A Fabry-kór a lizoszomális tárolási betegségek csoportjába tartozó, X-kromoszómához kötötten, rec... more A Fabry-kór a lizoszomális tárolási betegségek csoportjába tartozó, X-kromoszómához kötötten, recesszív módon öröklődő betegség, amely a globotriaosylceramid felhalmozódásához vezet a szervezet legkülönbözőbb szöveteiben. A betegség első tünetei többnyire gyermekkorban jelentkeznek, a progresszió során a betegek súlyos szervi károsodásokkal és korai halálozással számolhatnak. Elsősorban fi úk és férfi ak érintettek, azonban a betegség tüneteit heterozigóta nők esetében is megfi gyelhetjük, de náluk a kórkép súlyossága változó, általában enyhébb lefolyású. Az enzimpótló kezelések megjelenése szükségessé tette, hogy részletes diagnosztikus és terápiás protokollt dolgozzunk ki. A jelen dolgozatban megjelenő ajánlásokat egy, a magyarországi Fabry-betegek kezelésében aktívan részt vevő orvosokból, a diagnosztika területén dolgozó biológosukból és egyéb szakemberekből álló multidiszciplináris munkacsoport foglalta össze. A munkacsoport áttekintette a korábbi klinikai tanulmányokat, a publikált vizsgálatokat és a közelmúltban megjelent nemzetközi és nemzeti útmutatókat. Kulcsszavak: Fab y-betegség, diagnosztika, enzimpótló terápia Fabry disease diagnostic guideline Fabry disease is a rare, X-linked lysosomal storage disorder that leads to accumulation of globotriaosylceramide in different tissues of the body. The disease is progressive, fi rst symptoms usually present in childhood. Consequencies of the diseases are disability and premature death. The disease in females could be as severe as in males although women may also be asymptomatic. The possibility of enzyme replacement therapy has made it necessary to elaborate a comprehensive guideline for the diagnosis and treatment follow-up. The guideline was established by a Hungarian multidisciplinary working group, consisting of physicians who are involved in health care of Fabry patients. Previous clinical studies, published materials, and recently established international treatment guidelines were reviewed by the group.
The present investigation evaluates the capacity of Allium ursinum (wild garlic) leaf lyophilisat... more The present investigation evaluates the capacity of Allium ursinum (wild garlic) leaf lyophilisate (WGLL; alliin content: 0.261%) to mitigate cardiovascular damage in hypercholesterolemic rabbits. New Zealand rabbits were divided into three groups: (i) cholesterol-free rabbit chow (control); (ii) rabbit chow containing 2% cholesterol (hypercholesterolemic, HC); (iii) rabbit chow containing 2% cholesterol + 2% WGLL (hypercholesterolemic treated, HCT); for eight weeks. At the zero-and eight-week time points, echocardiographic measurements were made, along with the determination of basic serum parameters. Following the treatment period, after ischemia-reperfusion injury, hemodynamic parameters were measured using an isolated working heart model. Western blot analyses of heart tissue followed for evaluating protein expression and histochemical study for the atheroma status determination. WGLL treatment mediated increases in fractional shortening; right ventricular function; peak systolic velocity; tricuspidal annular systolic velocity in live animals; along with improved aortic and coronary flow. Western blot analysis revealed WGLL-associated increases in HO-1 protein and decreases in SOD-1 protein production. WGLL-associated decreases were observed in aortic atherosclerotic plaque coverage, plasma ApoB and the activity of LDH and CK (creatine kinase) in plasma. Plasma LDL was also significantly reduced. The results clearly demonstrate that WGLL has complex cardioprotective effects, suggesting future strategies for its use in prevention and therapy for atherosclerotic disorders.
QT interval prolongation on the electrocardiogram is considered a precursory sign for imminent, p... more QT interval prolongation on the electrocardiogram is considered a precursory sign for imminent, potentially lethal ventricular arrhythmias. Beside the inherited condition of long QT syndrome, numerous drugs, certain electrolyte disturbances and early transmural ischemia have been identified to induce reversible prolongation of the QT interval, collectively called as acquired long QT syndrome. Herein we describe a case of a patient with transient QT prolongation and Takotsubo cardiomyopathy, a rather infrequent cause of long QT development. Serial changes of the repolarization pattern were documented to demonstrate progression and resolution of the abnormal QT interval.
A Kawasaki-betegség egy főleg csecsemők és kisgyermekek körében előforduló akut immun vasculitis,... more A Kawasaki-betegség egy főleg csecsemők és kisgyermekek körében előforduló akut immun vasculitis, amelynek szövődményeként szívkoszorúér-aneurizmák és okklúziók alakulhatnak ki. Hosszú távú hatásaival kapcsolatban főként távol-keleti adatokra támaszkodhatunk, ahol a betegség epidemiológiai jellegzetességei eltérnek a kaukázusi populációban tapasztaltaktól. Célunk volt a Kawasaki-betegek hosszú távú nyomonkövetésével felmérendő, hogy a gyermekkori betegség után milyen kardiovaszkuláris szövődményük észlelhető. A betegekről regisztert is létrehozzunk, a könnyebb utánkövethetőség céljából. Módszerek: A kutatást a Semmelweis Egyetem Városmajori Szív-és Érgyógyászati Klinikán 1990 óta regisztrált, Kawasaki-betegségen átesettekből álló beteganyagon végeztük. A betegeken szív MR, koronária CT és echokardiográfiás vizsgálatokat végeztünk, utóbbi esetben a rutin paramétereken felül speckle-tracking deformációs analízist is használva, amelynek ezen betegcsoportban hosszú távú eredményiről nincs adat. A retrospektív vizsgálat keretében a páciensektől nyert adatokat összevetettük egy illesztett kontrollcsoport tagjainak eredményeivel. Eredmények: 54 beteg (átlagéletkor: 16±6 év) eredményeit hasonlítottuk össze egy 19 fős kontrollcsoport értékeivel. A betegség átlagosan 3 éves korban jelentkezett. Az átlagos nyomonkövetési idő 9±4,9 év. Mindenkinél készült szív MR-vizsgálat, a jelen tanulmányhoz felhasznált első vizsgálat átlagosan 11 éves korban készült és a betegek 24%-ában mutatott ki koszorúér-léziót. Az echokardiográfiával meghatározott bal kamrai végdiasztolés volumen és ejekciós frakció a kontrollcsoporthoz hasonlóan normáltartományban volt (Kawasaki vs. kontroll: EDV: 57±26 vs. 52±23 ml, EF: 67±8 vs. 67±7%) és a speckle-tracking echokardiográfiás deformációs paraméterek sem utaltak szisztolés diszfunkcióra (GLS:-18±5 vs.-18±4%). Következtetések: A fejlett kardiovaszkuláris képalkotó módszerek elérhetőségével növekvő számban lehetséges olyan gyermekek és felnőttek felismerése, akik korábban érintettek voltak a betegségben. Regiszterünk célja a betegség retrospektív jellemzése a magyar populációban, illetve a beválasztott betegek prospektív utánkövetése a betegség hosszú távú kardiovaszkuláris hatásainak felismerésére. Kawasakidiseasefromchildrentoadults-Roleofmultimodalityimaginginlongtermfollow-up Kawasaki disease (KD) is an acute immune vasculitis, occurring mainly in infants and children, where inflammation can lead to formation of coronary artery aneurysms and occlusions. Long-term effects of KD mainly rely on data from the Far East, where the epidemiological features of the disease are different from the Caucasian population. Our goal was to assess the cardiovascular complication after childhood KD. We created a registry that will help in the evaluation the incidence and long term outcome of KD. Methods: We have collected data from KD patients who have been registered since 1990 at Semmelweis University Heart and Vascular Center. The patients were studied using cardiac MR, coronary CT and echocardiography. In the latter method, we used speckle-tracking deformation analysis in addition to the routine parameters. Through the retrospective study we compared the results of patients who have had KD to data obtained from members of a matched control group. Results: The cardiac imaging results of a total of 54 patients (16±6 years average age) were compared to data obtained from a 19-person control group. The disease occurred among children at an average of 3 years of age. Follow-up 9±4,9 years. Each of our patients had cardiac MRI; the first examination used in this study was at an average of 11 years of age, and coronary lesions occurred in 24% of patients. Echocardiography measurements showed that end-diastolic volume and ejection fraction was in the normal range, similar to the control group (KD vs. control: EDV: 57±26 vs. 52 ± 23 ml, EF: 67±8 vs. 67±7 ml) and neither the speckle-tracking echocardiography deformation parameters showed systolic dysfunction (GLS:-18±5 vs.-18±4%). Conclusions:Using the advanced cardiovascular imaging methods, it is possible to recognize an increasing number of cases in children and adults, who were previously involved in KD. The purpose of our register is the retrospective characterization of this disease in the Hungarian population, and the prospective follow-up of enrolled patients, to detect long-term cardiovascular effects of the disease.
