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巨大先天性三尖瓣关闭不全的自然消退

Spontaneous resolution of massive congenital tricuspid insufficiency.

作者信息

Boucek R J, Graham T P, Morgan J P, Atwood G F, Boerth R C

出版信息

Circulation. 1976 Nov;54(5):795-800. doi: 10.1161/01.cir.54.5.795.

Abstract

Four cases of congenital tricuspid insufficiency (CTI) are presented in whom spontaneous improvement occured. There was no evidence of Ebstein's disease in three of the four. All presented with similar clinical and laboratory findings including massive cardiomegaly, mild to moderate congestive heart failure, and moderate to severe desaturation. Catheterization data demonstrated severe TI and right-to-left atrial shunting. During the period of follow-up, up to 3.5 years, all symptoms have spontaneously disappeared. Clinical and angiocardiographic evidence for minimal residual TI have remained. Right atrial and right ventricular volume measurements were markedly abnormal initially and returned to normal or near normal. These measurements may be a useful method for quantitating the severity of tricuspid insufficiency. The course of the three infants with massive CTI (without Ebstein's disease) differs from previous reports and may be important both in formulating future management plans and in defining the etiology of the disorder.

摘要

本文报告了4例先天性三尖瓣关闭不全(CTI)患者,其病情出现了自发改善。4例中有3例无埃布斯坦畸形的证据。所有患者均表现出相似的临床和实验室检查结果,包括心脏明显增大、轻度至中度充血性心力衰竭以及中度至重度血氧饱和度降低。心导管检查数据显示存在严重的三尖瓣关闭不全和右向左心房分流。在长达3.5年的随访期间,所有症状均自发消失。仍有微量残余三尖瓣关闭不全的临床和心血管造影证据。右心房和右心室容量测量最初明显异常,随后恢复正常或接近正常。这些测量可能是定量三尖瓣关闭不全严重程度的有用方法。3例患有严重CTI(无埃布斯坦畸形)的婴儿的病程与先前报道不同,这对于制定未来的治疗方案以及明确该疾病的病因可能都很重要。

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