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Az aortaívet érintő aortabetegségek miatt végzett endovascularis rekonstrukciók (thoracic endovascular aneurysm repair – TEVAR) során a sztentgraft proximalis rögzítése az ívben vagy az aorta ascendensen van. Ilyen esetben hagyományosan nyitott műtéttel előzetesen biztosítjuk a lefedésre kerülő supraaorticus ágak keringését (ún. ’debranching’ műtétek). Nyitott műtétre nem alkalmas betegek esetén azonban az ágak endovascularis módszerekkel történő megtartására kényszerülünk. Tanulmányunkban ezen komplex endovascularis aortaív-rekonstrukciók lehetőségeit mutatjuk be. A párhuzamos graftokat jellemzően sürgősségi körülmények között alkalmazzuk. Az ascendensen történő proximalis rögzítés esetén a jobb arteria (a.) carotisról indított ’debranching’ és a truncus brachiocephalicus párhuzamos grafttal történő biztosításával kombinált hibrid műtétet végeztünk. Létfontosságú ér véletlen lefedésével járó TEVAR esetén sürgősséggel végezhetünk konverziót például a bal a. carotis communis keringésének gyors helyreállítására. A bal a. subclavia előzetes revascularisatiója nélkül végzett sürgősségi TEVAR után ritkán jelentkező bal felső végtagi ischaemia esetén utólagos konverziót végezhetünk a bal a. subclavia lumenének helyreállítására ugyancsak párhuzamos grafttal. A kisgörbületen elhelyezkedő, saccularis morfológiájú penetráló aortafekélyek sikeres kirekesztését segítheti egyedi gyártású graft alkalmazása, melyen a nagygörbületen lévő supraaorticus érszájadék köré kivágást, ún. ’scallop’-ot helyezünk a graft proximalis végéhez, megnövelve így a proximalis nyak hosszát. Elektív körülmények között ugyancsak egyedileg gyártott elágazó graftot is alkalmazhatunk, melynek során akár mindhárom ág megtartható az ascendensről induló proximalis rögzítés mellett, így arra alkalmas anatómia esetén endovascularis ívcserére is lehetőségünk van. Orv Hetil. 2023; 164(11): 426–431.

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.

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. Az aortadissectio krónikus stádiumában kialakuló thoracoabdominalis tágulatok megoldása multidiszciplináris megközelítést, nagy felkészültséget és fejlett technológiát igényel. A jellemzően többlépcsős műtétsorozat mortalitása és morbiditása az endovascularis technológia fejlődésével csökkent, de még mindig jelentős. A fenesztrált endovascularis aortaműtét a thoracoabdominalis nyitott műtét alternatívája, mely kisebb mortalitással és morbiditással, rövidebb kórházi tartózkodással jár. Aortadissectio esetén történő alkalmazása az aorta lumenében lévő membrán miatt kihívást jelent. Esetbemutatásunkban egy 56 éves nőbeteget demonstrálunk, aki tíz évvel korábban A-típusú dissectio miatt aorta ascendens rekonstrukción esett át. A követés során csaknem a teljes aorta tágulata alakult ki, melynek megoldása három lépésben történt. Az első lépésben a disszekált aortaív nyitott műtétjét végeztük ’frozen elephant trunk’ technikával, majd az aorta descendens tágulatának endovascularis kezelése történt sztentgraft-implantációval. A műtétsorozat záró lépése egy fenesztrált endovascularis aortaműtét volt, mely egyben ezen technikának az aortadissectio esetében történt első hazai alkalmazását jelenti. Orv Hetil. 2021; 162(31): 1260–1264. Summary. Thoracoabdominal aortic aneurysms developing in the chronic phase of an aortic dissection require multidisciplinary approach, experienced operators and advanced technology. The mortality and morbidity rate of these multistage operations were reduced with the latest technical achievements in endovascular repair, but they are still significant. Fenestrated endovascular aortic repair, an alternative of thoracoabdominal open repair, is associated with less mortality and morbidity, shorter hospital stay. Using fenestrated devices in aortic dissection is usually technically demanding due to the dissection membrane. We report the case of a 56-year-old woman, who underwent ascending aortic repair due to type A aortic dissection. During the follow-up, a large thoracoabdominal aneurysm developed involving also the arch. We performed a three-stage operation starting with the open repair of the aortic arch using a ‘frozen elephant trunk’ device followed by a thoracic endovascular aortic repair of the descending aorta. The final stage was a fenestrated endovascular aortic repair, which is the first use of this technique in aortic dissection in Hungary. Orv Hetil. 2021; 162(31): 1260–1264.

