Gadofosveset-Trinatrium-Enhanced MR Angiography and MR Venography in the Diagnosis of Venous Thromboembolic Disease: A Single-Center Cohort Study.

BACKGROUND: The aim of this single-center combined prospective/retrospective cohort study was to analyze Gadolinium (Gd)-enhanced MRA (magnetic resonance angiography) and MRV (MR venography) for the diagnosis of pulmonary artery embolism and deep venous thrombosis. The gold standard methods result in major exposure to radiation and a high amount of nephrotoxic iodinated contrast media. This is the first larger contrast-enhanced MR imaging study of acute and chronic venous thromboembolic disease of various stages. METHODS: We prospectively examined 88 patients presenting clinical signs of deep vein thrombosis and/or pulmonary artery embolism. A single-session, one-stop shop Gd-enhanced MRA/MRV at 1.5 Tesla, using gradient echo sequences with very short repetition and echo times as well as low flip angles with subtraction and three-dimensional reconstruction, was performed. A diagnosis was made with the consensus of two experienced radiologists. RESULTS: We observed excellent MRA image quality in 87% and even higher diagnostic image quality of MRV in 90% of our examinations. Pulmonary artery embolism occurred with deep vein thrombosis in 22%. CONCLUSIONS: Gd-enhanced MRA/MRV provided excellent image quality for the diagnosis of venous thromboembolic disease in the majority of cases. It may be particularly useful to plan and follow-up filter implantation and retrieval in the inferior caval vein.

Cardiogenic shock and tumor resection due to cardiac synovial sarcoma: a case report.

BACKGROUND: Cardiac synovial sarcoma of the heart is a rare, aggressive mesenchymal tumor with poor prognosis, since complete resection is seldom feasible. CASE PRESENTATION: A 23-year-old man was referred in cardiogenic shock. Emergency computed tomography (CT) revealed a large tumor with obstruction of the right atrium (RA) and prolapse into the right ventricle (RV). Resection and pericardial patch plasty were performed. Histology confirmed a G-3 spindle-cell sarcoma. At 21 months postoperatively, CT and cardiac magnetic resonance (MR) angiography showed a tumor emerging from the lateral wall of the superior caval vein (SCV) and the RA. The RA and SCV were completely resected and replaced with a tailored Dacron tunnel prosthesis. Histology confirmed R0 resection of a G-3 spindle-cell sarcoma. Reverse transcription-polymerase chain reaction (RT-PCR) confirmed a monophasic fibrous synovial sarcoma. Echocardiography upon discharge showed normal biventricular function. The heart was tumor-free upon PET-CT 24 months thereafter. A sudden progression with innumerable pulmonary nodules caused only minimal exertional dyspnea, and the patient received palliative monochemotherapy with ifosfamide. Thirty months after the first operation, he succumbed to hemorrhage from a brain metastasis. CONCLUSIONS: We report an unusually long postoperative period of 30 months in our patient after resection of a very large right atrial sarcoma. Early diagnosis, aggressive surgical treatment, adjunctive chemotherapy and radiotherapy affect survival. Systematic inclusion of patients in multicenter initiatives, including biobanking, is necessary. Better knowledge of genetic defects relevant to these cardiac tumors will promote accurate diagnoses and suggest novel and personalized gene-based therapies.

[Popliteal Artery Entrapment Syndrome: Three Unusual Cases with Different Courses to Diagnosis]. / Poplitealarterien-Entrapment-Syndrom: 3 untypische Fälle mit 3 verschiedenen Diagnosewegen.