International Journal of Molecular Sciences, Sep 17, 2013
The present study evaluates a hypothesis that diet-related hypercholesterolemia increases oxidati... more The present study evaluates a hypothesis that diet-related hypercholesterolemia increases oxidative stress-related burden to cardiovascular tissue, resulting in progressively increased mortality, along with deterioration of electrophysiological and enzymatic function in rabbit myocardium. New Zealand white rabbits were divided into four groups, defined as follows: GROUP I, cholesterol-free rabbit chow for 12 weeks; GROUP II, cholesterol-free chow, 40 weeks; GROUP III, chow supplemented with 2% cholesterol, 12 weeks; GROUP IV, chow supplemented with 2% cholesterol, 40 weeks. At the 12 and 40 weeks time points, animals in each of the aforementioned cohorts were subjected to echocardiographic measurements, followed by sacrifice.
Background: Growing evidence exists for soluble Angiotensin Converting Enzyme-2 (sACE2) as a biom... more Background: Growing evidence exists for soluble Angiotensin Converting Enzyme-2 (sACE2) as a biomarker in definitive heart failure (HF), but there is little information about changes in sACE2 activity in hypertension with imminent heart failure and in reverse remodeling. Methods, Findings: Patients with systolic HF (NYHAII-IV, enrolled for cardiac resynchronisation therapy, CRT, n = 100) were compared to hypertensive patients (n = 239) and to a healthy cohort (n = 45) with preserved ejection fraction (EF.50%) in a single center prospective clinical study. The status of the heart failure patients were checked before and after CRT. Biochemical (ACE and sACE2 activity, ACE concentration) and echocardiographic parameters (EF, left ventricular enddiastolic (EDD) and end-systolic diameter (ESD) and dP/dt) were measured. sACE2 activity negatively correlated with EF and positively with ESD and EDD in all patient's populations, while it was independent in the healthy cohort. sACE2 activity was already increased in the hypertensive group, where signs for imminent heart failure (slightly decreased EF and barely increased NT-proBNP levels) were detected. sACE2 activities further increased in patients with definitive heart failure (EF, 50%), while sACE2 activities decreased with the improvement of the heart failure after CRT (reverse remodeling). Serum angiotensin converting enzyme (ACE) concentrations were lower in the diseased populations, but did not show a strong correlation with the echocardiographic parameters. Conclusions: Soluble ACE2 activity appears to be biomarker in heart failure, and in hypertension, where heart failure may be imminent. Our data suggest that sACE2 is involved in the pathomechanism of hypertension and HF.
Several transcatheter techniques based on radiofrequency energy were elaborated for the treatment... more Several transcatheter techniques based on radiofrequency energy were elaborated for the treatment of atrial fibrillation through the last decade. Recently, similar success rates with a better safety profile concerning life threatening complications were reported with the novel methode of cryoballon isolation of the pulmonary veins. This paper summarizes our initial experience with cryoballon ablation after the first 55 patients. Methode: Symptomatic patients refractory to aniarrhythmic medication mostly with paroxysmal atrial fibrillation without significant structural heart disease were enrolled. Cannulation and isolation of all pulmonary veins were attempted using a 28 mm double-wall cryoballon inflated at the ostium of the vein and abolishing eletrical activity of atrial tissue around its perimeter by freezing to –70 C. Intravenous heparin during and oral anticoagulant after the procedure was administered. Conventional ECGs, Holter ECGs and transtelephonic ECG recordings were used through 6 months follow-up for rhythm monitoring. Results: In 55 patients enrolled (18 female; age: 56±33,64 years) 165 out ot 192 (86%) pulmonary veins were successfully isolated. All pulmonary veins were isolated in 37 patients (67%). Procedure time was 155.67±100.66 min, while fluoroscopy time was 34.04±31.89 min. In 34 patients with 6 months follow-up 24 (70%) either remained free of arrhythmia (17 patients) or had a significant decrease in arrhythmia burden (7 patients). Conclusion: Based on our initial experience, cryoballon isolation of pulmonary veins appears to be a more simple procedure with similar efficacy to radiofrequency ablation in the treatment of atrial fibrillation.
Fabry disease is an X chromosome linked disorder caused by the inherited deficiency of lysosomal ... more Fabry disease is an X chromosome linked disorder caused by the inherited deficiency of lysosomal enzyme α- galactosidase A. The deficiency results in abnormal degradation of certain glycosphingolipids. Although the disease is known for more than hundred years and the underlying molecular basis is getting to be well defined, there are still a lot of unanswered questions regarding the different clinical presentations, available diagnostic procedures and therapeutic interventions. The scope of the article is to review the molecular basis of Fabry disease and summarize the available data about Fabry disease cardiomyopathy, highlight the controversies of current knowledge and evaluate future research directions.
The present study examined the efficacy of using longevinex, a commercially available resveratrol... more The present study examined the efficacy of using longevinex, a commercially available resveratrol formulation, to lower blood cholesterol in hypercholesteromic rabbits. New Zealand white rabbits were randomly divided into two groups (n = 6 per group), one group was given high cholesterol diet for 3 months while the other group fed regular diet served as control. The high cholesterol diet fed group was further subdivided into two groups (n = 6 per group), one group was given longevinex resveratrol while the other group given vehicle only served as control. Longevinex was given by gavaging up to a period of 6 months. Longevinex-treated rabbits exhibited lowering of plasma cholesterol level. Inhibition of arterial plaque formation was noticed even after 1 month. Longevinextreated hearts demonstrated improved ventricular recovery when isolated working hearts were subjected to 30 min of ischemia followed by 2 h of reperfusion. Aortic flow and developed pressure during post-ischemic reperfusion period were significantly higher for the longevinex-treated hearts compared to those in control group of hearts. Myocardial infarct size was also lower in the treated group compared to that for the untreated group. These results indicate cholesterol-lowering ability of longevinex, which appears to reflect in its ability to protect the hypercholesteromic hearts from ischemic reperfusion injury.