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.

We present a complex Marfan case, with previous type A aortic dissection, subsequent progressing aortic arch aneurysm, type B chronic aortic dissection, and Barlow disease with severe mitral regurgitation, all expressions of the same phenotype, all needing staged complex surgical therapies. (Level of Difficulty: Intermediate.), Graphical abstract

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.

BACKGROUND Marfan syndrome (MFS) is a systemic connective tissue disorder belonging to a group of rare diseases. Several psychologically distressing factors can challenge life for MFS patients. The aim of the present study was, therefore, to assess the psychological and psychosocial aspects of MFS with the goal of identifying a means of improving disease management for patients. METHODS A total of 66 adult patients with MFS were enrolled into the study prospectively and were divided into operated (OP) and non-operated (NOP) subgroups. Multiple questionnaire tests were used to determine the mental and physical state of our patients. Demographic and surgical data were collected. The results of the tests were also compared to the Hungarostudy (HS) population (representing the average Hungarian population) by using a propensity-matched control. RESULTS OP group scores yielded more alcohol consumption (P

BackgroundClinical evidence suggests that the currently recommended approach to estimate the risk of aortic dissection in Marfan syndrome (MFS) is not reliable enough. Therefore, we investigated the possible role of visceral arterial tortuosity in the risk stratification.Methods and resultsSplenic and renal arteries of 37 MFS patients and 74 age and gender matched control subjects were segmented using CT angiography imaging. To measure tortuosity, distance metric (DM), sum of angles metric (SOAM), inflection count metric (ICM), and the ratio of ICM and SOAM (ICM/SOAM) were calculated. DM of the splenic, right and left renal artery was significantly higher in MFS patients than in controls (2.44 [1.92-2.80] vs. 1.75 [1.57-2.18]p p = 0.011; 1.40 [1.29-1.70] vs. 1.13 [1.09-1.23]p p < 0.001; 26.52 [20.69-30.24] vs. 19.95 [16.47-22.95]p p ConclusionTo our knowledge this is the first demonstration of increased arterial tortuosity in MFS on visceral arteries. Visceral arterial tortuosity, dominated by curves of lower frequency but higher amplitude according to the observed opposite tendency between the DM and SOAM metrics, could be a possible new predictor of serious manifestations of MFS.

Heart transplantation remains the definitive therapy of end-stage heart failure. Ischemia-reperfusion injury occurring during transplantation is a primary determinant of long-term outcome of heart transplantation and primary graft insufficiency. Modification of the nitric oxide/soluble guanylate cyclase/cyclic guanosine monophosphate signaling pathway appears to be one of the most promising among the pharmacological interventional options. We aimed at characterizing the cardio-protective effects of the soluble guanylate cyclase stimulator riociguat in a rat model of heterotopic heart transplantation. Donor Lewis rats were treated orally with either riociguat or placebo for two days (n = 9) in each transplanted group and (n = 7) in donor groups. Following explantation, hearts were heterotopically transplanted. After one hour reperfusion, left ventricular pressure-volume relations and coronary blood flow were recorded. Molecular biological measurements and histological examination were also completed. Left ventricular contractility (systolic pressure: 117 ± 13 vs. 48 ± 5 mmHg, p max: 2963 ± 221 vs. 1653 ± 159 mmHg/s, p min: −2014 ± 305 vs. −1063 ± 177 mmHg/s, p = 0.02; all at 120 µl of left ventricular volume), and alteration of coronary blood flow standardized to heart weight (2.55 ± 0.32 vs. 1.67 ± 0.22 ml/min/g, p = 0.03) were markedly increased following preconditioning with riociguat. Myocardial apoptosis markers were also significantly reduced in the riociguat pretreated group as well as the antioxidant markers were elevated. Pharmacological preconditioning with riociguat decreases ischemia-reperfusion injury and improves donor organ function in our animal model of heart transplantation. Therefore, riociguat might be a potential cardioprotective agent.

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.

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.

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.

