Bicuspid aortic valve disease is the most common congenital cardiac disorder, being present in 1% to 2% of the general population. Associated aortopathy is a common finding in patients with bicuspid aortic valve disease, with thoracic aortic dilation noted in approximately 40% of patients in referral centers. Several previous consensus statements and guidelines have addressed the management of bicuspid aortic valve-associated aortopathy, but none focused entirely on this disease process. The current guidelines cover all major aspects of bicuspid aortic valve aortopathy, including natural history, phenotypic expression, histology and molecular pathomechanisms, imaging, indications for surgery, surveillance, and follow-up, and recommendations for future research. It is intended to provide clinicians with a current and comprehensive review of bicuspid aortic valve aortopathy and to guide the daily management of these complex patients.

Although there is adequate evidence that bicuspid aortic valve (BAV) is an inheritable disorder, there is a great controversy regarding the pathogenesis of dilatation of the proximal aorta. The hemodynamic theory was the first explanation for BAV aortopathy. The genetic theory, however, has become increasingly popular over the last decade and can now be viewed as the clearly dominant one. The widespread belief that BAV disease is a congenital disorder of vascular connective tissue has led to more aggressive treatment recommendations of the proximal aorta in such patients, approaching aortic management recommendations for patients with Marfan syndrome. There is emerging evidence that the 'clinically normal' BAV is associated with abnormal flow patterns and asymmetrically increased wall stress in the proximal aorta. Recent in vitro and in vivo studies on BAV function provide a unique hemodynamic insight into the different phenotypes of BAV disease and asymmetry of corresponding aortopathy even in the presence of a 'clinically normal' BAV. On the other hand, there is a subgroup of young male patients with BAV and a root dilatation phenotype, who may present the predominantly genetic form of BAV disease. In the face of these important findings, we feel that a critical review of this clinical problem is timely and appropriate, as the prevailing BAV-aortopathy theory undoubtedly affects the surgical approach to this common clinical entity. Thorough analysis of the recent literature shows a growing amount of evidence supporting the hemodynamic theory of aortopathy in patients with BAV disease. Data from recent studies requires a reevaluation of our overwhelming support of the genetic theory, and obliges us to acknowledge that hemodynamics plays an important role in the development of this disease process. Given the marked heterogeneity of BAV disease, further studies are required in order to more precisely determine which theory is the 'correct' one for explaining the obviously different types of BAV-associated aortopathy.

Preoperative malperfusion is a significant risk factor influencing perioperative and long-term survival after surgery for acute type A dissection. Percutaneous interventional procedures and delayed surgery should be considered in patients with clinically apparent mesenteric malperfusion because of the dismal prognosis of immediate surgical therapy.

Objective: Aortic surgical procedures requiring hypothermic circulatory arrest are associated with altered hemostasis and increased bleeding. In a randomized clinical trial, we evaluated effects of thromboelastometrically guided algorithm on transfusion requirements. Methods: Fifty-six consecutive patients (25 with acute type A dissection) undergoing aortic surgery with hypothermic circulatory arrest were enrolled in a randomized trial during a 6-month period. Patients were randomly allocated to treatment group (n ¼ 27) with thromboelastometrically guided transfusion algorithm or control group (n ¼ 29) with routine transfusion practices (clinical judgment-guided transfusion followed by transfusion according to coagulation test results). Primary end point was cumulative allogeneic blood units (red blood cells, fresh-frozen plasma, and platelets) transfused. Results: Transfusion of allogeneic blood was significantly reduced in the thromboelastometry group: median 9.0 units (interquartile range, 2.0-30.0 units) versus. 16.0 units (9.0-23.0 units, P ¼ .02). Most significant decrease was in the use of fresh-frozen plasma (3.0 units, 0-12.0 units, vs 8.0 units, 4.0-18.0 units, P ¼ .005). Postoperative blood loss (890 mL/d, 600-1250 mL/d vs 950 mL/d, 650-1400 mL/d, p ¼ 0.5) and rate of surgical re-exploration (19% vs 24%, P ¼ .7) were similar between groups. Thromboelastometrically guided algorithm significantly decreased need for massive perioperative transfusion (odds ratio, 0.45; 95% confidence interval, 0.2-0.9; P ¼ .03) in multivariable logistic regression analysis. Conclusions: Thromboelastometrically guided transfusion is associated with a decreased use of allogeneic blood units and reduced incidence of massive transfusion in patients undergoing aortic surgery with circulatory arrest.

The Hamburg City Health Study (HCHS) is a large, prospective, long-term, population-based cohort study and a unique research platform and network to obtain substantial knowledge about several important risk and prognostic factors in major chronic diseases. A random sample of 45,000 participants between 45 and 74 years of age from the general population of Hamburg, Germany, are taking part in an extensive baseline assessment at one dedicated study center. Participants undergo 13 validated and 5 novel examinations primarily targeting major organ system function and structures including extensive imaging examinations. The protocol includes validate self-reports via questionnaires regarding lifestyle and environmental conditions, dietary habits, physical condition and activity, sexual dysfunction, professional life, psychosocial context and burden, quality of life, digital media use, occupational, medical and family history as well as healthcare utilization. The assessment is completed by genomic and proteomic characterization. Beyond the identification of classical risk factors for major chronic diseases and survivorship, the core intention is to gather valid prevalence and incidence, and to develop complex models predicting health outcomes based on a multitude of examination data, imaging, biomarker, psychosocial and behavioral assessments. Participants at risk for coronary artery disease, atrial fibrillation, heart failure, stroke and dementia are invited for a visit to conduct an additional MRI examination of either heart or brain. Endpoint assessment of the overall sample will be completed through repeated follow-up examinations and surveys as well as related individual routine data from involved health and pension insurances. The study is targeting the complex relationship between biologic and psychosocial risk and resilience factors, chronic disease, health care use, survivorship and health as well as favorable and bad prognosis within a unique, large-scale long-term assessment with the perspective of further examinations after 6 years in a representative European metropolitan population.