German Registry for Acute Aortic Dissection Type A: Structure, Results, and Future Perspectives.

The German Registry for Acute Aortic Dissection Type A (GERAADA) as an international registry for acute aortic dissection type A (AADA) offers a unique opportunity to answer questions regarding acute dissections that cannot be answered by single institution's database alone. GERAADA was started in 2006 by the German Society for Thoracic and Cardiovascular Surgery (GSTCVS) and has collected more than 3,300 AADA patients' data from 56 centers in Germany, Austria, and Switzerland up to now. In the second generated validated dataset comprising the years from 2006 to 2010, 2,137 patients were surgically treated for AADA with an overall 30-day mortality of 16.9%, and a new postoperative neurologic dysfunction of 9.5%. Risk factors for neurologic dysfunction were malperfusion syndromes, dissections of the supra-aortic vessels, and longer operating time. Neuroprotective drugs had no influence on stroke rates. Hypothermic circulatory arrest and antegrade selective cerebral perfusion (ACP) led to similar results if arrest times were less than 30 minutes while ACP for longer arrest periods is advisable. Septuagenarians had an early mortality rate (15.8%) similar to the whole cohort's, but the mortality rate in octogenarians (34.9%) was much higher. GERAADA with its validated 2,137 patient files (2006-2010) is the largest database on AADA worldwide and continues to collect data. Structured follow-up of more than 5 years will be available in the future.

Endovascular treatment of symptomatic true-lumen collapse of the downstream aorta after open surgery for acute aortic dissection type A.

AIM: The aim of the present study was to evaluate the outcome of endovascular treatment of true-lumen collapse (TLC) of the downstream aorta after open surgery for acute aortic dissection type A (AADA). METHODS: Retrospective, observational study with follow-up of 16 ± 7.6 months. From April 2010 to January 2012, 89 AADA-patients underwent aortic surgery. Out of these, computed tomography revealed a TLC of the downstream aorta in 13 patients (14.6%). They all received additional thoracic endovascular aortic repair (TEVAR) in consequence of malperfusion syndromes. RESULTS: In all 13 TLC-patients, dissection after AADA-surgery extended from the aortic arch to the abdominal aorta and malperfusion syndromes occurred. Remodeling of the true-lumen was achieved by TEVAR with complemental stent disposal in abdominal and iliac arteries in all cases. One patient died on the third postoperative day due to intracerebral hemorrhage. Another patient, who presented under severe cardiogenic shock died despite AADA-surgery and TEVAR-treatment. Thirty-day mortality was 15.4% in TLC-patients (N = 2/13). In the follow-up period, 3 patients required additional aortic stents after the emergency TEVAR procedures. After 20 weeks, a third patient died secondary to malperfusion due to false-lumen recanalization. Therefore, late mortality was 23.1%. CONCLUSION: After proximal aortic repair for AADA, early postoperative computed tomography should be demanded in all patients to exclude a TLC of the descending aorta. Mortality is still substantial in these patients despite instant TEVAR application. Thus, in case of TLC and malperfusion syndrome of the downstream aorta, TEVAR should be performed early to alleviate or even prevent ischemic injury.

Acute aortic dissection type A.

BACKGROUND: Acute aortic dissection type A (AADA) is a life-threatening vascular emergency. Clinical presentation ranges from pain related to the acute event, collapse due to aortic rupture or pericardial tamponade, or manifestations of organ or limb ischaemia. The purpose of this review was to clarify important clinical issues of AADA management, with a focus on diagnostic and therapeutic challenges. METHODS: Based on a MEDLINE search the latest literature on this topic was reviewed. Results from the German Registry for Acute Aortic Dissection Type A (GERAADA) are also described. RESULTS: Currently, the perioperative mortality rate of AADA is below 20 per cent, the rate of definitive postoperative neurological impairment approaches 12 per cent and the long-term prognosis after surviving the acute phase of the disease is good. Many pathology- and therapy-associated factors influence the outcome of AADA, including prompt diagnosis with computed tomography and better cerebral protection strategies during aortic arch reconstruction. Endovascular technologies are emerging that may lead to less invasive treatment options. CONCLUSION: AADA is an emergency that can present with a wide variety of clinical scenarios. Advances in the surgical management of this complex disease are improving outcomes.

[German Registry for Acute Aortic Dissection Type A (GERAADA): initial results]. / Deutsches Register für akute Aortendissektion Typ A (GERAADA): Erste Ergebnisse.

