The role of transforming growth factor-beta in Marfan syndrome

The starting point, in Marfan syndrome (MFS) appears to be the mutation of fi brillin-1 gene whose deconstructed protein product cannot bind transforming growth factor beta (TGF-b), leading to an increased TGF-b tissue level. The aim of this review is to review the already known features of the cellular signal transduction downstream to TGF-b and its impact onthe tissue homeostasis of microfibrils, and elastic fi bers. We also investigate current data onthe extracellular regulation of TGF-b level including mechanotransduction and the feedback cycles of integrin-dependent and independent activation of the latent TGF-b complex. Togetherthese factors, by the destruction of the connective tissue fi bers, may play an important role inthe development of the diverse cardiac and extracardiac manifestations of MFS and many of them could be a target of conservative treatment. We present currently investigated drugs for thetreatment of the syndrome, and explore possible avenues of research into pathogenesis of MFS in order to improve understanding of the disease

Bentall procedure: quarter century of clinical experiences of a single surgeon

BACKGROUND: We retrospectively analyzed 25 years of experiences with the button Bentall procedure in patients with aortic root pathologies. Even though this procedure has become widespread, there are only a few very long term follow-ups available in the clinical literature, especially regarding single surgeon results. METHODS: Between 1988 and 2013, a total of 147 patients underwent the Bentall procedure by the same surgeon. Among them there were 62 patients with Marfan syndrome. At the time of the surgery the mean age was 46.5 +/- 17.6 years. The impact of surgical experience on long-term survival was evaluated using a cumulative sum analysis chart. RESULTS: The Kaplan-Meier estimated overall survival rates for the 147 patients were 91.8 +/- 2.3 %, 84.3 +/- 3.1 %, 76.3 +/- 4.9 % and 59.5 +/- 10.7 % at 1,5,10 and 20 years, respectively. Multivariate Cox regression analysis identified EuroSCORE II over 3 % (OR 4.245, 95 % CI, 1.739-10.364, p = 0.002), acute indication (OR 2.942, 95 % CI, 1.158-7.480, p = 0.023), use of deep hypothermic circulatory arrest (OR 3.267, 95 % CI, 1.283-8.323, p = 0.013), chronic kidney disease (OR 6.865, 95 % CI, 1.339-35.189, p = 0.021) and early complication (OR 3.134, 95 % CI, 1.246-7.883, p = 0.015) as significant risk factors for the late overall death. The survival rate for freedom from early complication was 94.3 +/- 2.2 %, 88.0 +/- 3.3 %, 82.9 +/- 4.7 % and 69.2 +/- 8.4 % at 1,5,10 and 20 years. The main pathological findings of the aortic wall were cystic medial degeneration in 75 %, fibrosis in 6 %, atherosclerosis in 13 % and no pathological alteration in 6 % of the samples. The overall survival rate was significantly lower in patients operated in first 15 years compared to patients operated in the last decade (log-rank p = 0.011). CONCLUSION: According to our long-term follow-up the Bentall operation provides an appropriate functional result by resolving the lesions of the ascending aorta. Based on our results, 25-30 operations done is necessary to gain such a level of confidence and experince to aquire better results on long-term survival. In addition, we discussed that there were no co-morbidities affecting on the survival of Marfan patients and prophylactic aortic root replacement ensures a longer survival among patients with Marfan syndrome

A mechanikus keringéstámogatás életet ment - a műszívprogram első három évének tapasztalata a Semmelweis Egyetemen

INTRODUCTION: Since the celebration of the 20th anniversary of the first heart transplantation in Hungary in 2012 the emerging need for modern heart failure management via mechanical circulatory support has evolved. In May 2012 the opening of a new heart failure and transplant unit with 9 beds together with the procurement of necessary devices at Semmelweis University accomplished this need. AIM: The aim of the authors was to report their initial experience obtained in this new cardiac assist device program. METHOD: Since May, 2012, mechanical circulatory support system was applied in 89 cases in 72 patients. Indication for support were end stage heart failure refractory to medical treatment and acute left or right heart failure. RESULTS: Treatment was initiated for acute graft failure after heart transplantation in 27 cases, for end stage heart failure in 24 cases, for acute myocardial infarction in 21 cases, for acute postcardiotomy heart failure in 14 cases, for severe respiratory insufficiency in 2 cases and for drug intoxication in one case. Among the 30 survivor of the whole program 13 patients were successfully transplanted. CONCLUSIONS: The available devices can cover all modalities of current bridge therapy from short term support through medium support to heart transplantation or long term support and destination therapy. These conditions made possible the successful start of a new cardiac assist device program. Orv. Hetil., 2015, 156(13), 521-527

Komplex endovascularis rekonstrukciók az aortaíven : műtéti esetbemutatások

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. ’de branching’ műtétek). Nyitott műtétre nem alkalmas betegek esetén azonban az ágak endovascularis módszerekkel történő megtartására kényszerülünk. Tanulmányunkban ezen komplex endovascularis aortaívrekonstrukciók lehető ségeit 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 hely reállítására. 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 ugyan csak 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

Krónikus aortadissectio talaján kialakult thoracoabdominalis aneurysma endovascularis kezelése fenesztrált sztentgrafttal = Fenestrated endovascular repair of a thoracoabdominal aortic aneurysm in chronic dissection

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 mortal-ity 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 ele-phant 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

A felnőttszív-átültetés fejlődése Magyarországon = Evolution of the Hungarian adult heart transplantation program

Absztrakt: A szerzők rövid történeti áttekintést adnak a felnőttszív-átültetés nemzetközi és hazai fejlődéséről, megvilágítva párhuzamokat, kiemelve egy program evolúciójának sajátosságait. Megvizsgálják a Magyarországon transzplantációra kerülő recipiensek betegadatait, melyeket összehasonlítanak nemzetközi adatokkal. Céljuk bemutatni, hogyan változott az elmúlt öt évben a szívtranszplantáció eredményessége, és ezt milyen módszerekkel, a programban végrehajtott változtatások révén érték el. Ehhez 496 transzplantáció adatait vizsgálták meg, és hasonlították össze a nemzetközi adatokkal. Orv Hetil. 2018; 159(46): 1869–1875. | 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