Nanoscale Structural Comparison of Fibrillin-1 Microfibrils Isolated from Marfan and Non-Marfan Syndrome Human Aorta.

Fibrillin-1 microfibrils are essential elements of the extracellular matrix serving as a scaffold for the deposition of elastin and endowing connective tissues with tensile strength and elasticity. Mutations in the fibrillin-1 gene (FBN1) are linked to Marfan syndrome (MFS), a systemic connective tissue disorder that, besides other heterogeneous symptoms, usually manifests in life-threatening aortic complications. The aortic involvement may be explained by a dysregulation of microfibrillar function and, conceivably, alterations in the microfibrils' supramolecular structure. Here, we present a nanoscale structural characterization of fibrillin-1 microfibrils isolated from two human aortic samples with different FBN1 gene mutations by using atomic force microscopy, and their comparison with microfibrillar assemblies purified from four non-MFS human aortic samples. Fibrillin-1 microfibrils displayed a characteristic "beads-on-a-string" appearance. The microfibrillar assemblies were investigated for bead geometry (height, length, and width), interbead region height, and periodicity. MFS fibrillin-1 microfibrils had a slightly higher mean bead height, but the bead length and width, as well as the interbead height, were significantly smaller in the MFS group. The mean periodicity varied around 50-52 nm among samples. The data suggest an overall thinner and presumably more frail structure for the MFS fibrillin-1 microfibrils, which may play a role in the development of MFS-related aortic symptomatology.

Blood-filled cyst of the tricuspid valve: Multiple cardiac disorders, one surgical case.

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.

Pseudoaneurysm of the ascending aorta: case report of a donor-derived Pseudomonas infection in a heart transplant recipient.

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.

Needle in the heart: a rare case of cardiac tamponade caused by a migrated foreign body and mimicking ST segment elevation myocardial infarction.

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.

Perioperative Low Tetraiodothyronine Levels and Adverse Outcomes After Heart Transplantation: A Retrospective, Observational Study.

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.

Lung Function Changes are More Common in Marfan Patients Who Need Major Thoracic Surgery.

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.

Heart re-transplantation in Eurotransplant.

Internationally 3% of the donor hearts are distributed to re-transplant patients. In Eurotransplant, only patients with a primary graft dysfunction (PGD) within 1 week after heart transplantation (HTX) are indicated for high urgency listing. The aim of this study is to provide evidence for the discussion on whether these patients should still be allocated with priority. All consecutive HTX performed in the period 1981-2015 were included. Multivariate Cox' model was built including: donor and recipient age and gender, ischaemia time, recipient diagnose, urgency status and era. The study population included 18 490 HTX, of these 463 (2.6%) were repeat transplants. The major indications for re-HTX were cardiac allograft vasculopathy (CAV) (50%), PGD (26%) and acute rejection (21%). In a multivariate model, compared with first HTX hazards ratio and 95% confidence interval for repeat HTX were 2.27 (1.83-2.82) for PGD, 2.24 (1.76-2.85) for acute rejection and 1.22 (1.00-1.48) for CAV (P < 0.0001). Outcome after cardiac re-HTX strongly depends on the indication for re-HTX with acceptable outcomes for CAV. In contrast, just 47.5% of all hearts transplanted in patients who were re-transplanted for PGD still functioned at 1-month post-transplant. Alternative options like VA-ECMO should be first offered before opting for acute re-transplantation.

[Evolution of the Hungarian adult heart transplantation program]. / A felnottszív-átültetés fejlodése Magyarországon.

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.

[The Semmelweis University extracorporeal membrane oxygenation program - 5 years' results and perspectives]. / A Semmelweis Egyetem extracorporalis membránoxigenizációs programja ­ az 5 éves Városmajori-eredmények függvényében.

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.

[Computer-assisted decision-making in cardiac surgery: from 3D preoperative planning to computational fluid dynamics in the design of surgical procedures]. / Számítástechnikai döntéstámogató rendszer kiépítése a szívsebészetben: a 3D tervezéstol a posztoperatív eredményekig.

