Severe hemolysis due to cloth wear 23 years after aortic valve replacement on a Starr-Edwards ball valve model 2320

Despite iron substitution therapy, a patient developed severe hemolytic anemia 23 yr after insertion of a cloth-covered Starr-Edwards model 2320 aortic valve prosthesis. The prosthesis showed no sign of significant dysfunction. Upon removal, it showed extensive cloth wear on the inner surface of all three struts; one strut was completely denuded of its cloth covering. Hemolysis immediately resolved after replacement with a St Jude aortic prosthesis.     

Aortic valve replacement for a patient with warm-reactive autoimmune hemolytic anemia

We treated a 68-year-old woman with warm-reactive type of autoimmune hemolytic anemia (AIHA) who underwent aortic valve replacement. In consideration of the different hemolytic mechanisms between cardiopulmonary bypass and warm-reactive AIHA, conventional surgical procedures could be performed after the discontinuation of steroid therapy.     

A surgical case for severe hemolytic anemia after mitral valve repair.

We report a rare case of severe hemolytic anemia accompanied by moderate renal insufficiency after mitral valve repair. Although the degree of the residual mitral regurgitation was less than 1+ during the first three weeks after the operation, the maximum lactate dehydrogenase (LDH) was up to 7,430 U/l and the minimum hemoglobin was 4.9 g/dl. The mitral valve replacement successfully resolved the hemolysis, but the renal function did not completely recover.     

Successful aortic valve replacement in severe pancytopenia related to myelodysplastic syndrome

We report a 60-year-old man with myelodysplastic syndrome (MDS) whose aortic valve was replaced successfully. He suffered from dyspnea on exertion and was confirmed by echocardiography and cardiac catheterization to have severe aortic stenosis. A peripheral blood examination showed pancytopenia and a bone marrow examination showed MDS with a subtype of refractory anemia with excess blasts. His preoperative neutrophil count was 468/mm³, hemoglobin 9.8 g/dl, and platelet count 27,000/mm³. We conducted aortic valve replacement (AVR) using a bioprosthesis in July 2001. Pre- and postoperatively, we injected granulocyte colony-stimulating factor to keep the neutrophil count at 2,000/mm³ or more. We also transfused platelet concentrate and infused tranexamic acid. Despite potential risks of infection and bleeding, the AVR involved no significant complications. Careful preoperative planning thus plays an important role in managing such cases.     

Paravalvular leakage causing hemolytic anemia at 8–9 years after mitral valve replacement

We report two similar cases of women, aged 52 and 71 years, who developed shortness of breath or easy fatigability at 8-9 years after mitral valve replacement of the mitral valve in each patient, the echocardiographic study revealed paravalvular leakage. Previous examination before the appearance of symptoms had revealed a well-functioned prosthetic valve, and no hematological abnormality had been detected. The hemoglobin levels at admission were 6.4 and 6.6 g/dl, and their serum lactate dehydrogenase levels were 5218 and 4608 mU/ml. Each successfully underwent another valve replacement, and after surgery, the hemolytic anemia and the symptoms disappeared.     

Successful mitral valve replacement in osteogenesis imperfecta--a case report]

A 34-year-old man with osteogenesis imperfecta who underwent successful mitral valve replacement due to mitral regurgitation was reported. Cardiac disease associated with osteogenesis imperfecta is very rare and only fifteen patients were operated under the extracorporeal circulation previously. While excessive hemorrhage due to tissue fragility was observed in 7 of 15 patients, perioperative course of the case reported here was completely uneventful. The difference of hemorrhagic tendency as well as etiology of osteogenesis imperfecta will be defined according to the advance of technology in collagen genetics and biochemistry in future.     

Successful surgical treatment of subdural hematoma following mitral valve replacement]

A 56-year-old woman, followed after mitral commissurotomy, was admitted to the hospital because of congestive heart failure of NYHA IV. Cardiac catheterization demonstrated severe MSR and TR. MVR using a SJM prosthetic valve and TAP with DeVega's method were performed under cardiopulmonary bypass with moderate hypothermia (25 degrees C). Postoperative hemodynamic condition was good with a uneventful convalescence. But disturbance of consciousness was seen with gradual deterioration and anisocoria developed on the 4th postoperative day. CT scan revealed a subdural hematoma in the right frontal and parietal region. Irrigation of the hematoma was performed immediately by neurosurgeons. She resumed full consciousness and was discharged from the hospital without any sequelae on the 49th postoperative day. The importance of the early diagnosis and the immediate treatment for the subdural hematoma following open heart surgery was emphasized.     

Intravascular haemolysis after valve replacement: comparative study between Starr-Edwards (ball valve) and Björk-Shiley (disc valve) prosthesis.

Seventy-four patients with single prosthetic valves (Björk-Shiley or Starr-Edwards) in the mitral or aortic position and 18 controls with rheumatic valvar heart disease were investigated for evidence of intravascular haemolysis. Serum lactate dehydrogenase (LDH) was used as the most sensitive indicator of haemolysis. Raised concentrations were found in a third of 39 patients with Björk-Shiley prostheses (mean 281 IU/l) and in all 35 patients with Starr-Edwards prostheses (mean 859 IU/l. Values were considerably higher in patients with Starr-Edwards prostheses and particularly in those with aortic prostheses (mean 927 IU/l). Eight out of 12 patients with haemosiderinuria had Starr-Edwards valves. Intravascular haemolysis was of little clinical significance in patients with Björk-Shiley prostheses, but some patients with Starr-Edwards prostheses became iron deficient as a result.     

Isolated replacement of the mitral valve with the Starr-Edwards prosthesis.

Isolated mitral valve replacement with the Starr-Edwards prosthesis has been performed on 657 patients at the Mayo Clinic during the 11 year period ending January, 1972. The most recent subset of that series comprised patients who received the Model 6120 prosthesis. In this group, the operative mortality rate was 9 per cent and the actuarial late death rate at 5 years was 20 per cent. This survival rate is a significant improvement over the natural history of severe mitral valve disease. Factors associated with operative deaths are large left artrial size, advanced functional class, and previous heart surgery. Variables associated with higher incidence of late deaths are large left atrial size, patient age at operation, and multivalve disease. Risk of thromboembolism is increased with large left atrial size, presence of left atrial thrombus, and inadequate anticoagulant therapy.     

Surgical treatment of hemolytic anemia]

Splenectomy was done in 448 patients with various forms of hemolytic anemia. Recovery was noted in 362 patients (of them 143 children) with hereditary microspherocytic hemolytic anemia (HMHA). Of 8 patients with hereditary nonmicrospherocytic hemolytic anemia (HNMHA) one died, in 7 general status had improved. Of 78 patients with autoimmune hemolytic anemia (AIHA) 3 died, in 47 (60%) satisfactory result was noted, general status improved--in 18 (23%). If surgical intervention is indicated the operation for HMHA is radical method of treatment, HNMHA--the selective one, and AIHA--one of the main.     

Aortic valve replacement for a patient with glucose-6-phosphate dehydrogenase deficiency and autoimmune hemolytic anemia

Autoimmune hemolytic anemia and deficiency of glucose-6-phosphate deyhdrogenase (G6PD) result in severe hemolysis with different mechanisms. In patients with both pathologies, the effects of cardiopulmonary bypass on red blood cells and thrombocytes demand special care before and after open heart surgery. We evaluated the preoperative management and postoperative care of a patient with severe aortic insufficiency associated with G6PD deficiency and autoimmune hemolytic anemia who underwent aortic valve replacement.