BACKGROUND: The working group "Aortic Surgery and Interventional Vascular Surgery" of the German Society for Thoracic and Cardiovascular Surgery (GSTCVS) set up the German registry for acute aortic dissection type A (GERAADA) in July 2006. This web-based database was developed to record data of patients who had undergone surgery for aortic dissection type A (AADA). The aim of GERAADA is to learn from analyzing the data of AADA patients how to improve the perioperative management and surgical treatment of patients with AADA and to identify possible parameters affecting patient risk and outcome. PATIENTS AND METHODS: Between July 2006 and June 2009 (2010), 1558 (2137) patients with AADA were enrolled in the multi-center, prospective GERAADA database by 50 cardiac surgery centers in German-speaking countries in Europe. Data on patients' preoperative and intraoperative status, postoperative complications, midterm results and circumstances of death were recorded. Data were analyzed to identify risk factors influencing the outcome of these patients. The Institute of Medical Biostatistics, Epidemiology and Informatics (IMBEI) in Mainz performed the statistical analyses. RESULTS: Analyses from GERAADA reveal a thirty-day mortality of 17% in 2137 AADA patients. Only short interventions in aortic arch surgery are safe during hypothermic circulatory arrest even without selective cerebral perfusion. If circulatory arrest times of over 30 min. are anticipated, antegrade cerebral perfusion is strongly recommended during the entire arch intervention using cardiopulmonary bypass. Surgical strategy in terms of isolated ascending aortic replacement versus ascending aortic replacement combined with aortic arch repair had no statistical relevant influence on 30-day mortality. AADA surgical results in elderly patients are more encouraging than those treated without surgery. Surgery is even feasible in octogenarians with a 35% mortality rate. CONCLUSION: The aim of this registry is to optimize AADA patients' medical care, thereby reducing their morbidity and mortality. AADA treatment should always involve open surgery. Initial analyses from GERAADA provide clinically relevant insights concerning patients with AADA, and may enable therapeutic recommendations for improving perioperative and surgical management. Our latest study detected significant influencing risk factors for the outcome of AADA patients and may contribute to a consensus in setting guidelines for standard medical treatment. PERSPECTIVE: A European Registry of Aortic Diseases ("EuRADa") is being established this year under the leadership of the "Vascular Domain" of the European Association for Cardio-Thoracic Surgery (EACTS). This database will collect parameters on all aortic diseases, dissection types A and B, aneurysms, perforating ulcer (PAU), intramural wall hematoma (IMH), traumatic aortic ruptures, and all potential treatment strategies (medical treatment, open surgical and endovascular).

German Registry for Acute Aortic Dissection Type A (GERAADA)--new software design, parameters and their definitions.

BACKGROUND: The working group for aortic surgery and interventional vascular surgery of the German Society for Thoracic and Cardiovascular Surgery (GSTCVS) initiated the web-based German Registry for Acute Aortic Dissection type A (GERAADA). It is the project's aim to collect standardized data from a large pool of patients with acute aortic dissections type A (AADA) to gain a deeper insight and knowledge to improve surgical therapies and perioperative management for these patients in the future. METHODS: In addition to new medical insights, the working group has gained more experience over the last 4 years in how to collect valid and high-quality data. This experience led us to revise the database completely. In this article we describe the new version of GERAADA, providing an overview as well as defining the parameters, and explaining the new features. This overview fulfills a request by the users of GERAADA in the participating centers. RESULTS: Since its inception, 50 cardiac centers in Germany, Austria and Switzerland have provided over 2000 records and the first statistical results have been published. CONCLUSION: GERAADA's new design allows it to stay abreast of changes in medicine and to focus on the essentials necessary for statistically relevant results, while keeping the work load low for the data providers at each cardiac center.

Long-term patency of open revascularization for visceral artery pathologies.

