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Az extracorporalis cardiopulmonalis resuscitatio alkalmazásának gyakorisága egyre nő mind a kórházon belüli, mind a kórházon kívüli keringésmegállás ellátásában. A legújabb újraélesztési irányelvek is egyre inkább beépítik javaslataik közé az extracorporalis keringéstámogató eszközök használatát elhúzódó újraélesztés esetén, bizonyos szelektált betegcsoportokban. Mindezek ellenére kevés bizonyíték áll rendelkezésünkre a beavatkozás hatékonyságáról, és még számos nyitott kérdés maradt megválaszolatlanul azt illetően, hogy milyen körülmények között, mely betegcsoportokban van a leginkább létjogosultsága ennek a modalitásnak. Fontos kérdés a terápia időzítése és helyszíne, illetve lényeges az extracorporalis technikákat alkalmazó személyzet megfelelő kiképzése. Összefoglalónkban röviden áttekintjük, hogy a jelenlegi szakirodalom és ajánlások szerint milyen esetekben merülhet fel az extracorporalis újraélesztés megkezdése, milyen típusú mechanikus keringéstámogatás az elsőként választandó az újraélesztés alatt, milyen tényezőket gondoljunk át alkalmazása során, illetve milyen szövődményekre számítsunk. Orv Hetil. 2023; 164(13): 510–514.

Összefoglaló. Bevezetés: A pericarditis constrictiva egy krónikus gyulladásos folyamat révén kialakuló betegség, melynek során a pericardium elveszíti rugalmasságát, gátolja a szív működését, végső soron szívelégtelenséghez vezet. Egyetlen oki terápiája sebészi. A műtéti megoldásként legelterjedtebben alkalmazott teljes pericardiectomia hosszú időtartamú műtét, amely akár 18%-os műtéti kockázattal járhat, és amelyhez az esetek jelentős részében szívmotor alkalmazása szükséges. Célkitűzés: Egy, az irodalomból már ismert, de csak ritkán és a legtöbbször csak a hagyományos pericardiectomia kiegészítéseként alkalmazott műtéti eljárás, a „turtle cage” pericardiectomia hatásosságának, eredményeinek, lehetséges előnyeinek vizsgálata. Módszer: 2008 és 2021 között Klinikánkon 33 „turtle cage” műtétet végeztünk pericarditis constrictiva miatt. A posztoperatív 30 napos időszak eredményeit több, a nemzetközi irodalomban megjelent közlemény adataival hasonlítottuk össze. Eredmények: Az intraoperatív kép alapján minden esetben sikeres volt a beavatkozás, a 33 beteg egyikénél sem volt szükség szívmotor alkalmazására (0%), szemben a vizsgált közleményekkel. A 33 beavatkozás során 1 beteget veszítettünk el (3%), valamint 1 páciensnél volt szükség vérzés miatti reoperációra (3%), 4 betegnél dialízisre (12,1%). Ezen eredményeink összevethetők a nagy esetszámot felvonultató közleményekkel, és szignifikánsan jobbak az egyik megjelenített európai centrum eredményeinél. Következtetés: Az általunk alkalmazott „turtle cage” pericardiectomia önmagában is megfelelő eljárás a pericarditis constrictiva szívsebészeti kezelésére. Alkalmazásával minimalizálható a szívmotor használatának szükségessége, ezáltal a műtéti kockázat. Eredményeink a technikának köszönhetően még a nagy esetszámú, sok tapasztalattal rendelkező centrumok eredményeivel is összevethetők, azokkal megegyezők. Orv Hetil. 2022; 163(10): 393–399. Summary. Introduction: Constrictive pericarditis is a disease caused by a chronic inflammatory process, which is characterized by the pericardium’s loss of flexibility, inhibiting the function of the heart, ultimately causing heart failure. The only definitive therapy is surgical. Total pericardiectomy, which is the most common surgical approach, is a lengthy procedure with up to 18% operative risk, and it often requires the use of cardiopulmonary bypass. Objective: The evaluation of the effectiveness, results and possible advantages of a surgical technique, “turtle cage” pericardiectomy, which is described in the literature, although rarely used, mainly in addition to conventional pericardiectomy. Method: Between 2008 and 2021, we performed 33 “turtle cage” procedures on patients with constrictive pericarditis in our Institute. We compared the results of the 30-day postoperative period with internationally published data from multiple sources. Results: Based on intraoperative findings, the procedure was successful in all cases, there were no instances when the use of cardiopulmonary bypass was required (0%). During the 33 procedures, we lost 1 patient (3%), reoperation was necessary for postoperative bleeding in 1 case (3%), and postoperative dialysis was necessary in 4 cases (12.1%). These results are comparable to those published by high-volume centres, and significantly better than those of one of the European centres published. Conclusion: The “turtle cage” pericardiectomy, as performed in our Institute, is suitable for the treatment of constrictive pericarditis on its own. With its use, we were able to minimize the use of cardiopulmonary bypass and the operative risk. Our results with this technique are comparable to those of the high-volume, highly experienced centres. Orv Hetil. 2022; 163(10): 393–399.

Heterozygous (HET) truncating mutations in the TTN gene (TTNtv) encoding the giant titin protein are the most common genetic cause of dilated cardiomyopathy (DCM). However, the molecular mechanisms by which TTNtv mutations induce DCM are controversial. Here we investigated 127 clinically identified DCM human cardiac samples with next-generation sequencing (NGS), high-resolution gel electrophoresis, Western blot analysis and super-resolution microscopy in order to dissect the structural and functional consequences of TTNtv mutations. The occurrence of TTNtv was found to be 15% in the DCM cohort. Truncated titin proteins matching, by molecular weight, the gene-sequence predictions were detected in the majority of the TTNtv samples. The total amount of expressed titin, which includes the truncated fragments, was comparable in the TTNtv+ and TTNtv-samples, indicating that titin haploinsufficiency is not the leading cause of the molecular pathogenesis. Proteomic analysis of washed cardiac myofibrils and Stimulated Emission Depletion (STED) super-resolution microscopy of myocardial sarcomeres labeled with sequence-specific anti-titin antibodies revealed that truncated titin is structurally integrated in the sarcomere. Sarcomere lengthdependent anti-titin epitope position, shape and intensity analysis pointed at structural defects in the I/A junction and the M-band of TTNtv+ sarcomeres, which may contribute, via faulty mechanosensor function, to the development of manifest DCM.

Összefoglaló. Az intrauterin echokardiográfiás és MR-diagnosztika fejlődése napjainkban jelentős szerepet tulajdonít már az első trimeszter idejében kimutatható aortaív-fejlődési rendellenességeknek. Célunk volt részletezni a vascularis gyűrűk megjelenési formáit, ezek különböző életkorokban jelentkező tüneteit, sebészi kezelését, hogy hozzájárulhassunk a helyes felvilágosításhoz, mely alapvető lehet a szülés helyének megválasztásában, így meghatározhassák a magzat sorsát, perinatalis ellátását. A situs inversustól eltekintve a jobb oldali aortaív jelenléte mindig felhívja a figyelmet vascularis gyűrű, társuló szívhibák, genetikai betegségek, kromoszómaanomáliák jelenlétére, tehát a magzat további vizsgálata szükséges. Kettős aortaív esetén jelentkezhetnek olyan súlyos tünetek, melyek a megszületés után, kora csecsemőkorban sebészi beavatkozást igényelhetnek (szükséges lehet a szülés helyének megválasztása!). Az aberráns jobb arteria subclavia önállóan nem alkot érgyűrűt, a ritkán társuló truncus caroticusszal csak későbbi életkorban okozhat sebészi beavatkozást igényló enyhe tüneteket. Orv Hetil. 2021; 162(48): 1920–1923. Summary. Nowadays, the development of intrauterine echocardiography and MR diagnostics plays a significant role in aortic arch malformations detected during the first trimester. Our aim was to detail the manifestations of vascular rings, their symptoms at different ages and their surgical treatment options in order to determine the fate of the fetus and its perinatal care. Apart from situs inversus, the presence of the right aortic arch always draws the attention to the possible presence of vascular rings, associated heart defects, genetic diseases, or chromosomal abnormalities, therefore further examinations of the fetus are necessary. In the case of a double aortic arch, severe symptoms may occur, which may require surgery after birth and in early infancy (it may be necessary to choose the place of birth!). The right aberrant subclavian artery does not form a ring and may cause mild symptoms requiring surgery at a later age with rarely associated truncus carotid. Orv Hetil. 2021; 162(48): 1920–1923.

