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Zusammenfassung: Das Poplitealaneurisma (PA) ist eine typische Erkrankung von Männern über 65 Jahren, bei denen häufig Aneurysmen der Aorta, der iliacalen, femoralen und kontralateralen Poplitealarterie vorkommen. Als Ursache des Poplitealaneurysmas (PA) werden prioritär die Degradation durch Matrixmetalloproteinasen, eine entzündliche Reaktion mit Bildung von reaktiven Sauerstoffradikalen sowie der oxidative Stress in der Arterienwand postuliert. Zwei Drittel der Patienten kommen wegen Symptomen zum Chirurgen, die übrigen wegen eines Zufallsbefundes oder weil bereits die Gegenseite operiert wurde. Die akute und chronische Ischämie mit ihrer hohen Morbidität stehen im Vordergrund. Asymptomatische PA sollten ab einem Durchmesser von 2cm therapeutisch angegangen werden, besonders wenn sie partiell thrombosiert sind. Zur Diagnostik reicht eine Duplexuntersuchung. Die digitale Subtraktionsangiographie ist die wichtigste Untersuchung für die Operationsplanung. Lokalisierte Befunde, die auf die Kniekehle begrenzt sind, können von dorsal, langstreckige PA müssen durch einen Zugang von medial mit einem Interponat überbrückt werden. Dabei ist eine autologe Vene dem Kunststoffinterponat vorzuziehen. Im Fall eines Veneninterponates oder -bypasses kann mit einer Offenheitsrate von 85% nach 5Jahren gerechnet werden. Endografts sollen nur ausnahmsweise oder im Rahmen von Studien eingesetzt werden

Using echocardiographic quantification of aortic pulsatility distal to the site of the surgical anastomosis, we evaluated whether the preoperatively impaired poststenotic aortic pulsatility returned to normal after repair of coarctation with a hemodynamically ideal result. Patients who underwent repair of aortic coarctation without residual obstruction were compared to a matched group of normal children. A standardized M-mode echocardiographic evaluation of the aorta at the diaphragmatic level was performed for all patients. Measurements consisted of maximum and minimum aortic diameters, time intervals, and a calculated pulsatility index. Compared to normal children (n = 19), 20 children with operated coarctation and with a hemodynamically ideal result showed a significantly smaller increase in aortic diameter in systole (mean of 29 ± 7% in patients versus 37 ± 7% in normals; p < 0.01). In contrast to patients with coarctation in whom the maximum aortic distension is reached much later during the cardiac cycle, hemodynamically normalized, operated patients in our study had no such delay (maximum aortic pulsation at 28% of cardiac cycle time compared to 27% in normals; p = not significant). The pulsatility index of the poststenotic aorta was clearly lower in operated children (mean, 130 ± 50%/sec) compared to a normal mean value of 202 ± 33%/sec but was still significantly higher than that in patients with unoperated coarctation, who showed a low mean value of 51 ± 24%/sec (p < 0.01). After correction of aortic coarctation with a hemodynamically ideal result, the pulsatility of the poststenotic aorta, severely impaired prior to repair, did not return to normal during the observation period in the patients studied

Zusammenfassung: Die technische Entwicklung im Bereich von Katheterinterventionen hat auch zunehmenden Einfluss auf das Tätigkeitsfeld der Gefäßchirurgen. Im Operationssaal werden häufiger additive Katheterinterventionen oder Kombinationsverfahren, auch Hybrideingriffe genannt, durchgeführt. Dies setzt zwingend kathetertechnische Fertigkeiten voraus. Deshalb werden an der Klinik und Poliklinik für Herz- und Gefäßchirurgie in Bern in Zusammenarbeit mit interventionell tätigen Angiologen und Radiologen die Gefäßchirurgen in kathetertechnischen Interventionen trainiert. Katheterinterventionen zwingen dazu, die technische Ausrüstung des Operationssaals laufend anzupassen und die Schulung des Assistenzpersonals zu fördern

Cardiopulmonary bypass (CPB) is associated with a systemic inflammatory response. Pre-bypass steroid administration may modulate the inflammatory response, resulting in improved postoperative recovery. We performed a prospective study in the departments of cardiovascular surgery and pediatric intensive care medicine of two university hospitals that included 50 infants who underwent heart surgery. Patients received either prednisolone (30 mg/kg) added to the priming solution of the cardiopulmonary bypass circuit (steroid group) or no steroids (nonsteroid group). Clinical outcome parameters include therapy with inotropic drugs, oxygenation, blood lactate, glucose, and creatinine, and laboratory parameters of inflammation include leukocytes, C-reactive protein, and interleukin-8. Postoperative recovery (e.g., the number, dosage, and duration of inotropic drugs as well as oxygenation) was similar in patients treated with or without steroids when corrected for the type of cardiac surgery performed. After CPB, there was an inflammatory reaction, especially in patients with a long CPB time. Postoperative plasma levels of interleukin-8 were correlated with the duration of CPB time (r = 0.62, p < 0.001). Administration of steroids had no significant impact on the laboratory parameters of inflammation. Administration of prednisolone into the priming solution of the CPB circuit had no measurable influence on postoperative recovery and did not suppress the inflammatory response

