@article{VeldhoenBehzadiLenzetal.2017,
  author    = {Veldhoen, Simon and Behzadi, Cyrus and Lenz, Alexander and Henes, Frank Oliver and Rybczynski, Meike and von Kodolitsch, Yskert and Bley, Thorsten Alexander and Adam, Gerhard and Bannas, Peter},
  title     = {Non-contrast MR angiography at 1.5 Tesla for aortic monitoring in Marfan patients after aortic root surgery},
  series = {Journal of Cardiovascular Magnetic Resonance},
  volume    = {19},
  journal   = {Journal of Cardiovascular Magnetic Resonance},
  number    = {82},
  doi       = {10.1186/s12968-017-0394-y},
  url       = {http://nbn-resolving.de/urn:nbn:de:bvb:20-opus-158693},
  year      = {2017},
  abstract  = {Background: Contrast-enhanced cardiovascular magnetic resonance angiography (CE-CMRA) is the established imaging modality for patients with Marfan syndrome requiring life-long annual aortic imaging before and after aortic root replacement. Contrast-free CMRA techniques avoiding side-effects of contrast media are highly desirable for serial imaging but have not been evaluated in the postoperative setup of Marfan patients. The purpose of this study was to assess the feasibility of non-contrast balanced steady-state free precession (bSSFP) magnetic resonance imaging for aortic monitoring of postoperative patients with Marfan syndrome. Methods: Sixty-four adult Marfan patients after aortic root replacement were prospectively included. Fourteen patients (22\%) had a residual aortic dissection after surgical treatment of type A dissection. bSSFP imaging and CE-CMRA were performed at 1.5 Tesla. Two radiologists evaluated the images regarding image quality (1 = poor, 4 = excellent), artifacts (1 = severe, 4 = none) and aortic pathologies. Readers measured the aortic diameters at defined levels in both techniques. Statistics included observer agreement for image scoring and diameter measurements and ROC analyses for comparison of the diagnostic performance of bSSFP and CE-CMRA. Results: Both readers observed no significant differences in image quality between bSSFP and CE-CMRA and found a median image quality score of 4 for both techniques (all p > .05). No significant differences were found regarding the frequency of image artifacts in both sequences (all p > .05). Sensitivity and specificity for detection of aortic dissections was 100\% for both readers and techniques. Compared to bSSFP imaging, CE-CMRA resulted in higher diameters (mean bias, 0.9 mm; p < .05). The inter-observer biases of diameter measurements were not significantly different (all p > .05), except for the distal graft anastomosis (p = .001). Using both techniques, the readers correctly identified a graft suture dehiscence with aneurysm formation requiring surgery. Conclusion: Unenhanced bSSFP CMR imaging allows for riskless aortic monitoring with high diagnostic accuracy in Marfan patients after aortic root surgery.},
  language  = {en}
}
@article{BuschHoffjanBergmannetal.2016,
  author    = {Busch, Albert and Hoffjan, Sabine and Bergmann, Frauke and Hartung, Birgit and Jung, Helena and Hanel, Daniela and Tzschach, Andeas and Kadar, Janos and von Kodolitsch, Yskert and Germer, Christoph-Thomas and Trobisch, Heiner and Strasser, Erwin and Wildenauer, Ren{\´e}},
  title     = {Vascular type Ehlers-Danlos syndrome is associated with platelet dysfunction and low vitamin D serum concentration},
  series = {Orphanet Journal of Rare Diseases},
  volume    = {11},
  journal   = {Orphanet Journal of Rare Diseases},
  number    = {111},
  doi       = {10.1186/s13023-016-0491-2},
  url       = {http://nbn-resolving.de/urn:nbn:de:bvb:20-opus-147757},
  year      = {2016},
  abstract  = {Background The vascular type represents a very rare, yet the clinically most fatal entity of Ehlers-Danlos syndrome (EDS). Patients are often admitted due to arterial bleedings and the friable tissue and the altered coagulation contribute to the challenge in treatment strategies. Until now there is little information about clotting characteristics that might influence hemostasis decisively and eventually worsen emergency situations. Results 22 vascular type EDS patients were studied for hemoglobin, platelet volume and count, Quick and activated partial thromboplastin time, fibrinogen, factor XIII, von Willebrand disease, vitamin D and platelet aggregation by modern standard laboratory methods. Results show a high prevalence of over 50 \% for platelet aggregation disorders in vascular type EDS patients, especially for collagen and epinephrine induced tests, whereas the plasmatic cascade did not show any alterations. Additionally, more than half of the tested subjects showed low vitamin D serum levels, which might additionally affect vascular wall integrity. Conclusion The presented data underline the importance of detailed laboratory screening methods in vascular type EDS patients in order to allow for targeted application of platelet-interacting substances that might be of decisive benefit in the emergency setting.},
  language  = {en}
}