[Genetic evolution of in situ follicular neoplasia to t(14;18)-positive aggressive B-cell lymphoma]. / Die genetische Evolution der in situ follikulären Neoplasie zum t(14;18)-positiven aggressiven B­Zell-Lymphom.

BACKGROUND: In situ follicular neoplasia (ISFN) is a t(14;18)(q32;q21)+ precursor lesion of follicular lymphoma (FL), which in turn can transform into diffuse large B­cell lymphoma (DLBCL). For DLBCL that arise de novo, no precursor lesion is known. Given the high frequency of the t(14;18) translocation in de novo DLBCL as well, we investigated whether they can also arise from ISFN without FL as an intermediate step. OBJECTIVES: To investigate the clonal evolution of ISFN to DLBCL - transformed from FL and de novo. MATERIALS AND METHODS: Identification of ISFN lesions in patients with DLBCL was performed by BCL2 staining of reactive lymphoid tissues. ISFN and DLBCL were subsequently analyzed by fluorescence in situ hybridization, clonality analyses, sequencing of the t(14;18) breakpoint, and targeted next-generation sequencing. RESULTS: 10 cases with paired ISFN and DLBCL samples were identified, 6 of which were de novo DLBCL and 4 transformed from FL. 3 DLBCL carried MYC-rearrangements in addition to the t(14;18) and were classified as high-grade B­cell lymphoma (HGBL). The clonal relationship of ISFN and DLBCL/HGBL was confirmed for all cases. CREBBP, KMT2D, EZH2, TNFRSF14, and BCL2 were the genes most frequently mutated, with the distribution of private and shared mutations pointing to 2 different scenarios of clonal evolution. In most cases, DLBCL/HGBL, ISFN, and, if also present, FL had evolved divergently from a common progenitor, whereas linear evolution was less frequent. CONCLUSION: We show for the first time that t(14;18)+ DLBCL/HGBL can arise directly from ISFN without FL as an intermediate step and that during this progression, divergent evolution is common.

Transradial and transulnar access for iliac artery interventions using sheathless guiding systems: A feasibility study.

PURPOSE: Our aim was to evaluate the acute success and complication rates of the transradial and transulnar access for iliac artery stenting using sheathless guiding systems. METHODS: Clinical and angiographic data from 156 consecutive patients with symptomatic iliac artery stenosis who were treated with transradial or transulnar access were evaluated. All patients underwent Duplex ultrasound before and after the intervention. The primary endpoints were the procedural success rate, major adverse events, and access site complication rates. The secondary endpoints were the angiographic result of the iliac artery intervention, fluoroscopy time, X-ray dose, procedure length, crossover rate to another puncture site and hospitalization duration. The impact of the learning curve was also investigated, along with right or left radial access. RESULTS: The indication for the intervention was intermittent claudication in 109 patients (69.9%), critical limb ischemia in 44 (28.2%) subjects and acute limb ischemia in three individuals (1.9%). Technical success was achieved in 155 patients (99.4%), with a crossover rate of 3.8%. Radial and ulnar artery access was used in 151 (96.8%) and 7 (4.5%) patients, respectively. The Ankle-brachial index increased from 0.69 [0.65-0.72] to 0.91 [0.88-0.95] as a result of the procedures (P < 0.001). The cumulative incidence of major adverse events was 3.8% at the 2-month follow-up (0% in patients with intermittent claudication and 13.8% in patients with critical limb ischemia). Radial artery access site complications were encountered in eight patients (5.1%). We documented decreased X-ray doses (1742.0 [783.9-2701] vs. 1435 [991.1-1879] vs. 692.8 [275.3-1110] Gy cm-2 P < 0.05) over time; however, the fluoroscopy time, procedure time, and contrast consumption were not significantly different. Left hand access was not associated with significantly better results than right radial artery access. CONCLUSIONS: Iliac artery stenting can be safely and effectively performed using radial or ulnar artery access and sheathless guiding catheters, with acceptable complication rates and high levels of technical success. The physician learning curve plays an important role in decreasing the X-ray dose. © 2016 The Authors. Catheterization and Cardiovascular Interventions Published by Wiley Periodicals, Inc.

Safety, clinical outcome, and fracture rate of femoropopliteal stenting using a 4F compatible delivery system.