Hypothesis. The present study evaluates the hypothesis that sour cherry seed extract (SCSE) prote... more Hypothesis. The present study evaluates the hypothesis that sour cherry seed extract (SCSE) protects against cardiovascular disease and inflammation in hypercholesterolemic rabbits, and that this protection correlates with SCSE-induced activity of heme oxygenase-1 (HO-1), a cytoprotective enzyme contributing to oxidative stress responses. Methods: 18 New Zealand white rabbits were divided into three groups receiving: I. cholesterol-free rabbit chow; II. chow containing 2% cholesterol; or III. 2% cholesterol plus SCSE for 16 weeks. Heart functions were monitored by echocardiography 0, 4, and 16 weeks after the initiation of cholesterol-supplemented feeding. At the 16-week time-point, isolated hearts were subjected to ischemia-reperfusion (I/R), followed by measurement of heart rate (HR), aortic flow (AF), coronary flow (CF), aortic pressure (AoP), and left ventricular developed pressure (LVDP). Myocardial infarct size was determined using triphenyl tetrazolium chloride (TTC). Quantification of fatty streaks was assessed using Sudan-III staining. Western blot analysis was used to determine the content of cytochrome c oxidase III (COX III), vascular endothelial growth factor (VEGF), and HO-1 in the myocardium. Results: Relative to cholesterol-treated animals not receiving SCSE, SCSE-treated animals exhibited significantly improved cardiac function and improved peak early diastolic velocity to peak atrial velocity ratio (E'/A'), along with decreased atherosclerotic plaque formation and infarct size. Increased HO-1 and COX III protein expression and COX activity were also noted in hearts from SCSE-treated rabbits. Conclusions: This study demonstrates SCSE cardioprotective effects on hypercholesterolemic hearts. Correlation of these outcomes with HO-1 expression suggests that the effect may be mediated by activity of this enzyme. However, definitive proof of HO-1 dependence requires further investigation.
Several transcatheter techniques based on radiofrequency energy were elaborated for the treatment... more Several transcatheter techniques based on radiofrequency energy were elaborated for the treatment of atrial fibrillation through the last decade. Recently, similar success rates with a better safety profile concerning life threatening complications were reported with the novel methode of cryoballon isolation of the pulmonary veins. This paper summarizes our initial experience with cryoballon ablation after the first 55 patients. Methode: Symptomatic patients refractory to aniarrhythmic medication mostly with paroxysmal atrial fibrillation without significant structural heart disease were enrolled. Cannulation and isolation of all pulmonary veins were attempted using a 28 mm double-wall cryoballon inflated at the ostium of the vein and abolishing eletrical activity of atrial tissue around its perimeter by freezing to –70 C. Intravenous heparin during and oral anticoagulant after the procedure was administered. Conventional ECGs, Holter ECGs and transtelephonic ECG recordings were used through 6 months follow-up for rhythm monitoring. Results: In 55 patients enrolled (18 female; age: 56±33,64 years) 165 out ot 192 (86%) pulmonary veins were successfully isolated. All pulmonary veins were isolated in 37 patients (67%). Procedure time was 155.67±100.66 min, while fluoroscopy time was 34.04±31.89 min. In 34 patients with 6 months follow-up 24 (70%) either remained free of arrhythmia (17 patients) or had a significant decrease in arrhythmia burden (7 patients). Conclusion: Based on our initial experience, cryoballon isolation of pulmonary veins appears to be a more simple procedure with similar efficacy to radiofrequency ablation in the treatment of atrial fibrillation.
Klinikánkon négy éve alkalmazunk perkután PD katéter-beültetést és peritoenális dialízis kezelést... more Klinikánkon négy éve alkalmazunk perkután PD katéter-beültetést és peritoenális dialízis kezelést a súlyosan csökkent bal kamra szisztolés funkcióval bíró, diuretikum-refrakter szívelégtelen betegek körében. Célul tűztük ki, hogy leírjuk centrumunk ebben a betegcsoportban szerzett tapasztalatát. Betegek, módszerek: 2014 áprilisától követtük betegeinket a követési idő vége 2018. január volt. Eredmények: Hat páciensünk közül háromnál nem iszkémiás etiológiájú csökkent ejekciós frakciójú szívelégtelenség, háromnál iszkémiás etiológiájú csökkent ejekciós frakciójú szívelégtelenség talaján alakult végstádiumú szívelégtelenség. Charlson komorbiditási index alapján 6-10 pont közötti értékeik voltak. A PD katéterbeültetés perkután technikával történt, az ultrafiltrációt azonnal el tudtuk kezdeni. Két betegünk sikeres szívtranszplantáción esett át. Három betegünk hunyt el, halálok nem PD asszociált szövődmény volt, 1 traumás, 1 egyéb infekció, 1 szívelégtelenség. Következtetések: A diuretikum-refrakter szívelégtelenség esetén a peritonealis dialízissel javulhat a jobb és bal kamra funkciója is. Centrumunkban kezeltünk elsőként PD-vel-áthidaló terápiaként-, hazánkban szívelégtelen beteget a szívtranszplantációig. Peritoneal ultrafiltration for refractory heart failure: single-center experience Introduction: In our centre we have been treated patients with therapy refracter congestive heart failure. The aim of this study was to describe a single-center experience in the treatment of refractory HF patients with peritoneal dialysis (PD). Methods: Our study included in a single PD Unit, showing symptoms and signs of severe refractory congestive HF to optimal pharmacological therapy (NYHA class IV). We followed them till January 2018. Results: Patients were followed for 10 months; population mean age was 58 years and Charlson's comorbidity index was 6-10. We could start peritoneal dialysis immediately after catheter implantation. Three patients died but not according to PD associated infection: 1 traumatic, 1 due to HF, another patient due to infection. Conclusion: PD, applied to refractory HF can improve functional class. In Hungary we used peritoneal dialysis first time as a bridge therapy to heart transplantation in diuretic refracter heart failure.
Esetismertetés Egy iszkémiás cardiomyopathiában szenvedő 55 éves férfi betegnél súlyos koronáriab... more Esetismertetés Egy iszkémiás cardiomyopathiában szenvedő 55 éves férfi betegnél súlyos koronáriabetegség miatt elvégzett koronária bypass-műtét és optimális gyógyszeres kezelés ellenére előrehaladott szívelégtelenség tüneteit, a balkamra-funkció progresszív romlását észleltük. Számos alkalommal hospitalizáltuk, mígnem diuretikum-refrakter ödémái miatt peritonealis dialízis (PD) elindítása mellett döntöttünk. Rendszeres napi háromszori PD-kezelés után a vesefunkció javulása és a testsúly csökkenése mellett a bal kamrai szisztolés és diasztolés funkció javulását észleltük. A Swan-Ganz-mérések alapján a PD-kezelés elindítását követően a pulmonalis vaszkuláris rezisztencia jelentősen csökkent. A beteg a PD-kezelés elkezdését követően kórházi felvételt szívelégtelenség tünetei miatt nem igényelt, végül sikeres szívtranszplantáción esett át. Peritoneal dialysis as bridge therapy to heart transplantation A 53-year old male patient suffering from ischaemic cardiomyopathy underwent coronary bypass operation showed progressive signs of cardiac decompensation and deterioration of left ventricular (LV) ejection fraction despite optimal medical treatment. The patient was hospitalized several times because of recurrent peripheral oedema, which proved to be refractory to diuretic treatment. Therefore peritoneal dialysis (PD) was initiated. Through regular (three times daily) fluid exchange renal function improved, which was accompanied by a remarkable reduction in the body weight and a significant improvement of LV systolic and diastolic function. Moreover, subsequent Swan-Ganz catheterizations showed a significant decrease in the pulmonary vascular resistance. After the initiation of the PD, the patient did not require hospitalization for worsening heart failure and underwent successful heart transplantation.
