Two hundred eighteen consecutive patients with tetralogy of Fallot (TOF) underwent surgical treatment under a prospective protocol during a three-year period (January 1978 to December 1980). No patient was refused the operation. Eighty-three patients had palliative operations without hospital deaths (50% less than 1 year of age). One hundred thirty-five had total correction with 5 hospital deaths (3.7%; 70% C.L. = 2.0 to 6.2). Thirty-five of them had had palliation in the first year of life (one hospital death at time of correction). Incremental risk factors were young age (p less than 0.0002), transannular patch (p = 0.13) and primary repair (p = 0.38). Significant stenosis in the pulmonary artery branches were eliminated utilizing an original table of relationship between the diameter of the expected normal pulmonary valve annulus and the calculated diameter of the branches. The immediate post-repair peak systolic pressure ratio between right and left ventricles (Prv/lv) was only 0.39 and the incidence of transannular patches was remarkably low (34%). It is concluded that surgical management of tetralogy of Fallot can be achieved, today, with a very low hospital mortality reserving a two-stage procedure only for small infants (less than 1 year of age). Accurate criteria for the reconstruction of the right ventricle outflow tract (RVOT) can easily allow very low rates of transannular patches and postoperative RV hypertension with an ultimate better preservation of the RV function.

Surgical treatment of mitral insufficiency and aortic valve stenosis associated with endocardial fibroelastosis has not been well documented. We treated 2 infants with this complex lesion: in one case it was possible to repair the mitral valve. The long-term results are quite encouraging and emphasize that this lesion should be treated early to avoid the risk of compromising the left ventricular function, although the chance of a valve replacement in an infant can be high.

The policy for surgical treatment of tetralogy of Fallot in younger patients is still controversial. Our overall 14-year experience has been reviewed with regard to the factors influencing mortality for both shunts and corrective procedures. An attempt has been made to evaluate our current expected cumulative mortality for two-stage correction in patients under 2 years of age. From November, 1966 through April, 1983, 440 shunts and 647 total corrections were performed. Patients under 2 years of age, and those with unfavorable anatomy and/or physiology, generally underwent two-stage correction. Early correction was occasionally performed in this age group on patients with very favorable anatomy, or in case of early shunt failure. Retrospective standard statistical analysis was carried out in order to evaluate the influences of the year of operation, age, and operative technique on mortality. The overall early mortality of shunt procedures was 5.7% (11.4% below and 3.5% over 6 months of age). Since 1978 it has dropped to 2.8% (4.2% below and 2.1% over 6 months). The Waterston shunt had a higher (7%) operative mortality than the Blalock (3%) or Goretex (2.6%) shunts. The overall early mortality of total corrections was 15.1% (25.2% below and 13.5% over 2 years of age). It has dropped to 6.9% since 1978 (29.9% below and 6.2% over 2 years).(ABSTRACT TRUNCATED AT 250 WORDS)