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.

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