OBJECTIVE: To determine the safety, clinical outcome, and fracture rate of femoropopliteal interventions using 4F stents. METHODS: Between January 2010 and December 2011, 112 symptomatic patients were treated by stent implantation. Ten patients were lost to follow up; therefore, 102 patients (62 men; mean age 66.4 ± 10.1 years) were retrospectively analyzed. The indication for femoropopliteal revascularization was severe claudication (Rutherford-Becker score = 3) in 63 (62%) patients and chronic critical limb ischemia (Rutherford-Becker score = 4-6) in 39 (38%). Follow up included palpation of peripheral pulses and measurement of ankle brachial index. In patients with suspected in-stent restenosis duplex ultrasonography was performed. In 2013, patients were asked to return for a fluoroscopic examination of the stents. RESULTS: 114 lesions (Trans-Atlantic InterSociety Consensus-C and D, n = 45) were treated with 119 stents (Astron Pulsar, n = 42; Pulsar-18, n = 77). Lesions were long (≥100 mm) in 49 cases and heavily calcified in 35. Stents were long (≥120 mm) in 46 cases. Ten stents were partially overlapped. The technical and clinical success rates were 100%. Two puncture related complications were noted, neither of which required surgical repair. Eleven patients died (myocardial infarction, n = 4; stroke, n = 2; cancer, n = 5) and nine patients underwent major amputation (above knee, n = 4). The primary patency rate was 83% at 6 months and 80% at 12 months. The primary assisted patency rate was 97% at 6 months and 94% at 12 months. The secondary patency rate was 86% at 6 months and 85% at 12 months. The prevalence of fractures was 26% (type III and IV, 10%) after an average follow up of 25 months. CONCLUSION: Femoropopliteal stenting using a 4F compatible delivery system can be accomplished with a low complication rate, acceptable fracture rate, and with similar 12 month patency and revascularization rates as their 6F counterparts.

Percutaneous endovascular treatment of innominate artery lesions: a single-centre experience on 77 lesions.

PURPOSE: To assess primary success and safety of percutaneous transluminal angioplasty and/or stenting of innominate artery lesions and to compare its 30-day stroke/mortality level with the literature data. METHODS: A total of 72 patients (77 stenoses, five recurrent, 58 symptomatic and 39 female) with seven innominate vessel occlusions, nine subocclusive lesions and 61 significant (>60%) stenoses of innominate artery treated between 2000 and 2009 were retrospectively reviewed. With the exception of seven, all procedures were performed using a transfemoral approach. A stent was implanted in 49 (63.6%) cases. Follow-up included neurological examination, carotid duplex scan and office/telephone interview. RESULTS: Primary technical success was 93.5% (72/77). There was neither periprocedural (<48 h) death, nor major neurological complication. Minor periprocedural neurological complications consisted of 2/72 (2.6%) ipsilateral TIAs. Access site complications included 4 (5.2%) access site bleedings. Follow-up was achieved in 65/72 (90.3%) of all patients and 68 (88.3%) of all procedures for a mean of 42.3 months and revealed neither major neurological complication, nor additional TIA. The cumulative primary patency rate was 100% at 12 months, 98+/-1.6% at 24 months, and 69.9+/-8.5% at 96 months. The cumulative secondary patency rate was 100% at 12 and at 24 months, and 81.5+/-7.7% at 96 months. Log-rank test showed no significant difference (p=0.79) in primary cumulative patencies between PTA alone (n=28) or PTA/stent (n=49). CONCLUSION: Transfemoral PTA with or without stent appears to be a safe treatment option for innominate artery lesions.

Post-traumatic pseudoaneurysm of the great saphenous vein: a case report.

Venous (pseudo)aneurysms are rare entities. Herewith we report a case of the right lower extremity in a 42-year-old woman in whom a non-pulsatile mass was diagnosed only by physical examination as a hematoma and was treated conservatively. Six months later ultrasound and phlebography identified a pseudoaneurysm of the great saphenous vein. Post-traumatic venous pseudoaneurysm should be considered among the differential diagnostic options of a subcutaneous non-pulsatile mass in patients with a history of physical trauma. Surgery was offered which was rejected by the patient. Further one month follow-up showed no change.

