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Inferior vena cava atresia is a rare and usually asymptomatic condition. However, when these patients undergo cardiac surgery, it can present an unexpected and challenging situation for the surgeon. Specifically, adequate venous drainage during cardiopulmonary bypass (CPB) is a critical issue here and may require an extension of cannulation strategies. Adequate preoperative diagnostics, ideally with imaging modalities such as CT angiography or MRI, are required for optimal surgical planning. Here, we describe a rare case of thoracic ascending aortic aneurysm with concomitant inferior vena cava atresia that was successfully operated on. With adequate preoperative planning, we were able to perform an operation without unforeseen complications with standard initialization of CPB.

Background: We aimed to test the hypothesis that there is an association between hypertrophic cardiomyopathy and dilated aorta in a case-control, matched-design fashion. Methods: Of 65,843 studies done from November 2011 to December 2015, we found, after detailed evaluation by a single author, 153 cases of hypertrophic cardiomyopathy and 3,213 controls who were classified as normal clinically and echocardiographically. Controls were defined as normal patients referred to the echocardiography laboratory with no diagnoses and no known risk factors for dilated aorta (e.g., aortic stenosis, hypertension, aortic regurgitation). Clinical chart review showed none of the risk factors for dilated aorta, and echocardiography did not reveal any abnormalities. Of these 3,213 patients, 153 controls were matched to cases by age and sex by propensity score. Dilated aorta was defined according to clinical, Goldstein, and Lang's criteria. Results: The prevalence of a dilated sinus of Valsalva was 9 times higher in hypertrophic cardiomyopathy patients than controls (OR = 9.4, P = 0.003). The 9-fold higher prevalence in hypertrophic cardiomyopathy patients persisted after adjusting for height, weight, and aortic pathology. Association of dilated mid-ascending aorta with hypertrophic cardiomyopathy was significant after adjustment for height and body surface area but became borderline insignificant after adjusting for weight and aortic valve pathology. Conclusion: Hypertrophic cardiomyopathy appears to be associated with a dilated sinus of Valsalva, even after adjusting for height, weight, and aortic valve pathology.

BackgroundBicuspid aortic valve (BAV) is associated with ascending aorta aneurysms and dissections. Presently, genetic factors and pathological flow patterns are considered responsible for aneurysm formation in BAV while the exact role of inflammatory processes remains unknown.MethodsIn order to objectify inflammation, we employ a highly sensitive, quantitative immunohistochemistry approach. Whole slides of dissected, dilated and non-dilated ascending aortas from BAV patients were quantitatively analyzed.ResultsDilated aortas show a 4-fold increase of lymphocytes and a 25-fold increase in B lymphocytes in the adventitia compared to non-dilated aortas. Tertiary lymphoid structures with B cell follicles and helper T cell expansion were identified in dilated and dissected aortas. Dilated aortas were associated with an increase in M1-like macrophages in the aorta media, in contrast the number of M2-like macrophages did not change significantly.ConclusionThis study finds unexpected large numbers of immune cells in dilating aortas of BAV patients. These findings raise the question whether immune cells in BAV aortopathy are innocent bystanders or contribute to the deterioration of the aortic wall.

Aim. To evaluate the impact of genetics in development of thoracic aneurysm in patients with tricuspid valve (TAV) and bicuspid aortic valve (BAV) based on the analysis and search for mutations in NOTCH1.Material and methods. In the study, 60 patients included with the dilation of thoracic aorta more than 40 mm and 200 patients with no aortic pathology, included in the comparison group. All patients underwent echocardiographic assessment on Vivid 7 (GE, USA) equipment, by standard protocol. For molecular genetics we utilized the strategy of targeted mutation screening, including the analysis of 10 of 34 exones of the gene NOTCH1, performed with the direct sequencing.Results. Patients with BAV were younger than those with no inborn defect. Arterial hypertension was verified only in every second BAV patient. Also, maximal rates of blood pressure were significantly lower in patients with inborn defects (р

Introduction. Hybrid procedures represent staged or simultaneous endovascular and open surgical techniques in the treatment of complex pathologies of the thoracic and abdominal aorta. We are presenting a patient with previous abdominal aortic surgery in whom hybrid vascular procedure for descending aorta and left subclavian artery aneurysm was performed. Case Outline. A 63-year-old female patient was admitted for computed tomography angiography. Descending aorta aneurysm (7.6 cm) as well as aneurysm of the left subclavian artery (LSA) was noted. Eight years ago she underwent abdominal aortic aneurysm resection and aortoiliac bypass. Standard TEVAR (thoracic endovascular aortic repair) procedure couldn’t be done due to small dimensions of previous “Y” graft (12.6 mm), so first we did LSA transposition and after three days hybrid procedure. After “Y” graft exposure, anastomosis between the corps of “Y” graft and tubular graft 10 mm was created and through this conduit thoracic stent-graft was placed followed by complete “Y” graft replacement. After 6 months angiography showed regular postoperative findings. Conclusion. Combined surgical and endovascular procedures in thoracic aorta pathology treatment could be useful solutions with favorable outcome. [Projekat Ministarstva nauke Republike Srbije, br. 41002]