Introduction Marfan syndrome is a genetic disorder affecting the connective tissue. Changes in lung tissue might influence respiratory function; however, a detailed respiratory functional assessment according to the need for major thoracic surgery is missing. Methods Comprehensive pulmonary examinations were performed in 55 Marfan patients including respiratory symptoms, lung function (LF) testing using European Coal and Steel Community (ECSC) reference values, TLCO and quality of life measurements. Groups included patients who did not need surgery (Mf, n = 32) and those who underwent major thoracic surgery (Mfop, n = 23). Results Respiratory symptoms affected 20% of patients. Scoliosis was significantly more frequent in the Mfop group. LF demonstrated in all Marfan patients a tendency towards airway obstruction (FEV1/FVC = 0.77 ± 0.10), more prominent in Mfop patients (0.74 ± 0.08 vs. Mf: 0.80 ± 0.11; p = 0.03). Correction of LF values using a standing height modification by arm span (Hcorrected) revealed additional changes in FVC and FEV1. TLCO and quality of life did not differ between groups. Conclusions Marfan syndrome is associated with airway obstruction, especially in patients who have undergone major thoracic surgery, indicative of more severe connective tissue malfunction. The use of arm span for height correction is suitable to evaluate LF changes in this special patient group including patients with significant scoliosis.

Background Marfan syndrome (MFS) is a genetically determined systemic connective tissue disorder, caused by a mutation in the FBN1 gene. In MFS mainly the cardiovascular, musculoskeletal and ocular systems are affected. The most dangerous manifestation of MFS is aortic dissection, which needs to be prevented by a prophylactic aortic root replacement. Main body The indication criteria for the prophylactic procedure is currently based on aortic diameter, however aortic dissections below the threshold defined in the guidelines have been reported, highlighting the need for a more accurate risk stratification system to predict the occurrence of aortic complications. The aim of this review is to present the current knowledge on the possible predictors of severe cardiovascular manifestations in MFS patients, demonstrating the wide range of molecular and radiological differences between people with MFS and healthy individuals, and more importantly between MFS patients with and without advanced aortic manifestations. These differences originating from the underlying common molecular pathological processes can be assessed by laboratory (e.g. genetic testing) and imaging techniques to serve as biomarkers of severe aortic involvement. In this review we paid special attention to the rapidly expanding field of genotype–phenotype correlations for aortic features as by collecting and presenting the ever growing number of correlations, future perspectives for risk stratification can be outlined. Conclusions Data on promising biomarkers of severe aortic complications of MFS have been accumulating steadily. However, more unifying studies are required to further evaluate the applicability of the discussed predictors with the aim of improving the risk stratification and therefore the life expectancy and quality of life of MFS patients.

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.

Marfan syndrome is a genetic disorder of the connective tissue, including involvement of the lungs.Pulmonary function test was performed in 32 asymptomatic adult Marfan patients using European Community for Coal and Steel (ECCS) and Global Lung Function Initiative (GLI) reference values.Using GLI equations for reference, significantly lower lung function values were noted for forced vital capacity (FVC) (87.0 ± 16.6% vs. 97.1 ± 16.9%; P < 0.01) and forced expiratory volume in the first second (FEV1) (79.6 ± 18.9% vs. 88.0 ± 19.1%; P < 0.01) predicted compared to ECCS. Obstructive ventilatory pattern was present in 25% of the cases when calculating with GLI lower limit of normal (LLN), and it was significantly more common in men as compared to women (n = 6, 50% vs. n = 2, 10%; P = 0.03).GLI is more suitable to detect early ventilatory changes including airway obstruction in young patients with special anatomic features, and should be used as a standard way of evaluation in asymptomatic Marfan population.

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.

Introduction Hungary joined Eurotransplant International (ET) to improve the chance of transplantation for Hungarian patients and patient outcomes, including access and graft and patient survival. After 5 years of full membership, the evaluation of numbers and quality indicators is possible. Method A comparison was made between 5 years prior to a preliminary cooperation agreement (2007-2011) and 5 years after full ET membership (2014-2018). During the 2 study periods, we analyzed numbers and circumstances of deceased organ donors, multiorgan donors, donated organs, and transplantations in Hungary and development of waiting lists along with international organ exchanges. Result The number of actual organ donors increased by 22.09% (729 vs 890), an additional 823 organ removals represents an increase of 42.71% (1927 vs 2750). There were 46.51% more transplants managed in the selected periods (1561 vs 2287). The number of new patients on the waiting list increased (2305 vs 3247; 40.87%). The mean kidney mismatch number decreased from 3.21 to 2.96. Conclusion Joining ET has been an effective and efficient in terms of increasing access to organs and the lives of patients on the Hungarian waiting list posttransplant. It is also a benefit for patients with special needs because the number of organ transplants is greater than the increased number of donors.