INTRODUCTION: Although surgical specialties utilize static models for preoperative planning, the evolution of dynamic planning methods and computer simulations created the opportunity for the introduction of dynamic parameters in cardiac surgery. Our aim was to apply 3D models in cardiac surgical practice, predicting fluid dynamic results, ventricular shape, volume and function before the operation. METHODS: Using a script developed by us, the raw DICOM files were imported, the dilated left ventricle was modeled and fluid dynamic parameters were simulated, such as flow kinematic and profile analysis, turbulence calculation and myocardial response to shear stress. Then step-by-step simulation of the surgical ventricle restoration procedure was accomplished and the calculated variables were imbedded in silico model. The length of resection lines was modified based on the previous computer simulation and applied during the operation, if feasible. RESULTS: The sphericity and conicity indexes were improved significantly in postoperative period (0.42 vs. 0.67 and 0.36 vs. 0.72, p < 0.05). The occurred shear stress at endocardium decreased 83% due to the normalization of flow kinematic pattern of the ventricle in postoperative period (132.21 ± 29.5 dyne/cm2 vs. 22.92 ± 10.3 p < 0.05 dyne/|cm2). The postoperative turbulent flow pattern significantly decreased, according to our computational method (2712 vs. 1823, p < 0.0001). CONCLUSION: With our method, the standardization of the surgical ventricle reconstruction was achievable and the surgical steps were predictable. Therefore, a new decision making support system was established in cardiac surgery for high risk patients. A personalized surgical technique was offered to our patients, improving their life expectancy and quality of life.

Uncommon presentation of a rare tumour - incidental finding in an asymptomatic patient: case report and comprehensive review of the literature on intrapericardial solitary fibrous tumours.

BACKGROUND: A solitary fibrous tumour is a rare, mainly benign spindle cell mesenchymal tumour most commonly originating from the pleura. An intrapericardial location of a solitary fibrous tumour is extremely unusual. We present a case of an asymptomatic patient with a slow-growing massive benign cardiac solitary fibrous tumour. CASE PRESENTATION: A 37-year-old asymptomatic female patient was referred to our hospital with an enlarged cardiac silhouette found on her screening chest X-ray. The echocardiographic examination revealed pericardial effusion and an inhomogeneous mobile mass located in the pericardial sac around the left ventricle. Cardiac magnetic resonance (MRI) examination showed an intrapericardial, semilunar-shaped mass attached to the pulmonary trunk with an intermediate signal intensity on proton density-weighted images and high signal intensity on T2-weighted spectral fat saturation inversion recovery images. First-pass perfusion and early and late gadolinium-enhanced images showed a vascularized mass with septated, patchy, inhomogeneous late enhancement. Coronary computed tomography angiography revealed no invasion of the coronaries. Based on the retrospectively analysed screening chest X-rays, the mass had started to form at least 7 years earlier. Complete resection of the tumour with partial resection of the pulmonary trunk was performed. Histological evaluation of the septated, cystic mass revealed tumour cells forming an irregular patternless pattern; immunohistochemically, the cells tested positive for vimentin, CD34, CD99 and STAT6 but negative for keratin (AE1-AE3), CD31 and S100. Thus, the diagnosis of an intrapericardial solitary fibrous tumour was established. There has been no recurrence for 3 years based on the regular MRI follow-up. CONCLUSION: Intrapericardial SFTs, showing slow growth dynamics, can present with massive extent even in completely asymptomatic patients. MRI is exceedingly useful for characterizing intrapericardial masses, allowing precise surgical planning, and is reliable for long-term follow up.

The effects of acute and elective cardiac surgery on the anxiety traits of patients with Marfan syndrome.

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 < 0.01). The mean score of Marfan syndrome patients was 48.56 (standard deviation (SD): 5.8) as compared to the STAI population mean score of 43.72 (SD: 8.53). No difference was found between groups on the BDI (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.

[Heart transplantation and long-term lvad support cost-effectiveness model]. / Szívtranszplantáció és muszívkezelés költséghatékonysági elemzési modellje.