Early restenosis after eversion carotid endarterectomy versus carotid stenting: a single-centre retrospective study.

AIM: The aim of our study was to compare the early restenosis rate between patients undergoing carotid artery stenting (CAS) and carotid endarterectomy (CEA) at a single cardiovascular institution. METHODS: In 2004, 368 carotid endarterectomies were carried out on 347 patients and 144 internal carotid artery stentings were performed on 140 patients. The mean follow-up time was 18.4 months (range 6-38 months). Restenosis rates were calculated with the Kaplan-Meyer method and the two groups were compared by using log-rank test. Perioperative outcome was also evaluated and the groups were compared with chi-square test. RESULTS: Significantly more perioperative complications occurred in the CAS group, mainly transient neurological (7.60% vs 2.20% in the CEA group, P<0.05) and cardiovascular symptoms (4.10% vs 1.10% in the CEA group, P<0.05). Moderate restenosis (50-69%) occurred in 11.41% (42/368) of CEA cases and in 4.86% (7/144) of CAS cases (P<0.05). Severe (70%) restenosis rates were 10.05 % in the CEA group and 3.47% in the CAS group (P<0.05). CONCLUSIONS: Incidence of restenosis after carotid artery stening was less common than after carotid endarterectomy. On the other hand, perioperative complications were recorded more often after CAS than following CEA.

Changes in the plasma concentration of soluble thrombomodulin in patients with severe carotid artery stenosis after eversion endarterectomy.

OBJECTIVE AND DESIGN: The purpose of the study was to investigate the putative role of soluble thrombomodulin (sTM) in severe carotid artery stenosis. MATERIALS AND METHODS: We prospectively studied 64 patients who were undergoing carotid endarterectomy (2001-2003). Plasma sTM concentration was determined in each patient before surgery and at 14 months postsurgery. -308 TNF-alpha promoter polymorphism was also determined. RESULTS: Strong negative correlation was found between the preoperative duplex scan values and the plasma sTM concentrations (R = -0.418, p = 0.0006). Patients with 308 A TNF-alpha genotype had significantly lower (p = 0.0415) preoperative sTM values than their counterparts with no such polymorphism. Soluble TM concentrations measured in plasma samples taken at the end of the postsurgical follow-up period of 14 months duration were significantly higher compared to the preoperative values (p < 0.0001). CONCLUSIONS: Our present findings indicate that sTM may be adsorbed to the atherosclerotic plaques or inflamed endothelium in carotid arteries. The pathological significance of this adsorption remains to be determined.

Endovascular treatment of peripheral artery disease with expanded PTFE-covered nitinol stents: interim analysis from a prospective controlled study.

PURPOSE: Current covered peripheral stent designs have significant drawbacks in terms of stent delivery characteristics and flexibility. The aim of this study was to analyze the technical performance, safety and initial clinical efficacy of expanded polytetrafluoroethylene (PTFE)-covered nitinol stents for arteriosclerotic peripheral artery disease. METHODS: Eighty-two patients underwent implantation of PTFE-covered nitinol stents for iliac and/or femoral obstructions. The study was conducted prospectively in seven European centers and one Canadian center. Patients were controlled clinically and by duplex ultrasound follow-up. Data up to discharge were collected in 79 patients. Seventy-four patients have thus far received 1 month follow-up and 32 patients, 6 month follow-up examinations. RESULTS: The average lesion length measured 47 mm for the common and external iliac arteries and 50 mm for the femoral arteries. The mean severity of the stenoses was reduced from 94% to 4% in the iliac arteries and from 98% to 7% in the femoral arteries after stent placement and dilatation. One device deviation (inadvertent stent misplacement) and one puncture-related severe adverse event with formation of a pseudoaneurysm occurred. There were occlusions of the stent in five patients. No infections were noticed. CONCLUSION: The interim analysis of this trial using PTFE-covered nitinol stents indicates that a strategy using primary implantation of this stent type is technically feasible, has an acceptable safety profile and is effective from a short-term perspective.

[Management of carotid injuries]. / Az arteria carotis sérülései és azok ellátása.