Összefoglaló. Az aortadissectio krónikus stádiumában kialakuló thoracoabdominalis tágulatok megoldása multidiszciplináris megközelítést, nagy felkészültséget és fejlett technológiát igényel. A jellemzően többlépcsős műtétsorozat mortalitása és morbiditása az endovascularis technológia fejlődésével csökkent, de még mindig jelentős. A fenesztrált endovascularis aortaműtét a thoracoabdominalis nyitott műtét alternatívája, mely kisebb mortalitással és morbiditással, rövidebb kórházi tartózkodással jár. Aortadissectio esetén történő alkalmazása az aorta lumenében lévő membrán miatt kihívást jelent. Esetbemutatásunkban egy 56 éves nőbeteget demonstrálunk, aki tíz évvel korábban A-típusú dissectio miatt aorta ascendens rekonstrukción esett át. A követés során csaknem a teljes aorta tágulata alakult ki, melynek megoldása három lépésben történt. Az első lépésben a disszekált aortaív nyitott műtétjét végeztük 'frozen elephant trunk' technikával, majd az aorta descendens tágulatának endovascularis kezelése történt sztentgraft-implantációval. A műtétsorozat záró lépése egy fenesztrált endovascularis aortaműtét volt, mely egyben ezen technikának az aortadissectio esetében történt első hazai alkalmazását jelenti. Orv Hetil. 2021; 162(31): 1260-1264. Summary. Thoracoabdominal aortic aneurysms developing in the chronic phase of an aortic dissection require multidisciplinary approach, experienced operators and advanced technology. The mortality and morbidity rate of these multistage operations were reduced with the latest technical achievements in endovascular repair, but they are still significant. Fenestrated endovascular aortic repair, an alternative of thoracoabdominal open repair, is associated with less mortality and morbidity, shorter hospital stay. Using fenestrated devices in aortic dissection is usually technically demanding due to the dissection membrane. We report the case of a 56-year-old woman, who underwent ascending aortic repair due to type A aortic dissection. During the follow-up, a large thoracoabdominal aneurysm developed involving also the arch. We performed a three-stage operation starting with the open repair of the aortic arch using a 'frozen elephant trunk' device followed by a thoracic endovascular aortic repair of the descending aorta. The final stage was a fenestrated endovascular aortic repair, which is the first use of this technique in aortic dissection in Hungary. Orv Hetil. 2021; 162(31): 1260-1264.

BackgroundMarfan syndrome (MFS) is a systemic connective tissue disorder with life-threatening manifestations affecting the ascending aorta. MFS is caused by dominant negative (DN) and haploinsufficient (HI) mutations of theFBN1gene. Our aim was to identify mutations of MFS patients with high detection rate and to investigate the use of a gene panel for patients with Marfanoid habitus. We also aimed to examine correlations between genotype and cardiovascular manifestations to predict “malignant” mutations.Methods136 individuals were enrolled. In the first phase, next-generation sequencing (NGS) and Sanger sequencing were performed for 57 patients to screen theFBN1gene, followed by multiplex ligation-dependent probe amplification (MLPA) in negative cases. For repeated negative results, NGS gene panel involving 9 genes was used. In the second phase, 79 patients were tested primarily with the same gene panel, negative samples were tested by MLPA.Results84 pathogenic mutations were detected, out of which 78 affectedFBN1, 6 non-FBN1mutations (2TGFB2, 1TGFBR2, 2TGFBR1, 1SMAD3) are associated with Loeys-Dietz syndrome (LDS). LDS patients had lower systemic score and they were younger, but their aortic involvement did not differ. MLPA detected 4 multi-exon deletions ofFBN1gene, which could not be identified by our first-step screening method. Aortic involvement (aortic dissection and/or dilation) did not differ significantly among HI and DN mutations (p = 0.061). Combined group of HI and DN mutations eliminating a disulphide-bonding cysteine (DN Cys) had significantly higher aortic involvement rate than DN mutations not eliminating a disulphide-bonding cysteine (DN non-Cys) (p p = 0.042 andp = 0.015, respectively).ConclusionsDue to the relevant number of mutations affecting genes other thanFBN1, preferred approach for testing individuals with Marfanoid habitus is using a gene panel rather than single-gene analysis, followed by MLPA for negative samples. DN Cys and HI mutations should be considered as risk factors for aortic involvement. Genetic testing for patients with Marfanoid features and a systemic score under 7 is recommended, as LDS patients may have lower scores, but they may have severe cardiovascular manifestations.