BACKGROUND Intracardiac blood cysts are an exceedingly rare occurrence in adulthood. Detailed imaging modalities aid in the diagnosis of such incidental lesions and procedure planning. METHODS We report the case of a 51-year-old male accusing dyspnea on exertion as a sole symptom which led to the discovery of multiple cardiac anomalies, namely, severe aortic valve insufficiency on a bicuspid aortic valve, ascending aortic aneurysm, a cystic mass on the tricuspid valve, patent foramen ovale, and an occluded right coronary artery. RESULTS The disorders were managed in a single surgical intervention, the resected mass being confirmed as a blood cyst. CONCLUSIONS Our case presents a unique association of cardiac disorders, including a highly uncommon intracardiac blood-filled cyst, and underlines the importance of multimodality imaging and interdisciplinary approach in the successful management of such complex cases.

Összefoglaló. A veleszületett szívbetegségek egyik gyakori formáját jelentő atrioventricularis septumdefektus korrekciós műtétjének kritikus pontja a közös atrioventricularis szájadék elválasztásával a mitralis billentyű kialakítása. A korrekció sikere számos anatómiai variáns függvénye, ezért nem lehet minden esetben a tökéletes anatómiai viszonyokat kialakítani. A fennmaradó billentyűstenosisok, regurgitatiók a későbbi életkorban progressziót mutatva olyan hemodinamikai kórképeket, keringési elégtelenséget okozhatnak, melyek további beavatkozásokat igényelhetnek. A mitralis billentyűnek az atrioventricularis septumdefektushoz társuló betegsége koraszülöttkortól aggkorig minden életkorban előfordul, más-más műtéti megoldást igényelve. A szerzők részletezik a különböző életkorokra vonatkozóan a napjainkban lehetséges és szükséges műtéti megoldásokat, sebészi kihívásokat. A mitralis billentyű műbillentyűre történő cseréjében a klasszikus sebészi megoldások mellett napjainkban új beavatkozásokként jelentős számban jelentkeznek a katéteres intervenciós és hibrid megoldások. A felnőttkort egyre nagyobb számban megélő betegek fokozott odafigyelést, speciális ellátást igényelnek a kardiológusoktól, szívsebészektől. Orv Hetil. 2021; 162(35): 1397–1401. Summary. The critical point of the atrioventricular septal defect correction is to separate the common atrioventricular orifice, which results in the reconstruction of the mitral valve. The success of the correction depends on many anatomical aspects, therefore a perfect anatomical outcome is not always possible. The remaining valvular stenoses and regurgitations, showing progression at a later age, may result in hemodynamic disorders and circulatory insufficiency that may require further interventions. Mitral valve disease associated with atrioventricular septal defect occurs at all ages from preterm to adulthood, requiring different surgical solutions. The authors detail the possible and necessary surgical solutions and surgical challenges at different ages. In addition to the classic surgical solutions, a significant number of catheter interventional and hybrid solutions are emerging as new interventions in the replacement of the mitral valve with an artificial valve. An increasing number of patients living in adulthood require increased attention and special care from cardiologists and cardiac surgeons. Orv Hetil. 2021; 162(35): 1397–1401.

Összefoglaló. Bevezetés: A Marfan-szindróma autoszomális domináns módon öröklődő, szisztémás kötőszöveti betegség. A hosszú távú túlélés szempontjából fontos a nagyérkatasztrófák megelőzése. Szívsebészeti szempontból a legfontosabb elváltozás az aortagyök tágulata. Aortagyök-rekonstrukciós beavatkozásaink Bentall–DeBono-, David I. és módosított Yacoub-műtétek, melyek mind preventív jelleggel, mind dissectio esetén jó eredménnyel végezhetők. Célkitűzés: A marfanos betegeknél eltérő technikával végzett aortagyök-rekonstrukciós műtéteink összehasonlítása. Módszer: A Semmelweis Egyetem Városmajori Szív- és Érgyógyászati Klinikáján 1993 és 2020 között Marfan-szindrómásoknál elvégzett Bentall–DeBono-, David I. és módosított Yacoub-féle aortagyök-rekonstrukciókat elemeztük. A műtét szerinti csoportok életkora a beavatkozás idején 29,69 (21,98–41,25) év, 29,15 ± 11,99 év és 35,29 ± 14,14 év volt, a fenti sorrendben. Az adatok forrásául a Magyar Marfan Regiszter és az Aortagyök-rekonstrukciós Regiszter szolgált. Eredmények: Az utánkövetési idő 132 ± 81,04 hónap volt a Bentall-, 76 ± 27,77 hónap a David-, valamint 4,5 (0,75–11,75) hónap a Yacoub-műtét esetén. A David- és a Yacoub-beavatkozások gyakrabban voltak profilaktikusak, mint a Bentall-operációk (p = 0,0153; p = 0,0085). A Bentall-műtéteknél ritkább volt a primer műtét esetleges későbbi elégtelenségéből fakadó reoperáció, mint a David-operációknál (pKövetkeztetés: Marfan-szindrómában a leggyakrabban halált okozó szövődmény az akut aortaruptura, illetve akut aortadissectio. Eredményeink alapján mindhárom profilaktikus aortagyök-rekonstrukciós műtéti típus jól reprodukálható és jó eredménnyel végezhető Marfan-szindrómában. Orv Hetil. 2021; 162(18): 696–704. Summary. Introduction: Marfan syndrome is an autosomal dominant, systemic connective tissue disorder. Preventing vascular complications is essential for long-term survival. Aortic dilation is the main cardiac surgical manifestation. Bentall–DeBono, David I and modified Yacoub aortic root reconstructions treat and prevent aortic dissections with great outcomes. Objective: Comparing results of aortic root reconstructions in Marfan syndrome. Method: We analysed the data of Bentall–DeBono, David I and modified Yacoub operations performed in Marfan syndrome at the Heart and Vascular Center, Semmelweis University between 1993 and 2020. Ages of surgical groups at the time of operation were 29.69 (21.98–41.25) years, 29.15 ± 11.99 years and 35.29 ± 14.14 years, respectively. Data were obtained from the Hungarian Marfan Register and the Aortic Root Reconstruction Register. Results: Follow-up time was 132 ± 81.04 months for Bentall, 76 ± 27.77 months for David and 4.5 (0.75–11.75) months for Yacoub groups. David and Yacoub operations were prophylactic more frequently than Bentall ones (p = 0.0153; p = 0.0085). Freedom from reoperation after primary surgery insufficiency was more common for Bentall than for David procedure (pConclusion: In Marfan syndrome, acute aortic rupture and dissection are the main contributors to mortality. Based on our results, the prophylactic aortic root reconstructions are reproducible and can be performed with great outcomes. Orv Hetil. 2021; 162(18): 696–704.

Since the establishment of highly active antiretroviral therapy, survival rates have improved among patients with human immunodeficiency virus infection giving them the possibility to become transplant candidates. Recent publications revealed that human immunodeficiency virus‐positive heart transplant recipients' survival is similar to non‐infected patients. We present the case of a 40‐year‐old human immunodeficiency virus infected patient, who was hospitalized due to severely decreased left ventricular function with a possible aetiology of acute myocarditis, that has later been confirmed by histological investigation of myocardial biopsy. Due to rapid progression to refractory cardiogenic shock, extracorporeal membrane oxygenation implantation had been initiated, which was upgraded to biventricular assist device later. On the 35th day of upgraded support, the patient underwent heart transplantation uneventfully. Our clinical experience confirms that implementation of temporary mechanical circulatory support and subsequent cardiac transplantation might be successful in human immunodeficiency virus‐positive patients even in case of new onset, irreversible acute heart failure.

Mechanical circulatory support (MCS) was introduced in the 1960s, of which there is short-, mid- and long-term forms. This brief review discusses our experiences with the primary modality used in Hungary, the veno-arterial extracorpo­real membrane oxygenator (VA-ECMO). We introduced the device with the most relevant information and summarized our clinic’s registry with patient characteristics and results in whom we applied this modality next to data of international studies. We collected data retro- and prospectively from 2012 to 2020 from patients treated with the VA-ECMO at our clinic. The primary endpoint we used was all cause mortality in this patient population of critical condition. Every indication, potential peripheral-central conversion and primary operation technique was involved in the analysis. We found that age above 65 and initial pH under 7.2 significantly affects mortality data. Acute coronary syndrome (ACS) as primary indication showed to be nearly significant, and acute rejection after hTX was a beneficial clinical scenario regarding survival. In international literature diabetes mellitus, elevated levels of lactate, kidney- and hepatic insufficiency were identified as contributing mortality predictors as well. Our results mostly coincide with the international mortality and risk factor data. Time is a very important factor for the survival of these patients. Therefore, it is very important to refer the patient’s refractory to non-MCS therapy to centers with VA-ECMO capability in time.

