diagnosis of the cardiac manifestation in this disease. Thus, we investigated the potential correlations between the levels of galectin-3, soluble suppression of tumorigenicity-2 (sST2) and the echocardiographic markers of the myocardial mechanics in SSc patients. Methods Forty patients (57.3 ± 13.7 years, 36 female) were investigated. In addition to the conventional echocardiography, tissue Doppler and speckle tracking-derived strain techniques were used to assess the function of both ventricles and atria. To estimate the correlations between galectin-3 and sST2 levels and the echocardiographic variables, partial correlation method was used with age as correcting factor. Results In age adjusted analysis galectin-3 level showed significant correlation with left ventricular global longitudinal strain (r = 0.460, p = 0.005); grade of left ventricular diastolic dysfunction (r = 0.394, p = 0.013); septal e’ (r = − 0.369, p = 0.021); septal E/e’ (r = 0.380, p = 0.017) and with the grade of mitral regurgitation (r = 0.323, p = 0.048). No significant correlation was found between sST2 levels and the echocardiographic variables. Conclusions Galectin-3 levels, but not sST2 levels show significant correlation with the parameters of the left ventricular systolic and diastolic function. Galectin-3 may be a useful biomarker for the screening and early diagnosis of SSc patients with cardiac involvement.

Funding Acknowledgements Type of funding sources: None. Background In systemic sclerosis (SSc) myocardial fibrosis contributes to the impairment of the myocardial function resulting in manifest diastolic and subclinical systolic dysfunction of the left ventricle (LV) and impaired left atrial (LA) function. The pathophysiology of this connective tissue disease is still not fully elucidated. Recently, however, elevated serum levels of galectin-3 and soluble suppression of tumorigenicity 2 (sST2) have been reported in SSc patients suggesting that these fibrotic markers may participate in the fibrotic process in this disease. Thus we aimed to investigate the potential associations between galectin-3 and sST2 levels and the echocardiographic markers of the LV and LA function in SSc patients. Methods Forty patients (57.3 ± 13.7 years, 36 female) were enrolled into the study. In addition to the conventional echocardiography, tissue Doppler and speckle-tracking-derived strain techniques were used to estimate the LV systolic and diastolic function as well as the LA size and function. To assess the correlations between galectin-3 and sST2 levels and the echocardiographic variables, partial correlation method was used with age as correcting factor. Results In age adjusted analysis galectin-3 level showed significant correlation with LV global longitudinal strain (r = 0.460, p = 0.005); grade of LV diastolic dysfunction (r = 0.394, p = 0.013); septal e’ (r=-0.369, p = 0.021); septal E/e’ (r = 0.380, p = 0.017) and with the grade of mitral regurgitation (r = 0.323, p = 0.048) (Figure 1). No significant correlation was found between sST2 levels and the echocardiographic variables. Conclusions Galectin-3 levels, but not sST2 levels show significant correlation with the parameters of the LV diastolic function and with LV global longitudinal strain, a parameter reflecting the subclinical impairment of the LV systolic function. Galectin-3 may be a useful biomarker for identifying SSc patients with high risk for cardiac involvement. Abstract Figure 1

Background Left ventricular diastolic dysfunction implies a worse prognosis in systemic sclerosis (SSc). Little is known, however, about the prognostic value of right ventricular (RV) and right atrial (RA) mechanics in this disease. Thus we aimed to investigate the long term prognostic value of the traditional and modern echocardiographic parameters of the RV and RA function as well as N-terminal pro B-type natriuretic peptide (NT-proBNP) levels in SSc patients. Patients and methods Seventy SSc patients (age: 57±12 years) were enrolled into the study. They underwent echocardiography in the years 2014–15. Parameters of the RV systolic function (tricuspid annular plane systolic excursion /TAPSE/, RV fractional area change /RVFAC/), inferior vena cava, collapsibility index, RV wall thickness were measured. Doppler data were collected: tricuspid E and A, peak velocity of tricuspid regurgitation (TR), tricuspid annular myocardial systolic (S), early- (e') and late- (a') diastolic velocities, tricuspid E/e' ratio. Maximal RA volume index as well as RA reservoir (εR), conduit (εCD) and contractile (εCT) strain were measured with the speckle tracking method. RA stiffness was calculated as ratio of E/e' to εR. Survival was assessed after 5 years. Since in some cases the cause of death was unknown, all-cause mortality was chosen as outcome. Results During the follow-up period of 4.7±0.9 years, 6 patients (8.6%) died. In univariate Cox regression analysis TAPSE, peak velocity of TR, tricuspid annular a' and S, tricuspid E/e' ratio, maximal RA volume index, RA stiffness and lnNT-proBNP were significantly associated with outcome. In multivariate Cox regression analysis RA stiffness was proved to be the only independent predictor of mortality (p=0.013). Using ROC analysis, RA stiffness ≥0.156 was the strongest predictor of the mortality (sensitivity=83.3%, specificity =89.1%, AUC=0.859). Conclusion Our results suggest that RA stiffness is an independent predictor of all-cause mortality in SSc. Larger prospective validation studies are required to prove our findings. ROC and Kaplan-Meier survival curve Funding Acknowledgement Type of funding source: None