OBJECTIVE: Aim of the study was to evaluate the long-term patency of bypass grafts used to treat occlusive and aneurysmal disease of the visceral arteries. METHODS: A retrospective analysis of our vascular surgery database identified 30 patients (11 men, mean age 59 ± 14 years) who underwent 32 operations for visceral artery pathology between January 1995 and December 2009. Acute mesenteric ischemia (aMI) was present in 10, chronic mesenteric ischemia (cMI) in 14 and visceral artery aneurysm (VAn) in 7 cases. The primary endpoint of this study was vessel patency, secondary endpoints were survival and freedom from reintervention. RESULTS: A total of 46 vessels were revascularized (26 bypass grafts) and additional revascularization procedures (thromboembolectomy, patch plasty, transposition) were performed in 21 cases. In the perioperative period, 6 deaths (5 aMI, 1 cMI) occurred, resulting in a mortality rate of 50 % for aMI and 7 % for cMI. At long-term follow-up (55 months), 22 patients (100 % follow-up) were interviewed and 21 were scheduled for clinical and imaging examinations. Four vessel (3 grafts) occlusions were found in these patients. CONCLUSION: We were able to show that bypass grafting for a visceral artery pathology, although associated with an increased perioperative mortality, is a successful and durable procedure.

Value and pitfalls of neurophysiological monitoring in thoracic and thoracoabdominal aortic replacement and endovascular repair.

BACKGROUND: The aim of our study was to analyze the neurophysiological monitoring method with regard to its potential problems during thoracic and thoracoabdominal aortic open or endovascular repair. Furthermore, preventive strategies to the main pitfalls with this method were developed. METHODS: Between 11/2000 and 05/2007 in 97 cases open surgery or endovascular stentgraft-implantation was performed on the thoracic or thoracoabdominal aorta. Intraoperatively, neurophysiologic motor- and somatosensory-evoked potentials were monitored. RESULTS: Our cases were divided into four groups: event-free patients with normal potentials (A, 63 cases), with correlation of modified evoked potentials and neurological outcome (B, 14 cases), false-positive or false-negative results (C, 4 cases), and medication interaction or technical issues (D, 16 cases). We observed a sensitivity of 93 % and a specificity of 96 % for the neurophysiological monitoring. CONCLUSIONS: Monitoring spinal cord function during surgical and endovascular interventions on the thoracic and thoracoabdominal aorta is necessary. It can be made more effective by precisely analyzing the interference factors of the neurophysiological monitoring method itself. Successful strategies of immediate troubleshooting could be identified.

German registry for acute aortic dissection type A (GERAADA)--lessons learned from the registry.

A German registry for acute aortic dissection type A (GERAADA) was initiated by the Working Group for Aortic Surgery and Interventional Vascular Surgery of the German Society for Thoracic and Cardiovascular Surgery (GSTCVS) in July 2006. This web-based database was developed to record the data of patients who had undergone surgery for aortic dissection type A. From analyzing the data, we aim to learn how to improve surgical treatment and to identify parameters affecting patient outcome. In the beginning, 33 cardiac centers participated via online access to the registry on the GSTCVS' homepage. Since then, 43 centers in Germany, Switzerland and Austria have begun entering data on the pre- and intraoperative status of their patients, postoperative complications, mid-term results and circumstances of death. We have succeeded in interpreting the initial results and trends from the registry now available to all of the participating centers, which benefit from this shared pool of analyzed data by optimizing their therapy regimes and comparing their success with that in the other centers.

Efficacy of the non-adenosine analogue A1 adenosine receptor agonist (BR-4935) on cardiovascular function after cardiopulmonary bypass.

BACKGROUND: We tested the hypothesis that pharmacological preconditioning with a newly developed, potent non-adenosine analogue A1AdoR agonist (BR-4935) improves biventricular cardiac and endothelial function after cardiopulmonary bypass. METHODS: Twelve anesthetized dogs underwent cardiopulmonary bypass. Dogs were divided into two groups: group 1 (n = 6) received saline vehicle, group 2 (n = 6) received BR-4935 before cardiopulmonary bypass. Biventricular hemodynamic variables were measured using a combined pressure-volume conductance catheter. Coronary blood flow, ATP content, malondialdehyde and myeloperoxidase levels and vasodilatative responses to acetylcholine and sodium nitroprusside were also determined. RESULTS: Administration of the A1AdoR agonist led to a significantly better recovery of left and right ventricular systolic function after 60 minutes of reperfusion. Although the vasodilatative response to sodium nitroprusside was similar in both groups, acetylcholine resulted in a significantly greater increase in coronary blood flow in the BR-4935 group. In addition, the ATP content was significantly higher in the same group. Furthermore, malondialdehyde and myeloperoxidase levels significantly decreased in the A1AdoR group. CONCLUSION: Pharmacological preconditioning with a new, potent non-adenosine analogue A1AdoR agonist improves biventricular function recovery and endothelial function after hypothermic cardiac arrest.

