Objectives. The study was done to determine the diagnostic accuracy of echo
cardiography alone in the preoperative diagnosis of children with major con
genital heart defects undergoing primary complete repair.
Background. Although echocardiography is well established as the first-line
imaging technique for the diagnosis of all forms of congenital heart disea
se, most institutions continue to perform cardiac catheterization prior to
complete repair of more complex defects.
Methods. To determine the diagnostic accuracy of echocardiography alone and
echocardiography plus catheterization, we reviewed the records of 503 chil
dren with major congenital heart defects who underwent primary complete rep
air at our institution between July 1992 and June 1997. We included childre
n with transposition of the great arteries, tetralogy of Fallot, double cha
mber right ventricle, interrupted aortic arch, aortic coarctation, atrioven
tricular septal defect, truncus arteriosus, aortopulmonary septal defect, a
nd totally anomalous pulmonary venous return. We excluded children with les
s complex defects such as isolated shunt lesions, as well as those with the
most complex defects that would require surgical palliation (e.g., functio
nal univentricular heart). We defined major errors as those that increased
the surgical risk and minor errors as those that did not. Errors in diagnos
is were determined at surgery.
Results. Eighty two percent of children (412 of 503) underwent surgery afte
r preoperative diagnosis by echocardiography alone. There were 9 major (2%)
and 10 minor errors in the echocardiography alone group and 7 major and 5
minor errors in the echocardiography plus catheterization group. The most c
ommon type of error was misidentification of coronary artery anatomy in pat
ients with transposition of the great arteries. No error in either group re
sulted in surgical morbidity or mortality.
Conclusions. This study suggests that echocardiography alone is an accurate
tool for the preoperative diagnosis of major congenital heart defects in m
ost children undergoing primary complete repair, and may obviate the need f
or routine diagnostic catheterization. (C) 1998 by the American College of
Cardiology.