OBJECTIVES To conduct a survey across European cardiac centres to evaluate the methods used for cerebral protection during aortic surgery involving the aortic arch. METHODS All European centres were contacted and surgeons were requested to fill out a short, comprehensive questionnaire on an internet-based platform. One-third of more than 400 contacted centres completed the survey correctly. RESULTS The most preferred site for arterial cannulation is the subclavian-axillary, both in acute and chronic presentation. The femoral artery is still frequently used in the acute condition, while the ascending aorta is a frequent second choice in the case of chronic presentation. Bilateral antegrade brain perfusion is chosen by the majority of centres (2/3 of cases), while retrograde perfusion or circulatory arrest is very seldom used and almost exclusively in acute clinical presentation. The same pumping system of the cardio pulmonary bypass is most of the time used for selective cerebral perfusion, and the perfusate temperature is usually maintained between 22 and 26°C. One-third of the centres use lower temperatures. Perfusate flow and pressure are fairly consistent among centres in the range of 10-15 ml/kg and 60 mmHg, respectively. In 60% of cases, barbiturates are added for cerebral protection, while visceral perfusion still receives little attention. Regarding cerebral monitoring, there is a general tendency to use near-infrared spectroscopy associated with bilateral radial pressure measurement. CONCLUSIONS These data represent a snapshot of the strategies used for cerebral protection during major aortic surgery in current practice, and may serve as a reference for standardization and refinement of different approaches

Between January 1983 and December 1990, 20 patients aged 80 years or older underwent valvular surgery. The patients' ages varied from 80 to 87 years (mean, 82 ± 1.5 years). The indication for operation was aortic stenosis in 19 patients, and mitral insufficiency after previous mitral valve replacement with a bioprosthesis in one. There were 15 elective, two urgent, and three emergency operations. Four of these patients had aortic valve replacement plus coronary artery bypass grafting. Six patients (30%) had an uneventful hospital stay, and the other 14 (70%) experienced several post-operative complications. The operative mortality rate was 15± (three patients). All patients before operation were in NYHA (New York Heart Association) class III and IV and all survivors remained in NYHA class I or II. The survivors have been followed from 6 to 70 months (mean 20 ± 8 months). The actuarial survival rate at 1 and 5 years was 78.5% and 67%, respectively. Valvular replacement in octogenarians can be performed, despite the high rate of post-operative complications, with increased but acceptable mortality. Long-term results are good

Objective: To compare three different techniques of distal aortic repair in acute type A (de Bakey type I) aortic dissection and to evaluate their impact on the late morphology of the aortic arch and descending aorta and on the incidence of reoperation. Methods: From 65 patients operated on due to an acute type A aortic dissection between 1989 and 1993, 54 long-term survivors underwent clinical and radiologic follow-up examination after a mean postoperative interval of 62±16 months. The surgical techniques of distal aortic reconstruction included closed repair using Teflon felt reinforcement under moderate hypothermic cardiopulmonary bypass (n=20) and open repair in deep hypothermic circulatory arrest using either Teflon felt reinforcement (n=16) or gelatin-resorcin-formaldehyde (GRF) glue (n=18) to readapt the dissected aortic layers. In all patients, MR imaging was performed on a 1.5-T whole body imaging system for the evaluation of the morphology and function of the heart, aorta and supraaortic branches. Results: Overall hospital mortality following surgical repair of type A aortic dissection was 15.4% during this time period. The highest rate of persistent false lumen perfusion (17/20, 85%) and presence of an intimal flap in the aortic arch (13/20, 65%) was observed in patients following closed repair of acute ascending aortic dissection, whereas the lowest rate of such findings was demonstrated in patients who had undergone open distal aortic repair using biological glue (false lumen perfusion 10/18, 55% and intimal flap in the arch 2/18, 11%). Redo-surgery was significantly reduced in the open repair group using GRF glue (1/18, 5.5%) as compared with the Teflon felt repair group (3/16, 18%) and the closed repair group (6/20, 30%). Conclusions: In patients with acute type A dissection, open distal aortic repair using GRF-glue favourably influences both (1) the severity of late morphologic alterations in the downstream aorta and (2) the incidence of reoperation

