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Treatment of Right Ventricle to Coronary Artery Connections in Infants wtih Pulmonary Atresia and Intact Ventricular Septum
S. Setty, J. Berry, P. Jain, L. Pyles, J. E. Foker. University of Minnesota, Minneapolis, MN,
BACKGROUND:
At the severe end of the spectrum of infants born with pulmonary atresia and intact ventricular septum (PAIVS), the likelihood of significant right ventricle-to-coronary-artery connections (RV-CACs) increases. With much or all of the coronary blood flow from the hypertensive RVs, hypoxic blood perfuses and presumably injures the myocardium. Moreover, arteritis and obstructions often develop in coronary arteries with retrograde flow. The consequences of hypertensive RVs and large RV-CACs presumably contribute to the increased mortality of PAIVS over tricuspid atresia. In addition, significant RV-CACs interfere with 2-ventricle repairs (2VR) because RV decompression might produce myocardial steal. The detrimental consequences of significant RV-CACs to the myocardium, patient survival and the goal of a 2VR led us to identify and eliminate them prior to decompressing the RV. Our purpose is to present the first series of RV-CACs ligated off bypass prior to RV decompression and evaluate the consequences of this approach.
METHODS:
From 1988-2005, 15 patients with PAIVS had a total of 53 RV-CACs identified preoperatively and 40 were judged large enough to warrant ligation. One newborn with no proximal CAs was the only patient excluded. Initial identification and localization was by transthoracic echo followed by angiography. These techniques also demonstrated three major obstructions and 12 narrowings of 25-75% in the major coronary arteries. Ligation was done epicardially off bypass using 6/0 prolene sutures with occlusion verified by echo. Direct visualization and echo assessment for regional wall motion abnormalities (WMA) were done. An RV outflow patch was placed in all. Echo studies were done at least at 1, 3 and 5 days postoperatively.
RESULTS:
After ligation, coronary flow converted from retrograde to antegrade. Ligation produced no visual myocardial consequences or immediate WMAs by echo. For 3 patients, however, apical WMAs appeared from 2 hours to 3 days postoperatively. One WMA resolved with ligation of an additional RV-CAC. The late-appearing RV-CAC involuted spontaneously, and the third patient developed a severe CNS hemorrhage on the fifth postoperative day, making the RV-CAC moot. Serial studies were done to assess longer-term effects in the 12/15 survivors. No later evidence for previous infarction was found, and two-ventricle repairs were accomplished in all.
CONCLUSIONS:
We present the first series of RV-CAC ligations as the initial surgical step in the treatment of the severe end of the PAIVS spectrum and conclude: (1) location and ligation of RV-CACs can be reliably accomplished without apparent myocardial damage; (2) coronary flow becomes antegrade and normoxic, improving myocardial oxygenation; (3) some small RV-CACs may enlarge secondarily while others involute; and (4) despite significant RV-CACs, 2-ventricle repairs become possible with their long-term benefits.
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