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.

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.

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.

A 9-day-old, 4.5-kg female infant was diagnosed with total anomalous pulmonary venous connections by echocardiography. The unusual nature of the pulmonary veins and their connections led to cardiac catheterization and angiography. Angiography demonstrated the left-sided veins connected, via a retropulmonary artery vertical vein, to the left inominate vein whereas the right-sided pulmonary veins connected to the right superior vena cava. We repaired this lesion uneventfully by creating a neo-common pulmonary vein and anastomosing this confluence to the left atrium.

Excessive surface fibrosis or fat limits effective electrode insertion in patients requiring epicardial pacing. We present chronic performance of a modified transvenous steroid lead used as an epi-intramyocardial electrode in a patient following repair of a univentricular heart after failure of both standard and steroid-eluting epicardial leads. Low implant threshold values remained stable during a 3-year postimplant interval demonstrating an effective and innovative approach to epicardial pacing.

Indications for pacemaker implantation in the pediatric population often include sinus or atrioventricular node dysfunction following surgery for congenital heart defects. However, patient size, cardiac defects, and vascular and valvular concerns may limit transvenous lead utilization. Since the epicardial surface of these patients often exhibits variable degrees of fibrosis from scar tissue formation or pericardial adhesions, chronic low output (2.5/1.6 V, 0.3 ms) epicardial pacing from implant is not currently recommended in children due to frequent threshold changes and electrode exit block. As a result, pacing in children is often viewed as a less efficient system than in adults. The addition of steroid combined with newer low threshold electrode designs however stabilizes the electrode‐tissue interface and eliminates postimplant changes seen with standard smooth surface electrodes potentially permitting efficient chronic pacemaker application to all patient ages. The stability of chronic low output epicardial pacing with steroid‐eluting electrodes was prospectively studied in 22 patients (ages 2 days‐18.5 years, median 3.5 years) for up to 6 years. Chronic pulse width thresholds were compared according to implant site and association of prior cardiac surgery. A total of 26 pacing leads were implanted. The acute implant mean pulse width threshold (2.5 V) for all the electrodes studied was 0.10 ms ± 0.05 ms. Stable low thresholds were maintained for up to 6 years without significant variation from implant. Mean ventricular pulse width thresholds (0.12 ms ± 0.05 ms) were significantly higher (P < 0.001) than atrial thresholds (0.06 ms ± 0.03 ms) at implant and throughout the study period. The thresholds in the patients following cardiac surgery were comparable to those without previous cardiac surgery (P = NS). Stable low thresholds may be chronically maintatined for up to 6 years for epicardial steroid‐eluting electrodes irrespective of pacing site or associated cardiac surgery.

We retrospectively compared the use of primary elective open sternum coupled with delayed sternal closure with the use of primary sternal closure in neonates after cardiac operations. Primary elective open sternum/delayed sternal closure was selectively used in patients who demonstrated hemodynamic or respiratory deterioration, or both, during an intraoperative trial of sternal closure; otherwise primary sternal closure was used. Primary elective open sternum was used in 55 (61.8%) and primary sternal closure in 34 (38.2%) of the 89 patients studied. Eleven (20%) patients having primary elective open sternum died compared with 5 (14.7%) patients having primary sternal closure (p = 0.6). Six (10.9%) of the patients with primary elective open sternum died before delayed sternal closure; the remaining 49 patients comprise the primary elective open sternum/delayed sternal closure group. The durations of mechanical ventilation (9.7 +/- 0.9 days [mean plus or minus standard error of the mean], median 7.7 versus 9.9 +/- 3.4 days, median 4.9; p = 0.0005) and hospital stay (21.1 +/- 1.4 days, median 17.7 versus 19.6 +/- 4.1 days, median 12.9; p = 0.004) were shorter in the primary sternal closure group. The overall morbidity and duration of inotropic support were not significantly different between the two groups, although seven (20.6%) of the patients with primary sternal closure did have to undergo delayed sternal reopening for refractory postoperative low cardiac output. There was one superficial wound infection in the primary elective open sternum/delayed sternal closure group. Primary elective open sternum/delayed sternal closure is an effective treatment for postoperative neonatal mediastinal compression for the following reasons: (1) the morbidity is low; (2) the mortality of the critically ill group of neonates in whom primary elective open sternum/delayed sternal closure was used was similar to that of the less critically ill primary sternal closure group; and (3) 20.6% of the primary sternal closure group eventually had to undergo delayed sternal reopening to treat refractory postoperative low cardiac output.

Between 1972 and 1990, 18 patients (median age 3 years, range 0.1-14 years) with coronary artery fistulae (CAF) were seen at this institution. Of the 16 patients without associated heart defects, two patients presented with congestive heart failure and the remaining 14 were asymptomatic. Fifteen patients had normal origin of two coronary arteries. Two patients had atresia of the proximal right coronary artery and, in one patient, the right coronary artery originated from the left main coronary artery. The QP/QS ranged between 1.0 and 2.8, with a mean of 1.4. Fifteen patients underwent operative closure without any deaths. One patient is being followed medically at present. Two patients showed spontaneous clinical improvement of CAF (complete closure in one and near complete in the other) and remained asymptomatic during a decade of follow-up. Review of the literature suggests operative closure of symptomatic and asymptomatic small CAF during childhood. However, the natural history of minute CAF remains unclear. In addition, symptoms from CAF may spontaneously improve with time.