Introduction Mechanical circulatory support (MCS) has been established as a means of augmenting circulation in patients with critically decreased systolic function due to a variety of underlying clinical reasons. Different methods of MCS may be used, with the venous-arterial extracorporeal membrane oxygenation system (VA-ECMO) being one of the most utilized devices in everyday care. Aim To determine independent predictors influencing mortality outcomes following VA-ECMO therapy in a large, unselected, adult, critically ill patient population in cardiogenic shock (CS). Material and methods Data on 235 consecutive, real-world VA-ECMO treatments were assessed. Analysis was conducted for all subjects requiring MCS with the VA-ECMO as the first instalment, regardless of underlying cause or eventual upgrade. All potential clinical factors influencing mortality were examined and evaluated. Results Overall mortality was ~66% at median 28 days follow-up and significantly depended upon pH < 7.3 (HR = 3.56; p < 0.001), and age ≥ 65 years (HR = 1.96; p = 0.001). Acute coronary syndrome (ACS) as an indication for VA-ECMO displayed a nearly significant value (HR = 1.44; p = 0.07). Heart transplant (hTX) primary graft failure as an indication for the VA-ECMO displayed a clearly favorable outcome (HR = 0.51, p = 0.025); all data based on multivariate Cox regression analysis. Conclusions Mortality in patients requiring VA-ECMO remains high. We conclude that only decreased pH values and advanced age clearly influence mortality in this MCS scenario. ACS also bodes unfavorably, whereas hTX as an indication clearly shows better survival.

Objectives Thyroid dysfunction has been shown to be associated with increased all-cause mortality and severity of chronic heart failure in critical illness and severe cardiac diseases. The present study was conducted to ascertain the relationship between perioperative free triiodothyronine and free tetraiodothyronine (fT4) levels and postoperative adverse outcomes after heart transplantation (HTX). Design Retrospective, observational study. Setting Single-center study in a quaternary care university clinical center. Participants The study comprised adult patients who underwent HTX between 2015 and 2019 and had at least 1 perioperative thyroid hormone laboratory test on the day of surgery or in the 24 hours before/after the procedure (free triiodothyronine, fT4, and thyroid-stimulating hormone). Interventions No interventions were applied. Measurements and Main Results The primary outcome was primary graft dysfunction (PGD), defined by the consensus conference of the International Society for Heart and Lung Transplantation. A total of 151 patients were included in the final analyses. Twenty-nine (19.2%) patients had PGD. Fourteen (9.3%) patients had low fT4 levels. An independent association was found between fT4 and PGD (odds ratio 6.49; 95% confidence interval 2.26-18.61; p = 0.001), with adjusted multivariate Cox regression models. Conclusion The perioperative fT4 level could be a prognostic marker of adverse outcomes in HTX. The authors suggest appropriate perioperative monitoring of fT4 levels. Additional research is warranted to examine the optimal timing, dosage, and method of replacement.

Absztrakt: A veleszületett szívhibák sebészi kezelése a technika fejlődésével folyamatosan változik. Az egyik legegyszerűbb, legközismertebb fejlődési rendellenesség a coarctatio aortae. A szerzők az elmúlt évtizedek során alkalmazott műtéti megoldásokat (’end-to-end’ anasztomózis, foltplasztikák, interpositumok), a műtéti lehetőségeket mutatják be, hazai és nemzetközi tapasztalatok alapján ismertetik a különböző megoldások eredményeit, előnyeit, hátrányait. Nagy sebészi kihívást jelent az újszülöttkori súlyos aorta-hypoplasiával járó eseteknek, a későbbiekben a kialakuló restenosisoknak és az esetleges szövődményeknek (aneurysmák, dissectiók) a megoldása. A coarctatio aortae megoldásai még napjainkban is nehézségeket, sebészi kihívást jelentenek, hiszen ezeknek mások a megoldásai csecsemőkben és nagyobb gyermekekben, illetve felnőttkorban. Itt már sokszor kell alkalmazni a katéterintervenciós megoldásokat is, melyek helyes időzítése még napjainkban is nagy kihívást jelent. Orv Hetil. 2020; 161(21): 861–866.

Introduction: Veno-arterial extracorporeal membrane oxygenation is a valuable therapeutic approach in patients with severe heart failure due to different etiologies. Current prognosis with veno-arterial extracorporeal membrane oxygenation is unsatisfactory, and the risk stratification is still challenging. Therefore, we aimed to evaluate the predictive value of different baseline model for end-stage liver disease scores for survival in patients with veno-arterial extracorporeal membrane oxygenation. Methods: We conducted an observational, retrospective study of consecutive veno-arterial extracorporeal membrane oxygenation-treated patients between January 2012 and August 2018. The four types of model for end-stage liver disease scores—model for end-stage liver disease, international normalized ratio-excluded model for end-stage liver disease, modified model for end-stage liver disease, and model for end-stage liver disease with sodium—were calculated preoperatively. Veno-arterial extracorporeal membrane oxygenation was used based on the four clinical indications: primer graft failure after heart transplantation, weaning failure from cardiopulmonary bypass, acute myocardial infarction with refractory cardiogenic shock, and bridge to transplantation or bridge to candidacy. The primary endpoint of the study was overall mortality. The secondary endpoint was in-hospital mortality. We performed univariable and multivariable Cox regression analyses. Results: Data from 135 patients were analyzed. The median follow-up was 952 days (interquartile range = 417–1555 days). In-hospital mortality was 62.2%, and overall mortality was 71.1%. The multivariable Cox regression analysis is adjusted for indication, and the survival after veno-arterial extracorporeal membrane oxygenation score showed that the following scores were associated with overall mortality: model for end-stage liver disease (hazard ratio = 1.04; 95% confidence interval = 1.01–1.07; p = 0.016), modified model for end-stage liver disease (hazard ratio = 1.04; 95% confidence interval = 1.01–1.06; p = 0.006), and model for end-stage liver disease with sodium (hazard ratio = 1.05; 95% confidence interval = 1.02–1.08; p = 0.001). Conclusion: Model for end-stage liver disease, modified model for end-stage liver disease, and model for end-stage liver disease with sodium scores could be useful in the risk stratification of veno-arterial extracorporeal membrane oxygenation treatment in varying clinical indications.

Background Mycotic aortic pseudoaneurysm is a rare complication after heart transplantation (HTX) with remarkable mortality. Intrathoracic infection is a well-documented predisposing factor for this disease. Staphylococcus aureus, Pseudomonas aeruginosa or Candida species are commonly isolated from resected specimens of the pseudoaneurysms. We demonstrate a unique case of mycotic pseudoaneurysm caused by presumably donor-derived Pseudomonas infection in a heart transplant recipient. Case presentation Our 67-year-old male patient treated with diabetes mellitus underwent HTX. The donor suffered from epiglottic abscess and pneumonia with known microorganisms including Pseudomonas, therefore both the donor and recipient received targeted antimicrobial therapy and prophylaxis. Five months after the uneventful HTX, lab test of the asymptomatic patient showed moderate, increasing C-reactive protein level without obviuos source of infection. Chest computed tomography showed a large (90 mm) saccular dilatation of the tubular portion of ascending aorta. Urgent surgical intervention identified a pseudoaneurysm, histological examinations and cultures of the resected aorta verified Pseudomonas aeruginosa aortitis, while all blood cultures remained negative. Retrospective interrogation of other transplanted organs of the donor supported donor-derived infection as the transport fluid of the right kidney grew Pseudomonas. The patient received 3 weeks of ceftazidime followed by 7 months of oral ciprofloxacin therapy. One year after the operation the patient was asymptomatic with normal inflammatory markers. Conclusions Donor-derived infection is a rare but potential cause of aortitis. Early diagnosis, surgical intervention and adjuvant antibiotic therapy seem to be the keys to successful management of mycotic pseudoaneurysms after HTX.