INTRODUCTION: Popliteal artery entrapment syndrome (PAES) is a pathology characterised by anatomical anomalies of the relative position of the popliteal artery (PA) to the medial head of the gastrocnemius muscle (MHGM), resulting in mechanical compression causing damage to and occlusion of the artery. PATIENTS: From 2012 to 2018, we operated on 3 male patients aged 17 to 48 years, who presented with PAES in our department. The first and oldest patient had previously undergone femoro-popliteal bypass surgery in 2003 when aged 23, with the underlying diagnosis of "posttraumatic PAOD II b" followed by several reoperations. In 2012, during the preparation of the popliteal fossa for the interposition of the aneurysmatic bypass vein a strong tendinous strand of the MHGM was found intraoperatively to be constricting the vessels. This aberrant part of the MHGM was resected and the vein replaced. The second patient was referred to us in 2014 with the diagnosis "PAOD II b with thrombosed popliteal aneurysm" after having undergone intra-arterial lysis at age 33. Due to the remaining wall adherent thrombi and position of the aneurysm, interposition of the PA with a venous graft was planned. Intraoperatively a tendon was found proximal to the aneurysm, causing significant stenosis of the PA. This structure was resected and the interposition performed as planned. The third patient was a 17 year old adolescent, who presented with plantar and calf claudication in his right leg with paleness and pulselessness. Imaging showed occlusion of the popliteal artery in both legs. After initial intra-arterial lysis, HRMRI of the knee showed the atypical course of the PA undercrossing the MHGM from the medial side. Open surgical treatment was performed by myotomy, leading to refixation of the tendon of the MHGM in its physiological position. All patients received individual medical treatment for prophylaxis of rethrombosis. All three patients declined surgical treatment of the asymptomatic contralaterally diagnosed PAES. CONCLUSION: Popliteal artery entrapment syndrome is rare and usually associated with athletic, largely young male patients with pronounced calf muscles. Delayed diagnosis of PAES leads to severe vascular defects, unnecessary reintervention and prolonged illness as is shown in our first case. Therefore, calf and foot claudication in young patients should always bring to mind the differential diagnosis of popliteal entrapment syndrome.

Gadobutrol in Renally Impaired Patients: Results of the GRIP Study.

OBJECTIVE: The aim of this study was to assess the potential risk of gadobutrol-enhanced magnetic resonance imaging (MRI) in patients with moderate to severe renal impairment for the development of nephrogenic systemic fibrosis (NSF). MATERIALS AND METHODS: We performed a prospective, international, multicenter, open-label study in 55 centers. Patients with moderate to severe renal impairment scheduled for any gadobutrol-enhanced MRI were included. All patients received a single intravenous bolus injection of gadobutrol at a dose of 0.1 mmol/kg body weight. The primary target variable was the number of patients who develop NSF within a 2-year follow-up period. RESULTS: A total of 908 patients were enrolled, including 586 with moderate and 284 with severe renal impairment who are at highest risk for developing NSF. The mean time since renal disease diagnosis was 1.83 and 5.49 years in the moderate and severe renal impairment cohort, respectively. Overall, 184 patients (20.3%) underwent further contrast-enhanced MRI with other gadolinium-based contrast agents within the 2-year follow-up. No patient developed symptoms conclusive of NSF. CONCLUSIONS: No safety concerns with gadobutrol in patients with moderate to severe renal impairment were identified. There were no NSF cases.

Gadobenate dimeglumine and gadofosveset trisodium for MR angiography of the renal arteries: Multicenter intraindividual crossover comparison.