Ein Handbuch für Betroffene seltener Krankheiten und ihr Umfeld. Das Bewältigen einer chronischen Krankheit stellt an die betroffenen Menschen hohe Anforderungen. Erst recht, wenn die Krankheit selten ist. Wie gehen sie damit um? Welche Unterstützung erhalten sie von ihrem sozialen und vom professionellen Umfeld? Was bedeuten «Gesundheits- kompetenz» und «Empowerment» für sie – in der Gesundheitsversorgung und im Alltag? Am Beispiel des Marfan-Syndroms sind Betroffene und Fachleute diesen Fragen aus verschiedenen Blickwinkeln nachgegangen.

Mutations in the FBN1 gene are the major cause of Marfan syndrome (MFS), an autosomal dominant connective tissue disorder, which displays variable manifestations in the cardiovascular, ocular, and skeletal systems. Current molecular genetic testing of FBN1 may miss mutations in the promoter region or in other noncoding sequences as well as partial or complete gene deletions and duplications. In this study, we tested for copy number variations by successively applying multiplex ligation-dependent probe amplification (MLPA) and the Affymetrix Human Mapping 500 K Array Set, which contains probes for approximately 500,000 single-nucleotide polymorphisms (SNPs) across the genome. By analyzing genomic DNA of 101 unrelated individuals with MFS or related phenotypes in whom standard genetic testing detected no mutation, we identified FBN1 deletions in two patients with MFS. Our high-resolution approach narrowed down the deletion breakpoints. Subsequent sequencing of the junctional fragments revealed the deletion sizes of 26,887 and 302,580 bp, respectively. Surprisingly, both deletions affect the putative regulatory and promoter region of the FBN1 gene, strongly indicating that they abolish transcription of the deleted allele. This expectation of complete loss of function of one allele, i.e. true haploinsufficiency, was confirmed by transcript analyses. Our findings not only emphasize the importance of screening for large genomic rearrangements in comprehensive genetic testing of FBN1 but, importantly, also extend the molecular etiology of MFS by providing hitherto unreported evidence that true haploinsufficiency is sufficient to cause MFS.

Cardiopulmonary bypass (CPB) is associated with a systemic inflammatory response. Pre-bypass steroid administration may modulate the inflammatory response, resulting in improved postoperative recovery. We performed a prospective study in the departments of cardiovascular surgery and pediatric intensive care medicine of two university hospitals that included 50 infants who underwent heart surgery. Patients received either prednisolone (30 mg/kg) added to the priming solution of the cardiopulmonary bypass circuit (steroid group) or no steroids (nonsteroid group). Clinical outcome parameters include therapy with inotropic drugs, oxygenation, blood lactate, glucose, and creatinine, and laboratory parameters of inflammation include leukocytes, C-reactive protein, and interleukin-8. Postoperative recovery (e.g., the number, dosage, and duration of inotropic drugs as well as oxygenation) was similar in patients treated with or without steroids when corrected for the type of cardiac surgery performed. After CPB, there was an inflammatory reaction, especially in patients with a long CPB time. Postoperative plasma levels of interleukin-8 were correlated with the duration of CPB time (r = 0.62, p < 0.001). Administration of steroids had no significant impact on the laboratory parameters of inflammation. Administration of prednisolone into the priming solution of the CPB circuit had no measurable influence on postoperative recovery and did not suppress the inflammatory response

Between January 1983 and December 1990, 20 patients aged 80 years or older underwent valvular surgery. The patients' ages varied from 80 to 87 years (mean, 82 ± 1.5 years). The indication for operation was aortic stenosis in 19 patients, and mitral insufficiency after previous mitral valve replacement with a bioprosthesis in one. There were 15 elective, two urgent, and three emergency operations. Four of these patients had aortic valve replacement plus coronary artery bypass grafting. Six patients (30%) had an uneventful hospital stay, and the other 14 (70%) experienced several post-operative complications. The operative mortality rate was 15± (three patients). All patients before operation were in NYHA (New York Heart Association) class III and IV and all survivors remained in NYHA class I or II. The survivors have been followed from 6 to 70 months (mean 20 ± 8 months). The actuarial survival rate at 1 and 5 years was 78.5% and 67%, respectively. Valvular replacement in octogenarians can be performed, despite the high rate of post-operative complications, with increased but acceptable mortality. Long-term results are good

