Ventriculo-coronary arterial connections in pulmonary atresia with intact ventricular septum, and their influences on ventricular performance and clinical course.

To define the influence of ventriculo-coronary (VC) arterial connections on ventricular performance and late outcome, and associated wall motion abnormalities in patients with pulmonary atresia and intact ventricular septum, 21 patients aged > or = 6 months (mean 4.3 +/- 3.3 years, range 0.5 to 11.8) were reviewed. Ventricular volumes, mass, systolic functional variables and regional wall motion were measured using biplane ventricular cineangiography. Fourteen patients had VC connections, 11 of whom had coronary artery abnormalities. In comparing patients with VC connections and coronary abnormalities (group I) with those without coronary abnormalities (group II), no hemodynamic differences were observed, except the expected increased right-to-left ventricular systolic pressure ratio in group I (1.33 +/- 0.24 vs 0.72 +/- 0.50). Wall motion abnormalities were observed in 8 patients (7 in group I, and only 1 in group II [p < 0.05]). Ejection fraction in patients with abnormal wall motion was significantly lower (50 +/- 6% vs 57 +/- 7%; p < 0.05) and ventricular systolic pressure ratio significantly higher (1.46 +/- 0.16 vs 0.80 +/- 0.45; p < 0.05) than in those with normal wall motion. Five patients died during follow-up, all of whom had wall motion abnormalities. The findings suggest that patients with VC connections and coronary abnormalities have a higher incidence of wall motion abnormalities, which may reflect ongoing ischemia, and are at risk for late death.