Heart transplantation is a high priority project at Semmelweis University. In accordance with this, the funding of heart transplantation and mechanical circulatory support also constitutes an important issue. In this report, the authors discuss the creation of a framework with the purpose of comparing the cost-effectiveness of heart transplantation and artificial heart implantation. Our created framework includes the calculation of cost, using the direct allocation method, calculating the incremental cost-effectiveness ratio and creating a cost-effectiveness plane. Using our model, it is possible to compare the initial, perioperative and postoperative expenses of both the transplanted and the artificial heart groups. Our framework can possibly be used for the purposes of long term follow-up and with the inclusion of a sufficient number of patients, the creation of cost-effectiveness analyses and supporting strategic decision-making.

[Migration of an epicardial pacemaker into the abdominal cavity]. / Hasüregbe vándorolt epicardialis pacemaker.

Migration of a permanent pacemaker generator from their intramuscular pocket to the abdominal cavity is a less frequent, but potentially life-threatening complication. The authors present the case of a 69-year-old woman, who visited the emergency department of the clinic, with complains of non-specific abdominal symptoms. Her past medical history included a complete atrioventricular block diagnosed in 2009 during the mitral valve replacement and since then she had an epicardial permanent pacemaker; the pulse generator was placed into an intramuscular pouch created in the left subcostal region. Surprisingly, radiologic examinations showed that the generator migrated into the pouch of Douglas. Considering patient safety, first a new intracardiac pacemaker was implanted and then the migrated device was removed surgically. The patient was discharged on the seventh postoperative day.

[Mechanical circulatory support saves lives -- three years' experience of the newly established assist device program at Semmelweis University, Budapest, Hungary]. / A mechanikus keringéstámogatás életet ment -- a muszívprogram elso 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.

Possible extracardiac predictors of aortic dissection in Marfan syndrome.

BACKGROUND: According to previous studies, aortic diameter alone seems to be insufficient to predict the event of aortic dissection in Marfan syndrome (MFS). Determining the optimal schedule for preventive aortic root replacement (ARR) aortic growth rate is of importance, as well as family history, however, none of them appear to be decisive. Thus, the aim of this study was to search for potential predictors of aortic dissection in MFS. METHODS: A Marfan Biobank consisting of 79 MFS patients was established. Thirty-nine MFS patients who underwent ARR were assigned into three groups based on the indication for surgery (dissection, annuloaortic ectasia and prophylactic surgery). The prophylactic surgery group was excluded from the study. Transforming growth factor-ß (TGF-ß) serum levels were measured by ELISA, relative expression of c-Fos, matrix metalloproteinase 3 and 9 (MMP-3 and -9) were assessed by RT-PCR. Clinical parameters, including anthropometric variables - based on the original Ghent criteria were also analyzed. RESULTS: Among patients with aortic dissection, TGF-ß serum level was elevated (43.78 ± 6.51 vs. 31.64 ± 4.99 ng/l, p < 0.0001), MMP-3 was up-regulated (Ln2α = 1.87, p = 0.062) and striae atrophicae were more common (92% vs. 41% p = 0.027) compared to the annuloaortic ectasia group. CONCLUSIONS: We found three easily measurable parameters (striae atrophicae, TGF-ß serum level, MMP-3) that may help to predict the risk of aortic dissection in MFS. Based on these findings a new classification of MFS, that is benign or malignant is also proposed, which could be taken into consideration in determining the timing of prophylactic ARR.

[Successful surgical treatment of an acute type-A aortic dissection complicated with pericardial tamponade and ST-segment elevation . Case report]. / Pericardialis tamponáddal, ST-szakasz-elevációval szövodött akut "A" típusú aortadissectio sikeresen operált esetének ismertetése.

Successful treatment of type A acute aortic dissection depends on the promptness of diagnostic evaluation and therapy. Fast diagnosis can be challenged by numerous complications such as myocardial ischemia, acute aortic insufficiency, and disturbances in organ perfusion and pericardial tamponade. The authors report the case history of a 72-year-old woman, who was admitted after resuscitation with ST segment elevation. Echocardiography revealed acute type A aortic dissection with signs of pericardial tamponade. An emergency operation consisting of the resection of the ascending aorta and the reconstruction of the aortic root was performed, which took six hours from admission until the end of the operation. Follow-up examinations demonstrated good left ventricular function and competent aortic valve. The authors propose that with the development of diagnostic and therapeutic options, faster and less invasive interventions will be introduced in near future for the treatment of acute aortic dissection, which may reduce the morbidity and mortality rates of this lethal illness.