Patient Forum The content of these European Society of Cardiology (ESC) / European Association fo... more Patient Forum The content of these European Society of Cardiology (ESC) / European Association for Cardio-Thoracic Surgery (EACTS) Guidelines has been published for personal and educational use only. No commercial use is authorized. No part of the ESC/EACTS Guidelines may be translated or reproduced in any form without written permission from the ESC and the EACTS. Permission can be obtained upon submission of a written request to Oxford University Press, the publisher of the European Heart Journal and the party authorized to handle such permissions on behalf of the ESC ([email protected]). Disclaimer: The ESC/EACTS Guidelines represent the views of the ESC and the EACTS and were produced after careful consideration of the scientific and medical knowledge and the evidence available at the time of their publication. The ESC and the EACTS are not responsible in the event of any contradiction, discrepancy and/or ambiguity between the ESC/EACTS Guidelines and any other official recommendations or guidelines issued by the relevant public health authorities, in particular in relation to good use of healthcare or therapeutic strategies. Health professionals are encouraged to take the ESC/EACTS Guidelines fully into account when exercising their clinical judgment, as well as in the determination and the implementation of preventive, diagnostic or therapeutic medical strategies; however, the ESC/EACTS Guidelines do not override, in any way whatsoever, the individual responsibility of health professionals to make appropriate and accurate decisions in consideration of each patient's health condition and in consultation with that patient and, where appropriate and/or necessary, the patient's caregiver. Nor do the ESC/EACTS Guidelines exempt health professionals from taking into full and careful consideration the relevant official updated recommendations or guidelines issued by the competent public health authorities, in order to manage each patient's case in light of the scientifically accepted data pursuant to their respective ethical and professional obligations. It is also the health professional's responsibility to verify the applicable rules and regulations relating to drugs and medical devices at the time of prescription.
Fabry disease is an X chromosome linked disorder caused by the inherited deficiency of lysosomal ... more Fabry disease is an X chromosome linked disorder caused by the inherited deficiency of lysosomal enzyme α- galactosidase A. The deficiency results in abnormal degradation of certain glycosphingolipids. Although the disease is known for more than hundred years and the underlying molecular basis is getting to be well defined, there are still a lot of unanswered questions regarding the different clinical presentations, available diagnostic procedures and therapeutic interventions. The scope of the article is to review the molecular basis of Fabry disease and summarize the available data about Fabry disease cardiomyopathy, highlight the controversies of current knowledge and evaluate future research directions.
A Fabry-kór a lizoszomális tárolási betegségek csoportjába tartozó, X-kromoszómához kötötten, rec... more A Fabry-kór a lizoszomális tárolási betegségek csoportjába tartozó, X-kromoszómához kötötten, recesszív módon öröklődő betegség, amely a globotriaosylceramid felhalmozódásához vezet a szervezet legkülönbözőbb szöveteiben. A betegség első tünetei többnyire gyermekkorban jelentkeznek, a progresszió során a betegek súlyos szervi károsodásokkal és korai halálozással számolhatnak. Elsősorban fi úk és férfi ak érintettek, azonban a betegség tüneteit heterozigóta nők esetében is megfi gyelhetjük, de náluk a kórkép súlyossága változó, általában enyhébb lefolyású. Az enzimpótló kezelések megjelenése szükségessé tette, hogy részletes diagnosztikus és terápiás protokollt dolgozzunk ki. A jelen dolgozatban megjelenő ajánlásokat egy, a magyarországi Fabry-betegek kezelésében aktívan részt vevő orvosokból, a diagnosztika területén dolgozó biológosukból és egyéb szakemberekből álló multidiszciplináris munkacsoport foglalta össze. A munkacsoport áttekintette a korábbi klinikai tanulmányokat, a publikált vizsgálatokat és a közelmúltban megjelent nemzetközi és nemzeti útmutatókat. Kulcsszavak: Fab y-betegség, diagnosztika, enzimpótló terápia Fabry disease diagnostic guideline Fabry disease is a rare, X-linked lysosomal storage disorder that leads to accumulation of globotriaosylceramide in different tissues of the body. The disease is progressive, fi rst symptoms usually present in childhood. Consequencies of the diseases are disability and premature death. The disease in females could be as severe as in males although women may also be asymptomatic. The possibility of enzyme replacement therapy has made it necessary to elaborate a comprehensive guideline for the diagnosis and treatment follow-up. The guideline was established by a Hungarian multidisciplinary working group, consisting of physicians who are involved in health care of Fabry patients. Previous clinical studies, published materials, and recently established international treatment guidelines were reviewed by the group.
The present investigation evaluates the capacity of Allium ursinum (wild garlic) leaf lyophilisat... more The present investigation evaluates the capacity of Allium ursinum (wild garlic) leaf lyophilisate (WGLL; alliin content: 0.261%) to mitigate cardiovascular damage in hypercholesterolemic rabbits. New Zealand rabbits were divided into three groups: (i) cholesterol-free rabbit chow (control); (ii) rabbit chow containing 2% cholesterol (hypercholesterolemic, HC); (iii) rabbit chow containing 2% cholesterol + 2% WGLL (hypercholesterolemic treated, HCT); for eight weeks. At the zero-and eight-week time points, echocardiographic measurements were made, along with the determination of basic serum parameters. Following the treatment period, after ischemia-reperfusion injury, hemodynamic parameters were measured using an isolated working heart model. Western blot analyses of heart tissue followed for evaluating protein expression and histochemical study for the atheroma status determination. WGLL treatment mediated increases in fractional shortening; right ventricular function; peak systolic velocity; tricuspidal annular systolic velocity in live animals; along with improved aortic and coronary flow. Western blot analysis revealed WGLL-associated increases in HO-1 protein and decreases in SOD-1 protein production. WGLL-associated decreases were observed in aortic atherosclerotic plaque coverage, plasma ApoB and the activity of LDH and CK (creatine kinase) in plasma. Plasma LDL was also significantly reduced. The results clearly demonstrate that WGLL has complex cardioprotective effects, suggesting future strategies for its use in prevention and therapy for atherosclerotic disorders.
QT interval prolongation on the electrocardiogram is considered a precursory sign for imminent, p... more QT interval prolongation on the electrocardiogram is considered a precursory sign for imminent, potentially lethal ventricular arrhythmias. Beside the inherited condition of long QT syndrome, numerous drugs, certain electrolyte disturbances and early transmural ischemia have been identified to induce reversible prolongation of the QT interval, collectively called as acquired long QT syndrome. Herein we describe a case of a patient with transient QT prolongation and Takotsubo cardiomyopathy, a rather infrequent cause of long QT development. Serial changes of the repolarization pattern were documented to demonstrate progression and resolution of the abnormal QT interval.