Összefoglaló. Bevezetés: A pericarditis constrictiva egy krónikus gyulladásos folyamat révén kialakuló betegség, melynek során a pericardium elveszíti rugalmasságát, gátolja a szív működését, végső soron szívelégtelenséghez vezet. Egyetlen oki terápiája sebészi. A műtéti megoldásként legelterjedtebben alkalmazott teljes pericardiectomia hosszú időtartamú műtét, amely akár 18%-os műtéti kockázattal járhat, és amelyhez az esetek jelentős részében szívmotor alkalmazása szükséges. Célkitűzés: Egy, az irodalomból már ismert, de csak ritkán és a legtöbbször csak a hagyományos pericardiectomia kiegészítéseként alkalmazott műtéti eljárás, a "turtle cage" pericardiectomia hatásosságának, eredményeinek, lehetséges előnyeinek vizsgálata. Módszer: 2008 és 2021 között Klinikánkon 33 "turtle cage" műtétet végeztünk pericarditis constrictiva miatt. A posztoperatív 30 napos időszak eredményeit több, a nemzetközi irodalomban megjelent közlemény adataival hasonlítottuk össze. Eredmények: Az intraoperatív kép alapján minden esetben sikeres volt a beavatkozás, a 33 beteg egyikénél sem volt szükség szívmotor alkalmazására (0%), szemben a vizsgált közleményekkel. A 33 beavatkozás során 1 beteget veszítettünk el (3%), valamint 1 páciensnél volt szükség vérzés miatti reoperációra (3%), 4 betegnél dialízisre (12,1%). Ezen eredményeink összevethetők a nagy esetszámot felvonultató közleményekkel, és szignifikánsan jobbak az egyik megjelenített európai centrum eredményeinél. Következtetés: Az általunk alkalmazott "turtle cage" pericardiectomia önmagában is megfelelő eljárás a pericarditis constrictiva szívsebészeti kezelésére. Alkalmazásával minimalizálható a szívmotor használatának szükségessége, ezáltal a műtéti kockázat. Eredményeink a technikának köszönhetően még a nagy esetszámú, sok tapasztalattal rendelkező centrumok eredményeivel is összevethetők, azokkal megegyezők. Orv Hetil. 2022; 163(10): 393-399.Constrictive pericarditis is a disease caused by a chronic inflammatory process, which is characterized by the pericardium's loss of flexibility, inhibiting the function of the heart, ultimately causing heart failure. The only definitive therapy is surgical. Total pericardiectomy, which is the most common surgical approach, is a lengthy procedure with up to 18% operative risk, and it often requires the use of cardiopulmonary bypass.The evaluation of the effectiveness, results and possible advantages of a surgical technique, "turtle cage" pericardiectomy, which is described in the literature, although rarely used, mainly in addition to conventional pericardiectomy.Between 2008 and 2021, we performed 33 "turtle cage" procedures on patients with constrictive pericarditis in our Institute. We compared the results of the 30-day postoperative period with internationally published data from multiple sources.Based on intraoperative findings, the procedure was successful in all cases, there were no instances when the use of cardiopulmonary bypass was required (0%). During the 33 procedures, we lost 1 patient (3%), reoperation was necessary for postoperative bleeding in 1 case (3%), and postoperative dialysis was necessary in 4 cases (12.1%). These results are comparable to those published by high-volume centres, and significantly better than those of one of the European centres published.The "turtle cage" pericardiectomy, as performed in our Institute, is suitable for the treatment of constrictive pericarditis on its own. With its use, we were able to minimize the use of cardiopulmonary bypass and the operative risk. Our results with this technique are comparable to those of the high-volume, highly experienced centres. Orv Hetil. 2022; 163(10): 393-399.

Abstract: Use of 3D planning and 3D printing is expanding in healthcare. One of the common applications is the creation of anatomical models for the surgical procedure from DICOM files. These patient-specific models are used for multiple purposes, including visualization of complex anatomical situations, simulation of surgical procedures, patient education and facilitating communication between the different disciplines during clinical case discussions. Cardiac and thoracic surgical applications of this technology development include the use of patient-specific 3D models for exploration of ventricle and aorta function and surgical procedural planning in oncology. The 3D virtual and printed models provide a new visualization perspective for the surgeons and more efficient communication between the different clinical disciplines. The 3D project was started at the Semmelweis University with the cooperation of the Thoracic Surgery Department of the National Institute of Oncology in 2018. The authors want to share their experiences in 3D designed medical tools. Orv Hetil. 2019; 160(50): 1967–1975.

Abstract: Introduction and aim: Pediatric coronary artery surgery for congenital heart disease has become increasingly important in newborns and infants. It is life-saving in unsuccessful coronary transfer surgery, injury of coronary artery during surgery, in pediatric coronary artery bypass surgery (PCABS) due to acquired Kawasaki disease. Method: We review the current surgical role of congenital and acquired coronary artery diseases in newborns and infants. Results: The 7 main challenges are: 1) anomalous origin of the left coronary artery from the pulmonary artery; 2) different variations of the origins of the coronary arteries in transposition of the great arteries (TGA); 3) acute and late coronary events in the arterial switch operation for TGA; 4) complications after coronary transfer procedures: Ross-, Nikaidoh operation; 5) inadvertent coronary artery injury during heart surgery; 6) Kawasaki disease; 7) coronary artery procedures in premature infants. Direct coronary reimplantation in most, surgical angioplastic procedures in selected patients is the first choice; however, PCABS with internal thoracic artery (ITA) grafts can be life-saving in emergency or severe myocardial hypoperfusion conditions. Since the patency of saphenous vein grafts is poorer than that of ITA grafts, their use should be avoided in growing children. The procedures can be performed safely in neonates and infants, using high-power magnifying glasses or a surgical microscope. Conclusion: Pediatric coronary artery surgery technique is now established as the standard safety surgical choice, as a new area in cardiac surgery. Congenital heart surgeons after proper training are able to use coronary transfer and bypass surgery safely even in premature babies. Orv Hetil. 2019; 160(49): 1935–1940.

Background Aortopathy is a common phenomenon in tetralogy of Fallot (TOF). The current study was designed to detect left ventricular (LV) deformation abnormalities and its relation to aortic stiffness in corrected TOF (cTOF) using the novel three-dimensional (3D) speckle-tracking echocardiography (3DSTE). Detailed comparative analysis between patients with early palliation-late correction (pcTOF) and early total reconstruction (etrTOF) was also performed. Methods The present study consisted of 28 cTOF patients (35.0±15.7 years, 11 males) from which 15 and 13 proved to be pcTOF and etrTOF, respectively. Their clinical parameters were compared to those of 39 matched healthy adults (35.5±6.0 years, 16 males). Results cTOF patients showed significantly lower global LV longitudinal, circumferential and area strains as compared to controls. In etrTOF patients, global LV 3D strain was higher than in controls. In pcTOF patients, all LV strains proved to be significantly lower as compared to those of etrTOF patients and controls. In all cTOF patients, several moderate correlations could be detected between LV strain parameters and aortic elastic properties. Conclusions Significant LV deformational abnormalities could be demonstrated in cTOF patients. etrTOF patients have beneficial LV strain parameters as compared to those of pcTOF patients. LV strains show correlations with aortic elastic properties.

Background Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease (CHD). Abnormal aortic dimensions and elasticity parameters have been long described for corrected TOF (cTOF) together with left ventricular (LV) rotational abnormalities, but results are conflicting. The present study focuses on investigating LV rotational mechanics in cTOF, and possible correlation of these parameters with aortic elasticity. It was also aimed to be examined whether different surgical strategies have any effect on these results. Methods The study involved 26 adult cTOF patients, from which 14 had palliative surgery first and a late total correction (pcTOF), while early total correction was the treatment of choice in 12 patients (etrTOF). Their results were compared to those of 37 age- and gender-matched healthy adults. Routine transthoracic two-dimensional Doppler echocardiography extended with assessment of aortic elastic properties and three-dimensional speckle-tracking echocardiography (3DSTE) was performed in all cTOF patients and controls. Results Sixteen out of 26 cTOF patients showed normally directed LV rotational mechanics, while apical or basal LV rotations were in the same clockwise or counterclockwise directions in 7 and 3 cTOF cases, respectively (LV 'rigid body rotation', RBR). Significantly reduced LV apical rotation and twist could be demonstrated in all cTOF patients with preserved LV basal rotation regardless of previous procedure. pcTOF patients showed significantly reduced LV apical rotation as compared to that of etrTOF cases. Significant correlations could be demonstrated between LV apical rotation and aortic stiffness index (r=-0.55, P=0.03) and aortic distensibility (r=0.52, P=0.04). Conclusions Significant LV rotational abnormalities could be demonstrated in cTOF with the high prevalence of LV-RBR. pcTOF patients showed significantly reduced LV apical rotation as compared to that of etrTOF cases. Unexpected abnormal physiologic response of LV rotational mechanics to increased aortic stiffness can be detected in cTOF patients without LV-RBR.