Reevaluation of two techniques of harvesting the skeletonized internal mammary artery.

BACKGROUND: Selective skeletonization of the internal mammary artery (IMA) without adjacent vasculo-muscular structures reduces trauma to the chest wall, results in elongated grafts, makes ideal graft positioning possible, and eliminates the need to implant a dissected or hypoplastic graft with direct visual control of the vessel. We compared two techniques of skeletonizing the IMA in a prospective randomized trial. METHODS: 51 IMAs were randomly harvested and divided into two groups according to the technique of skeletonization. In group I (n = 31), IMAs were harvested in a skeletonized fashion with the Harmonic Ultrasonic scalpel, and in group II (n = 20) using scissors and hemostatic clips. We compared arterial wall histology, harvesting time, spasm frequency, and the use of hemostatic clips between the two groups. RESULTS: There were no significant morphological differences in the arterial wall in the two groups. Use of an ultrasonically-activated scalpel reduced the IMA's harvesting time (p < 0.001), the frequency of spasm (p = 0.01), and the use of hemostatic clips (p < 0.001). CONCLUSIONS: Ultrasonic harvesting of a skeletonized IMA is a non-traumatic preparatory technique that reduces the costs of surgical clips and that can be performed safely and quickly.

Bail-out visceral bypass grafting for acute intestinal ischemia after endovascular stent-graft placement in a complicated type B dissection.

We report on the case of a 35-year-old male who underwent emergency stent-graft placement in March 2007 due to a complicated type B dissection. One week after this procedure the patient developed critical visceral malperfusion. Subsequently, autologous iliaco-mesenteric as well as iliaco-hepatic bypass grafting was performed. At 6-month follow-up, aortic remodelling has occurred and visceral perfusion is regular.

Efficacy and frequency of cerebrospinal fluid drainage in operative management of thoracoabdominal aortic aneurysms.

BACKGROUND: Paraplegia remains the most dreaded complication following thoracoabdominal aortic repair. We investigated the efficacy of cerebrospinal fluid drainage as a spinal cord-protecting modality. We also evaluated the correlation between the frequency of cerebrospinal fluid drainage and the Crawford classification. METHODS: Spinal cord function was monitored during 20 open surgical procedures (group I) and 27 stent-graft implantations (group II). Evoked potentials and intracranial pressure were monitored in each operation. If intracranial pressure exceeded 15 mmHg, cerebrospinal fluid was drained. RESULTS: Cerebrospinal fluid drainage was necessary in 75 % of patients in group I (Crawford type I: 33 %, type II: 40 %, type III: 20 %, type IV: 7 %) and in 22 % of patients in group II (Crawford type I: 33 %, type II: 66 %). Evoked potential alterations correlated with an increase in intracranial pressure. Timely cerebrospinal fluid drainage reversed these changes in 72 %. Three patients remained paraplegic. CONCLUSION: Cerebrospinal fluid drainage is a valuable neuroprotective interventional tool to lower the risk of spinal cord ischemia. The combination of neurophysiological monitoring and cerebrospinal fluid drainage optimizes the prevention of paraplegia during aortic repair.

Actual management of patients with familial ascending aortic aneurysms and type-A aortic dissections.

BACKGROUND: There are few families with the diagnosis of ascending aortic aneurysm and acute type-A aortic dissection inherited as an autosomal-dominant disorder in the absence of a known genetic syndrome. METHODS: We investigated a family with 26 members in whom ascending aortic aneurysms and acute type-A aortic dissections occurred over three generations. Examinations were performed to identify family members at specific risk. RESULTS: Six members presented with acute type-A aortic dissections and three relatives had ascending aortic aneurysms. Clinical examinations showed no characteristics of a known genetic syndrome. Molecular genetic analysis revealed no mutations known to cause a form of autosomal-dominant inherited aortic disease. CONCLUSION: Adequate diagnostic measures are mandatory in families with ascending aortic aneurysms or type-A aortic dissections to identify or exclude family members at risk for aortic diseases. Even in the absence of identifiable mutations causing isolated aortic aneurysms or aortic dissections, we recommend standardised examinations of all first-degree relatives of affected families. An indication for prophylactic aortic root replacement should be considered for patients at risk.

Evaluation of three different measurement methods for dural ectasia in Marfan syndrome.

