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Background Low superior vena cava (SVC) blood flow has recently been identified as a marker for bidirectional superior cavopulmonary connection (SCPC) failure and death. Prior innominate vein occlusion is considered a significant anatomic risk factor for SCPC failure. We therefore evaluated the outcomes of infants who underwent SCPC with known upper-body venous obstruction. Methods Between February 1995 and June 2014, SCPC was carried out in 8 patients who had either a single SVC with known prior occlusion of the innominate vein (n = 6) or bilateral SVCs without a bridging vein with occlusion of one SVC (n = 2). The cause of the occlusion was an indwelling catheter in 5 patients. These patients were compared with 8 patients with normal upper-body venous drainage who underwent SCPC. Patients were evaluated for preoperative risk factors (including SVC size, pulmonary artery size, Nakata index, pulmonary vascular resistance), operative factors, and clinical outcomes to determine the impact of prior upper-body venous occlusion on SCPC failure or death. Results There were no significant differences in preoperative risk factors between the two groups, except for a significantly lower Nakata index in the study group with a trend toward smaller pulmonary artery branch size. There were no SCPC takedowns or mortalities. There was no significant difference in postoperative length of stay (median of 7 days [range, 5 to 32 days] versus 5 days [range, 4 to 32 days]; p = 0.17. Study patients had a lower mean systemic oxygen saturation at discharge, 81% versus 85% ( p = 0.05). In the study group, at a median follow-up of 42 months, 3 patients underwent successful Fontan completion and 5 are still awaiting Fontan completion. Conclusions Although patients with prior upper-body venous obstruction may have lower systemic oxygen saturations at hospital discharge, they do not demonstrate an increased SCPC failure or mortality rate. Innominate vein occlusion or its equivalent in patients with bilateral SVCs should not preclude the performance of SCPC. Physiologic rather than anatomic evaluation of preoperative systemic venous return may be more useful to predict outcome after SCPC.

Hybrid Norwood palliation is gaining acceptance as an alternative to open Norwood palliation in high-risk neonates with hypoplastic left heart syndrome. Aortic dissection after hybrid Norwood palliation is a rare but ominous complication that has not been reported.

Objective To compare the interstage cardiac catheterization hemodynamic and angiographic findings between shunt types for the Pediatric Heart Network Single Ventricle Reconstruction trial. The trial, which randomized subjects to a modified Blalock-Taussig shunt (MBTS) or right ventricle-to-pulmonary artery shunt (RVPAS) for the Norwood procedure, demonstrated the RVPAS was associated with a smaller pulmonary artery diameter but superior 12-month transplant-free survival. Methods We analyzed the pre–stage II catheterization data for the trial subjects. The hemodynamic variables and shunt and pulmonary angiographic data were compared between shunt types; their association with 12-month transplant-free survival was also evaluated. Results Of 549 randomized subjects, 389 underwent pre–stage II catheterization. A smaller size, lower aortic and superior vena cava saturation, and higher ventricular end-diastolic pressure were associated with worse 12-month transplant-free survival. The MBTS group had a lower coronary perfusion pressure (27 vs 32 mm Hg; P P = .009). A greater pulmonary blood flow/systemic blood flow ratio increased the risk of death or transplantation only in the RVPAS group ( P = .01). The MBTS group had fewer shunt (14% vs 28%, P = .004) and severe left pulmonary artery (0.7% vs 9.2%, P = .003) stenoses, larger mid-main branch pulmonary artery diameters, and greater Nakata indexes (164 vs 134, P Conclusions Compared with the RVPAS subjects, the MBTS subjects had more hemodynamic abnormalities related to shunt physiology, and the RVPAS subjects had more shunt or pulmonary obstruction of a severe degree and inferior pulmonary artery growth at pre–stage II catheterization. A lower body surface area, greater ventricular end-diastolic pressure, and lower superior vena cava saturation were associated with worse 12-month transplant-free survival.