The occurrence of carotid arterial injuries is relatively infrequent but have serious outcome. At the Department of Cardiovascular Surgery we have performed surgical interventions in 16 instances (7 penetrating and 9 blunt trauma patients) due to carotid injuries. Preoperatively all these patients developed neurological deficits and in the postoperative period 7 patients became asymptomatic. We lost two patients owing to stroke. Vascular injuries are generally characterized by hemorrhage and ischemia in the area supplied. In addition to the symptoms of hemorrhagic shock, penetrating vascular injuries may lead to embolism from the injured intimal surface, thrombus formation and subsequent occlusion may occur. In other instances pulsating hematomas can be formed and with combined arterial and venous trauma A-V fistula can develop. In blunt trauma patients stretching or compression of the vessel may cause intimal rupture with subsequent formation of subintimal hematoma, dissection and later pseudoaneurysm. The disruption of the atherosclerotic plaque or the accumulation on the injured intimal surface may serve as source of embolism and thrombosis. In symptomatic patients the urgent performance of the surgical correction of the carotid arterial injuries is mandatory, nevertheless in asymptomatic patients and in chronic cases the prevention of the late complications is also justified as soon as possible.

[Percutaneous angioplasty of the innominate artery in 89 patients: experience of 19 years]. / Az arteria anonyma percutan angioplasztikás kezelése: 19 év tapasztalata 89 betegen.

PURPOSE: To assess retrospectively the success of percutaneous transluminal angioplasty (PTA) in treating innominate artery stenoses and occlusion in a large series of patients with long-term follow-up results. METHODS: In symptomatic patients with high degree (> 60%) stenosis, innominate artery PTA was performed. Long-term follow-up was undertaken by blood pressure measurements on both arms as well as subclavian, right common carotid and right vertebral duplex scan. RESULTS: Between 1981 and 1999, primary success rate of 89 innominate artery PTA (84 stenoses, 5 occlusions) was 96.4%. Complications included 1 left occipital lobe infarction (1.5%), 2 puncture-site thrombosis (2.9%), and 4 TIA (5.8%). Two patients with restenosis were successfully treated with rePTA. Cumulative primary patency was 98 +/- 2% at 6 months, 93 +/- 4% at 16 to 117 months, secondary patency was 100% at 6 months, 98 +/- 2% at 12 to 117 months; 61% of the patients became symptomless, 32% improved, 7% showed no improvement. CONCLUSION: Angioplasty of the innominate artery has been proven to be safe and effective on a large series of patients. For innominate artery stenosis and short occlusion, PTA should be the first treatment of choice.

[Simultaneous carotid and coronary operations: experience and results of 11 years (1990-2000)]. / Szimultán végzett carotis-coronaria mútétek: 11 év tapasztalatai és eredményei (1990-2000).

Between 1990-2000 163 coronary + carotid procedures were performed. The mean age was 4 years higher than it was at patients underwent isolated CABG (63.4 y vs 59.8 y). Clinically proven obliterative artery diseases in other anatomical regions were also present in more than 60% of patients. The half of the procedures were performed under emergency and urgent circumstances. The cardiac status and the coronary morphology were found to be unstable in half of the patients. The "Euroscore" risk evaluation model was used for risk scoring. The mean score value was as high as 6.26. According to this the estimated surgical lethality could have been as high as 11.2%. The real surgical lethality value was far under this estimated level (7.36%). The 1, 5, 10 year survival rate were as high as 89%, 82%, 68% (Kaplan-Meier). The majority of the patients was in NYHA I-II at the end of the follow-up period. The majority of the early and late deaths were found to be myocardial in origin. The estimated surgical risk of the simultaneous procedures could be reduce by accepting of the severe surgical indications existing at this surgical field and with the availability of an experienced operating team.

[Primary percutaneous coronary angioplasty in acute myocardial infarction. Measures of quality control for the work of the hemodynamics laboratory]. / Primer koszorúér angioplasztika heveny szívizom infarktusban. Minóségbiztosítási módszerek a hemodinamikai laboratórium munkájának értékelésére.

The results of primary percutane coronary angioplasty in acute myocardial infarction were studied and compared to international standards. We tried to develop new quality indicators for objective comparison. Seventy nine patients with acute myocardial infarction were included in the study, 47 of them from the pre-stent, and 32 from the stent era. The primary success rate was 91.5 and 81 per cent, respectively. Six months event free period was achieved in 74 and in 69 per cent. The mortality reduced from 10.6 per cent to 3.1 per cent. No emergency by-pass operation was needed in the stent group.