AIM: Dural ectasia is a major diagnostic criterion for Marfan syndrome using the Ghent nosology. Our aim was to evaluate the efficacy of three different radiological methods previously proposed for the assessment of dural sac diameter in Marfan syndrome. METHODS: Marfan syndrome was diagnosed in our study using the Ghent criteria, disregarding dural ectasia as a criterion. Three proposed radiological methods were applied to measure dural sac diameter, examined for 41 patients (18 patients with and 23 without Marfan syndrome) by computed tomography or magnetic resonance imaging. RESULTS: Using Oosterhof's method, 94% of the patients with and 44% of the patients without Marfan syndrome fulfilled the criteria of dural ectasia. According to Villeirs, dural ectasia was diagnosed in 18% of the patients with and in none of the patients without Marfan syndrome. With Ahn's method, dural ectasia was found in 72% of the patients with and in 44% of the patients without Marfan syndrome. In only two patients with Marfan syndrome was dural ectasia diagnosed by all three methods. CONCLUSION: Our results reveal overt discrepancy between the three methods of assessing dural ectasia. Considering the key role played by dural ectasia in reinforcing the diagnosis of Marfan syndrome according to the Ghent nosology, a standardized and reliable method should be sought.

Volume changes of experimental carotid sidewall aneurysms due to embolization with liquid embolic agents: a multidetector CT angiography study.

Iodine-containing polyvinyl alcohol polymer (I-PVAL) is a novel precipitating liquid embolic that allows for artifact-free evaluation of CT angiography (CTA). As accurate aneurysm volumetry can be performed with multidetector CTA, we determined volumes of experimental aneurysms before, immediately after, and 4 weeks after embolization of 14 porcine experimental carotid sidewall aneurysms with this liquid embolic. An automated three-dimensional software measurement tool was used for volumetric analysis of volume-rendering CTA data. Furthermore, intra-aneurysmal pressure changes during liquid embolization were measured in four silicone aneurysms and potential polymer volume changes within 4 weeks were assessed in vitro. Liquid embolic injection was performed during temporary balloon occlusion of the aneurysm neck, resulting in a mean occlusion rate of 98.3%. Aneurysms enlarged significantly during embolization by 61.1 +/- 28.9%, whereas a significant shrinkage of 5.6 +/- 2.7% was observed within the follow-up period. Histologic analysis revealed an inflammatory foreign body reaction with partial polymer degradation. In silicone aneurysm models, intra-aneurysmal pressure remained unchanged during liquid embolic injection, whereas balloon inflation resulted in a mean pressure increase of 31.2 +/- 0.7%. No polymer shrinkage was observed in vitro. The aneurysm enlargement noted was presumably due to pressure elevation after balloon inflation, which resulted in dilatation of the weak venous wall of the newly constructed aneurysm--another shortcoming of this experimental aneurysm model. The volume decrease after 4 weeks expressed partial polymer degradation.

Development of a special balloon occlusion device to prevent adverse events in high-risk patients during open aortic surgery.

OBJECTIVE: To prevent clamp injury that may occur during aortic surgery, we aimed to develop a special balloon occlusion (BO) device to lower the thromboembolic risk in patients with severe atherosclerosis during aortic aneurysm repair. METHODS: The study comprised two test phases: a laboratory-testing series focussing on flexible artificial aortas, and an experimental series conducted on 10 pigs. RESULTS: The device proved to be effective during the laboratory tests and the experiments on pigs. No complications such as intraoperative balloon rupture, dislocation, or occlusion leaks occurred. No damage to the aortic vessels was observed in further histological examinations. CONCLUSIONS: This BO device has the potential to become an alternative to cross-clamping for vascular surgeons in patients with severely atherosclerotic vessels.

Thoracoabdominal aortic aneurysm repair: interplay of spinal cord protecting modalities.

