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Pulmonary atresia with Ventricular Septal Defect and Major Aortopulmonary Collaterals (PA/VSD/MAPCA's) is a complex form of congenital heart disease. Although it has been recognized that coronary artery anomalies are frequently associated with many other conotruncal defects, the incidence of coronary anomalies in PA/VSD/MAPCA's has not been documented. The purpose of this study was to evaluate the incidence of coronary anomalies in PA/VSD/MAPCA's.
64 consecutive patients undergoing their first surgical procedure for repair of PA/VSD/MAPCA's were included in this study. The coronary artery patterns were documented based on the observations made intra-operatively. These data were then compared to the coronary artery patterns identified at cardiac catheterization.
There were 6 different coronary artery patterns (one normal and five anomalous) observed. 73% had a normal coronary artery pattern, while 27% had an anomalous coronary origin and course. Abnormal coronary artery patterns included:
15% left anterior descending originating from the right coronary artery
5% branch of left comes from right and supplies septum(dual LAD)
3% right coronary originating from left coronary artery
3% branch of right comes from left and supplies free wall
1% right coronary originating from the non-coronary sinus
In addition to these anomalous coronary artery patterns, there were two additional coronary artery abnormalities noted. This included the pulmonary artery arising from the coronary system (n=3) and a MAPCA arising from a coronary (n=2).
Cardiac catheterization corroborated the coronary artery patterns found intra-operatively in 91% of cases. The 9% that did not correlate were all false negatives.
The incidence of anomalous coronary arteries was 27% in PA/VSD/MAPCA's. An additional 8% had coronary artery abnormalities noted. This incidence of coronary artery anomalies is significantly higher than for most other forms of conotruncal defects. We would emphasize the importance of recognizing these anomalous patterns during placement and/or replacement of conduits from the right ventricle to the reconstructed pulmonary arteries.
Poster Sessions, Expo North
Saturday, March 09, 2013, 3:45 p.m.-4:30 p.m.
Session Title: Congenital Cardiology Solutions: Prenatal Diagnosis, Coronary Anomalies and More
Abstract Category: 13. Congenital Cardiology Solutions: Pediatric
Presentation Number: 1162-127
- 2013 American College of Cardiology Foundation