A new method for the quantitative standardization of cross-sectional areas of the pulmonary arteries in congenital heart diseases with decreased pulmonary blood flow.

A new angiographic method for quantitative standardization of cross-sectional area of bilateral pulmonary arteries, the PA-index, and retrospective analysis of the PA-index in different types of operative procedures are presented. This study included 40 subjects in the normal control group, 46 patients in the tetralogy group, 26 patients in the Rastelli group, and 15 patients in the Fontan group. The normal value of the PA-index was 330 +/- 30 mm2/BSA and was consistent in a wide range of body surface areas from infancy to adolescence. The PA-index in the tetralogy and Rastelli groups ranged from 100 to 400 mm2/BSA. There were no early deaths in the tetralogy group, but the incidence of low cardiac output was higher in patients with a smaller PA-index, especially when the PA-index was less than 150 mm2/BSA. Low cardiac output was more severe in the Rastelli group. The operative mortality was significantly affected by the PA-index. In the Rastelli group, all of the patients with a PA-index of less than 200 mm2/BSA died, whereas the mortality rate in patients with a PA-index of more than 200 was only 6% (p less than 0.01). The mortality rate was not influenced by any other factors, such as aortic cross-clamp time or age at operation. In the Fontan group, two patients with a PA-index of less than 250 mm2/BSA died of severe heart failure, and 12 of 13 patients with a PA-index of more than 250 survived (p less than 0.01). Our results indicated the validity of the PA-index in predicting the postoperative prognosis of the various entities. In tetralogy, all patients with a PA-index over 100 mm2/BSA can undergo correction safely; in Rastelli operation, those with a PA-index under 200 should have a palliative procedure first, whereas those with a PA-index over 250 can be considered good candidates for the Fontan procedure. The PA-index may also serve a useful guide in comparing surgical results from different institutions with patients having anomalies of varying severity.