Recurrent Pediatric Respiratory Distress From a Challenging Vascular Anomaly: The Uncrossing Operation for Circumflex Aorta.

The authors report a case of circumflex aorta causing persistent respiratory distress in a 9-year-old boy who had previously undergone vascular ring division and multiple aortopexies. The uncrossing operation was performed, with symptomatic relief of both posterior and right-sided tracheal compression from the transverse aorta and right aortic arch, respectively.

The number of postoperative surgical or diagnostic procedures following congenital heart surgery correlates with both mortality and hospital length of stay.

BACKGROUND: Outcomes for congenital heart disease have dramatically improved over the past several decades. However, there are patients who encounter intraoperative or postoperative complications and ultimately do not survive. It was our hypothesis that the number of postoperative procedures (including surgical and unplanned diagnostic procedures) would correlate with hospital length of stay and operative mortality. METHODS: This was a retrospective review of 938 consecutive patients undergoing congenital heart surgery at a single institution over a 2-year timeframe. The number of postoperative surgical and unplanned diagnostic procedures were counted and the impact on hospital length of stay and mortality was assessed. RESULTS: 581 of the 938 (62%) patients had zero postoperative diagnostic or surgical procedures. These patients had a median length of stay of 6 days with a single operative mortality (0.2%). 357 of the 938 (38%) patients had one or more postoperative diagnostic or surgical procedures. These patients had a total of 1586 postoperative procedures. There was a significant correlation between the number of postoperative procedures and both hospital length of stay and mortality (p < .001). Patients who required 10 or more postoperative procedures had a median hospital length of stay of 89 days and had a 50% mortality. There were no survivors in patients who had 15 or more postoperative procedures. CONCLUSIONS: The data demonstrate that the number of postoperative procedures was highly correlated with both hospital length of stay and mortality.

Pseudoaneurysm of Innominate Artery Graft Stump in an Infant.

Innominate artery grafts are often utilized in pediatric cardiac surgery and very rarely lead to complications, including infection. Here, we present a unique case of an infant who underwent repair of coarctation of the aorta and hypoplastic arch using a GORE-TEX graft (W. L. Gore and Associates, Newark, DE) for antegrade cerebral perfusion. The graft subsequently became infected with Pseudomonas and formed a pseudoaneurysm with resultant tracheal compression. The presentation, diagnosis, and management of this mycotic pseudoaneurysm are described.

Long-term Results Using Glutaraldehyde-treated Homograft Pericardium in Congenital Heart Surgery.

BACKGROUND: This study reports the long-term outcomes using glutaraldehyde-treated cryopreserved homograft pericardium (CPH) in neonates, infants, children, and young adults undergoing congenital cardiac surgery. METHODS: A retrospective review was performed of all patients at a single institution (Rady Children's Hospital, San Diego, CA) who had undergone surgical implantation with CPH between 2006 and 2016. The study identified 134 consecutive patients who underwent implantation of a total of 276 patches. The baseline demographic characteristics, primary cardiac diagnosis, surgical characteristics, operative reports, and postoperative catheterization and reoperation reports were analyzed. The use of CPH was categorized by specific anatomic insertion site. RESULTS: The median age at patch implantation was 1.47 years (range, 1 day to 31.6 years). The numbers and locations of patch use were 124 for pulmonary arterial repair, 57 for repair of the aorta, 49 for septal repair, and 43 at other sites. At a median follow-up of 5.29 years, 9 patients had died (6.7%), but none of those deaths were related to CPH. Twelve patients (8.96%) underwent reoperations, and 18 patients (13.4%) underwent catheter interventions at sites of CPH implantation. The 10-year freedom from patch-induced reoperation and catheter intervention rates were 88.5% and 86.9%, respectively. Overall patch failure-free survival was 85.8% and 79.0% at 5 and 10 years, respectively. CONCLUSIONS: The use of CPH patch in the surgical correction of congenital heart disease is effective and durable, as evidenced by the low reintervention rates. These results are comparable to the early and midterm outcomes of other similarly used surgical patches.

Prevalence of Complications Following Unifocalization and Pulmonary Artery Reconstruction Procedures.