Background Behçet’s disease is an auto-inflammatory disorder categorized as a primer systemic vasculitis of unknown aetiology. Genetic factors, infectious agents and the irregularity of T-cell homeostasis are presumed to be responsible for the emergence of Behçet’s disease. Characteristic symptoms are multisystemic. Although cardiovascular involvement is rare, it should be noted due to the difficulty of surgical treatment options. Case presentation Our 44-year-old male patient underwent aortic valve replacement due to aortic regurgitation. At the 15-month follow-up, echocardiography showed detachment of the prosthetic valve and in the aortic root, multiple pseudo-aneurysms were identified. We performed an aortic root reconstruction with a Bentall procedure using a special „skirted” conduit to reduce strain in the suture line between the conduit and the extremely dilated left ventricular outflow tract. Conclusions The surgical treatment of cardiovascular manifestations of Behçet’s disease remains challenging. This new technique may be beneficial in well-selected cases where the annulus of the aorta is extremely dilated or annular tissue disorder is present.

Összefoglaló. Az intrauterin echokardiográfiás és MR-diagnosztika fejlődése napjainkban jelentős szerepet tulajdonít már az első trimeszter idejében kimutatható aortaív-fejlődési rendellenességeknek. Célunk volt részletezni a vascularis gyűrűk megjelenési formáit, ezek különböző életkorokban jelentkező tüneteit, sebészi kezelését, hogy hozzájárulhassunk a helyes felvilágosításhoz, mely alapvető lehet a szülés helyének megválasztásában, így meghatározhassák a magzat sorsát, perinatalis ellátását. A situs inversustól eltekintve a jobb oldali aortaív jelenléte mindig felhívja a figyelmet vascularis gyűrű, társuló szívhibák, genetikai betegségek, kromoszómaanomáliák jelenlétére, tehát a magzat további vizsgálata szükséges. Kettős aortaív esetén jelentkezhetnek olyan súlyos tünetek, melyek a megszületés után, kora csecsemőkorban sebészi beavatkozást igényelhetnek (szükséges lehet a szülés helyének megválasztása!). Az aberráns jobb arteria subclavia önállóan nem alkot érgyűrűt, a ritkán társuló truncus caroticusszal csak későbbi életkorban okozhat sebészi beavatkozást igényló enyhe tüneteket. Orv Hetil. 2021; 162(48): 1920-1923. Summary. Nowadays, the development of intrauterine echocardiography and MR diagnostics plays a significant role in aortic arch malformations detected during the first trimester. Our aim was to detail the manifestations of vascular rings, their symptoms at different ages and their surgical treatment options in order to determine the fate of the fetus and its perinatal care. Apart from situs inversus, the presence of the right aortic arch always draws the attention to the possible presence of vascular rings, associated heart defects, genetic diseases, or chromosomal abnormalities, therefore further examinations of the fetus are necessary. In the case of a double aortic arch, severe symptoms may occur, which may require surgery after birth and in early infancy (it may be necessary to choose the place of birth!). The right aberrant subclavian artery does not form a ring and may cause mild symptoms requiring surgery at a later age with rarely associated truncus carotid. Orv Hetil. 2021; 162(48): 1920-1923.

Background Pericardial tamponade is a serious condition which may eventually lead to severe haemodynamic disturbances and cardiac arrest. It is most often caused by the accumulation of fluid inside the pericardium, as a result of different aetiological factors such as pericarditis, neoplastic diseases, lymphatic dysfunctions, or idiopathic pericardial disease. Pericardial tamponade can develop after cardiac surgical procedures or as a complication of myocardial infarction. Collection of blood inside the pericardial sack can be the result of pericardial or cardiac trauma. It is exceedingly rare for the injury to be caused by a migrating foreign body. Although a typical picture of pericardial tamponade has been previously described, the disorder may clinically resemble an acute myocardial infarction. Case presentation We report the case of a 58-year-old female patient complaining of new onset thoracic pain and shortness of breath. Electrocardiographic examination results were suggestive of an acute inferior myocardial infarction. However, echocardiography revealed significant pericardial tamponade. The cause was found to be a needle which remained inside the pelvis following a previous cesarean delivery, which the patient had undergone 18 years prior. In emergency setting, the needle was removed and the pericardial tamponade was resolved. Due to the prompt and efficient management, the patient had an uneventful postoperative recovery and presented no recurrence at the follow-up examinations. Conclusions The migration of foreign bodies through tissues is exceedingly rare. If present, it may cause life-threatening complications. Since the aetiology of pericardial tamponade is vast, a thorough assessment is highly important. Therefore, echocardiography is the imaging modality of choice. We wish to highlight the possibility of migrating foreign bodies as probable cause for pericardial tamponade, as well as the importance of echocardiographic methods in the fast-track evaluation of such critical conditions.

Összefoglaló. A veleszületett szívbetegségek egyik gyakori formáját jelentő atrioventricularis septumdefektus korrekciós műtétjének kritikus pontja a közös atrioventricularis szájadék elválasztásával a mitralis billentyű kialakítása. A korrekció sikere számos anatómiai variáns függvénye, ezért nem lehet minden esetben a tökéletes anatómiai viszonyokat kialakítani. A fennmaradó billentyűstenosisok, regurgitatiók a későbbi életkorban progressziót mutatva olyan hemodinamikai kórképeket, keringési elégtelenséget okozhatnak, melyek további beavatkozásokat igényelhetnek. A mitralis billentyűnek az atrioventricularis septumdefektushoz társuló betegsége koraszülöttkortól aggkorig minden életkorban előfordul, más-más műtéti megoldást igényelve. A szerzők részletezik a különböző életkorokra vonatkozóan a napjainkban lehetséges és szükséges műtéti megoldásokat, sebészi kihívásokat. A mitralis billentyű műbillentyűre történő cseréjében a klasszikus sebészi megoldások mellett napjainkban új beavatkozásokként jelentős számban jelentkeznek a katéteres intervenciós és hibrid megoldások. A felnőttkort egyre nagyobb számban megélő betegek fokozott odafigyelést, speciális ellátást igényelnek a kardiológusoktól, szívsebészektől. Orv Hetil. 2021; 162(35): 1397-1401. Summary. The critical point of the atrioventricular septal defect correction is to separate the common atrioventricular orifice, which results in the reconstruction of the mitral valve. The success of the correction depends on many anatomical aspects, therefore a perfect anatomical outcome is not always possible. The remaining valvular stenoses and regurgitations, showing progression at a later age, may result in hemodynamic disorders and circulatory insufficiency that may require further interventions. Mitral valve disease associated with atrioventricular septal defect occurs at all ages from preterm to adulthood, requiring different surgical solutions. The authors detail the possible and necessary surgical solutions and surgical challenges at different ages. In addition to the classic surgical solutions, a significant number of catheter interventional and hybrid solutions are emerging as new interventions in the replacement of the mitral valve with an artificial valve. An increasing number of patients living in adulthood require increased attention and special care from cardiologists and cardiac surgeons. Orv Hetil. 2021; 162(35): 1397-1401.