A Kawasaki-betegség egy főleg csecsemők és kisgyermekek körében előforduló akut immun vasculitis,... more A Kawasaki-betegség egy főleg csecsemők és kisgyermekek körében előforduló akut immun vasculitis, amelynek szövődményeként szívkoszorúér-aneurizmák és okklúziók alakulhatnak ki. Hosszú távú hatásaival kapcsolatban főként távol-keleti adatokra támaszkodhatunk, ahol a betegség epidemiológiai jellegzetességei eltérnek a kaukázusi populációban tapasztaltaktól. Célunk volt a Kawasaki-betegek hosszú távú nyomonkövetésével felmérendő, hogy a gyermekkori betegség után milyen kardiovaszkuláris szövődményük észlelhető. A betegekről regisztert is létrehozzunk, a könnyebb utánkövethetőség céljából. Módszerek: A kutatást a Semmelweis Egyetem Városmajori Szív-és Érgyógyászati Klinikán 1990 óta regisztrált, Kawasaki-betegségen átesettekből álló beteganyagon végeztük. A betegeken szív MR, koronária CT és echokardiográfiás vizsgálatokat végeztünk, utóbbi esetben a rutin paramétereken felül speckle-tracking deformációs analízist is használva, amelynek ezen betegcsoportban hosszú távú eredményiről nincs adat. A retrospektív vizsgálat keretében a páciensektől nyert adatokat összevetettük egy illesztett kontrollcsoport tagjainak eredményeivel. Eredmények: 54 beteg (átlagéletkor: 16±6 év) eredményeit hasonlítottuk össze egy 19 fős kontrollcsoport értékeivel. A betegség átlagosan 3 éves korban jelentkezett. Az átlagos nyomonkövetési idő 9±4,9 év. Mindenkinél készült szív MR-vizsgálat, a jelen tanulmányhoz felhasznált első vizsgálat átlagosan 11 éves korban készült és a betegek 24%-ában mutatott ki koszorúér-léziót. Az echokardiográfiával meghatározott bal kamrai végdiasztolés volumen és ejekciós frakció a kontrollcsoporthoz hasonlóan normáltartományban volt (Kawasaki vs. kontroll: EDV: 57±26 vs. 52±23 ml, EF: 67±8 vs. 67±7%) és a speckle-tracking echokardiográfiás deformációs paraméterek sem utaltak szisztolés diszfunkcióra (GLS:-18±5 vs.-18±4%). Következtetések: A fejlett kardiovaszkuláris képalkotó módszerek elérhetőségével növekvő számban lehetséges olyan gyermekek és felnőttek felismerése, akik korábban érintettek voltak a betegségben. Regiszterünk célja a betegség retrospektív jellemzése a magyar populációban, illetve a beválasztott betegek prospektív utánkövetése a betegség hosszú távú kardiovaszkuláris hatásainak felismerésére. Kawasakidiseasefromchildrentoadults-Roleofmultimodalityimaginginlongtermfollow-up Kawasaki disease (KD) is an acute immune vasculitis, occurring mainly in infants and children, where inflammation can lead to formation of coronary artery aneurysms and occlusions. Long-term effects of KD mainly rely on data from the Far East, where the epidemiological features of the disease are different from the Caucasian population. Our goal was to assess the cardiovascular complication after childhood KD. We created a registry that will help in the evaluation the incidence and long term outcome of KD. Methods: We have collected data from KD patients who have been registered since 1990 at Semmelweis University Heart and Vascular Center. The patients were studied using cardiac MR, coronary CT and echocardiography. In the latter method, we used speckle-tracking deformation analysis in addition to the routine parameters. Through the retrospective study we compared the results of patients who have had KD to data obtained from members of a matched control group. Results: The cardiac imaging results of a total of 54 patients (16±6 years average age) were compared to data obtained from a 19-person control group. The disease occurred among children at an average of 3 years of age. Follow-up 9±4,9 years. Each of our patients had cardiac MRI; the first examination used in this study was at an average of 11 years of age, and coronary lesions occurred in 24% of patients. Echocardiography measurements showed that end-diastolic volume and ejection fraction was in the normal range, similar to the control group (KD vs. control: EDV: 57±26 vs. 52 ± 23 ml, EF: 67±8 vs. 67±7 ml) and neither the speckle-tracking echocardiography deformation parameters showed systolic dysfunction (GLS:-18±5 vs.-18±4%). Conclusions:Using the advanced cardiovascular imaging methods, it is possible to recognize an increasing number of cases in children and adults, who were previously involved in KD. The purpose of our register is the retrospective characterization of this disease in the Hungarian population, and the prospective follow-up of enrolled patients, to detect long-term cardiovascular effects of the disease.
International Journal of Molecular Sciences, Sep 17, 2013
The present study evaluates a hypothesis that diet-related hypercholesterolemia increases oxidati... more The present study evaluates a hypothesis that diet-related hypercholesterolemia increases oxidative stress-related burden to cardiovascular tissue, resulting in progressively increased mortality, along with deterioration of electrophysiological and enzymatic function in rabbit myocardium. New Zealand white rabbits were divided into four groups, defined as follows: GROUP I, cholesterol-free rabbit chow for 12 weeks; GROUP II, cholesterol-free chow, 40 weeks; GROUP III, chow supplemented with 2% cholesterol, 12 weeks; GROUP IV, chow supplemented with 2% cholesterol, 40 weeks. At the 12 and 40 weeks time points, animals in each of the aforementioned cohorts were subjected to echocardiographic measurements, followed by sacrifice.
Background: Growing evidence exists for soluble Angiotensin Converting Enzyme-2 (sACE2) as a biom... more Background: Growing evidence exists for soluble Angiotensin Converting Enzyme-2 (sACE2) as a biomarker in definitive heart failure (HF), but there is little information about changes in sACE2 activity in hypertension with imminent heart failure and in reverse remodeling. Methods, Findings: Patients with systolic HF (NYHAII-IV, enrolled for cardiac resynchronisation therapy, CRT, n = 100) were compared to hypertensive patients (n = 239) and to a healthy cohort (n = 45) with preserved ejection fraction (EF.50%) in a single center prospective clinical study. The status of the heart failure patients were checked before and after CRT. Biochemical (ACE and sACE2 activity, ACE concentration) and echocardiographic parameters (EF, left ventricular enddiastolic (EDD) and end-systolic diameter (ESD) and dP/dt) were measured. sACE2 activity negatively correlated with EF and positively with ESD and EDD in all patient's populations, while it was independent in the healthy cohort. sACE2 activity was already increased in the hypertensive group, where signs for imminent heart failure (slightly decreased EF and barely increased NT-proBNP levels) were detected. sACE2 activities further increased in patients with definitive heart failure (EF, 50%), while sACE2 activities decreased with the improvement of the heart failure after CRT (reverse remodeling). Serum angiotensin converting enzyme (ACE) concentrations were lower in the diseased populations, but did not show a strong correlation with the echocardiographic parameters. Conclusions: Soluble ACE2 activity appears to be biomarker in heart failure, and in hypertension, where heart failure may be imminent. Our data suggest that sACE2 is involved in the pathomechanism of hypertension and HF.
Several transcatheter techniques based on radiofrequency energy were elaborated for the treatment... more Several transcatheter techniques based on radiofrequency energy were elaborated for the treatment of atrial fibrillation through the last decade. Recently, similar success rates with a better safety profile concerning life threatening complications were reported with the novel methode of cryoballon isolation of the pulmonary veins. This paper summarizes our initial experience with cryoballon ablation after the first 55 patients. Methode: Symptomatic patients refractory to aniarrhythmic medication mostly with paroxysmal atrial fibrillation without significant structural heart disease were enrolled. Cannulation and isolation of all pulmonary veins were attempted using a 28 mm double-wall cryoballon inflated at the ostium of the vein and abolishing eletrical activity of atrial tissue around its perimeter by freezing to –70 C. Intravenous heparin during and oral anticoagulant after the procedure was administered. Conventional ECGs, Holter ECGs and transtelephonic ECG recordings were used through 6 months follow-up for rhythm monitoring. Results: In 55 patients enrolled (18 female; age: 56±33,64 years) 165 out ot 192 (86%) pulmonary veins were successfully isolated. All pulmonary veins were isolated in 37 patients (67%). Procedure time was 155.67±100.66 min, while fluoroscopy time was 34.04±31.89 min. In 34 patients with 6 months follow-up 24 (70%) either remained free of arrhythmia (17 patients) or had a significant decrease in arrhythmia burden (7 patients). Conclusion: Based on our initial experience, cryoballon isolation of pulmonary veins appears to be a more simple procedure with similar efficacy to radiofrequency ablation in the treatment of atrial fibrillation.