BACKGROUND: Unifocalization and pulmonary artery reconstructions have been developed to treat complex disorders of pulmonary artery development. These procedures require extremely long periods of cardiopulmonary bypass (CPB) to facilitate surgical repair. The objective of this study was to document the prevalence of complications in patients undergoing unifocalization or pulmonary artery reconstructions associated with prolonged periods of CPB. METHODS: This was a retrospective review of 100 consecutive patients who underwent unifocalization (n = 66) or pulmonary artery reconstructions (n = 34) with CPB times in excess of five hours. Thirty-eight of these operations were primary procedures, whereas 62 were reoperations. RESULTS: The median age at surgery was 15 months, median duration of CPB was 473 minutes, median number of postoperative complications was 5, and the median length of hospital stay was 24 days. The most frequently encountered complications were low cardiac output (43%), open sternum (40%), reintubation (24%), arrhythmia (17%), and bronchoscopy (17%). There was a correlation between the total number of complications and overall length of hospital stay (R2 = 0.64). Major adverse cardiac events (MACE) occurred in 11 patients with one hospital mortality. Patients who experienced MACE had a median length of stay that was 35 days longer (56 vs 21 days) than patients who did not experience MACE. CONCLUSIONS: The data demonstrate that complications were relatively frequent in this cohort of patients and had a linear association with hospital length of stay. Major adverse cardiac events were encountered at a modest prevalence but had a profound impact on measures of outcome.

Arterial Switch Operation in a Patient With Ehlers-Danlos Syndrome Type IV.

Ehlers-Danlos syndrome vascular type IV is characterized by translucent skin, easy bruising, and fragility of arteries. A full-term female infant presented at four weeks of age with a diagnosis of d-transposition of the great arteries with restrictive atrial septal defect. She successfully underwent emergent balloon atrial septostomy and placement of patent ductus arteriosus (PDA) stent. She required restenting of the PDA and pulmonary artery banding prior to arterial switch procedure. At 16 months of age, the patient successfully underwent arterial switch procedure without complication. This report demonstrates the feasibility of an arterial switch operation along with long-term follow-up of this rare condition.

Epicardial Intraoperative Three-Dimensional Mapping of Wolff-Parkinson-White Syndrome in a Child With Ebstein's Anomaly.

Catheter ablation of arrhythmias in small patients has inherent significant risk because of the patient, catheter, and lesion size. Heart block, perforation, and coronary damage may occur. This report shows adaptation of a three-dimensional electroanatomic mapping system for intraoperative use in a small patient with Ebstein's anomaly who was undergoing a cone procedure and a bidirectional Glenn procedure. Use of this technique gave clear identification of the epicardial insertion points of two accessory pathways causing Wolff-Parkinson-White syndrome and resistant episodes of supraventricular tachycardia. Successful three-dimensional mapping and cryoablation were performed with a short procedure time and no complications. The patient remains without preexcitation or arrhythmia 6 months postoperatively.

Intraoperative Three-Dimensional Mapping of Supraventricular Tachycardia in a Young Fontan Patient.

Preoperative tachycardia is uncommon before an initial Fontan procedure. Catheter intervention can risk complications in small patients. A patient with fetal tachycardia and double inlet left ventricle underwent Norwood and bidirectional Glenn procedures. She had persistent tachycardia. A three-dimensional electroanatomic mapping system was modified for the operative procedure; it was used to identify the arrhythmia substrate and cryoablate an atrioventricular nodal tachycardia. Mapping added 20 minutes to the operation. She is arrhythmia-free and off medication after 4 months. This three-dimensional system was successfully adapted for an intraoperative SVT ablation in this small patient, and it may be useful in other settings.

Transcatheter stenting of the systemic-to-pulmonary artery shunt: A 7-year experience from a single tertiary center.

BACKGROUND: Systemic-to-pulmonary artery shunt (SPS) dysfunction can be deleterious in shunt dependent patients and traditionally have undergone surgical revision. Data on transcatheter stenting of SPS is limited. We sought to evaluate feasibility, safety and outcomes of stenting SPS. METHODS: Retrospective review of all patients who underwent transcatheter SPS stenting from 1/2006 to 12/2013. RESULTS: Of 229 surgically implanted SPS, 25 transcatheter stent interventions were performed in 22 patients. The majority had pulmonary atresia (n = 9) or HLHS (n = 10). Their median age was 4 ms (range 10 days to 4 years) and median weight 4.9 kg (range 3-14). Nine had a central and 15 had a BT shunt with a median shunt size of 3.75 mm (range 3-6). The interval from shunt placement to intervention was 1.9 ms (range 4 days-3.8 years). The indication for intervention was increasing cyanosis in10 patients and delaying final repair in 9. Two patients were on ECMO at the time of intervention. The median shunt diameter increased from 2.3 to 4.1 mm and oxygen saturation from 72 to 85% (P < 0.001). No intra-procedural complications were encountered. One patient died from aspiration (autopsy demonstrated a patent shunt), 13 progressed to repair or next stage, 6 remain palliated with shunts as they are deemed unfit for the next stage and 2 are awaiting surgery. CONCLUSIONS: Stenting of systemic to pulmonary artery shunt is a safe and effective procedure and avoids surgical re-intervention. It can be performed both as a rescue procedure in patients with acute shunt occlusion and as an elective procedure to palliate patients not yet suitable for subsequent corrective or staged repair.