Traumatic aortic injuries occur rarely in the pediatric population. Adult vascular interventionalists more frequently encounter aortic injury, and endovascular graft placement is a commonly used management approach. The standard of care in pediatric traumatic aortic injury is an open surgical repair, although it is not always the optimal approach. Endovascular graft placement has been trialed in pediatric patients, but its use has technical limitations. We describe the case of an 8 year old female passenger in a motor vehicle collision, resulting in formation of a traumatic aortic pseudoaneurysm. Due to her co-morbidities, she was a high risk surgical candidate, and therefore, underwent successful percutaneous implantation of an investigational balloon-expandable covered Cheatham platinum (CP) stent (NuMed, Inc., Hopkinton, New York). This case demonstrates the utility of balloon-expandable covered stents for treatment of pediatric traumatic aortic injury (TIA) as an alternative to open surgical repair or percutaneous endograft implantation.

Background Traditional palliation of infants with biventricular hearts and inadequate pulmonary blood flow is a modified Blalock-Taussig shunt. The aim of this report is to assess the results of an alternative, right ventricle-to-pulmonary artery (RV-PA) shunt. Methods Between August 2004 and July 2007, 10 infants with biventricular hearts and inadequate pulmonary blood flow underwent palliation with an RV-PA shunt. Median age was 9 days (range, 4 to 86), weight was 3.0 kg (1.7 to 4.5), and 4 of 10 patients weighed less than 2.5 kg. Shunts were nonvalved Gore-Tex (W.L. Gore Assoc, Flagstaff, AZ), and size was 6 mm (n = 5) or 5 mm (n = 5). Results There were no operative deaths. Median oxygen saturation at hospital discharge was 95% (87 to 98). In 2 patients the shunt was partially narrowed with a metal clip; they underwent successful balloon dilation 6 months after shunt placement. Eight patients have undergone two-ventricle repair 6 to 17 months after shunt placement. At the time of complete repair, oxygen saturation was 86 ± 1% and weight was 7.7 ± 1.7 kg. Repairs included a valved RV-to-PA conduit, 14 to 16 mm in diameter. There was one interstage death. Conclusions The RV-PA shunt provides successful palliation in infants with biventricular heart disease and inadequate pulmonary blood flow. It can be used in low birth weight infants and allows significant growth with protection of oxygen saturation prior to complete repair. Partial clipping of the shunt with subsequent balloon dilation is an option to prolong palliation. These results compare favorably with those of a modified Blalock-Taussig shunt or single stage complete repair.

Neonates with hypoplastic left heart syndrome and high-risk factors for an open Norwood procedure may benefit from a hybrid stage I procedure. The presence of a giant patent ductus arteriosus prevents safe deployment of the ductus arteriosus stent. We describe a new technique that involves banding the patent ductus arteriosus, therefore allowing stent implantation during hybrid stage I palliation.

Background Complete repair of conotruncal defects frequently uses maintenance of an interatrial communication. Postoperative right ventricular dysfunction may be characterized by elevated right atrial pressure and decreased systemic oxygen saturation owing to right-to-left shunting at the atrial level. Methods From January 1996 to December 2005, 112 patients younger than 6 months of age underwent complete repair of tetralogy of Fallot or truncus arteriosus. An interatrial communication was used in 80 of 112 patients (71%). Hemodynamic data were determined during the first 48 hours after surgery. Results In patients with an atrial communication, mean oxygen saturation reached a nadir of 94% ± 6%, and mean arterial Po 2 a nadir of 73 ± 25 mm Hg at 16 to 24 hours after surgery; both increased during the second 24 hours. At hospital discharge, median oxygen saturation was 98% (range, 86% to 100%). During the first 48 hours, mean oxygen saturation was less than 90% in 13 patients; the only multivariate risk factor was younger patient age. Mean right atrial pressure was greater than 10 mm Hg in 30 patients; multivariate risk factors were older patient age and repair with a transannular patch. Conclusions After complete repair of conotruncal defects using an interatrial communication, systemic oxygenation reaches a nadir at 24 hours after surgery, and improves by the time of hospital discharge. Clinically significant desaturation occurs in a small minority of patients. Infants undergoing repair before 2 months of age are at higher risk for systemic desaturation. The effects of an interatrial communication on systemic oxygenation should not be considered a contraindication to complete repair in early infancy.

