重症拡張型心筋症の幼児に対する血漿交換療法の経験(<特集>心疾患とアフェレシス)

概要

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Cardiodepressant antibodies such as anti β1-adrenergic receptor (β1-AR) antibody are suggested as one of the causes of dilated cardiomyopathy (DCM). Immunoadsorption for removing the antibodies is reported to induce hemodynamic improvement in adulthood. We administered plasma exchange (PE) to a 4-year-old male DCM patient with serious heart failure. PE was performed three times and effected improvement in his cardiac function at the first and second time. Left ventricular ejection fraction and urine volume increased, and the dobutamine and diuretic infusion were discontinued briefly. His titer of anti β1-AR antibody decreased after the first PE. Ultimately he died from progressive heart failure two weeks after the third PE. However, the apheretic treatment may be effective against DCM in patients with the cardiodepressant antibodies positive in infancy or childhood as well as in adulthood. Consequently the PE had better be administered to DCM patients early before the heart failure became progressive.
2010-05-31