Intermediate Results of Hybrid Versus Primary Norwood Operation.

BACKGROUND: In 2007 we began a hybrid program for hypoplastic left heart syndrome (HLHS) variants to potentially improve outcome in high-risk patients. During implementation we offered both hybrid and Norwood approaches to all risk categories. The purpose of this study was to perform a comparative analysis of intermediate survival. METHODS: Newborns were evaluated jointly for high-risk characteristics, including birth weight less than 2.5 kg, prematurity (especially < 35 weeks), central nervous system abnormalities, multiorgan failure, intact or severely restrictive atrial septum, severe ventricular dysfunction, and severe atrioventricular valve regurgitation. We prefer Norwood for standard risk and hybrid for high risk, but all groups crossed over into all treatment pathways resulting in the following 5 treatment groups: standard risk Norwood; high-risk Norwood; standard risk hybrid ductal stent (HDS); high-risk hybrid DS; and high-risk hybrid prostaglandin E1 (HPGE). We reviewed all consecutive patients from 2007 to 2012, obtained follow-up, and analyzed the results. RESULTS: Sixty-eight newborns presented (median 2.96 kg, 8 days); 29 (43%) were high and 39 (57%) were standard risk. There were 14 stage I hospital deaths strongly associated with risk: 3 of 39 standard (7.7%) and 11 of 29 high (38%, p = 0.002). Stage I discharge mortality was highest for high-risk Norwood and high-risk HPGE groups (p < 0.001). Actuarial survival up to 5 years demonstrated superior survival for Norwood versus hybrid (78.1% vs 56.4%, p = 0.0182). With risk stratification there was suboptimal survival for all 3 high-risk groups (p = 0.003); HDS fared better than HPGE but had higher birth weight (p < 0.001). CONCLUSIONS: While a risk-stratified approach for HLHS variant patients with selective use of hybrid palliation resulted in acceptable stage I mortality, the longer term mortality for high-risk patients remains higher than for standard risk regardless of treatment modality. Intrinsic patient risk factors (rather than treatment modality) likely determine long-term outcome in experienced centers. Our current high-risk approach has evolved to HPGE application with Norwood conversion whenever deemed medically possible.

Comparison of ductal stenting versus surgical shunts for palliation of patients with pulmonary atresia and intact ventricular septum.

OBJECTIVES: We sought to compare ductal stenting (DS) to systemic-pulmonary artery shunt (SPS) in a contemporary cohort of patients having pulmonary atresia with intact ventricular septum (PAIVS). BACKGROUND: Alternative palliative strategies (interventional versus surgical) are currently available for initial palliation of ductal dependent patients with various forms of congenital heart disease. There is little data available to guide strategy selection. METHODS: A retrospective review of patients with PAIVS at our institution from Jan 2006 to Dec 2013 was performed. Demographic, echocardiographic, procedural, and follow-up data were reviewed and analyzed. RESULTS: Twenty-nine patients were identified. Thirteen underwent DS and 16 underwent SPS. In the DS group, 6 had radiofrequency perforation of the pulmonary valve, and 12 underwent balloon pulmonary valvuloplasty (BPV) prior to DS. SPS was performed after perforation of the pulmonary valve in 1 patient and after BPV in 4 patients. Procedural success was 92.8% in the DS group versus 93.3% in the SPS group. In the DS group, one patient required emergency shunting due to intra-procedural ductal spasm. In the SPS group there was one procedure related death, and there were six significant procedure-related complications. Five patients required re-interventions prior to hospital discharge. The median length of hospital stay for the DS versus SPS group was 10 versus 23 days, respectively (P < 0.05). Post discharge, there were seven reinterventions in the DS group and two reinterventions in the SPS group. CONCLUSIONS: DS may provide a safe and effective alternative to SPS in selected patients with PAIVS.

QTc prolongation in children following congenital cardiac disease surgery.

