Aortic Valve and Root Replacement for an adolescent with Sickle Cell
Disease, Hodgkin's Lymphoma and History of Cerebrovascular Accident.
Background: Sickle cell anemia is an autosomal recessive inherited
disorder that affects approximately 5% of the world population. These
patients are at greater risk for developing Hodgkin’s lymphoma.
Cardiopulmonary bypass can trigger lethal vaso-occlusive crises in those
patients if they are subjected to hypoxia, hypothermia, acidosis, or
low-ﬂow states. Case presentation: This case report describes a patient
with sickle cell anemia and history of stroke was diagnosed with
Bicuspid aortic valve stenosis and aneurysmal dilatation of the
ascending aorta complicated with infective endocarditis. During routine
workup he was discovered to have Hodgkin’s Lymphoma. He successfully
underwent mechanical aortic valve and aortic root replacement. He
underwent exchange transfusion preoperatively and one time immediately
before initiating of Cardiopulmonary bypass. There was no major
vaso-occlusive crisis occurred throughout the surgery. Patient was
discharged in stable condition, and was scheduled for involved site
radiation therapy for Hodgkin’s Lymphoma management. Conclusion: Sickle
Cell Disease can be very challenging during cardiopulmonary bypass.
Exchange transfusion can reduce HbS, and increase hematocrit level. Mild
hypothermia can be used if sufficient CPB flows and venous saturation