Sickle cell anemia and mitral valve replacement:Case Report

Abstract

An 8-year-old black boy with sickle cell disease and severe hemolytic anemia crisis (95% hemoglobin S) also had mitral incompetence due to rheumatic valve disease. A 27 mm monostrut Björk-Shiley valve prosthesis was implanted after partial exchange transfusions had reduced the hemoglobin S to less than 40%. High-flow normothermic perfusion was used during extracorporeal circulation, with care taken to avoid hypoxia and acidosis. Postoperative recovery was uneventful.

Perioperative care of children with sickle cell disease: A systematic review and clinical recommendations

“…Some authors advocate for preoperative transfusion without giving a threshold, despite evidence from the abovementioned RCTs. Exchange transfusions (manual and automated) for a leve of HbS <30% is recommended for high risk surgery either preoperatively, or on cardiopulmonary bypass . In cardiac pulmonary bypass, some authors suggest a stricter HbS limit of <5% .…”

“…Intraoperative monitoring varies. Per standard anesthetic care, blood pressure, cardiac rhythm and rate and oxygenation are monitored strictly to avoid hypoxia . Some groups perform more invasive systematic monitoring such as the continuous measurement of central venous pressure, arterial blood pressure, regular arterial blood gas exchange and/or urine output determination (by Foley catheterization) .…”

“…Oxygenation has to be strictly controlled and hypoxia needs to be avoided. Acidosis has to be controlled and bicarbonate is used to reverse it . Mild alkalosis induced by bicarbonate therapy has sometimes been advocated for, mostly older articles .…”

“…Complex and high‐risk procedures in the area of cardiac surgery are now seen as feasible and safe for patients with SCD. Cardiopulmonary bypass can be done in normothermic or hypothermic conditions . Cold vs warm cardioplegia have been used without consensus.…”

Cirurgia e anestesia na doença falciforme

“…Nos casos de cirurgia cardíaca em pacientes com DF, em que é necessário bypass cardiopulmonar, a maioria dos autores relata o emprego exsangüíneo-transfusão 28,29 embora a transfusão simples também tenha se mostrado eficaz. 30 A indução de hipotermia permanece controversa nos casos de pacientes com DF submetidos a bypass cardiopulmonar, 30 sendo que não há uma contra-indicação absoluta para sua realização.…”