[Surgical considerations in type-B aortic dissection]. / Sebészi megfontolások B típusú aorta disszekció esetében.

Inspite of remarkable progress in both diagnostic and therapeutic development in treating aortic dissections this continues to be one of the most severe vascular catastrophies complicated by high mortality. While aortic dissections Type A are to treat surgically in our days, in majority of patients with Type B dissections hypotensive treatment is the method of choice providing better chance for survival preventing effectively aneurysm formation and rupture at the site of intimal tear. However in certain cases Type B dissection produces either isthmic aneurysm formation, or with distal progression may afflict long segmental thoracic, thoracoabdominal or abdominal deterioration of the aorta and its side branches. The spinal, visceral, renal and lower limb ischemia threatens viability of these organs and hypotensive medication may enhance risk. Prompt evaluation and selection of these cases with proper surgical reconstruction help us to reduce mortality. At our department we have performed 33 thoracoabdominal endoaortectomies with meticulous reconstruction of renal and visceral orifices. The mortality rate in this subgroup of patients was 6%, meanwhile almost 70% in those, treated medically but had the severe distal branch involvement. Based on our experiences in selected patients with Type B aortic dissections we recommend this procedure in order to achieve improvement of results.

Aneurysmal bone cyst: its pathogenesis based on angiographic, immunohistochemical and electron microscopic studies.

Based on angiographic, immunohistochemical as well as electron microscopic findings, authors outline a hypothesis for the etiopathogenesis of aneurysmal bone cysts. No changes were found at the arterial site in 16 studied aneurysmal bone cysts, with no signs of an arteriovenous shunt. In certain cases, however, dilated and tortous efferent veins became visible in the late venous phase. Due to the impedance of venous flow, the intracystic pressure increases and the small veins become dilated causing formation of aneurysmal slits. This is supported by the immunohistochemical finding that S-actin shows concentric arrangement around the aneurysmal cavities. Endothelial lining and basal membrane remnants were detectable in places, though the aneurysmal slits were devoid of continuous endothelial lining and basal membrane. We suggested that the aneurysmal bone cyst corresponds to a hemodynamic disturbance and is due to primary or secondary venous malformation of the bones.

[Total proximal reconstruction of chronic aortic dissection in patients with Marfan syndrome]. / Marfan-kóros betegeknél észlelt krónikus aorta dissectio teljes proximalis korrekciója.

Authors present two patients suffering from Marfan syndrome. Asymptomatically developed serious cardiovascular complications being characteristic of the syndrome itself were revealed at both cases. Type "A" aortic dissection was found at the first, and type "B" aortic dissection involving even the arch as well revealed at the other patient. They were operated on an elective base. Total proximal aortic reconstructions (aortic root-ascending aorta-arch) were performed under deep hypothermia using total circulatory arrest (35 and 87 min). Both patients are doing well without complaints 3-4 months following the surgical interventions. Control angiographic imagination has found normal anatomic conditions at one of the patients. Anastomosis stenosis at the borderline of the arch and descending aorta was revealed at the other case which should be necessary to operate at an later occasion.

Proximal bleeding control obtained by a balloon catheter in the surgical repair of a left supraclavicular traumatic arteriovenous fistula.

Endovascular procedures offer various methods for the treatment of posttraumatic arteriovenous fistulas. We report the treatment of a posttraumatic arteriovenous fistula between the left subclavian artery and internal jugular vein combined with a large subclavian false aneurysm. To avoid left thoracotomy we attained control of arterial bleeding by means of a transfemorally introduced balloon catheter advanced into the left subclavian artery. We found that the combination of endovascular procedures with classical surgery can reduce the risks in the treatment of arteriovenous fistulas.

Multimodality treatment of pancreatic pseudoaneurisms.

