Treatment options for complex aortic aneurysms ­ own experiences / Complex aortaaneurysmák kezelési lehetoségei - saját tapasztalatok

Complex aortic aneurysms extend to more aortic segments, and/or include one or more orifices of highly important side branches. Meanwhile complex aneurysms need reconstructive solutions in one sitting or hybrid procedures timely close to each other, multiple aneurysms can be treated technically and timely separated. Previously, open surgery was the only opportunity to intervene, which was associated with significant surgical trauma and was not suitable for high risk patients when devastating complications were likely. Recently combination of lower risk surgery with endovascular treatment options ­ the so called hybrid techniques ­ resulted in that indications for treatment remarkably widened. In addition, permanent technical progress made available pure endovascuar solutions, so a wide range of surgical procedures provide number of options for treatment. In this paper we report on the treatment options of complex aortic aneurysms, and present our own relevant experience.

Surgical ischemia and reperfusion of the spinal cord ­ effect on specific perfusion and neurological outcome. Experimental study / A sebészi gerincvelo ischemia és reperfusio hatása a specifikus átáramlásra és a neurológiai következményekre

Clamping of the thoracoabdominal aorta reduces perfusion of the spinal cord significantly, which clinically may present as paraparesis or paraplegia ­ devastating and unpredictable complications of open thoracoabdominal aortic surgery. Introduction of monitoring of evoked potentials and/or biochemical markers, methods increasing distal arterial pressure, indirect procedures enhancing residual flow (like liquor drainage), drugs, and use of hypothermia contributed to achieve better outcome. Preconditioning of spinal cord circulation is also a promising method. New endovascular techniques for thoracoabdominal aortic aneurysms and dissections reduced surgical trauma significantly. Despite all these progressions spinal cord ischemic damage is still a significant risk. To address this problem we carried out an experimental work using a canine model focusing on the protective effect of distal arterial perfusion, spinal fluid drainage, and their combination in a one hour setting of thoracoabdominal aortic clamping. In this paper we publish our data of circulatory and specific perfusion parameters of the spinal cord during and after declamping in correlation of final neurologic outcome.

The presence of small joint contractures is a risk factor for survival in 439 patients with systemic sclerosis.

OBJECTIVES: Analysis of risk factors and mortality of 439 patients with systemic sclerosis (SSc) in a tertiary care centre. METHODS: The mean follow up time was 8.4±5.6 years. Lost to follow up rate was 6.4%. Female to male ratio was 366 to 73. Two hundred sixty patients had limited and 179 diffuse cutaneous SSc (dcSSc). A standard protocol including musculoskeletal examinations was used for the assessment of patients. RESULTS: By Kaplan-Meier analysis the overall 5-, 10- and 15 year survival were 88.2%, 79.9% and 73.6%, respectively. Univariate analysis showed that dcSSc, male gender, presence of small joint contractures, pulmonary interstitial, cardiac, oesophageal involvement, scleroderma renal crisis, arterial hypertension, anti-topoisomerase antibody, anemia, hypalbuminemia, coexistent malignancies and elevated erythrocyte sedimentation were associated with poor survival. Lack of giant capillaries, avascular zones or neo-angiogenesis on capillaroscopy, and presence of anti-centromere antibodies were associated with favourable outcome. Multivariate regression analysis showed presence of small joint contractures, history of arterial hypertension, male gender, diffusing capacity of carbon monoxide <50%, right ventricular pressure >40 mmHg on echocardiography, less than 50% ejection fraction, anti-topoisomerase I positivity, anemia, and serum albumin concentration < 35 g/l as well as current or history of coexistent malignancy were independent poor prognostic factors. CONCLUSIONS: In addition to well-known factors predicting poor outcome in SSc, the presence of small joint contractures was a newly identi ed independent risk factor of mortality. Our data also confirmed a recent finding showing that history of arterial hypertension was also a poor prognostic factor.

Malignancies in Patients with Anti-RNA Polymerase III Antibodies and Systemic Sclerosis: Analysis of the EULAR Scleroderma Trials and Research Cohort and Possible Recommendations for Screening.

