Surgical treatment of tricuspid atresia

From April 1984 to July 1989, 6 consecutive patients underwent an orthoterminal repair for classical tricuspid atresia (TA). Their ages ranged from 6 to 14 years with a mean of 8.3 years. Four patients had ventriculoarterial concordance (VA-con) and 2 had ventriculoarterial discordance (VA-dis). One patient with VA-dis underwent a total cavopulmonary connection. The other five patients had modified Fontan operation. All 6 patients survived the operation. At a mean follow-up of 28 months, all patients were in the New York Heart Association Functional Class I. A follow-up color Doppler echocardiogram showed that the forward pulmonary flow was mainly during the ventricular diastolic phase except in one case. In this case, a tripartite right ventricle (RV) could be identified by angiocardiogram 4.5 years after the atrioventricular (A-V) connection without obliteration of the trabecular portion. A big "V" wave was demonstrated in the RV and pulmonary trunk. The pulmonary valve was closed in the ventricular diastolic phase. In the other case with an A-V connection and obliteration of the trabecular portion, revealed a fixed-open pulmonary valve with a forward flow more in the atrial systolic phase than in the ventricular systolic phase. The graded exercise test in these 2 patients with an A-V connection revealed a normal functional capacity. The mean maximal oxygen uptake was 8.4 +/- 0.1 METS. In the other 3 children without an A-V connection the mean maximal oxygen uptake was 6.6 +/- 0.5 METS. We concluded that the Fontan-like repair was the procedure of choice for TA, and that excellent surgical results could be obtained.(ABSTRACT TRUNCATED AT 250 WORDS)