Introduction Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease. Abnormal aortic dimensions and elasticity parameters have been long described for corrected TOF (cTOF) together with left ventricular (LV) rotational abnormalities. The present study focuses on investigating LV rotational mechanics in cTOF, and possible correlation of these parameters with aortic elastic properties. It was also aimed to be examined whether different surgical strategies has any effect on the results. Methods The study involved 26 adult cTOF patients, from which 14 had palliative surgery first [Blaloc-Taussig (n=10), Waterstone-Cooley (n=2) shunts, or Brock procedure (n=2)] (mean age at the repair: 7.3±10.2 years) and a late total correction (mean age at the repair: 10.0±13.3 years) (pcTOF), while the early total correction was the treatment of choice in 12 patients (mean age at the repair: 4.2±3.2 years) (etrTOF). Their results were compared to that of 37 age- and gender-matched healthy adults. Routine transthoracic two-dimensional Doppler echocardiography extended with assessment of aortic elastic properties and three-dimensional speckle-tracking echocardiography (3DSTE) was performed in all cTOF patients and controls. Results Sixteen out of 26 cTOF patients showed normally directed LV rotational mechanics, while apical and basal LV rotations were in the same clockwise and counterclockwise directions in 7 and 3 cTOF cases, respectively (38%). This sort of LV movement is called as LV rigid body rotation (RBR). The ratio of LV-RBR between etrTOF and pcTOF patients did not differ significantly (33% vs. 43%, p=0.70). Significantly reduced LV apical rotation (5.2±3.7 degree vs. 10.2±4.5 degree, p Conclusions Significant LV rotational abnormalities could be demonstrated in cTOF with the high prevalence of LV-RBR. cTOF patients with early total reconstruction proved to have beneficial results. Abnormal physiologic response of LV rotational mechanics to increased aortic stiffness can be detected in cTOF patients without LV-RBR. Funding Acknowledgement Type of funding source: None

Introduction In diabetic patients, multiple cardiovascular outcome trials consistently showed the robust cardioprotective effects of the novel antidiabetic agents, sodium glucose cotransporter 2 (SGLT2) inhibitors. However, the DAPA-HF study using the SGLT2 inhibitor dapagliflozin have extended these observations onto non-diabetic patients with heart failure (HF), urging previous hypotheses regarding the cardioprotective effects of SGLT2 inhibitors to be revised. This is further complicated by the fact that SGLT2 is not expressed in the human myocardium neither under normal nor diseased states. Hence, it has been postulated that SGLT2 inhibitors might exert direct cardioprotection via non-specific inhibition of SGLT1, which is in turn highly expressed in the myocardium. Purpose Because literature data is scarce regarding the expression profile of myocardial SGLT1, we aimed to characterize left ventricular SGLT1 expression in humans with end-stage HF accordingly to HF aetiology and to investigate whether cardiac resynchronization therapy (CRT) affects SGLT1 expression. Methods From patients undergoing mitral valve replacement with otherwise no myocardial disease and preserved LV function, we collected control papillary muscles (Control, n=9). From patients with end-stage HF undergoing heart transplantation (n=72), we obtained LV anterior wall samples according to the following HF aetiology groups: hypertrophic cardiomyopathy (HCM, n=7); idiopathic dilated cardiomyopathy (DCM, n=12); ischaemic heart disease (IHD, n=14), IHD with type 2 diabetes mellitus (IHD+T2DM, n=11); and patients with CRT (CRT-DCM, n=9; CRT-IHD, n=9; CRT-IHD+T2DM, n=10). We measured LV SGLT1 expression on the gene and protein expression levels using qRT-PCR and western blotting, respectively. Echocardiography-derived LV end-diastolic diameter (LVEDD) and LV ejection fraction (LVEF) were registered prior to surgery. Results Compared to controls, LV SGLT1 mRNA and protein expressions were significantly upregulated in patients with DCM, IHD and IHD+T2DM (all P Conclusions Myocardial SGLT1 is upregulated in patients with HF (except HCM), and correlated strongly with parameters (LVEDD, LVEF) related to adverse LV remodelling. CRT was associated with reduced SGLT1 expression in DCM and IHD patients, but not in those with IHD+T2DM. Our results suggest that SGLT1 is upregulated in HF and might be implicated in adverse myocardial remodelling. Accordingly, whether SGLT2 inhibitors exert direct cardioprotection in HFrEF via non-specific inhibition of SGLT1 needs to be further elucidated. Funding Acknowledgement Type of funding source: Public grant(s) – National budget only. Main funding source(s): National Research, Development and Innovation Fund of Hungary, Higher Education Institutional Excellence Programme of the Ministry of Human Capacities of Hungary

Background When indicated, cardiac resynchronization therapy (biventricular pacing, CRT) decreases mortality in patients with heart failure (HF) and reduced ejection fraction, especially in those with non-ischemic cardiomyopathy. This is reflected by relatively rapid improvement of left ventricular (LV) end-diastolic diameter (LVEDD) and LV ejection fraction (LVEF) indicating reverse remodelling. These LV structural and functional improvements are accompanied by characteristic changes in LV gene expression profile. However, whether beneficial gene expression alterations related to biventricular pacing are sustained independently of structural and functional reverse remodelling is unclear. Purpose We aimed to compare LV fibrosis-related mRNA expression profile in end-stage HF patients with idiopathic dilated cardiomyopathy (DCM) who were not on CRT versus to those on CRT. Methods Left ventricular myocardial samples were harvested from end-stage HF patients undergoing heart transplantation (HTX). Inclusion criteria were negative family history of DCM, negative coronarography (i.e. non-ischemic), no relevant comorbidity (e.g. diabetes, hypertension) and no history of myocarditis. Accordingly, the following patient groups were included: 1.) DCM (n=12, 17% female, mean age [±standard deviation] 46.8±11.8 years) without CRT and 2.) CRT-DCM (n=12, 42% female, mean age 47.8±12.3 years) which comprised DCM patients on active CRT for mean 3.2±2.4 years until HTX. LV RNA was extracted and subjected to a commercially available mRNA expression panel interrogating 760 genes related to the development and regulation of fibrosis. Normalization to 10 housekeeping genes and batch corrections were conducted as per protocol. LV mRNA expression of atrial-natriuretic peptide (ANP) was quantified using qRT-PCR. Results Markers of reverse remodelling including LVEDD (73.4±8.3 mm vs 75.4±9.9 mm), LVEF (21.9±3.7% vs 18.5±6.8%) and LV ANP mRNA expression (arbitrary units: 1.05±1.80 vs 1.04±0.88) were comparable between DCM and CRT-DCM patients (all P>0.05), respectively. High-throughput mRNA expression screening revealed significant (all P Conclusions Independently of structural and functional reverse remodelling, CRT might be associated with slightly favourable LV expression profile of genes related to the regulation and development of fibrosis. This suggests that biventricular pacing might be beneficial on the molecular level beyond improvement of LV structure and function. Funding Acknowledgement Type of funding source: Public grant(s) – National budget only. Main funding source(s): National Research, Development and Innovation Fund of Hungary, Higher Education Institutional Excellence Programme of the Ministry of Human Capacities of Hungary

Összefoglaló. Bevezetés: A Marfan-szindróma autoszomális domináns módon öröklődő, szisztémás kötőszöveti betegség. A hosszú távú túlélés szempontjából fontos a nagyérkatasztrófák megelőzése. Szívsebészeti szempontból a legfontosabb elváltozás az aortagyök tágulata. Aortagyök-rekonstrukciós beavatkozásaink Bentall-DeBono-, David I. és módosított Yacoub-műtétek, melyek mind preventív jelleggel, mind dissectio esetén jó eredménnyel végezhetők. Célkitűzés: A marfanos betegeknél eltérő technikával végzett aortagyök-rekonstrukciós műtéteink összehasonlítása. Módszer: A Semmelweis Egyetem Városmajori Szív- és Érgyógyászati Klinikáján 1993 és 2020 között Marfan-szindrómásoknál elvégzett Bentall-DeBono-, David I. és módosított Yacoub-féle aortagyök-rekonstrukciókat elemeztük. A műtét szerinti csoportok életkora a beavatkozás idején 29,69 (21,98-41,25) év, 29,15 ± 11,99 év és 35,29 ± 14,14 év volt, a fenti sorrendben. Az adatok forrásául a Magyar Marfan Regiszter és az Aortagyök-rekonstrukciós Regiszter szolgált. Eredmények: Az utánkövetési idő 132 ± 81,04 hónap volt a Bentall-, 76 ± 27,77 hónap a David-, valamint 4,5 (0,75-11,75) hónap a Yacoub-műtét esetén. A David- és a Yacoub-beavatkozások gyakrabban voltak profilaktikusak, mint a Bentall-operációk (p = 0,0153; p = 0,0085). A Bentall-műtéteknél ritkább volt a primer műtét esetleges későbbi elégtelenségéből fakadó reoperáció, mint a David-operációknál (p0,001). David-beavatkozásnál a Bentall-műtéthez képest hosszabb volt a cardiopulmonaris bypass (p = 0,0013) és az aortalefogás ideje (p = 0,0048), valamint David- és Yacoub-műtét esetén gyakrabban lépett fel korai posztoperatív szövődmény, mint Bentall-operációnál (p = 0,0005; p = 0,0037). A késői szövődmények és a túlélés tekintetében a csoportok nem különböztek. Következtetés: Marfan-szindrómában a leggyakrabban halált okozó szövődmény az akut aortaruptura, illetve akut aortadissectio. Eredményeink alapján mindhárom profilaktikus aortagyök-rekonstrukciós műtéti típus jól reprodukálható és jó eredménnyel végezhető Marfan-szindrómában. Orv Hetil. 2021; 162(18): 696-704.Marfan syndrome is an autosomal dominant, systemic connective tissue disorder. Preventing vascular complications is essential for long-term survival. Aortic dilation is the main cardiac surgical manifestation. Bentall-DeBono, David I and modified Yacoub aortic root reconstructions treat and prevent aortic dissections with great outcomes.Comparing results of aortic root reconstructions in Marfan syndrome.We analysed the data of Bentall-DeBono, David I and modified Yacoub operations performed in Marfan syndrome at the Heart and Vascular Center, Semmelweis University between 1993 and 2020. Ages of surgical groups at the time of operation were 29.69 (21.98-41.25) years, 29.15 ± 11.99 years and 35.29 ± 14.14 years, respectively. Data were obtained from the Hungarian Marfan Register and the Aortic Root Reconstruction Register.Follow-up time was 132 ± 81.04 months for Bentall, 76 ± 27.77 months for David and 4.5 (0.75-11.75) months for Yacoub groups. David and Yacoub operations were prophylactic more frequently than Bentall ones (p = 0.0153; p = 0.0085). Freedom from reoperation after primary surgery insufficiency was more common for Bentall than for David procedure (p0.001). Compared to Bentall, David surgeries required longer cardiopulmonary bypass (p = 0.0013) and aortic cross clamp time (p = 0.0048), more early postoperative complications occurred after David and Yacoub, than after Bentall operations (p = 0.0005; p = 0.0037). Late complications and survival did not differ among the groups.In Marfan syndrome, acute aortic rupture and dissection are the main contributors to mortality. Based on our results, the prophylactic aortic root reconstructions are reproducible and can be performed with great outcomes. Orv Hetil. 2021; 162(18): 696-704.