of an early aggressive treatment was life saving. Compared with the scheduled device implantation in patients deteriorating on the waiting list, management of emergencies requiring circulatory support is still a challenging situation. We have developed in our unit a mobile kit of emergency circulatory support to deal with this problem. This ECLS has become a useful adjunct to our cardiac assist device program, allowing patient transfer and secondary implantation of a more long-term sophisticated device. As expected in this catecholamine-induced cardiomyopathy, the patient recovered after surgical resection of the tumor, but recovery is not so fast as to allow the use of an ECLS device alone, and implantation of a long-term device was mandatory.

Background A modification to the Norwood procedure involving a right ventricle-to-pulmonary artery (RV-PA) shunt may improve early postoperative outcomes. Concerns remain about the effect of the right ventriculotomy required with this shunt on long-term ventricular function. Methods Between January 2000 and April 2005, 76 patients underwent the Norwood procedure, 35 with a modified Blalock-Taussig shunt (mBTS) and 41 with a RV-PA shunt. Patients were monitored until death or September 1, 2009, with an average follow-up of 6.8 years. Cardiac catheterization, echocardiograms, perioperative Fontan courses, and need for cardiac transplantation were compared between groups. Results Cumulative survival was 63% (22 of 35) in the mBTS group vs 78% (32 of 41) in the RV-PA group (p = 0.14). Pre-Fontan echocardiography revealed poorer ventricular function in RV-PA patients (p = 0.03). Cardiac transplantation was required in 6 of 32 (19%) patients with a prior RV-PA shunt vs 1 of 23 (4%) in the mBTS group (p = 0.06). This results in an almost identical cumulative transplant-free survival between groups; 60% (21 of 35) in the mBTS group and 63% (26 of 41) in the RV-PA group (p = 0.95). Conclusions Neither shunt offers a clear survival advantage through an average follow-up of 6.8 years. The RV-PA shunt results in impaired late ventricular function that may result in an increased need for cardiac transplantation.

Despite dramatic progress in neonatal cardiac surgery, prematurity and low birth weight remain risk factors for poor outcome. Attempts to delay intervention with supportive therapy have been shown to increase morbidity and mortality. We present a case of an 840 gram, 28-week gestation newborn with tetralogy of Fallot, in whom palliation was achieved with a right ventricular outflow tract stent. This management allowed subsequent successful complete repair.

Objective Prognostic factors for survival after bidirectional cavopulmonary anastomosis for functionally single ventricle are not well defined. We analyzed preoperative hemodynamic and echocardiographic data to determine risk factors for death or transplantation at least 1 year after bidirectional cavopulmonary anastomosis. Methods Data for all patients who underwent bidirectional cavopulmonary anastomosis before 5 years of age at our institution from September 1995 through June 2005 were analyzed. Available preoperative echocardiograms and catheterizations were reviewed. Survivors were compared with those who died or underwent transplantation. Bivariable associations between demographic and clinical risk factors and survival status (alive without transplantation vs dead or transplanted) were assessed with Wilcoxon rank sum test and χ 2 or Fisher exact tests. Survival functions were constructed with Kaplan–Meier estimates, and event times compared between subgroups with log–rank tests. Cox proportional hazard modeling was used for multivariable modeling of risk of death or transplantation. Results One hundred sixty-seven patients underwent bidirectional cavopulmonary anastomosis with hemi-Fontan (n = 62) or bidirectional Glenn (n = 105) operations. Three patients died before discharge, 11 died later, and 1 has undergone transplantation. Freedom from death or transplantation after bidirectional cavopulmonary anastomosis was 96% at 1 year and 89% at 5 years. Multivariable analysis of preoperative variables showed atrioventricular valve regurgitation to be an independent risk factor for death or transplantation (hazard ratio 2.8, 95% confidence interval 1.1–7.1, P = .02). Conclusion Although survival after bidirectional cavopulmonary anastomosis is high, preoperative atrioventricular valve regurgitation is an important risk factor for death or transplantation.