Although new “low threshold” epicardial electrodes combine steroid with a porous, platinized‐platinum surface, the actual contribution of steroid elution has not been established. We evaluated this new electrode surface design with and without steroid in 13 children, ages 1–22 years. Both electrodes are unipolar and of similar surface area. The Medtronic Model 4951‐P is a barb design for epimyocardial insertion without steroid while the Model 10295A is a steroid elufing, epicardial disk‐shaped design. Both electrodes were implanted for atriai and ventricular pacing. At implant, sensed P and R waves, and pacing impedances were comparable between both electrodes. There were no significant differences between initial measured pulse width or calculated energy thresholds for the first 2 months following implant. Strength‐duration curves for both electrodes at 1 month were comparable to implant values. After 2 months, the threshold of the nonsleroid electrode peaked and stabilized at a significantly higher fP < 0.05) level than the more constant steroid eluting electrode. This difference continued for the first year following implant. We conclude that the new porous, platinized‐platinum electrode design intrinsically limits initial electrode‐tissue interface reactivity in children and improves epicardial pacing with low chronic threshold values. Steroid elution augments these intrinsic qualities by maintaining fibrous capsule stability with more constant low thresholds over time.

The clinical and pathological features of primary pulmonary artery sarcoma in two children are reported. The first patient presented with right ventricular outflow obstruction and underwent successful surgical resection of his tumor. The second patient developed cardiac arrest following a relatively short period of symptoms of right heart failure and could not be revived. The pathological diagnosis in both patients was hemangiopericytoma. To the best of our knowledge, primary pulmonary artery sarcoma in children has not been previously reported.

The files of 121 patients who presented to Children's Hospital of Michigan over the last 10 years with complete atrioventricular (AV) canal were reviewed to evaluate long-term management and overall outcome. Of 121 patients, 70 underwent corrective surgery, 21 (30%) of whom died perioperatively. The surgical mortality rate was 13% when patients with hypoplastic left or right ventricle (n = 6), double-orifice mitral valve or extreme deficiency of mitral tissue (n = 5), and pulmonary vascular obstructive disease (n = 5) were excluded. Of the 49 patients who survived operation, 36 are in New York Heart Association class I, 1 patient requires a pacemaker and 3 died late. In 34 of the 51 patients (28%) who did not undergo operation, pulmonary vascular obstructive disease developed; it occurred within 12 months in 10 patients (8%). Eight other patients who did not undergo operation died before planned surgery (age 1 to 9 months). Although surgical prognosis in good candidates is acceptable, the overall prognosis for children with complete AV canal is guarded because of the risk of early death or early pulmonary vascular obstructive disease and frequently unfavorable anatomy.

Fifty-two infants with a large ventricular septal defect (VSD) underwent primary surgical closure within the first 12 months of life. Operation was done because of medically intractable congestive heart failure in 39 patients (75%), failure to thrive in nine patients (17.3%), and increased pulmonary vascular resistance (PVR) (>8 units/m2) in four patients (7.6%). Four patients (7.6%) died in the early postoperative period. Surgically induced heart block did not occur in any patient. Relief of heart failure and normalization of growth and weight gain was evident in all survivors. Late postoperative hemodynamic evaluation in 15 patients with preoperative elevation of PVR revealed normal pulmonary artery pressure and resistance in each instance. Residual VSD was found in only one patient (2%) with partial disruption of a muscular VSD originally closed by direct suture. There was one late postoperative death from unrelated causes. The results of primary closure of VSD in infancy are compared with those of preliminary pulmonary artery banding (PAB) and of VSD closure after PAB. Fifty-six patients with isolated VSD or with VSD associated with atrial septal defect, patent ductus arteriosus, or aortic arch anomalies underwent initial palliative PAB. There were 10 early postoperative deaths (17.8%). Four additional patients died in the late postoperative period. Severe elevation of PVR persisted in three patients despite PAB. Closure of VSD and pulmonary artery debanding was done in 43 patients, with four early postoperative deaths (9.3%). Distal migration of the band necessitated extensive reconstruction of one or both pulmonary arteries in four patients. Placement of the PAB too close to the pulmonary anulus necessitated transannular patching in three patients. The staged surgical management of symptomatic VSD in infancy carries significant mortality and complication rates. Early primary closure is the treatment of choice because of its lower postoperative mortality rate and superior long-term results.

Consecutive series of 50 transvenous and 50 sutureless pacemaker insertions were reviewed and compared with a nonsimultaneous series of 50 patients who received sutured epicardial pacemakers. It is concluded that: (1) the sutureless electrode has a lower initial stimulation threshold than the transvenous type; (2) complications from sutureless electrodes are greater than with transvenous electrodes and similar to the incidence with sutured epicardial pacemakers; (3) initial pacing is more satisfactory with the sutureless electrode compared with the transvenous model; and (4) late electrode failure due to threshold rise is greater with the sutureless electrode than with the transvenous or sutured epicardial type.