Fabry disease is an X chromosome linked disorder caused by the inherited deficiency of lysosomal ... more Fabry disease is an X chromosome linked disorder caused by the inherited deficiency of lysosomal enzyme α- galactosidase A. The deficiency results in abnormal degradation of certain glycosphingolipids. Although the disease is known for more than hundred years and the underlying molecular basis is getting to be well defined, there are still a lot of unanswered questions regarding the different clinical presentations, available diagnostic procedures and therapeutic interventions. The scope of the article is to review the molecular basis of Fabry disease and summarize the available data about Fabry disease cardiomyopathy, highlight the controversies of current knowledge and evaluate future research directions.
The present study examined the efficacy of using longevinex, a commercially available resveratrol... more The present study examined the efficacy of using longevinex, a commercially available resveratrol formulation, to lower blood cholesterol in hypercholesteromic rabbits. New Zealand white rabbits were randomly divided into two groups (n = 6 per group), one group was given high cholesterol diet for 3 months while the other group fed regular diet served as control. The high cholesterol diet fed group was further subdivided into two groups (n = 6 per group), one group was given longevinex resveratrol while the other group given vehicle only served as control. Longevinex was given by gavaging up to a period of 6 months. Longevinex-treated rabbits exhibited lowering of plasma cholesterol level. Inhibition of arterial plaque formation was noticed even after 1 month. Longevinextreated hearts demonstrated improved ventricular recovery when isolated working hearts were subjected to 30 min of ischemia followed by 2 h of reperfusion. Aortic flow and developed pressure during post-ischemic reperfusion period were significantly higher for the longevinex-treated hearts compared to those in control group of hearts. Myocardial infarct size was also lower in the treated group compared to that for the untreated group. These results indicate cholesterol-lowering ability of longevinex, which appears to reflect in its ability to protect the hypercholesteromic hearts from ischemic reperfusion injury.
Hypothesis. The present study evaluates the hypothesis that sour cherry seed extract (SCSE) prote... more Hypothesis. The present study evaluates the hypothesis that sour cherry seed extract (SCSE) protects against cardiovascular disease and inflammation in hypercholesterolemic rabbits, and that this protection correlates with SCSE-induced activity of heme oxygenase-1 (HO-1), a cytoprotective enzyme contributing to oxidative stress responses. Methods: 18 New Zealand white rabbits were divided into three groups receiving: I. cholesterol-free rabbit chow; II. chow containing 2% cholesterol; or III. 2% cholesterol plus SCSE for 16 weeks. Heart functions were monitored by echocardiography 0, 4, and 16 weeks after the initiation of cholesterol-supplemented feeding. At the 16-week time-point, isolated hearts were subjected to ischemia-reperfusion (I/R), followed by measurement of heart rate (HR), aortic flow (AF), coronary flow (CF), aortic pressure (AoP), and left ventricular developed pressure (LVDP). Myocardial infarct size was determined using triphenyl tetrazolium chloride (TTC). Quantification of fatty streaks was assessed using Sudan-III staining. Western blot analysis was used to determine the content of cytochrome c oxidase III (COX III), vascular endothelial growth factor (VEGF), and HO-1 in the myocardium. Results: Relative to cholesterol-treated animals not receiving SCSE, SCSE-treated animals exhibited significantly improved cardiac function and improved peak early diastolic velocity to peak atrial velocity ratio (E'/A'), along with decreased atherosclerotic plaque formation and infarct size. Increased HO-1 and COX III protein expression and COX activity were also noted in hearts from SCSE-treated rabbits. Conclusions: This study demonstrates SCSE cardioprotective effects on hypercholesterolemic hearts. Correlation of these outcomes with HO-1 expression suggests that the effect may be mediated by activity of this enzyme. However, definitive proof of HO-1 dependence requires further investigation.
Several transcatheter techniques based on radiofrequency energy were elaborated for the treatment... more Several transcatheter techniques based on radiofrequency energy were elaborated for the treatment of atrial fibrillation through the last decade. Recently, similar success rates with a better safety profile concerning life threatening complications were reported with the novel methode of cryoballon isolation of the pulmonary veins. This paper summarizes our initial experience with cryoballon ablation after the first 55 patients. Methode: Symptomatic patients refractory to aniarrhythmic medication mostly with paroxysmal atrial fibrillation without significant structural heart disease were enrolled. Cannulation and isolation of all pulmonary veins were attempted using a 28 mm double-wall cryoballon inflated at the ostium of the vein and abolishing eletrical activity of atrial tissue around its perimeter by freezing to –70 C. Intravenous heparin during and oral anticoagulant after the procedure was administered. Conventional ECGs, Holter ECGs and transtelephonic ECG recordings were used through 6 months follow-up for rhythm monitoring. Results: In 55 patients enrolled (18 female; age: 56±33,64 years) 165 out ot 192 (86%) pulmonary veins were successfully isolated. All pulmonary veins were isolated in 37 patients (67%). Procedure time was 155.67±100.66 min, while fluoroscopy time was 34.04±31.89 min. In 34 patients with 6 months follow-up 24 (70%) either remained free of arrhythmia (17 patients) or had a significant decrease in arrhythmia burden (7 patients). Conclusion: Based on our initial experience, cryoballon isolation of pulmonary veins appears to be a more simple procedure with similar efficacy to radiofrequency ablation in the treatment of atrial fibrillation.
Klinikánkon négy éve alkalmazunk perkután PD katéter-beültetést és peritoenális dialízis kezelést... more Klinikánkon négy éve alkalmazunk perkután PD katéter-beültetést és peritoenális dialízis kezelést a súlyosan csökkent bal kamra szisztolés funkcióval bíró, diuretikum-refrakter szívelégtelen betegek körében. Célul tűztük ki, hogy leírjuk centrumunk ebben a betegcsoportban szerzett tapasztalatát. Betegek, módszerek: 2014 áprilisától követtük betegeinket a követési idő vége 2018. január volt. Eredmények: Hat páciensünk közül háromnál nem iszkémiás etiológiájú csökkent ejekciós frakciójú szívelégtelenség, háromnál iszkémiás etiológiájú csökkent ejekciós frakciójú szívelégtelenség talaján alakult végstádiumú szívelégtelenség. Charlson komorbiditási index alapján 6-10 pont közötti értékeik voltak. A PD katéterbeültetés perkután technikával történt, az ultrafiltrációt azonnal el tudtuk kezdeni. Két betegünk sikeres szívtranszplantáción esett át. Három betegünk hunyt el, halálok nem PD asszociált szövődmény volt, 1 traumás, 1 egyéb infekció, 1 szívelégtelenség. Következtetések: A diuretikum-refrakter szívelégtelenség esetén a peritonealis dialízissel javulhat a jobb és bal kamra funkciója is. Centrumunkban kezeltünk elsőként PD-vel-áthidaló terápiaként-, hazánkban szívelégtelen beteget a szívtranszplantációig. Peritoneal ultrafiltration for refractory heart failure: single-center experience Introduction: In our centre we have been treated patients with therapy refracter congestive heart failure. The aim of this study was to describe a single-center experience in the treatment of refractory HF patients with peritoneal dialysis (PD). Methods: Our study included in a single PD Unit, showing symptoms and signs of severe refractory congestive HF to optimal pharmacological therapy (NYHA class IV). We followed them till January 2018. Results: Patients were followed for 10 months; population mean age was 58 years and Charlson's comorbidity index was 6-10. We could start peritoneal dialysis immediately after catheter implantation. Three patients died but not according to PD associated infection: 1 traumatic, 1 due to HF, another patient due to infection. Conclusion: PD, applied to refractory HF can improve functional class. In Hungary we used peritoneal dialysis first time as a bridge therapy to heart transplantation in diuretic refracter heart failure.