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.

Marfan syndrome is a genetic disorder of the connective tissue, including involvement of the lungs. Pulmonary function test was performed in 33 asymptomatic adult Marfan patients using European Community for Coal and Steel (ECCS) and Global Lung Function Initiative (GLI) reference values. Using GLI equations for reference, significantly lower lung function values were noted for forced vital capacity (FVC) (87.02±16.62 vs. 97.11±16.89; p Airway obstruction is dependent on reference equations used in Marfan patients. GLI reference equations are more suitable to evaluate lung function changes in patients with connective tissue disorders.

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 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.

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.Absztrakt: A 3D tervezés és 3D nyomtatás nyújtotta lehetőségek folyamatosan bővülnek az orvosi gyakorlatban. A technológia leggyakoribb felhasználási területe a 3D anatómiai modellek nyomtatása sebészi döntéstámogatás céljából. Az így személyre szabott és kinyomtatott modelleknek számos egyéb felhasználási területük van: komplex anatómiai szituációk pontos megjelenítése, az adott beteg sebészi beavatkozásának szimulációja a tényleges beavatkozást megelőzően, betegoktatás és a különböző diszciplínák között az eset megbeszélésének megkönnyítése. A technológia szívsebészeti vonatkozásában kiemelendő a kamrákat és a nagyereket érintő elváltozások 3D anatómiai modellezése és funkcionális elemzése, míg a mellkassebészetben az onkológiai betegek erősen vaszkularizált tumorának eradikálásakor lehet a sebészi terápia felállításában szerepe. A virtuális és 3D nyomtatott modellek új diagnosztikai lehetőséget jelentenek, melyek segítségével egyes sebészi beavatkozások standardizálhatók, így személyre szabott terápiás döntéseket lehet kidolgozni. A 3D projekt a Semmelweis Egyetemen 2018-ban kezdődött a Semmelweis Egyetem Városmajori Szív- és Érgyógyászati Klinikájának és az Országos Onkológiai Intézet Mellkassebészeti Osztályának kooperációja során. A szerzők a technológia ismertetése mellett az eddigi 121 tervezés és 49 személyre szabott 3D nyomtatás során megszerzett tapasztalataikat és a technológia orvosi szempontból való előnyeit ismertetik. Orv Hetil. 2019; 160(50): 1967–1975.

Copy number variations (CNVs) comprise about 10% of reported disease-causing mutations in Mendelian disorders. Nevertheless, pathogenic CNVs may have been under-detected due to the lack or insufficient use of appropriate detection methods. In this report, on the example of the diagnostic odyssey of a patient with Marfan syndrome (MFS) harboring a hitherto unreported 32-kb FBN1 deletion, we highlight the need for and the feasibility of testing for CNVs (>1 kb) in Mendelian disorders in the current next-generation sequencing (NGS) era.

Introduction: The majority of patients with Marfan syndrome undergo Bentall procedure. Several periprocedural complications may arise; cardiac complications play the most dominant role. Case presentation: A patient with Marfan syndrome developed symptoms two years after the Bentall procedure. Coronary CT angiography revealed bilateral coronary ostial stenosis. Percutaneous coronary intervention was performed, and two drug-eluting stents were implanted. Conclusions: Coronary CT angiography is a valuable tool for the examination of symptomatic patients following surgical aortic root replacement.

While heart transplantation (HTX) is the definitive therapy of heart failure, donor shortage is emerging. Pharmacological activation of soluble guanylate cyclase (sGC) and increased cGMP-signalling have been reported to have cardioprotective properties. Gemfibrozil has recently been shown to exert sGC activating effects in vitro. We aimed to investigate whether pharmacological preconditioning of donor hearts with gemfibrozil could protect against ischemia/reperfusion injury and preserve myocardial function in a heterotopic rat HTX model. Donor Lewis rats received p.o. gemfibrozil (150 mg/kg body weight) or vehicle for 2 days. The hearts were explanted, stored for 1 h in cold preservation solution, and heterotopically transplanted. 1 h after starting reperfusion, left ventricular (LV) pressure-volume relations and coronary blood flow (CBF) were assessed to evaluate early post-transplant graft function. After 1 h reperfusion, LV contractility, active relaxation and CBF were significantly (p

