Noninvasive quantitative evaluation of the morphology of the major pulmonary artery branches in cyanotic congenital heart disease. Angiocardiographic and echocardiographic correlative study.

BACKGROUND Precise noninvasive evaluation of pulmonary artery (PA) morphology is extremely important for medical and surgical management of patients with cyanotic heart disease. In this study, the accuracy of two-dimensional echocardiography combined with color Doppler flow mapping to assess the size, stenosis, and atresia of the major PA branches was examined using a new parasternal approach. METHODS AND RESULTS With the use of right and left high parasternal windows, we visualized each of the major portions along the right (R-PA) and left (L-PA) pulmonary arteries in 45 of the 47 examinations (96%) in 38 patients with cyanotic heart disease. The patients were between 13 days and 20 years old (mean age, 2.9 years). The internal diameters of the major PA branches were measured at three points along the R-PA (the proximal, mid, and distal portions) and at the proximal and distal portions on the L-PA in systole by both two-dimensional echocardiography and angiography. In addition, the diameter of the stenosis in the PA branch was measured. These PA values as determined by two-dimensional echocardiography correlated well with those obtained by angiography (r = .95 to .97). By two-dimensional echocardiography with color Doppler flow mapping, 17 of 19 lesions with stenoses or atresia of the major PA branches were predicted as defined by angiography (sensitivity, 89.5%; specificity, 100%). Differences between the distal parts of the L-PA and R-PA of > 30% in diameter were determined by angiography in 15 examinations and by two-dimensional echocardiography in 12 examinations (sensitivity, 80%; specificity, 97.4%). CONCLUSIONS Our technique permits noninvasive evaluation of the size, stenoses, and atresia of the major portions of the PA branches in patients with cyanotic heart disease both before and after surgery.