Vascular lesions of pancreatitis manifest in the form of haemorrhage into the pseudocyst (PC), the development of pseudoaneurisms (PA) or splenic lesions. Between 1987 and 1996 31 patients were found to develop vascular lesions either in the form of haemorrhage into a PC (12) or PA (19). Diagnosis of pancreatic PA was established preoperatively in 8 cases only. Gastrointestinal (GI) bleeding manifested in 12 patients, but only in 6 of them was the pancreatic origin of the bleeding considered. All patients were operated. For the management of the lesions resection of the pancreas (11 cases) or ligation of the bleeding vessel with external or internal drainage of the PC was performed (12 cases). Simple external drainage of a haemorrhaged PC in 3, and cystoduodenostomy or cystogastrostomy was performed in 5 cases respectively. Intraoperatively moderate bleeding gave some concern (7 cases), while post operatively pancreatic fistula developed in 9 patients drained externally. All stopped spontaneously. In two cases severe GI bleeding occurred post operatively. In both cases embolization of the bleeding vessels was performed successfully. No operative mortality occurred. The mean follow-up time was 40.6 months (5-106). Five patients died of unrelated causes, 3 patients underwent subsequent pancreatic operation, and 74.2% of the patients are doing well. Development of pancreatic PA was associated with a longer observation or conservative treatment period. Angiography should be considered whenever severe upper GI bleeding occurs in patients with known pancreatic disease and the source of bleeding is obscure. In selected cases selective embolization of the bleeding site may provide definitive treatment.

[Endocrinologic complications of neurofibromatosis type 1]. / Neurofibromatosis 1. típusának endokrinológiai szövódményei.

Neurofibromatosis type 1 is the most common autosomal dominant inheritable disease, which is often associated with secondary forms of hypertension and with tumors of neuroectodermal origin. The authors present the results of evaluation of 60 members of 3 families. Of the 60 family members, 13 subjects had symptoms of neurofibromatosis type 1 disease, of which 7 subjects were evaluated. The case histories of patients are discussed: (1) An incidentally discovered adrenal tumor was proved to be a pheochromocytoma. (2) Because of complaints similar to thyrotoxicosis, thyrostatic drugs were administered for years without effect and, finally, an adrenal phaeochromocytoma was diagnosed after the presence of neurofibromatosis was established. (3) Preeclamptic pregnancy of a young primigravida complicated with severe HELLP syndrome (hemolysis-elevated liver enzymes-low platelet count) led to thorough evaluation which revealed renal artery stenosis. In this patient, percutaneous renal artery angioplasty resulted in a complete cessation of hypertension. (4) Glucocortocoid replacement therapy in a patient with neurofibromatosis type 1 resulted in a complete normalization of both secondary adrenal insufficiency and a previously unexplained iron-refractor iron-deficient anemia. The case histories of the patients demonstrate a lack of in-depth knowledge of neurofibromatosis in clinical practice. A regular follow-up of neurofibromatosis patients is suggested in specialized health centers.

[Incidence of stenosis of the internal carotid artery among patients waiting for coronary surgery]. / Arteria carotis interna szükületek gyakorisága coronaria mütétre váró betegek között.

1273 patients awaiting for coronary surgery were screened towards coexisting internal carotid artery stenoses. Non-significant carotid stenosis was revealed in 7.3% of these patients. In 48 patients (3.8%) the revealed stenoses were found to be hemodynamically significant. The coexistence of significant carotid stenoses with coronary artery disease was especially high at elderly patients. Among male patients over 70 years of age the occurrence of high grade carotid stenosis was as high as 10%. The 75% of significant stenoses was found to be asymptomatic. These would not been revealed without the carotid screening. The goal of this study is drawing attention to the frequent coexistence of coronary artery disease with carotid artery stenoses.

[Percutaneous transluminal angioplasty of the subclavian artery]. / Az arteria subclavia perkután transzluminális angioplasztikája.

Over a period of 12 years, percutaneous transluminal angioplasty was used to dilate 227 subclavian obliterations (216 stenoses, 11 occlusions) in 208 patients. Immediate success rate was 96%. 152 dilated arteries long-term patency are known. The average follow-up time was 32 months (1-120 months). 14 restenosis occurred. In 7 of the 14 patients redilation were performed. Complication: 3 puncture site thrombosis, 1 haematoma, 4 transient confusion occurred. In 2 patients shoulder pain developed with unknown origin, and last for a few weeks. There were no irreversible neurologic deficit. Percutaneous transluminal angioplasty of subclavian artery stenoses should be the procedure of choice in symptomatic patients.