Background: Behçet’s disease is an auto-inflammatory disorder categorized as a primer systemic vasculitis of unknown aetiology. Genetic factors, infectious agents and the irregularity of T-cell homeostasis are presumed to be responsible for the emergence of Behçet’s disease. Characteristic symptoms are multisystemic. Although cardiovascular involvement is rare, it should be noted due to the difficulty of surgical treatment options.Case Presentation: Our 44-year-old male patient underwent aortic valve replacement due to aortic regurgitation. At the 15-month follow-up, echocardiography showed detachment of the prosthetic valve and in the aortic root, multiple pseudo-aneurysms were identified. We performed an aortic root reconstruction with a Bentall procedure using a special „skirted” conduit to reduce strain in the suture line between the conduit and the extremely dilated left ventricular outflow tract. Conclusions: The surgical treatment of cardiovascular manifestations of Behçet’s disease remains challenging. This new technique may be beneficial in well-selected cases where the annulus of the aorta is extremely dilated or annular tissue disorder is present.

Background Pituitary dysfunction after brainstem death can cause various hormone deficiencies in potential heart donors. The aim of this study was to evaluate the relationship between hormone replacement therapy (HRT; including antidiuretic hormone analog, thyroid hormone, and methylprednisolone) in heart donors and the recipients’ outcomes after heart transplantation (HTx). Methods We retrospectively analyzed HTxs performed between January 2012 and October 2018. Donor and recipient characteristics were retrieved with a focus on endocrine parameters and HRT. The primary outcome was primary graft dysfunction (PGD). Secondary outcomes were the 30-day and 2-year mortality of the recipients. Univariate and multivariate Cox regression analyses were applied. Results The study included 297 HTxs. PGD occurred in 56 recipients (18.9%). In the multivariable Cox analysis, methylprednisolone and thyroxine treatment in donors were associated with a lower odds for PGD (odds ratio [OR], 0.43; 95% CI, 0.19-1.01; P = .052; and OR,: 0.34; 95% CI, 0.15-0.76; P = .009, respectively). In multivariate analysis, thyroxine treatment in donors was associated with a lower odds of PGD (OR, 0.38; 95% CI, 0.17-0.86; P = .020). Donor thyroxine supplementation also had a beneficial effect on recipients’ 2-year survival (OR, 0.53; 95% CI, 0.29-0.96; P = .036). Conclusions Combined thyroxine and methylprednisolone treatment could be a protective factor against PGD. Thyroxine administration was associated with better 2-year survival in recipients.

Rationale The beta-O-linkage of N-acetylglucosamine (i.e., O-GlcNAc) to proteins is a pro-adaptive response to cellular insults. To this end, increased protein O-GlcNAcylation improves short-term survival of cardiomyocytes subjected to acute injury. This observation has been repeated by multiple groups and in multiple models; however, whether increased protein O-GlcNAcylation plays a beneficial role in more chronic settings remains an open question. Objective Here, we queried whether increasing levels of cardiac protein O-GlcNAcylation would be beneficial during infarct-induced heart failure. Methods and results To achieve increased protein O-GlcNAcylation, we targeted Oga, the gene responsible for removing O-GlcNAc from proteins. Here, we generated mice with cardiomyocyte-restricted, tamoxifen-inducible haploinsufficient Oga gene. In the absence of infarction, we observed a slight reduction in ejection fraction in Oga deficient mice. Overall, Oga reduction had no major impact on ventricular function. In additional cohorts, mice of both sexes and both genotypes were subjected to infarct-induced heart failure and followed for up to four weeks, during which time cardiac function was assessed via echocardiography. Contrary to our prediction, the Oga deficient mice exhibited exacerbated—not improved—cardiac function at one week following infarction. When the observation was extended to 4 wk post-MI, this acute exacerbation was lost. Conclusions The present findings, coupled with our previous work, suggest that altering the ability of cardiomyocytes to either add or remove O-GlcNAc modifications to proteins exacerbates early infarct-induced heart failure. We speculate that more nuanced approaches to regulating O-GlcNAcylation are needed to understand its role—and, in particular, the possibility of cycling, in the pathophysiology of the failing heart.

Background: In complete or dextro-transposition of the great arteries (dTGA), the aorta and the pulmonary artery are transposed. The present study was designed to examine dTGAassociated left atrial (LA) volumetric and functional abnormalities in adult patients late after repair and to compare their results to those of healthy controls. Methods: The present study consisted of 15 dTGA patients (30.3 &plusmn, 8.1 years, 9 males), the patients had Mustard (n = 8) or Senning (n = 7) procedure performed. Their results were compared to those of 36 age- and gender-matched healthy subjects (28.7 &plusmn, 1.5 years, 24 males). Results: Increased maximum LA volume and reduced LA emptying fractions respecting the cardiac cycle could be demonstrated in our dTGA patients. LA stroke volumes representing all LA functions were significantly reduced. Peak LA circumferential, longitudinal, and area strains and LA circumferential, longitudinal, and area strains measured at atrial contraction were reduced in our dTGA patients. Most LA strains were reduced in patients having Mustard surgery compared to controls and patients undergoing Senning operation. Conclusions: Significant LA volumetric and functional abnormalities could be demonstrated in adult patients with dTGA late after repair. Senning procedure seems to have more beneficial long-term effects on LA volumetric and functional features as compared to the Mustard procedure.

The surgical management of congenital heart defects is changing continuously due to technical development. Aortic coarctation is one of the most frequent congenital heart diseases often with serious symptoms. The authors review the different surgical techniques (end-to-end anastomosis, pathplasty, interposition) and present the up-to-date procedures from infancy to adulthood. We assess the results, the benefits and disadvantages of the different procedures based on our and international experiences. The greatest surgical challenges are the management of the serious hypoplastic aortic arch in infancy and later evolving restenosis and complications (aneurysm, dissection). Nowadays, the management of aortic coarctation is very difficult because the surgical procedures are different in infancy, childhood and adult age. In the latter two cases, it is necessary to apply catheter interventions, too, the correct timing of which also present great challenges nowadays. Orv Hetil. 2020; 161(21): 861-866.

