Echocardiographic and Surgical Correlation of Coronary Artery Patterns in Transposition of the Great Arteries.

OBJECTIVE: Determine the accuracy of echocardiography to diagnose coronary anatomy in transposition of the great arteries and to evaluate the effect of accuracy on surgical outcomes and changes in accuracy over time. DESIGN: Retrospective chart review of neonates admitted February 1999 to March 2013 with transposition. Coronary pattern from the preoperative echocardiogram and operative reports were collected and compared with determine diagnostic accuracy. Coronary patterns were further confirmed by intraoperative images taken during surgery. SETTING: Tertiary care children's hospital. PATIENTS: Neonates with transposition of the great arteries and planned arterial switch operation with an echo and operative report or image describing the coronaries. INTERVENTIONS: Not applicable. OUTCOME MEASURES: Accuracy of echocardiography to diagnose coronary anatomy in transposition, and to identify factors related to correct diagnosis. RESULTS: One hundred forty-two patients met inclusion criteria with 122 correctly diagnosed, 16 incorrect, and 4 inconclusive. Accuracy was 86%, with 95% accuracy in patients with typical coronary patterns, 85% with the most common variant (left coronary from the leftward sinus and right and circumflex from the rightward sinus), and 61% with less common patterns. Typical and common variants were more likely to be correct than atypical patterns (P < .001). Cases with ventricular septal defect were more likely to have correctly diagnosed coronaries than with an intact ventricular septum (94% vs. 79%, P = .01). There was no change in accuracy over time (P > .05). There was no difference in duration of cardiopulmonary bypass, cross-clamp times, length of stay, or postoperative stay between the correct and incorrectly diagnosed groups (P > .05). CONCLUSIONS: In our center, accuracy of echocardiographic imaging of the coronary arteries in transposition was 86% without improvement over time, and perioperative outcomes were not affected by diagnostic accuracy. Further invasive imaging may not be necessary to determine the coronary pattern in this lesion.

Takotsubo cardiomyopathy after transcatheter Edwards Sapien pulmonary valve placement in a patient with tetralogy of Fallot.

A 34-year-old woman who had history of repaired tetralogy of Fallot with absent pulmonary valve syndrome presented for transcatheter intervention to reestablish pulmonary valve competence. A 26-mm Edwards Sapien valve was implanted, and coronary angiography after implantation demonstrated no coronary compression. Hemodynamically stable ventricular tachycardia was documented after the procedure. A 12-lead electrocardiogram showed ST segment elevation, and cardiac enzymes were moderately elevated. An echocardiogram demonstrated the stable position of the new pulmonary valve, the marked circumferential hypokinesia of the mid-to-apical left ventricular myocardium, and the normally contracting basal-to-mid myocardial segments. No intervention was required. An echocardiogram obtained 4 days after discharge showed qualitatively improved left ventricular systolic function, particularly in the apical region.