OBJECTIVE: This prospective multicenter intraindividual crossover study was designed to compare gadobenate dimeglumine and gadofosveset trisodium at approved doses with respect to the image quality and diagnostic performance of contrast-enhanced MR angiography (CE-MRA) in the detection of clinically relevant renal artery stenosis. SUBJECTS AND METHODS: Thirty-nine subjects (17 men, 22 women; age range, 30-86 years; mean 62 +/- 13.3 [SD] years) with known or suspected renovascular disease underwent a first CE-MRA examination with 0.1 mmol/kg gadobenate dimeglumine and a second examination 3-12 days later with 0.03 mmol/kg gadofosveset. Identical T1-weighted spoiled gradient-refocused echo coronal first-pass images were acquired for 38 of the 39 patients. For 15 of the 38 patients, additional sagittal or axial images or both were acquired with gadofosveset during the steady-state phase. Thirty-four patients underwent digital subtraction angiography, which was the reference standard. Three independent blinded readers assessed source images and maximum-intensity-projection reconstructions to detect clinically relevant (> 50%) renal artery stenosis. Diagnostic performance (sensitivity, specificity, accuracy, positive and negative predictive values) was evaluated with the McNemar and Wald tests. Matched-pair determinations of diagnostic preference were evaluated with Wilcoxon's signed rank test. Reader agreement was determined with kappa analysis, and safety was assessed. RESULTS: Comparison of first-pass images revealed superior sensitivity (75.7-86.5% vs 68.4-76.3%), specificity (92.1-98.6% vs 90.5-93.9%), accuracy (88.9-96.2% vs 85.9-90.3%), positive predictive value (70.0-94.1% vs 65.0-76.3%), and negative predictive value (94.0-96.6% vs 91.7-93.9%) with gadobenate dimeglumine. Significant superiority was noted for specificity (p < or = 0.02), accuracy (p < or = 0.005), and positive predictive value (p < or = 0.018). Steady-state images showed no benefit of gadofosveset. Reader agreement was good to excellent (gadobenate dimeglumine, kappa = 0.855; gadofosveset, kappa = 0.776). Reader preference was for gadobenate dimeglumine in 11, 17, and 13 patients and for gadofosveset in five, four, and five patients. No safety concerns were noted. CONCLUSION: Better diagnostic performance and reader preference were found for gadobenate dimeglumine than gadofosveset in first-pass renal CE-MRA.

Efficacy and safety of gadodiamide (Gd-DTPA-BMA) in renal 3D-magnetic resonance angiography (MRA): a phase II study.

PURPOSE: To determine the most efficacious dose of gadodiamide for three-dimensional (3D) contrast-enhanced (CE) magnetic resonance angiography (MRA) of the renal arteries on a patient level based on the sensitivity in detecting the main hemodynamically relevant (> or =50% or occlusion) renal artery stenosis (RAS) using intra-arterial digital subtraction angiography (IA DSA) as the gold standard. MATERIALS AND METHODS: This prospective, randomized, double-blind, parallel-group, multicenter study included 273 patients referred to IA DSA for suspected RAS. Patients underwent 3D CE MRA after injection of 0.01, 0.05, 0.1, or 0.2mmol/kg of body weight gadodiamide (0.5mmol/ml). The images were assessed for location and degree of RAS by independent blinded readers (MRA: three readers, IA DSA: one reader). Hypothesis testing for a significant trend in sensitivity across dose groups was based on the one-sided Cochran-Armitage style trend test for each independent MRA reader. RESULTS: The lowest dose group (0.01mmol/kg) proved non-efficacious in detecting hemodynamically relevant (i.e., > or =50% or occlusion) RAS. A statistically significant dose trend (p<0.001) was shown for each of the three independent readers. Depending on reader, the sensitivity obtained with 0.05, 0.1, and 0.2mmol/kg was 63.9-86.1%, 75.8-91.4% and 80.6-90.6%, the specificity was 66.7-73.9%, 59.3-75.0%, and 59.3-75.0% and accuracy was 67.8-78.9%, 75.4-77.4%, and 76.3-81.0%, for the three dose groups, respectively. There were eight non-severe adverse events (AEs). Three serious AEs occurring in one patient were judged not related to gadodiamide by the on-site investigator. CONCLUSION: A significant dose trend between the four doses examined was observed. The lowest dose (0.01mmol/kg) differed significantly from those of the other three doses. Based on the analysis of the primary and secondary endpoints, 0.1mmol/kg gadodiamide appears to be the most suitable dose in diagnosing hemodynamically relevant RAS. The present study also demonstrated gadodiamide to be safe and well tolerated.

Clinical, radiological, and pathological findings in four children with gastrointestinal stromal tumors of the stomach.