Using echocardiographic quantification of aortic pulsatility distal to the site of the surgical anastomosis, we evaluated whether the preoperatively impaired poststenotic aortic pulsatility returned to normal after repair of coarctation with a hemodynamically ideal result. Patients who underwent repair of aortic coarctation without residual obstruction were compared to a matched group of normal children. A standardized M-mode echocardiographic evaluation of the aorta at the diaphragmatic level was performed for all patients. Measurements consisted of maximum and minimum aortic diameters, time intervals, and a calculated pulsatility index. Compared to normal children (n = 19), 20 children with operated coarctation and with a hemodynamically ideal result showed a significantly smaller increase in aortic diameter in systole (mean of 29 ± 7% in patients versus 37 ± 7% in normals; p < 0.01). In contrast to patients with coarctation in whom the maximum aortic distension is reached much later during the cardiac cycle, hemodynamically normalized, operated patients in our study had no such delay (maximum aortic pulsation at 28% of cardiac cycle time compared to 27% in normals; p = not significant). The pulsatility index of the poststenotic aorta was clearly lower in operated children (mean, 130 ± 50%/sec) compared to a normal mean value of 202 ± 33%/sec but was still significantly higher than that in patients with unoperated coarctation, who showed a low mean value of 51 ± 24%/sec (p < 0.01). After correction of aortic coarctation with a hemodynamically ideal result, the pulsatility of the poststenotic aorta, severely impaired prior to repair, did not return to normal during the observation period in the patients studied

Zusammenfassung: Die technische Entwicklung im Bereich von Katheterinterventionen hat auch zunehmenden Einfluss auf das Tätigkeitsfeld der Gefäßchirurgen. Im Operationssaal werden häufiger additive Katheterinterventionen oder Kombinationsverfahren, auch Hybrideingriffe genannt, durchgeführt. Dies setzt zwingend kathetertechnische Fertigkeiten voraus. Deshalb werden an der Klinik und Poliklinik für Herz- und Gefäßchirurgie in Bern in Zusammenarbeit mit interventionell tätigen Angiologen und Radiologen die Gefäßchirurgen in kathetertechnischen Interventionen trainiert. Katheterinterventionen zwingen dazu, die technische Ausrüstung des Operationssaals laufend anzupassen und die Schulung des Assistenzpersonals zu fördern

Cardiopulmonary bypass (CPB) is associated with a systemic inflammatory response. Pre-bypass steroid administration may modulate the inflammatory response, resulting in improved postoperative recovery. We performed a prospective study in the departments of cardiovascular surgery and pediatric intensive care medicine of two university hospitals that included 50 infants who underwent heart surgery. Patients received either prednisolone (30 mg/kg) added to the priming solution of the cardiopulmonary bypass circuit (steroid group) or no steroids (nonsteroid group). Clinical outcome parameters include therapy with inotropic drugs, oxygenation, blood lactate, glucose, and creatinine, and laboratory parameters of inflammation include leukocytes, C-reactive protein, and interleukin-8. Postoperative recovery (e.g., the number, dosage, and duration of inotropic drugs as well as oxygenation) was similar in patients treated with or without steroids when corrected for the type of cardiac surgery performed. After CPB, there was an inflammatory reaction, especially in patients with a long CPB time. Postoperative plasma levels of interleukin-8 were correlated with the duration of CPB time (r = 0.62, p < 0.001). Administration of steroids had no significant impact on the laboratory parameters of inflammation. Administration of prednisolone into the priming solution of the CPB circuit had no measurable influence on postoperative recovery and did not suppress the inflammatory response

Between January 1983 and December 1990, 20 patients aged 80 years or older underwent valvular surgery. The patients' ages varied from 80 to 87 years (mean, 82 ± 1.5 years). The indication for operation was aortic stenosis in 19 patients, and mitral insufficiency after previous mitral valve replacement with a bioprosthesis in one. There were 15 elective, two urgent, and three emergency operations. Four of these patients had aortic valve replacement plus coronary artery bypass grafting. Six patients (30%) had an uneventful hospital stay, and the other 14 (70%) experienced several post-operative complications. The operative mortality rate was 15± (three patients). All patients before operation were in NYHA (New York Heart Association) class III and IV and all survivors remained in NYHA class I or II. The survivors have been followed from 6 to 70 months (mean 20 ± 8 months). The actuarial survival rate at 1 and 5 years was 78.5% and 67%, respectively. Valvular replacement in octogenarians can be performed, despite the high rate of post-operative complications, with increased but acceptable mortality. Long-term results are good