OBJECTIVE: To analyze the characteristics of anti-RNA polymerase III antibodies (anti-RNAP3)- positive patients with systemic sclerosis (SSc) in the European League Against Rheumatism Scleroderma Trials and Research group (EUSTAR) registry with a focus on the risk of cancer and the characteristics of malignancies, and the aim to provide guidelines about potential cancer screening in these patients. METHODS: (1) Analysis of the EUSTAR database: 4986 patients with information on their anti-RNAP3 status were included. (2) Case-control study: additional retrospective data, including malignancy history, were queried in 13 participating EUSTAR centers; 158 anti-RNAP3+ cases were compared with 199 local anti-RNAP3- controls, matched for sex, cutaneous subset, disease duration, and age at SSc onset. (3) A Delphi exercise was performed by 82 experts to reach consensus for cancer screening in anti-RNAP3+ patients. RESULTS: In the EUSTAR registry, anti-RNAP3 were associated in multivariable analysis with renal crisis and diffuse cutaneous involvement. In the case-control study, anti-RNAP3 were associated with gastric antral vascular ectasia, rapid progression of skin involvement, and malignancies concomitant to SSc onset (OR 7.38, 95% CI 1.61-33.8). When compared with other anti-RNAP3+ patients, those with concomitant malignancies had older age (p < 0.001) and more frequent diffuse cutaneous involvement (p = 0.008). The Delphi exercise highlighted the need for malignancy screening at the time of diagnosis for anti-RNAP3+ patients and tight followup in the following years. CONCLUSION: Anti-RNAP3+ patients with SSc have a high risk of concomitant malignancy. These results have implications for clinical practice and suggest regular screening for cancer in anti-RNAP3+ patients.

Galectin-3 is an independent predictor of survival in systemic sclerosis.

BACKGROUND: Galectin-3 is a beta-galactoside-binding lectin that may be related to tissue sclerosis or aberrant activation of angiogenesis in systemic sclerosis (SSc). The aim of our study was to determine the associations between galectin-3 levels and patient characteristics, as well as to investigate the long term prognostic value of galectin-3 in a large cohort of SSc patients. METHODS: 152 patients with SSc (55±11years, 138 female) were included in our follow-up study. Blood samples and clinical data were collected at baseline. Primary and secondary outcomes were all-cause and cardiovascular mortality, respectively. RESULTSS: Galectin-3 levels showed positive correlation with the grade of left ventricular diastolic function (r=0.193; p=0.026), erythrocyte sedimentation rate (r=0.172; p=0.036) and serum level of C-reactive protein (r=0.200; p=0.015) while negative correlation with diffusing capacity for carbon monoxide (r=-0.228; p=0.006), in age, gender and BSA adjusted analyses. During the follow-up of 7.2±2.3years, 35 SSc patients (23%) died. In multivariate Cox regression analyses adjusted for age, gender, BSA, creatinine and NT-proBNP levels, galectin-3 was an independent predictor both of the all-cause mortality (HR: 2.780, 95% CI: 1.320-5.858, p=0.007) and cardiovascular mortality (HR: 3.346, 95% CI: 1.118-10.012, p=0.031). Using receiver-operating characteristic analysis, galectin-3>10.25ng/ml was found to be the best predictor of the all-cause mortality. CONCLUSIONS: Our results suggest that galectin-3 is an independent predictor of all-cause and cardiovascular mortality in SSc. Validation studies are required to establish whether galectin-3 may be proposed as simple biomarker for identifying patients with high mortality risk in SSc.

[Surgical treatment of atypical aortic coarctation -- long-term results]. / Atípusos aortacoarctatiók sebészi kezelésének hosszú távú eredményei.

INTRODUCTION: Aortic coarctation is a frequent congenital cardiovascular disorder representing 5-8% of all cases. It is typically localized in the isthmic region. However, in about 1% of cases coarctation may develop in atypical sites of the aorta and it is frequently complicated with severe hypertension. AIM: The aim of the authors was to present diagnostic and surgical methods used in 27 patients with atypical aortic coarctation during the last 35 years with special interest on long-term results. METHOD: There was a great advance in diagnostic and surgical treatment methods during the time period analyzed in this study. Nowadays morphologic diagnosis is most commonly obtained using computed tomography angiography and magnetic resonance angiography. Some cases were treated with endovascular techniques, but the authors used also a wide variety of surgical approaches in these patients with atypical aortic coarctation. RESULTS: No patient died after surgery and hypertension was reduced in all patients, too. Reintervention was necessary in patients operated in childhood due to change of body measures. CONCLUSIONS: Atypical aortic coarctation can be treated surgically with good early and late outcomes. Somatic growth of children may indicate surgical revision.