The incidence of gastrointestinal stromal tumors (GISTs) in children is exceptionally low. However, during the last decade these tumors attracted increasing attention, because they were found to express the cell surface transmembrane receptor kit (CD117) that has tyrosine kinase activity. This tyrosine kinase can be semi-selectively inhibited by signal transduction inhibitors such as imatinib mesylate (Glivec), which is a competitive inhibitor of c-kit, c-abl, platelet-derived growth factor receptor-alpha (PDGFR-alpha) and PDGFR-beta, and abl-related gene (arg). The authors present the clinical, radiographic, and pathological findings of 4 children who were diagnosed with gastric GIST. One of them had an incomplete Carney triad including GIST and mediastinal paraganglioma. All 4 patients presented with anemia and anemia-related symptoms and underwent total resection of the tumor. One patient received additional chemotherapy (in the pre-imatinib era) and 2 patients received a short course of imatinib mesylate. With a follow-up of 116, 55, 23, and 10 months all patients are alive in first complete continuous remission. In children and adolescents, particularly in female patients, GISTs should be included in the differential diagnosis of anemia secondary to gastrointestinal hemorrhage. Complete surgical resection is the mainstay of treatment for this tumor, with imatinib mesylate restricted to patients with advanced or metastatic tumors. Since late recurrences (up to 30 years following initial diagnosis) are reported, a life-long follow-up is mandatory in these patients.

Three-dimensional gadolinium-enhanced magnetic resonance angiography used as a "one-stop shop'' imaging procedure for venous thromboembolism: a pilot study.

Pulmonary embolism and deep venous thrombosis are individual manifestations of a single entity, venous thromboembolic disease. This study aimed to assess the feasibility of 3-dimensional gadolinium-enhanced magnetic resonance angiography used as an "one-stop shop'' imaging procedure visualizing both the pulmonary arteries and the deep lower venous system within a single investigation. The inclusion criterion was a proven or excluded venous thromboembolism. Diagnosis was based on an imaging work-up for pulmonary embolism including either perfusion lung scan or contrast-enhanced spiral computed tomography, or both, and an imaging work-up for deep venous thrombosis including either venous color-coded duplex sonography or ascending phlebography, or both. A gadolinium-enhanced "one-stop shop'' magnetic resonance angiography was performed within 24 hours of completed diagnostic imaging work-up for pulmonary embolism and deep venous thrombosis in 20 patients. Results of pulmonary magnetic resonance angiography were concordant with perfusion lung scan and/or computed tomography in 90% of patients. Magnetic resonance angiography results of the deep lower venous system were concordant with venous duplex sonography and/or phlebography in 75% of patients and seemed to be more precise in 25% of patients. The "one-stop shop'' imaging procedure using gadolinium-enhanced magnetic resonance angiography was feasible and proved to offer a reliable and rapid diagnostic approach in thromboembolic disease, sparing patients' exposure to ionizing radiation and iodinated contrast media.

Congenital familial vascular anomalies: a study of patients with an anomalous inferior vena cava, and of their first-degree relatives.

With magnetic resonance angiography and computed tomography, congenital anomalies of the inferior vena cava are diagnosed more frequently than they used to be. Accessory renal arteries identified by magnetic resonance angiography in a patient with an anomalous inferior vena cava indicated a combination of arterial and venous abnormalities. The study was initiated to screen consecutive patients with an anomalous inferior vena cava for concomitant abdominal and pelvic arterial abnormalities, and their first-degree relatives for congenital vascular anomalies. Magnetic resonance angiography identified in 2 of 5 patients with an anomalous inferior vena cava concomitant accessory renal arteries and in 5 of 11 first-degree relatives major abdominal vascular anomalies including accessory renal arteries, accessory renal veins, and anomalies of the hepatic artery. None of the relatives showed abnormalities of the inferior vena cava. The familial occurrence of vascular anomalies strongly suggests an underlying pathogenetic component in affected family members. In patients with a congenital anomaly of the inferior vena cava, concomitant arterial abnormalities should be considered. First-degree relatives may be at risk for congenital vascular anomalies.

Value of a blood pool contrast agent in MR venography of the lower extremities and pelvis: preliminary results in 12 patients.