Heart failure with preserved ejection fraction (HFpEF) is common in systemic sclerosis (SSc) and implies a worse prognosis therefore non-invasive assessment of left ventricular (LV) filling pressure is pivotal. Besides E/eʹ the use of maximal left atrial volume (LA Vmax index) is recommended. LA reservoir strain was also reported to be useful. The utility of LA stiffness, however, was never investigated in SSc. Thus we aimed to compare the diagnostic power of LA Vmax index, reservoir strain and stiffness in predicting elevated LV filling pressure in SSc patients. 72 SSc patients (age: 57 ± 11 years) were investigated. LA stiffness was calculated as ratio of E/eʹ to LA reservoir strain. Elevated LV filling pressure was defined as NT-proBNP > 220 pg/ml. Receiver-operating characteristic (ROC) curves were used to estimate the diagnostic performance of the investigated parameters. Average NT-proBNP level was 181 ± 154 pg/ml. NT-proBNP > 220 pg/ml was found in 21 SSc patients. LA stiffness showed the highest diagnostic performance in predicting NT-pro-BNP > 220 pg/ml, with a cut off value of 0.314 (Area under the curve: 0.719, specificity: 89.4%, sensitivity: 42.1%). AUC values for LA reservoir strain and Vmax index were 0.595 and 0.521, respectively. LA stiffness was superior to Vmax index and reservoir strain in predicting elevated NT-proBNP levels in SSc patients. Although invasive validation studies on larger samples are required, our data suggest, that the use of LA stiffness may significantly contribute to diagnostic precision in populations with a high suspicion of HFpEF.

Right ventricular (RV) systolic failure is rare in patients with COPD, but they often develop RV diastolic dysfunction. Left ventricular (LV) diastolic dysfunction is also common in this population. Nevertheless, data are scarce regarding the effect of diastolic dysfunction on the functional capacity in patients with COPD. We investigated the correlation between echocardiographic parameters of RV and LV diastolic function and the exercise capacity in COPD, by using conventional echocardiographic methods and tissue Doppler imaging. 65 patients with COPD (61 ± 9 years) in stages GOLD II-IV were investigated. Functional capacity was measured with 6-minute walk test (6MWT). Right (RA) and left atrial (LA) area index were measured; collapsibility index inferior vena cava was calculated. Parameters of the mitral and tricuspid inflow (E, A) as well as annular systolic (S), early- (e') and late- (a') diastolic myocardial longitudinal velocities were measured. E/A, E/e' and e'/a' ratios were calculated. 6MWT distance was 330 ± 76 m. LV diastolic dysfunction was found in 48 (74%) patients. LV and RV filling pressures were elevated in 28 (43%) and in 29 (45%) patients, respectively. In the left heart, LA area index showed significant correlation with the functional capacity (r = -0.319; p = 0.011). In stepwise multiple linear regression analysis tricuspid e'/a' (r = 0.611; p = 0.000), collapsibility index (r = 0.505; p = 0.000), RA area index (r = -0.445; p = 0.000) and body surface area (r = 0.314; p = 0.011) were independent predictors of 6MWT distance. Right ventricular diastolic function and filling pressure have strong influence on the functional capacity in patients with COPD.