[Late complications and treatment options of aortic coarctation operated in childhood]. / Gyermekkorban operált aortacoarctatiók késoi szövodményei és ellátásuk.

INTRODUCTION: The prevalence of congenital aortic coarctation is 4 in 10 000 live birth. Aortic coarctation is typically located in the aortic isthmus, but it may occur at atypical sites. Treatment options include both surgical and endovascular interventions. In patients undergoing surgical or endovascular intervention late complications such as recoarctation or aortic aneurysm may develop. AIM: The aim of the authors was to analyse their own experience in late complication and treatment options of aortic coarctation operated in childhood. METHOD: Retrospective analysis of data of 32 patients treated between 1980 and 2014 for late complications 8-42 years after surgical treatment of aortic coarctation. RESULTS: In 28 patients aneurysm formation after isthmic patch plasty was found. Two patients had aortobronchial fistula, 2 patients showed anastomosis disruption and 2 patients had graft stenosis. During operation hybrid solution was performed in 23 patients, isthmic aorto-aortic inlay graft interposition in 5 patients, aorto-aortic bypass in 2 patients, subclavio-aortic bypass in 2 patients, graft patch plasty in one patient and ilio-renal bypass in one patient. Complications included severe intraoperative bleeding in one patient and pneumothorax in one patient. No early or late mortality occurred. CONCLUSIONS: The authors conclude that life long control is mandatory in order to detect late complications in patients who underwent operation of aortic coarctation in childhood.

[Penetrating atheromatous ulcer of the thoracic aorta. Treatment options]. / A mellkasi aorta penetráló atheromás ulcusa. Kezelési lehetoségek.

INTRODUCTIONS: Atherosclerosis is a generalized degenerative disease of arteries. A rare manifestation of that is the penetrating atheromatous aortic ulceration of the aortic wall which may be complicated by subintimal hematoma, dissection, false aneurysm and rupture (acute aortic syndrome). AIM: The aim of the authors was to analyse their experience with this rare disease. METHOD: In the department of the authors 10,212 patients underwent surgery for cardiovascular diseases. Among these, 18 patients were diagnosed with penetrating atheromatous aortic ulceration located in the thoracic aorta (ascending aorta 2, aortic arch 8, descending aorta 6 and thoracoabdominal aorta 2 patients). Severe comorbidities were present in the majority of patients. RESULTS: Depending on the localisation the authors used various treatment options such as open surgery (4 patients), hybrid techniques (7 patients) and pure endografting (7 patients). One patient died on postoperative day 3. Two patients had late complications due to endoleaks treated successfully by open surgery. CONCLUSIONS: The authors conclude that when this dangerous condition detected in time, a wide variety of open, hybrid and endovascular methods can be applied with good results.

[Adverse events during biological therapy -- focusing on dermatological side-effects]. / Biológiai terápia során észlelt mellékhatások -- különös tekintettel a borgyógyászati szövodményekre.

UNLABELLED: Biological response modifiers, especially tumour necrosis factor inhibitors have been proved to be very effective in the treatment of various immune mediated rheumatological, gastroenterological and dermatological diseases in the last 15 years. With their increasing use, the incidence of their adverse effects are more precisely defined. The aim of this cohort study was to analyse the adverse effects occurred within the study period in patients receiving biological therapy for rheumatological and dermatological autoimmune diseases. METHODS: 324 patients within a 3 years study period were treated with biological response modifiers (adalimumab: 92, etanercept: 107, infliximab: 125). The primary diagnoses were rheumotoid arthritis (n = 174), ankylosing spondylitis (n = 60), psoriatic arthritis (n = 11), and psoriasis vulgaris (79). RESULTS: Hypersensitive reactions were observed in 11 of the patients (3.4%), 7 of which were serious and needed treatment termination. Infections constituted the majority of side effects, which were localised to skin in 10 (3.1%) and to respiratory tract in 9 (2.8%). However, most of these were mild or moderate reactions. Malignant skin tumour developed in 1 case (0.3%) only. Drug induced inflammatory disorders occurred in some cases: onset of new psoriasis was observed in 1 and flares of the existing disease were detected in additional three. Lichenoid exanthema developed in one. (n = 5, 1.5%) CONCLUSION: The use of TNF-α blockers may provoke a broad spectrum of dermatological side effects. Our results suggest that the majority of these are infectious and inflammatory disorders, the latter may relatively often appear as drug induced psoriasis. The occurrence of malignancies was very low in our series.