Esetismertetés Egy iszkémiás cardiomyopathiában szenvedő 55 éves férfi betegnél súlyos koronáriab... more Esetismertetés Egy iszkémiás cardiomyopathiában szenvedő 55 éves férfi betegnél súlyos koronáriabetegség miatt elvégzett koronária bypass-műtét és optimális gyógyszeres kezelés ellenére előrehaladott szívelégtelenség tüneteit, a balkamra-funkció progresszív romlását észleltük. Számos alkalommal hospitalizáltuk, mígnem diuretikum-refrakter ödémái miatt peritonealis dialízis (PD) elindítása mellett döntöttünk. Rendszeres napi háromszori PD-kezelés után a vesefunkció javulása és a testsúly csökkenése mellett a bal kamrai szisztolés és diasztolés funkció javulását észleltük. A Swan-Ganz-mérések alapján a PD-kezelés elindítását követően a pulmonalis vaszkuláris rezisztencia jelentősen csökkent. A beteg a PD-kezelés elkezdését követően kórházi felvételt szívelégtelenség tünetei miatt nem igényelt, végül sikeres szívtranszplantáción esett át. Peritoneal dialysis as bridge therapy to heart transplantation A 53-year old male patient suffering from ischaemic cardiomyopathy underwent coronary bypass operation showed progressive signs of cardiac decompensation and deterioration of left ventricular (LV) ejection fraction despite optimal medical treatment. The patient was hospitalized several times because of recurrent peripheral oedema, which proved to be refractory to diuretic treatment. Therefore peritoneal dialysis (PD) was initiated. Through regular (three times daily) fluid exchange renal function improved, which was accompanied by a remarkable reduction in the body weight and a significant improvement of LV systolic and diastolic function. Moreover, subsequent Swan-Ganz catheterizations showed a significant decrease in the pulmonary vascular resistance. After the initiation of the PD, the patient did not require hospitalization for worsening heart failure and underwent successful heart transplantation.
Patient Forum The content of these European Society of Cardiology (ESC) / European Association fo... more Patient Forum The content of these European Society of Cardiology (ESC) / European Association for Cardio-Thoracic Surgery (EACTS) Guidelines has been published for personal and educational use only. No commercial use is authorized. No part of the ESC/EACTS Guidelines may be translated or reproduced in any form without written permission from the ESC and the EACTS. Permission can be obtained upon submission of a written request to Oxford University Press, the publisher of the European Heart Journal and the party authorized to handle such permissions on behalf of the ESC ([email protected]). Disclaimer: The ESC/EACTS Guidelines represent the views of the ESC and the EACTS and were produced after careful consideration of the scientific and medical knowledge and the evidence available at the time of their publication. The ESC and the EACTS are not responsible in the event of any contradiction, discrepancy and/or ambiguity between the ESC/EACTS Guidelines and any other official recommendations or guidelines issued by the relevant public health authorities, in particular in relation to good use of healthcare or therapeutic strategies. Health professionals are encouraged to take the ESC/EACTS Guidelines fully into account when exercising their clinical judgment, as well as in the determination and the implementation of preventive, diagnostic or therapeutic medical strategies; however, the ESC/EACTS Guidelines do not override, in any way whatsoever, the individual responsibility of health professionals to make appropriate and accurate decisions in consideration of each patient's health condition and in consultation with that patient and, where appropriate and/or necessary, the patient's caregiver. Nor do the ESC/EACTS Guidelines exempt health professionals from taking into full and careful consideration the relevant official updated recommendations or guidelines issued by the competent public health authorities, in order to manage each patient's case in light of the scientifically accepted data pursuant to their respective ethical and professional obligations. It is also the health professional's responsibility to verify the applicable rules and regulations relating to drugs and medical devices at the time of prescription.
Fabry disease is an X chromosome linked disorder caused by the inherited deficiency of lysosomal ... more Fabry disease is an X chromosome linked disorder caused by the inherited deficiency of lysosomal enzyme α- galactosidase A. The deficiency results in abnormal degradation of certain glycosphingolipids. Although the disease is known for more than hundred years and the underlying molecular basis is getting to be well defined, there are still a lot of unanswered questions regarding the different clinical presentations, available diagnostic procedures and therapeutic interventions. The scope of the article is to review the molecular basis of Fabry disease and summarize the available data about Fabry disease cardiomyopathy, highlight the controversies of current knowledge and evaluate future research directions.
Hypothesis. The present study evaluates the hypothesis that sour cherry seed extract (SCSE) prote... more Hypothesis. The present study evaluates the hypothesis that sour cherry seed extract (SCSE) protects against cardiovascular disease and inflammation in hypercholesterolemic rabbits, and that this protection correlates with SCSE-induced activity of heme oxygenase- 1 (HO-1), a cytoprotective enzyme contributing to oxidative stress responses.
Methods: 18 New Zealand white rabbits were divided into three groups receiving: I. cholesterol-free rabbit chow; II. chow containing 2% cholesterol; or III. 2% cholesterol plus SCSE for 16 weeks. Heart functions were monitored by echocardiography 0, 4, and 16 weeks after the initiation of cholesterol-supplemented feeding. At the 16-week time-point, isolated hearts were subjected to ischemia-reperfusion (I/R), followed by measurement of heart rate (HR), aortic flow (AF), coronary flow (CF), aortic pressure (AoP), and left ventricular developed pressure (LVDP). Myocardial infarct size was determined using triphenyl tetrazolium chloride (TTC). Quantification of fatty streaks was assessed using Sudan-III staining. Western blot analysis was used to determine the content of cytochrome c oxidase III (COX III), vascular endothelial growth factor (VEGF), and HO-1 in the myocardium.
Results: Relative to cholesterol-treated animals not receiving SCSE, SCSE-treated animals exhibited significantly improved cardiac function and improved peak early diastolic velocity to peak atrial velocity ratio (E'/A'), along with decreased atherosclerotic plaque formation and infarct size. Increased HO-1 and COX III protein expression and COX activity were also noted in hearts from SCSE-treated rabbits.
Conclusions: This study demonstrates SCSE cardioprotective effects on hypercholesterolemic hearts. Correlation of these outcomes with HO-1 expression suggests that the effect may be mediated by activity of this enzyme. However, definitive proof of HO-1 dependence requires further investigation.
Hypothesis. The present study evaluates the hypothesis that sour cherry seed extract (SCSE) prote... more Hypothesis. The present study evaluates the hypothesis that sour cherry seed extract (SCSE) protects against cardiovascular disease and inflammation in hypercholesterolemic rabbits, and that this protection correlates with SCSE-induced activity of heme oxygenase- 1 (HO-1), a cytoprotective enzyme contributing to oxidative stress responses.