Funding Acknowledgements Project no. NVKP_16-1-2016-0017 has been implemented with the support provided from the National Research, Development and Innovation Fund of Hungary Introduction Bland-White-Garland syndrome or ALCAPA (anomalous left coronary artery from pulmonary artery) syndrome is a rare congenital disorder with a prevalence of 1: 300,000. This condition is one the most common causes of infant myocardial infarction. It can ultimately lead to heart failure before the age of one, however when pronounced left and right coronary artery collateralization is present, patients may remain asymptomatic until older age. Due to the development and availability of non-invasive imaging techniques, these patients diagnosed more frequently in recent years. Case description: We present a 53-year-old patient with treated hypertension and five uncomplicated pregnancies. At the age of 40, the patient was referred to coronary angiography due to her dyspnea on exertion. The examination showed that the left main coronary artery (LMCA) originates from the pulmonary artery 7 mm above the pulmonary valve and the right coronary artery is significantly dilated. Based on the mild complaints and good left ventricle systolic function, conservative therapy was recommended by the cardiac surgery team. Since 2018 the patient has had gradually worsening complaints despite of the therapy adjusted for chronic heart failure. Q-waves were depicted on the 12-lead ECG in aVL and V1-V6 leads. Echocardiography showed dilated left ventricle (LV), LV hypertrophy, diffuse hypokinesia, decreased LV systolic function and diastolic dysfunction. At our clinic, we performed cardiac magnetic resonance imaging (CMR) which showed moderately reduced LV ejection fraction, diffuse LV hypokinesia, increased LV volume, septal LV hypertrophy. We visualized the retrograde flow on the LMCA, the shunt volume at the anomalous artery was 1,27 L/min. The CMR also showed irreversible damage of the subendocardial region of the myocardium via late gadolinium enhancement in the area supplied by the LMCA. Since we planned to reevaluate the surgical possibilities, the collateral network with tortuous and dilated left and right coronary arteries were visualized by coronary CT angiography. The surgical team at our institute decided to perform myocardial revascularization and restoration of dual coronary artery supply. Discussion ALCAPA is usually diagnosed in infancy however, in about 10-15% of cases the disease is only recognized in adulthood. The long-term morphological and functional characteristics of the syndrome may include reversible/irreversible ischemic damage, systolic and diastolic dysfunction, mitral regurgitation, significant collateral circulation, left-to-right shunt, dilated coronary arteries. Surgical myocardial revascularization and restoration of dual coronary artery supply is recommended even in adult patients. Abstract P1338 Figure. CTA reconstruction and LGE CMR

Background Sinus of Valsalva aneurysm (SVA) is an uncommon cardiac anomaly, with an incidence of less than 1% of open heart surgery cases. Its evolution is most frequently silent, being found incidentally or discovered in the event of its acute rupture. Non-ruptured giant SVAs may cause unusual clinical manifestations, as a consequence of their protrusion into the heart chambers or compression of the coronary vessels and are frequently associated with aortic insufficiency of various degrees of severity. The gold standard treatment for SVAs consists of complete replacement of the aortic root and valve. However, in certain cases, valve-sparing procedures may prove to be a more suitable alternative. Case presentation A 68-year-old male patient presented with dyspnea as symptom caused by a large (> 5 cm) right sinus of Valsalva aneurysm. The aneurysm was occupying most of the right ventricle and was associated with severe aortic regurgitation. The surgical treatment of the condition involved valve-sparing root reconstruction procedure (remodeling technique), completed with external stabilization of the aortic valve annulus via running suture annuloplasty. Following the uneventful intervention, the patient did well and his status improved. The follow-up transthoracic echocardiography obtained 1 month after surgery showed a fully competent aortic valve with no regurgitation. Conclusions Despite complete aortic root and valve replacement being considered the safest approach to large SVAs complicated with aortic insufficiency, valve-sparing procedures should not be overlooked in case of a dilated aortic root with uncalcified aortic valve. Performing valve-sparing by applying a remodeling technique operation completed with annuloplasty reduces aortic valve insufficiency, avoiding side-effects related to implanted valves.

Abstract: The authors give a short introduction of the Hungarian and the international history of adult heart transplantation, and highlight the similarities in the evolution of the two programs. Their aim was to show how the Hungarian post-transplant survival changed in the last five years. They wanted to investigate how all the changes they had made in the program affected the post-transplant results. They investigated 496 heart transplantation data and compared to international data. Orv Hetil. 2018; 159(46): 1869–1875.

Abstract: The mechanical circulatory support (MCS) program of the Semmelweis University Heart and Vascular Centre has become established over the last five years. The main requirements of our MCS program to be developed first were the Heart Transplantation and Heart Failure Intensive Care Unit and a well trained medical team. The wide range of mechanical circulatory support devices provides suitable background for the adequate treatment of our patients in all indications. In this review, we present our results related to extracorporeal membrane oxygenation (ECMO) supports performed in the last five years. Between 2012 and 2017, we applied MCS support in 140 cases, among them 111 patients received ECMO support. The leading indications of ECMO support were the following: primary graft failure after heart transplantation (33 cases), postcardiotomy cardiogenic shock (18 patients), acute decompensation of end-stage heart failure (14 patients), acute myocardial infarction complicated with refractory cardiogenic shock (37 patients), cardiogenic shock developed after transcatheter aortic valve implantation (3 patients), malignant arrhythmia due to drug intoxication (1 patient) and acute respiratory distress syndrome (4 cases). The mortality of patients receiving ECMO support was 46%. The analysis of the results of ECMO support needs to change our approach. The mortality results show that we lost the half of our patients. However, the mortality in the conventionally treated patients would have been 100% without ECMO. In fact, we could save the life of half of these patients. Orv Hetil. 2018; 159(46): 1876–1881.

Absztrakt: Bevezetés: A sebészi diszciplínákban a műtétetek megtervezése statikus modelleken alapulnak. Azonban a számítástechnikai fejlődése lehetőséget nyitott a dinamikus modellek preoperatív megtervezésére és intraoperatív alkalmazásukra. Célunk volt, olyan döntéstámogató rendszer megalkotása a szívsebészeti gyakorlatban, melynek segítségével az áramlástani, kamramorfológiai, térfogat és posztoperatív kamrafunkció predikcióját végezhetjük kamrarekonstrukciós műtéteknél. Módszerek: Saját fejlesztésű szoftver használatával DICOM fájlok importálását követően a dilatált bal kamrát három dimenzióban modellezzük. Ezt követő áramlástani modellezések segítségével az idővel és a kamratérfogat változásával dinamikusan változó paramétereket: az áramlástani profilt, turbulenciát és a bal kamrában fellépő nyíróerők hatását a myocardium funkciójára kiszámítjuk. In silico modellben – a prediktált adatokat felhasználva – megtervezzük a balkamra-rekonstrukciós műtét egyes lépéseit. A tervezés alapján a kiszámított rezekciós vonalak hosszát alkalmazva történtek a műtétek. Eredmények: Összesen 50 esetben történt 3D tervezés. A bal kamra geometriáját jellemző konicitási és sphericitási indexek szignifikánsan javultak a műtétet követően (0,42 vs. 0,67 és 0,36 vs. 0,72, p < 0,05). A bal kamra izomzatára ható nyíróerő szignifikánsan 83%-kal csökkent, illetve a kamra turbulens áramlásprofilja szignifikánsan javult (2712 vs. 1823, p < 0,05). Következésképpen a szív pumpafunkciója javult a műtétet követően. Konklúzió: Technikánk alkalmazásával a balkamra-rekonstrukciós műtétek egyes lépéseinek standardizálásával és e lépések intraoperatív alkalmazásának kidolgozásával sikerült egy új sebészi döntéstámogató rendszert létrehoznunk. Az így megtervezett és személyre szabott műtétekkel növelhettük ezen magas kockázatú betegcsoport posztoperatív életminőségét és túlélését.

Abstract: Low output syndrome significantly increases morbidity and mortality of cardiac surgery and lengthens the durations of intensive care unit and hospital stays. Its treatment by catecholamines can lead to undesirable systemic and cardiac complications. Levosimendan is a calcium sensitiser and adenosine triphosphate (ATP)-sensitive potassium channel (IK,ATP) opener agent. Due to these effects, it improves myocardium performance, does not influence adversely the balance between O2 supply and demand, and possesses cardioprotective and organ protective properties as well. Based on the scientific literature and experts’ opinions, a European recommendation was published on the perioperative use of levosimendan in cardiac surgery in 2015. Along this line, and also taking into consideration cardiac surgeon, anaesthesiologist and cardiologist representatives of the seven Hungarian heart centres and the children heart centre, the Hungarian recommendation has been formulated that is based on two pillars: literature evidence and Hungarian expert opinions. The reviewed fields are: coronary and valvular surgery, assist device implantation, heart transplantation both in adult and pediatric cardiologic practice. Orv Hetil. 2018; 159(22): 870–877.