Background Marfan syndrome is a genetic disease, presenting with dysfunction of connective tissues leading to lesions in the cardiovascular and skeletal muscle system. Within these symptoms, the most typical is weakness of the connective tissue in the aorta, manifesting as aortic dilatation (aneurysm). This could, in turn, become annuloaortic ectasia, or life-threatening dissection. As a result, life-saving and preventative cardiac surgical interventions are frequent among Marfan syndrome patients. Aortic aneurysm could turn into annuloaortic ectasia or life-threatening dissection, thus life-saving and preventive cardiac surgical interventions are frequent among patients with Marfan syndrome. We hypothesized that patients with Marfan syndrome have different level of anxiety, depression and satisfaction with life compared to that of the non-clinical patient population. Methods Patients diagnosed with Marfan syndrome were divided into 3 groups: those scheduled for prophylactic surgery, those needing acute surgery, and those without need for surgery (n = 9, 19, 17, respectively). To examine the psychological features of the patients, Spielberger’s anxiety (STAI) test, Beck’s Depression questionnaire (BDI), the Berne Questionnaire of Subjective Well-being, and the Satisfaction with Life scale were applied. Results A significant difference was found in trait anxiety between healthy individuals and patients with Marfan syndrome after acute life-saving surgery (p 0.1). Finally, a significant, medium size effect was found between patient groups on the Joy in Living scale (F (2.39) = 3.51, p = 0.040, η2 = 0.15). Conclusions Involving psychiatric and mental-health care, in addition to existing surgical treatment interventions, is essential for more successful recovery of patients with Marfan syndrome.

Heterotopic abdominal rat heart transplantation has been extensively used to investigate ischemic-reperfusion injury, immunological consequences during heart transplantations and also to study remodeling of the myocardium due to volume unloading. We provide a unique review on the latter and present a summary of the experimental studies on rat heart transplantation to illustrate changes that occur to the myocardium due to volume unloading. We divided the literature based on whether normal or failing rat heart models were used. This analysis may provide a basis to understand the physiological effects of mechanical circulatory support therapy.

Purpose The key goal of cold storage is to maintain cell viability for a prolonged time during solid organ transplantation. Methane (CH4) has been recognized as novel therapeutic gas exerting anti-inflammatory effects in ischemia-reperfusion (IR) injuries. We aimed to investigate whether cold storage of donor hearts in CH4-enriched Custodiol preservation solution could protect against IR and preserve myocardial function in a rat model of heterotopic heart transplantation (HTX). Methods The hearts of donor Lewis rats were explanted and stored for 1 h in cold Custodiol (CS group, n=12) or in CH4-saturated (0.054 mg/100 ml) Custodiol (CS-CH4 group, n=12). 60 min after HTX left ventricular (LV) pressure-volume relations and coronary blood flow (CBF) were assessed to evaluate early post-transplant graft function. At the end of haemodynamic measurements, samples were taken for qPCR of endoplasmic reticulum (ER) stress and mitochondria-related apoptosis markers (CHOP, GRP78, GSK3β, VLDR, Caspase 3 and 9, Bcl2, Bax), biochemical parameters and mitochondrial functional analysis with high-resolution respirometry (Oxygraph2K, Austria). Results LV contractility (LV systolic pressure at 120µl of LV volume: 86±6 vs. 57±7mmHg, p=0.01; dP/dtmax: 2326±167 vs. 1583±139mmHg, p=0.007) and active relaxation (dP/dtmin at 120µl of LV volume: -1660±185 vs. -1043±169mmHg, p=0.04) improved significantly after an hour of reperfusion, while alteration of CBF standardized to heart weight (2.11±0.35 vs. 1.13±0.2ml/min/g, p=0.04) was also significantly improved following pretreatment. CS-CH4 storage significantly reduced the transcription of pro-apoptotic proteins and Bcl2/Bax ratios as compared to CS grafts. Increased mitochondrial oxidative phosphorylation, reduced leak respiration and cytochrome c release were demonstrated in response to CS-CH4 preservation. Conclusion These results might provide reliable evidence for the benefit of CH4-enriched preservation solution during HTX, through a mechanism which involves the inhibition of pro-apoptotic signals. Hence CH4-enriched preservation solution could be a potential cardioprotective agent in the inventory of heart transplantation surgery and other cardiac surgical procedures requiring prolonged cardioplegia.