BACKGROUND: The purpose of this study was to assess the complementary use of different methods of measuring spinal cord perfusion during thoracoabdominal aortic surgery. METHODS: The spinal cords of 28 patients undergoing surgery on the thoracoabdominal aorta were monitored with transcranial electrical stimulation (tcMEP) and somatosensory-evoked potentials (SSEP). Available approaches of spinal cord-protection included: Moderate systemic hypothermia, constant cerebrospinal fluid (CSF) drainage and pressure monitoring, reimplantation of segmental arteries, cardiopulmonary bypass (CPB), and staged clamping. RESULTS: Fourteen of 19 patients (75%) undergoing open surgical treatment (Group I) exhibited loss of tcMEP after proximal aortic clamping. In nine cases (47%), we observed recovery of tcMEP after intraoperative interventions, while two patients subsequently developed paraplegia and three died. Seventeen of 19 patients showed loss of SSEP, with recovery in 12 cases (63%). During stent-graft implantation (Group II), one of nine patients (11%) demonstrated tcMEP loss with intraoperative, intervention-related recovery. The SSEP-recording course remained stable. CONCLUSIONS: tcMEP/SSEP monitoring has proved to be an excellent means of detecting spinal cord ischaemia during surgery on thoracoabdominal aortic aneurysms. The prognostic value of tcMEP monitoring should be considered superior to that of SSEP measurements, because of its direct and rapid response to spinal malperfusion. Through combined neurophysiological monitoring, vital parameter balancing and intraoperative interventions, spinal cord perfusion improves and recovery of tcMEP and SSEP is achievable, reducing the prevalence of postoperative paraplegia.

Improved spinal cord perfusion during thoracoabdominal aortic repair.

OBJECTIVES: The study's aim is to evaluate whether intraoperative neurophysiological monitoring with transcranial motor-evoked potentials (tcMEP) permits early recognition of neuronal lesions, thus making interventions to prevent irreversible spinal cord damage possible. MATERIAL AND METHODS: TcMEP monitoring was carried out in twelve patients (mean age 60 years) during open surgical thoracoabdominal aortic replacement. Current approaches for corrective, spinal cord-protecting interventions consist of: raising distal perfusion by increasing cardiopulmonary bypass (CPB) flow, catecholamine application, reducing central venous pressure, reimplantation of segmental arteries, and cerebrospinal fluid (CSF) drainage. RESULTS: Nine patients exhibited loss of tcMEP after segmental aorta clamping. In five patients we observed a recovery of tcMEP through counteractive measures. Three patients died intraoperatively, one patient presented with postoperative paraplegia and loss of tcMEP. CONCLUSION: TcMEP loss is associated with spinal cord ischaemia, causing postoperative paraplegia. TcMEP monitoring is an excellent method to detect spinal cord ischaemia at an early stage.

Setup of neurophysiological monitoring with tcMEP/SSEP during thoracoabdominal aneurysm repair.

OBJECTIVES: The article describes a procedure for the intraoperative neurophysiological placement of electrodes to control the spinal cord function during thoracoabdominal aortic aneurysm repair. MATERIAL AND METHODS: Intraoperative monitoring is performed by motor-evoked myogenic potentials after transcranial electric stimulation (tcMEP) and somatosensory-evoked potentials (SSEP). In tcMEP, the stimulating percutaneous needle electrodes are placed at C3 and C4 according to the 10 - 20 system for EEG recordings. TcMEP are recorded from the anterior tibial and gastrocnemius muscles on both sides. The SSEP electrodes are placed laterally and caudally onto the malleolus medialis in order to stimulate the tibial nerve. The stimulus is documented via electrodes attached to the scalp within the sensory cortex region. RESULTS: The application of the electrodes is both easy to learn and can be performed without further difficulties. Once attached, the electrodes provide a quick assessment and interpretation of spinal cord function. The identification of external sources of disturbance during the monitoring (e. g. insufficient impedance, unfavourable electrode positioning, and technical interference caused by medical equipment) enables the supervisor to differentiate between normal and abnormal neurological responses. CONCLUSIONS: TcMEP and SSEP allow an adequate, direct, and reliable intraoperative assessment of spinal cord function, enabling the surgeon to diagnose an impending ischaemia and act accordingly. This measurement technique provides the surgical team with a means of integrating neurological aspects during thoracoabdominal aneurysm repair.

Epithelioid angiosarcoma of the aorta.

Primary malignant tumors of the aorta are extremely rare. Review of the literature indicates that there are nearly 100 recorded cases of primary malignant tumors of the aorta. The purpose of this article is to present an additional case of the primary malignant tumors of the aorta which initially has been misinterpretated as atherosclerosic disease. This aortic tumor was of endothelial origin and immunohistochemical studies classified the tumor as an epithelioid angiosarcoma. The treatment resulted in an abdominal aortic repair. MRI of the spine revealed multifocal metastatic disease of the axial skeleton and a subsequent chemotherapy was performed. The patient died 17 months after the initial diagnosis.