The purpose of this study was to determine the value of a blood-pool contrast media (NC100150, Nycomed Imaging (now Amersham Health) Oslo, Norway) for evaluation of venous thrombosis of the deep veins of the pelvis and lower extremities. Twelve patients were prospectively evaluated with conventional X-ray venography (XRV) and MR venography (MRV) after injection of NC100150 (2 ml/kg body weight). The source images and 3D maximum intensity projection (MIP) were viewed on an independent workstation. Diagnosis was made in consensus from two radiologists. Diagnostic image quality was achieved in 87 veins with XRV and MRV. As determined by XRV, thrombus was present in 30 out of 87 veins (34.5%). There was agreement concerning absence or presence of thrombi in 83 out of 87 veins (95.4%; kappa = 0.9 +/- 0.05). Compared to XRV, overall sensitivity and specificity of blood-pool MRV were 93.3% and 96.5%, respectively. Two venous thromboses of the popliteal and posterior tibial vein were diagnosed in MRV, but not in XRV. Conversely, two venous thromboses below the knee had been missed by MRV. NC100150 allows prolonged and improved visualization of the peripheral vasculature and may overcome some limitations of gadolinium contrast media. A more complete examination of the proximal venous tree may be possible than with conventional XRV. Arterial and venous enhancement and motion artifacts can limit image interpretation.

Gadobutrol-enhanced moving-table magnetic resonance angiography in patients with peripheral vascular disease: a prospective, multi-centre blinded comparison with digital subtraction angiography.

The purpose of this study was to compare moving-table three-dimensional contrast-enhanced magnetic resonance angiography (CE MRA), using 1.0-mol gadobutrol, with intra-arterial digital subtraction angiography (i.a. DSA) for evaluation of pelvic and peripheral arteries in patients with peripheral arterial occlusive disease. A total of 203 patients were examined in a prospective, multi-centre study at 1.0/1.5 T. Ten vessel segments of one leg were evaluated on-site and by three independent blinded reviewers off-site. One hundred eighty-two patients were evaluable in blinded reading. For pelvis and thigh, there was statistically significant diagnostic agreement between CE MRA and i.a. DSA on-site (94%) and off-site (86-88%). Overall, for detection of clinically significant stenoses, 93% sensitivity and 90% specificity were achieved in on-site evaluation, with 71-76 and 87-93% off-site; for detection of occlusion, sensitivity and specificity on-site were 91 and 97%, with 75-82 and 94-98% off-site. Evaluation was more sensitive on-site than off-site for detection of stenoses and occlusion, whereas specificity was similar. The CE MRA with 1.0-mol gadobutrol gave results comparable to those of i.a. DSA for the larger arteries of pelvis and thigh. Results for calf arteries were compromised by spatial resolution and technical limitations.

Anomalies of the inferior vena cava in patients with iliac venous thrombosis.

BACKGROUND: Cases of deep venous thrombosis in the lower extremities triggered by abnormalities of the vena cava have been reported. OBJECTIVE: To describe anomalies of the inferior vena cava in patients with deep venous thrombosis. DESIGN: Prospective, consecutive case series. SETTING: University Hospital, Graz, Austria. PATIENTS: 97 patients with deep venous thrombosis. INTERVENTION: Sonography, venography, or both to diagnose deep venous thrombosis; magnetic resonance angiography to image the inferior vena cava. MEASUREMENTS: Anomalies of the inferior vena cava imaged by magnetic resonance angiography. RESULTS: 31 of 97 patients showed thrombotic occlusion of iliac veins (common and external iliac vein [ n = 29] or external iliac vein [ n = 2]). Five of 31 patients (3 men, 2 women) had an anomaly of the inferior vena cava. Anomalies were missing inferior vena cava, hypoplastic hepatic segment, and missing renal or postrenal segments. Patients with anomalies were significantly younger than the 92 patients without (mean age+/-SD, 25+/-6 years vs. 53+/-19 years; P = 0.002). In 2 patients with anomalies, the thrombotic occlusion was recurrent. CONCLUSIONS: An anomaly of the inferior vena cava should be suspected if thrombosis involving the iliac veins is seen in patients 30 years of age or younger. Patients with both an anomaly and thrombosis may be at higher risk for thrombotic recurrence.