Abnormal origin of the right subclavian artery from the right pulmonary artery in a patient with D-transposition of the great vessels and left juxtaposition of the right atrial appendage: an unusual anatomical variant.

Isolation of the left subclavian artery (LSCA) or its anomalous origin from the pulmonary artery (PA) has been documented in several cases, especially in association with a right-sided aortic arch. Similar anomalies involving the right subclavian artery (RSCA) are less frequent. Anomalous origin of the RSCA from the PA in association with D-transposition of the great arteries (D-TGA) is exceedingly rare and only two cases have been reported so far. We present here, a case of aberrant origin of the RSCA from the right PA in a patient with D-TGA, in whom the diagnosis was rendered difficult due to the partial occlusion of the intervening ductus arteriosus (DA). We discuss the embryological basis of this anomaly and review its clinical and surgical implications.

Restrictive interatrial communication in hypoplastic left heart syndrome after modified Fontan repair.

The occurrence of pulmonary venous obstruction after total cavopulmonary connection with intraatrial lateral tunnel is a rare occurrence. We present two cases of hypoplastic left heart syndrome with restrictive interatrial communication presenting late after this type of modified Fontan repair. This occurred even after complete excision of the atrial septum at the time of Stage 1 Norwood in both cases. A novel approach to this problem of resecting the roof of the coronary sinus was utilized to enlarge the interatrial communication.

Abrupt aortic root dilation after the Ross procedure--is this a progressive phenomenon?

This study provides evidence of aortic root dilation in children, adolescents, and young adults who have undergone the Ross procedure. Several mechanisms are described.

Chronic performance of steroid-eluting epicardial leads in a growing pediatric population: a 10-year comparison.

Patient size and congenital heart defects complicate pacemaker therapy in children favoring an initial epicardial approach. Steroid-eluting (SE) epicardial (EPI) leads maintain stable, low pacing thresholds in the short-term when compared to the nonsteroid (NSE) epicardial (EPI) leads. The purpose of this study was to evaluate chronic, 10-year performance of SE leads in growing children compared with NSE EPI leads implanted during the same time interval. From 1990 to 2000, 35 patients (age 1 month to 18 year, median 3 years), 28 with and 7 without congenital heart disease (CHD) received 51 SE leads: 27 ventricular and 24 atrial. NSE leads were implanted in 27 patients (age 1-28 years, median 8 years), 24 with and 3 without CHD: 27 ventricular and 1 atrial. Pacing lead threshold, impedance, and energy were measured at implant and during a 10-year follow-up. Unpaired t-test showed that impedance remained stable for all leads with lower mean values for the SE(376 +/- 55 vs 443 +/- 109 Omega) (P = NS). The mean energy requirement for SE leads at 10 years(1.2 +/- 0.9 microJ)was significantly lower than for NSE(4.4 +/- 5.5 microJ) (P < 0.05). At 2.5-V output, chronic thresholds for SE leads did not significantly differ from implant values for atrial (0.08 vs 0.09 ms) or ventricular (0.08 vs 0.08 ms) sites. There were no differences in SE lead performances among patients with or without CHD. Fracture or dislodgement occurred in two SE (4%) and four NSE (14%) leads. SE outperform NSE EPI leads and show stable, chronic low thresholds over time in all growing children.