Journal of cardiac surgery
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From 1985 to 1996, 18 patients with Marfan's syndrome underwent the Bentall procedure at Mount Sinai Hospital. They are compared with 38 patients aged < 40 without Marfan's syndrome who also underwent composite valve-graft replacement of the ascending aorta. The mean age of the non-Marfan group was 33 while that of the Marfan group was 29. ⋯ No difference in immediate operative mortality following the Bentall procedure was noted between patients with and without Marfan's syndrome, but young patients without Marfan's syndrome seem to have better event-free and long-term survival. In patients with Marfan's syndrome, the presence of acute dissection makes reoperation more likely, and sudden death from rupture still occurs despite careful postoperative surveillance. A higher incidence of severe mitral valve disease was found among young patients with Marfan syndrome than in controls.
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Until recently the surgical treatment of aneurysms of the aortic root in patients with the Marfan syndrome consisted of composite replacement of the aortic valve and ascending aorta. At the present, almost one-half of these patients can have reconstruction of the aortic root with preservation of the aortic valve. The mitral valve can also be frequently preserved. ⋯ Although the number of patients in each group is small and the follow-up relatively short, aortic valve-sparing operations have given gratifying results and may prove superior to valve replacement in patients with the Marfan syndrome.
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Hemostasis is a significant problem in aortic surgery requiring profoundly hypothermic techniques. Aprotinin, a serine protease inhibitor, reduces blood loss in high-risk coronary and valve surgery, but its use in profound hypothermia is controversial. ⋯ This data does not support the adverse effect of aprotinin upon early survival. Although early reports were of concern, the role of aprotinin as an adjunct to hemostasis requires further investigation.
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Hemostasis abnormalities in cardiovascular and aortic surgery remain a major source of morbidity and mortality in patients undergoing such complex procedures. The need for frequent transfusions of red cell and other blood products increases risks and costs to patients and institutions providing patient care. Specifically in cardiovascular and aortic surgery, the nature of the surgery is, at best, semi-elective, and careful preparation to preserve the hemostatic mechanisms of the body is essential. ⋯ Aorto intimal disease initiates fibrinolysis by the release of tissue plasminogen activator. Due to the numerous etiologies of bleeding, a combination of blood conservation strategies is suggested. The ideal combination of interventions has yet to be determined and is currently dependent on patient variables, physician and institutional practices, and economic pressures.
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The problem of altered hemostasis remains a major challenge during thoracic aortic surgery. Bleeding is associated with a marked increase in morbidity and mortality. The hemostatic derangements are caused by multiple interrelated factors including interference with the vascular integrity, extensive surgical dissection, transient need for complete inhibition of the normal coagulation process, large blood products and fluid requirements, hemodilution, hypothermia, extensive ischemia and reperfusion, activation of systemic inflammatory responses, interference with fibrinolysis, and the use of extracorporeal circulation systems. ⋯ Rewarming may produce clear procoagulant effects by improving the efficacy of platelets and clotting factors. Platelet dysfunction can be reduced by several pharmacological interventions including acid aminocaproic, desmopressin and aprotinin; however, efficacy and safety are still being established. The most important factorS regarding safety in thoracic aortic surgery are a secure suture line and the experience of the surgical and anesthesiology teams.