Methods: 18 New Zealand white rabbits were divided into three groups receiving: I. cholesterol-free rabbit chow; II. chow containing 2% cholesterol; or III. 2% cholesterol plus SCSE for 16 weeks. Heart functions were monitored by echocardiography 0, 4, and 16 weeks after the initiation of cholesterol-supplemented feeding. At the 16-week time-point, isolated hearts were subjected to ischemia-reperfusion (I/R), followed by measurement of heart rate (HR), aortic flow (AF), coronary flow (CF), aortic pressure (AoP), and left ventricular developed pressure (LVDP). Myocardial infarct size was determined using triphenyl tetrazolium chloride (TTC). Quantification of fatty streaks was assessed using Sudan-III staining. Western blot analysis was used to determine the content of cytochrome c oxidase III (COX III), vascular endothelial growth factor (VEGF), and HO-1 in the myocardium.
Results: Relative to cholesterol-treated animals not receiving SCSE, SCSE-treated animals exhibited significantly improved cardiac function and improved peak early diastolic velocity to peak atrial velocity ratio (E'/A'), along with decreased atherosclerotic plaque formation and infarct size. Increased HO-1 and COX III protein expression and COX activity were also noted in hearts from SCSE-treated rabbits.
Conclusions: This study demonstrates SCSE cardioprotective effects on hypercholesterolemic hearts. Correlation of these outcomes with HO-1 expression suggests that the effect may be mediated by activity of this enzyme. However, definitive proof of HO-1 dependence requires further investigation.
Hypothesis. The present study evaluates the hypothesis that sour cherry seed extract (SCSE) prote... more Hypothesis. The present study evaluates the hypothesis that sour cherry seed extract (SCSE) protects against cardiovascular disease and inflammation in hypercholesterolemic rabbits, and that this protection correlates with SCSE-induced activity of heme oxygenase- 1 (HO-1), a cytoprotective enzyme contributing to oxidative stress responses.
Methods: 18 New Zealand white rabbits were divided into three groups receiving: I. cholesterol-free rabbit chow; II. chow containing 2% cholesterol; or III. 2% cholesterol plus SCSE for 16 weeks. Heart functions were monitored by echocardiography 0, 4, and 16 weeks after the initiation of cholesterol-supplemented feeding. At the 16-week time-point, isolated hearts were subjected to ischemia-reperfusion (I/R), followed by measurement of heart rate (HR), aortic flow (AF), coronary flow (CF), aortic pressure (AoP), and left ventricular developed pressure (LVDP). Myocardial infarct size was determined using triphenyl tetrazolium chloride (TTC). Quantification of fatty streaks was assessed using Sudan-III staining. Western blot analysis was used to determine the content of cytochrome c oxidase III (COX III), vascular endothelial growth factor (VEGF), and HO-1 in the myocardium.
Results: Relative to cholesterol-treated animals not receiving SCSE, SCSE-treated animals exhibited significantly improved cardiac function and improved peak early diastolic velocity to peak atrial velocity ratio (E'/A'), along with decreased atherosclerotic plaque formation and infarct size. Increased HO-1 and COX III protein expression and COX activity were also noted in hearts from SCSE-treated rabbits.
Conclusions: This study demonstrates SCSE cardioprotective effects on hypercholesterolemic hearts. Correlation of these outcomes with HO-1 expression suggests that the effect may be mediated by activity of this enzyme. However, definitive proof of HO-1 dependence requires further investigation.
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Papers by Attila Kertész
Methods: 18 New Zealand white rabbits were divided into three groups receiving: I. cholesterol-free rabbit chow; II. chow containing 2% cholesterol; or III. 2% cholesterol plus SCSE for 16 weeks. Heart functions were monitored by echocardiography 0, 4, and 16 weeks after the initiation of cholesterol-supplemented feeding. At the 16-week time-point, isolated hearts were subjected to ischemia-reperfusion (I/R), followed by measurement of heart rate (HR), aortic flow (AF), coronary flow (CF), aortic pressure (AoP), and left ventricular developed pressure (LVDP). Myocardial infarct size was determined using triphenyl tetrazolium chloride (TTC). Quantification of fatty streaks was assessed using Sudan-III staining. Western blot analysis was used to determine the content of cytochrome c oxidase III (COX III), vascular endothelial growth factor (VEGF), and HO-1 in the myocardium.
Results: Relative to cholesterol-treated animals not receiving SCSE, SCSE-treated animals exhibited significantly improved cardiac function and improved peak early diastolic velocity to peak atrial velocity ratio (E'/A'), along with decreased atherosclerotic plaque formation and infarct size. Increased HO-1 and COX III protein expression and COX activity were also noted in hearts from SCSE-treated rabbits.
Conclusions: This study demonstrates SCSE cardioprotective effects on hypercholesterolemic hearts. Correlation of these outcomes with HO-1 expression suggests that the effect may be mediated by activity of this enzyme. However, definitive proof of HO-1 dependence requires further investigation.
Methods: 18 New Zealand white rabbits were divided into three groups receiving: I. cholesterol-free rabbit chow; II. chow containing 2% cholesterol; or III. 2% cholesterol plus SCSE for 16 weeks. Heart functions were monitored by echocardiography 0, 4, and 16 weeks after the initiation of cholesterol-supplemented feeding. At the 16-week time-point, isolated hearts were subjected to ischemia-reperfusion (I/R), followed by measurement of heart rate (HR), aortic flow (AF), coronary flow (CF), aortic pressure (AoP), and left ventricular developed pressure (LVDP). Myocardial infarct size was determined using triphenyl tetrazolium chloride (TTC). Quantification of fatty streaks was assessed using Sudan-III staining. Western blot analysis was used to determine the content of cytochrome c oxidase III (COX III), vascular endothelial growth factor (VEGF), and HO-1 in the myocardium.
Results: Relative to cholesterol-treated animals not receiving SCSE, SCSE-treated animals exhibited significantly improved cardiac function and improved peak early diastolic velocity to peak atrial velocity ratio (E'/A'), along with decreased atherosclerotic plaque formation and infarct size. Increased HO-1 and COX III protein expression and COX activity were also noted in hearts from SCSE-treated rabbits.
Conclusions: This study demonstrates SCSE cardioprotective effects on hypercholesterolemic hearts. Correlation of these outcomes with HO-1 expression suggests that the effect may be mediated by activity of this enzyme. However, definitive proof of HO-1 dependence requires further investigation.
Methods: 18 New Zealand white rabbits were divided into three groups receiving: I. cholesterol-free rabbit chow; II. chow containing 2% cholesterol; or III. 2% cholesterol plus SCSE for 16 weeks. Heart functions were monitored by echocardiography 0, 4, and 16 weeks after the initiation of cholesterol-supplemented feeding. At the 16-week time-point, isolated hearts were subjected to ischemia-reperfusion (I/R), followed by measurement of heart rate (HR), aortic flow (AF), coronary flow (CF), aortic pressure (AoP), and left ventricular developed pressure (LVDP). Myocardial infarct size was determined using triphenyl tetrazolium chloride (TTC). Quantification of fatty streaks was assessed using Sudan-III staining. Western blot analysis was used to determine the content of cytochrome c oxidase III (COX III), vascular endothelial growth factor (VEGF), and HO-1 in the myocardium.
Results: Relative to cholesterol-treated animals not receiving SCSE, SCSE-treated animals exhibited significantly improved cardiac function and improved peak early diastolic velocity to peak atrial velocity ratio (E'/A'), along with decreased atherosclerotic plaque formation and infarct size. Increased HO-1 and COX III protein expression and COX activity were also noted in hearts from SCSE-treated rabbits.
Conclusions: This study demonstrates SCSE cardioprotective effects on hypercholesterolemic hearts. Correlation of these outcomes with HO-1 expression suggests that the effect may be mediated by activity of this enzyme. However, definitive proof of HO-1 dependence requires further investigation.