UNLABELLED: IntroductionQTc prolongation has been reported in adults following cardiopulmonary bypass; however, this phenomenon has not been studied in children with congenital cardiac disease. This study's aim was to formally assess QTc in children undergoing cardiac surgery. METHODS: Pre-operative and post-operative electrocardiograms during hospital stays were prospectively analysed on 107 consecutive patients under 18 years of age undergoing cardiac surgery. QTc was measured manually in leads II, V4, and V5. Measurements of 440 and 480 milliseconds were used to categorise patients. Peri-procedural data included bypass and cross-clamp time, medications, and electrolyte measurements. Outcome data included arrhythmias, length of mechanical ventilation, and hospital stay. Patients with post-operative new bundle branch block or ventricularly paced rhythm were excluded. RESULTS: In all, 59 children were included, out of which 26 had new QTc over 440 milliseconds and 6 of 59 had new QTc over 480 milliseconds post-operatively. The mean increase in post-operative QTc was 25 milliseconds, p=0.0001. QTc over 480 was associated with longer cross-clamp time, p=0.003. Other risk factors were not associated with post-operative QTc prolongation. This phenomenon was transient with normalisation occurring in 67% of patients over 60 hours on average. One patient with post-operative QTc over 440 milliseconds developed ventricular tachycardia. There was no correlation between prolonged QTc and duration of mechanical ventilation, or hospital stay.ConclusionA significant number of children undergoing cardiac surgery showed transient QTc prolongation. The precise aetiology of QT prolongation was not discerned, though new QTc over 480 milliseconds was associated with longer cross-clamp time. In this cohort, transient QTc prolongation was not associated with adverse sequela.

Can randomized clinical trials impact the surgical approach for hypoplastic left heart syndrome?

The Eighth International Conference of the Pediatric Cardiac Intensive Care Society was held in Miami, Florida, December 8 to 11, 2010. The program included a session dedicated to the management of hypoplastic left heart syndrome (HLHS), with particular emphasis on the innovations that have led to contemporary schemes of management and the role of clinical trials in the evolution and acceptance of these strategies. An invited panel of experts reviewed the historical evolution of staged surgical reconstruction, the randomized clinical trials that have been undertaken thus far, and the extent to which these have, or have not, influenced individual and institutional approaches to management of HLHS.

An epicardial pacing safety net: an alternative technique for pacing in the young.

Epicardial pacing is the standard approach for permanent pacing in small children and patients with functionally univentricular physiology. The longevity of epicardial leads, however, is compromised by increased occurrences of exit block and lead fractures. We report our experience with a technique of placing a second ventricular lead, and attaching it to the atrial port of a dual chamber pacemaker to prevent the need for early re-operation in the event of failure of the primary epicardial lead. A retrospective review showed that, over the period from 2001 through 2007, epicardial ventricular pacemakers had been placed in 88 patients. In 6 of these, we had placed 2 ventricular leads, their median weight being 8.0 kilograms, with a range from 4.2 to 31.8 kilograms. Fracture of a lead occurred in 1 of the patients (17%) 8 months after placement, requiring reprogramming to pace from the atrial port. This possibility avoided the need for repeated emergent surgery. At a median follow-up of 1.5 years, with a range from 0.3 to 4.4 years, there have been no complications. During the same time period, overall failure of epicardial leads at our institution was 13%. Placement of a second ventricular epicardial pacing lead, attached to the atrial port of a dual chamber pacemaker, therefore, may provide a safe and effective means of ventricular pacing in the setting of epicardial lead failure, and may obviate the need for repeat, potentially urgent, pacemaker surgery.

Outcome analysis for a small, start-up congenital heart surgery program.

There will be a continuing need to start new congenital heart surgery programs to serve communities experiencing significant population growth. However, small congenital heart programs frequently underperform their larger counterparts. This study summarizes the clinical outcome data for the first 42 months of a small, start-up congenital heart surgery program. Clinical outcomes were summarized from the start of the program in September 2003 through March 2007. Risk adjustment analysis was performed using the risk adjustment in congenital heart surgery (Rachs-1) risk adjustment model and Society of Thoracic Surgeons (STS) congenital database. Three hundred eighty-six operations have been performed during this time period, including 47 newborns, 96 infants, 217 children/adolescents, and 26 adults (greater than 18 years). There have been two operative mortalities (0.5%). Assigning these cases to the Rachs-1 categories, there were 64 level I, 188 level II, 90 level III, 12 level IV cases, with 32 "others." The predicted mortality for the 354 categorized cases calculates to be 17.4 (Rachs-1) and 10.2 (STS). The data demonstrate that a start-up program with a relatively modest surgical volume can achieve satisfactory clinical results. This model has relied upon careful case selection and direct, senior-level surgeon involvement through an affiliation with a university-based program. These results suggest that a small congenital heart surgery program can be successfully started if the circumstances are carefully controlled.