大動脈弁上狭窄および低形成弓部大動脈術後の鎖骨下動脈瘤 : von Willebrand 病の小児における1治験例

概要

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A 7-year-old girl was first admitted to our institute at 3 years of age with a history of heart murmur and epistaxis. Cardiac catheterization and angiography showed diffuse stenosis of the aortic segment extending from supravalvular region to the distal aortic arch. The concomitantly performed hematological evaluation made the diagnosis of von Willebrand's disease. With the aid of hypothermic cardiopulmonary bypass, patch aortoplasty was successfully performed. The coexisting hemophilic disease required supplemental transfusion of fresh whole blood and plasma and also administration of massive doses of AHF, 20 units/kg, during the perioperative periods. Three years after the aortoplasty, however, the chest roentgenogram demonstrated a growing calcined mass at the left upper mediastinum, which, by the combined evaluation of angiography and computed tomography, was found to be the aneurysm of the left subclavian artery. Aneurysmectomy was successfully performed with the aid of normothermic partial cardiopulmonary bypass and AHF was again administered with a lesser dosage. The pathological findings was false aneurysm.
日本小児外科学会の論文
1988-08-20