Right ventricular decompression and left ventricular function in pulmonary atresia with intact ventricular septum. The influence of less extensive coronary anomalies.

BACKGROUND: Right ventricular decompression (RVD) may cause myocardial ischemia in patients with pulmonary atresia with intact ventricular septum and associated coronary artery abnormalities. Although we have previously shown that mortality is very high when two or more coronary arteries are obstructed, the effects of lesser degrees of coronary abnormalities are unknown. We therefore evaluated the effect of RVD on left ventricular (LV) function in those with less extensive coronary artery abnormalities. METHODS AND RESULTS: Preoperative cineangiograms demonstrated fistulas with or without one coronary artery stenosis in 12 of 24 patients aged 2 days to 33 months at the time of RVD. Preoperative and postoperative two-dimensional echocardiograms were analyzed for global and regional LV function. One infant with fistulas involving two coronary arteries and stenosis of the right coronary artery died from severe global LV dysfunction after RVD. Despite this, mean LV end-diastolic volume (66 +/- 17 mL/m2) and mean LV ejection fraction (60 +/- 9%) were similar in patients with and without coronary artery abnormalities before and after RVD. Before RVD, regional LV dysfunction was seen in 8 of 132 (6%) regions in those with coronary artery abnormalities and in 3 of 132 (2%) in those without coronary artery abnormalities. After RVD, there were 16 of 132 (12%) abnormal regions in those with coronary artery abnormalities and 1 of 132 (< 1%) in those without coronary artery abnormalities. In regions with normal wall motion before RVD, the presence of coronary artery abnormalities was related to regional LV dysfunction after RVD (P < .001). CONCLUSIONS: Regional LV dysfunction was rare in patients without coronary artery abnormalities. In those with less extensive coronary artery abnormalities not involving obstruction to multiple coronary arteries, regional LV dysfunction was common before and increased after RVD, but severe global LV dysfunction was unusual.