malignant hematologic disorders ( multiple myeloma , myelodysplasia , chronic lymphocytic leukemia , and hodgkin 's and non - hodgkin 's lymphoma ) and idiopathic aplastic anemia that occur in the population with an incidence of 0.5 - 5/100,000 increase the surgical operative risk due to coagulation defects , changes of blood viscosity , immunosuppression , and bone marrow insufficiency ( 1 ) .
pancytopenia associated with idiopathic aplastic anemia may pose an increased risk for postoperative bleeding and infection ( 2 ) . here , we report a patient with severe aortic valve insufficiency undergoing bioprosthetic aortic valve replacement who suffered from idiopathic aplastic anemia .
a 66-yr - old man was admitted to the hospital because of shortness of breath .
his dyspnea was classified by the new york heart association ( nyha ) as functional class iii .
the patient 's history included treatment for severe aplastic anemia with anti - thymocyte globulin and prednisone for 5 yr prior to admission .
echocardiography revealed reduced left ventricular function and severe aortic valve regurgitation ( grade iv ) with left ventricular end diastolic dimension measuring 87 mm .
the presence of severe dyspnea and echocardiographically documented severe aortic valve insufficiency led us to schedule a surgery for elective aortic valve replacement . on admission ,
laboratory testing showed leukocytopenia ( white blood cells 1,900/l ) , anemia ( hemoglobin 6.7 mm / l ) , and thrombocytopenia ( platelets 34,000/l ) .
the differential blood count revealed 42.9% neutrophils ( 50 - 70% ) , 42.3% lymphocytes ( 25 - 40% ) , 6.9% monocytes ( 2 - 8% ) , and 4.3% eosinophils ( 2 - 4% ) .
preoperative preparation included two weeks of granulocyte colony - stimulating factor ( neupogen 300 g , amgen , subcutaneously three times a week ) , transfusion of four units of packed red blood cells ( rbcs ) , and ten units of platelet concentrations ( pcs ) one day before the surgical procedure resulting in a hemoglobin level of 9.8 mm / l , a leukocyte level of 10,300/l , and a platelet level of 120,000/l preoperatively .
the patient received aortic valve replacement with a 21-mm bioprosthetic aortic valve ( carpentier - edwards , edwards lifesciences , irvine , ca , u.s.a . ) by a standard median sternotomy ; 2 milrione units of aprotinin were added to the cardiopulmonary bypass circuit priming solution .
on the second day after surgery , atrial fibrillation deveolped and was successfully converted with intravenous amiodarone .
antibiotic prophylaxis was given using ceftriaxon ( rocephin , roche , basel , swiss ) 2 g intravenously over 6 days .
the patient was discharged from the intensive care unit 4 days after surgery and was discharged from the hospital postoperative on day 11 with a leukocyte count 4,300/l , hemoglobin of 6.2 mm / l , and a platelet count of 62,000/l . after the surgery , the patient received 5 units of rbcs and 20 units of pcs until discharge .
perioperative laboratory findings are summarized in table 1 . during the 6 months of follow - up ,
the patient did well with a functional class of nyha class i. the chest radiography at 3 months post surgery showed a decreased cardiac size .
according to the definition of the severity of aplastic anemia ( 5 ) , the patient 's aplastic anemia could be categorized as non - severe aplastic anemia . regardless of the severity of aplastic anemia ,
( 1 ) , two questions have to be considered when cardiac surgery is indicated in patients with a hematologic disorder causing pancytopenia . given the increased operative risk and the potential benefit with regard to the life expectancy and quality of life , aggressive surgical treatment has to be compared with conservative treatment and the natural course of both , the hematologic disorder and the cardiac disease .
our patient suffered from severe dyspnea on exertion ; the patient had stable idiopathic aplastic anemia and was under hematological supervision .
progressive deterioration of ventricular function was documented on serial echocardiographic examinations . both symptomatic aortic regurgitation and
impaired left ventricular function led us to the decision to perform aortic valve replacement for this patient .
another important issue for this specific subset of patients is perioperative management to decrease morbidity and mortality ( 1 ) .
potential complications are caused by a decreased number or impaired function of blood cells ; this condition is aggravated by the cell damaging properties of extracorporeal circulation .
in addition to meticulous surgical hemostasis , substitution of various blood products is important to reduce the risk of bleeding complications .
total amount of the blood product transfused to this patient is regarded to be acceptable considering the higher risk of bleeding in these specific subset of patients .
aprotinin has been shown to decrease blood loss and reduce the use of blood products by at least 30% , in open heart surgery ( 4 ) .
thoracic drainage in our patient was 785 ml , which was similar to that of other patients who required cardiac surgery with a variety of malignant hematological disorders ( 1 , 3 ) .
another important aspect of perioperative care in these patients is the increased risk for infections .
it is of great importance to perform surgery in aseptic conditions and to avoid transmission of pathogenic microorganisms during the perioperative period .
the patient received warfarin sodium 2 days after surgery , and inr was maintained between 1.5 to 2.0 .
follow up , we have noticed neither thrombotic nor hemorrhagic complications related to the anticoagulation therapy .
( 3 ) , cardiac surgery with extracorporeal circulation is feasible in patients with idopathic aplastic anemia , and it was not associated with excessive complications that might be expected in a patient with this disorder .
 major surgery in a patient with pancytopenia might be associated with increased surgical risks , especially for bleeding and infection . 
 a 66-yr - old man was admitted to the hospital due to shortness of breath . 
 his dyspnea was classified by the new york heart association ( nyha ) as functional class iii . prior to admission 
 , he had a 5-yr history of medical management for idiopathic aplastic anemia . 
 the severity of aplastic anemia of the patient was graded as non - severe aplastic anemia . 
 echocardiography revealed reduced left ventricular function and severe aortic valve regurgitation ( grade iv ) with left ventricular end diastolic dimension measuring 87 mm . because of dyspnea and echocardiographically documented aortic valve insufficiency , the patient underwent elective aortic valve replacement . 
 although extracorporeal circulation for valve operations might be associated with aggravation of impaired blood cell function , the patient recovered from surgery uneventfully . here , we report a successful cardiac surgery with extracorporeal cardiopulmonary bypass in a patient with severe aortic valve insufficiency and concomitant idiopathic aplastic anemia . 