Right ventricular (RV) dysfunction has adverse effects on long-term outcome
in patients with repaired tetralogy of Fallot (TOF), We employed serial ra
dionuclide angiography (RNA) to examine RV and left ventricular (LV) systol
ic function in adults late after TOF repair and its relation to clinical ou
tcome. We reviewed 10-year records of 95 patients (53 men) with TOF followe
d in our clinic (mean age at repair 12.6 +/- 10.5 years, mean age at last f
ollow-up 37.7 +/- 9.8 years) who underwent at least 2 RNAs between 1987 and
1997, Most patients were well by the end of the study (80% were New York H
eart Association class I, 17% were class II, and 3% were in class III). Six
teen patients experienced sustained tachyarrhythmias (8 had atrial; 8 patie
nts had ventricular). One patient died suddenly. Fifteen patients underwent
RV outflow reoperations(15 underwent pulmonary valve replacement; 7 had re
lief of RV outflow obstruction); RV systolic function during exercise in th
ese 15 patients was significantly impaired before and returned to similar l
evels after surgery, compared with the rest of the patients, Overall, RV an
d LV function remained stable in the whole group at a mean interval of 5.7
+/- 2.2 years between first and last RNA. This group of closely followed ad
ults with TOF remained well over 10 years with a low incidence of sudden de
ath and stable RV and LV systolic function, despite a relatively large numb
er of RV outflow reoperations, Aggressive intervention for right-sided hemo
dynamic abnormalities may have contributed to this outcome. Preserved ventr
icular function may herald a favorable long-term outlook in this group, (C)
2000 by Excerpta Medica, Inc.