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高度肺高血圧を合併した僧帽弁狭窄症には弁置換術を施行しても肺動脈圧が下降せず,術後管理に難渋するものが少なくない。著者らは高度肺高血圧を合併した僧帽弁狭窄兼三尖弁閉鎖不全症(MS+TR,PH)に対し,僧帽弁置換,三尖弁形成術を施行し,術後肺動脈圧が下降せず,肺高血圧クリーゼを起こしたが,プロスタグランディンE1(PGE1)の投与が有効であった症例を経験したので,臨床的,病理学的に検討を加え報告する。また,本症例は全身性エリテマトーデス(SLE)を合併していたため弁病変についても病因論的に検討を加え、あわせて報告する。
In some patients with severe pulmonary hyperten-sion secondary to mitral stenosis, we sometimes encounter much difficulty in postoperative care even after mitral valve surgery. We reported pulmonary hypertensive crisis in a patient with mitral stenosis, tricuspid regurgitation associated with pulmonary hypertension after mitral valve replacement and tricuspid annuloplasty. Low dose infusion of prosta-glandin E1 (0.1 ng/kg/min) reduced pulmonary arte-rial pressure and consequently resulted in improve-ment in postoperative hemodynamics. Histologic study of the pulmonary vasculature, in this case, reve-aled marked medial hypertrophy and concentric-laminar intimal fibrosis of the muscular arteries. This case was associated with systemic lupus erythemato-sus, but neither clinical data nor pathological find-ings could clarify the etiology of mitral lesion.
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