[2012 -- the year of success in the 20 year-old adult heart transplant program of Hungary]. / A 2012-es év a húszéves hazai felnottszív-transzplantáció sikeréve -- és ami mögötte van.

The Hungarian adult heart transplant program, which started in 1992, has changed gradually in the past 20 years. After the early enthusiasm of the first cases it changed significantly and it became an organized programme. However, low donation activity and moderate referral numbers to the national transplant waiting list slowed down the process therefore, heart transplant numbers did not fulfill expectations in the early years. After a moderate increase in 2007 transplant numbers have dropped again until recently when Hungary partially joined Eurotransplant network. Excess fundamental resources allocated to cardiac transplantation by health care professionals and reorganizing transplant coordination as well as logistics forced dramatic changes in clinical management. In 2011 and 2012 major structural changes had been made at Semmelweis University. The newly established transplant intensive care unit and the initiation of mechanical circulatory support and assist device programme increased transplant numbers by 131% compared to previous years, as well as it resulted an 86.63% 30-day survival rate, hence last year was the most successful year of cardiac transplantation ever.

[Complex endovascular repairs of the aortic arch.] / Komplex endovascularis rekonstrukciók az aortaíven.

Thoracic endovascular aneurysm repair (TEVAR) of the aortic arch has started to spread in recent years. We present our initial experience with TEVAR involving supra-aortic branches using parallel and branched grafts. Parallel grafts are typically used in emergency cases. In the case of Z0 proximal fixation, we can perform a combined hybrid surgery with Z1 debranching and securing of the innominate artery with chimney graft, which can also be used instead of Z0 debranching when the patient is unfit for sternotomy. In the case of TEVAR with planned Z2 position with inadvertent covering of the left common carotid artery, we can perform chimney conversion to rapidly recover the circulation of the left common carotid artery (LCCA). Instead of prior revascularization of the left subclavian artery, chimney graft can be used to recover the lumen, in the case of left upper limb ischemia after Z2 TEVAR. Exclusion of penetrating aortic ulcers located in the lesser curvature can be facilitated by use of a custom-made graft, where a scallop is placed around the origin of the supra-aortic vessel, thus increasing the length of the proximal neck. For elective interventions, custom-made branching grafts can be allowed to save all three branches with Z0 fixation. Technical success was obtained in all cases. Orv Hetil. 2023; 164(11): 426-431.

Extracorporeal photopheresis in the treatment of cardiac allograft rejection: A single-centre experience.

Despite novel immunosuppressive (IS) protocols, adverse effects of IS drugs continue to have notable negative impact on patient and cardiac allograft survival after heart transplantation (HTx). Therefore, IS regimens with less toxic side effects are sorely needed. We aimed to evaluate the efficacy of extracorporeal photopheresis (ECP) in combination with tacrolimus-based maintenance IS therapy in the treatment of allograft rejection in adult HTx recipients. Indications for ECP included acute moderate-to-severe or persistent mild cellular rejection, or mixed rejection. Twenty-two patients underwent a median of 22(2-44) ECP treatments after HTx. Median duration of ECP course was 173.5(2-466) days. No relevant adverse effects of ECP were noted. Reduction of methylprednisolone doses was safe throughout the ECP course. ECP, used in conjunction with pharmacological anti-rejection therapy, had a successful reversal of cardiac allograft rejection, decreased the rates of subsequential rejection episodes and normalized the allograft function in patients completing the ECP course. Short- and long-term survivals were excellent (91% at 1 and 5 years post-ECP) and comparable to International Society for Heart and Lung Transplantation registry data on HTx recipient overall survival. In conclusion, ECP can be safely used for the treatment and prevention of cardiac allograft rejection in conjunction with traditional IS regimen.