OBJECTIVES To conduct a survey across European cardiac centres to evaluate the methods used for cerebral protection during aortic surgery involving the aortic arch. METHODS All European centres were contacted and surgeons were requested to fill out a short, comprehensive questionnaire on an internet-based platform. One-third of more than 400 contacted centres completed the survey correctly. RESULTS The most preferred site for arterial cannulation is the subclavian-axillary, both in acute and chronic presentation. The femoral artery is still frequently used in the acute condition, while the ascending aorta is a frequent second choice in the case of chronic presentation. Bilateral antegrade brain perfusion is chosen by the majority of centres (2/3 of cases), while retrograde perfusion or circulatory arrest is very seldom used and almost exclusively in acute clinical presentation. The same pumping system of the cardio pulmonary bypass is most of the time used for selective cerebral perfusion, and the perfusate temperature is usually maintained between 22 and 26°C. One-third of the centres use lower temperatures. Perfusate flow and pressure are fairly consistent among centres in the range of 10-15 ml/kg and 60 mmHg, respectively. In 60% of cases, barbiturates are added for cerebral protection, while visceral perfusion still receives little attention. Regarding cerebral monitoring, there is a general tendency to use near-infrared spectroscopy associated with bilateral radial pressure measurement. CONCLUSIONS These data represent a snapshot of the strategies used for cerebral protection during major aortic surgery in current practice, and may serve as a reference for standardization and refinement of different approaches

Objective: To compare three different techniques of distal aortic repair in acute type A (de Bakey type I) aortic dissection and to evaluate their impact on the late morphology of the aortic arch and descending aorta and on the incidence of reoperation. Methods: From 65 patients operated on due to an acute type A aortic dissection between 1989 and 1993, 54 long-term survivors underwent clinical and radiologic follow-up examination after a mean postoperative interval of 62±16 months. The surgical techniques of distal aortic reconstruction included closed repair using Teflon felt reinforcement under moderate hypothermic cardiopulmonary bypass (n=20) and open repair in deep hypothermic circulatory arrest using either Teflon felt reinforcement (n=16) or gelatin-resorcin-formaldehyde (GRF) glue (n=18) to readapt the dissected aortic layers. In all patients, MR imaging was performed on a 1.5-T whole body imaging system for the evaluation of the morphology and function of the heart, aorta and supraaortic branches. Results: Overall hospital mortality following surgical repair of type A aortic dissection was 15.4% during this time period. The highest rate of persistent false lumen perfusion (17/20, 85%) and presence of an intimal flap in the aortic arch (13/20, 65%) was observed in patients following closed repair of acute ascending aortic dissection, whereas the lowest rate of such findings was demonstrated in patients who had undergone open distal aortic repair using biological glue (false lumen perfusion 10/18, 55% and intimal flap in the arch 2/18, 11%). Redo-surgery was significantly reduced in the open repair group using GRF glue (1/18, 5.5%) as compared with the Teflon felt repair group (3/16, 18%) and the closed repair group (6/20, 30%). Conclusions: In patients with acute type A dissection, open distal aortic repair using GRF-glue favourably influences both (1) the severity of late morphologic alterations in the downstream aorta and (2) the incidence of reoperation

Zusammenfassung: Das Poplitealaneurisma (PA) ist eine typische Erkrankung von Männern über 65 Jahren, bei denen häufig Aneurysmen der Aorta, der iliacalen, femoralen und kontralateralen Poplitealarterie vorkommen. Als Ursache des Poplitealaneurysmas (PA) werden prioritär die Degradation durch Matrixmetalloproteinasen, eine entzündliche Reaktion mit Bildung von reaktiven Sauerstoffradikalen sowie der oxidative Stress in der Arterienwand postuliert. Zwei Drittel der Patienten kommen wegen Symptomen zum Chirurgen, die übrigen wegen eines Zufallsbefundes oder weil bereits die Gegenseite operiert wurde. Die akute und chronische Ischämie mit ihrer hohen Morbidität stehen im Vordergrund. Asymptomatische PA sollten ab einem Durchmesser von 2cm therapeutisch angegangen werden, besonders wenn sie partiell thrombosiert sind. Zur Diagnostik reicht eine Duplexuntersuchung. Die digitale Subtraktionsangiographie ist die wichtigste Untersuchung für die Operationsplanung. Lokalisierte Befunde, die auf die Kniekehle begrenzt sind, können von dorsal, langstreckige PA müssen durch einen Zugang von medial mit einem Interponat überbrückt werden. Dabei ist eine autologe Vene dem Kunststoffinterponat vorzuziehen. Im Fall eines Veneninterponates oder -bypasses kann mit einer Offenheitsrate von 85% nach 5Jahren gerechnet werden. Endografts sollen nur ausnahmsweise oder im Rahmen von Studien eingesetzt werden