Extended Application of the Hybrid Procedure in Neonates with Left-Sided Obstructive Lesions in an Evolving Cardiac Program

The hybrid approach to management of hypoplastic left heart syndrome (HLHS) was developed as an alternative to neonatal Norwood surgery, providing a less invasive initial palliation for HLHS. We describe our experience in extending the concept of the hybrid procedure to palliate neonates with anatomically compromised systemic arterial blood flow in a variety of congenital cardiac anomalies and supporting its application as first-line palliation in centers developing their HLHS programs. Retrospective review of patients undergoing therapy for HLHS at a single institution from June 2008 to December 2014 was performed. Subject demographics, clinical and procedural data, along with follow-up, were collected. Thirteen patients had initial hybrid palliation for HLHS during the time frame indicated at a median age of 8 days (range 1–29 days) and median weight of 3.4 kg (range 2.4–4.6 kg). Diagnoses included typical HLHS (n = 6), right-dominant unbalanced atrioventricular septal defect with arch hypoplasia (n = 4), double outlet right ventricle [subpulmonic VSD (n = 1) and intact ventricular septum (n = 1)] with hypoplastic transverse aortic arch and borderline left ventricular dimensions. Standard approach with bilateral pulmonary artery banding and ductal stenting was carried out in all thirteen patients. Two patients required two ductal stents at the time of index procedure. There were no intraprocedural complications. Median intubation length post-procedure was 4 days (range 1–74 days). Median hospital stay post-procedure was 47 days (range 15–270 days). The overall mortality rate on follow-up through comprehensive stage 2 over the 6-year experience was 38 % (5 out of 13). Of note, the mortality rate was significantly lower in the latter 3 years of the study period when the procedure was adopted as a primary palliation for HLHS (14 % or 1 out of 7) compared to the initial 3-year period when it was reserved for higher risk cohorts (67 % or 4 out of 6). Median time to subsequent surgery was 3 months (range 1–4 months). One patient required further ductal stenting on follow-up and developed subsequently airway compression. On median follow-up of 24 months, two patients required pulmonary artery arterioplasty. The hybrid procedure may be used for palliation for a variety of cardiac lesions to avoid high-risk surgery in the neonatal period. This approach may be also an alternative in centers performing lower number of Norwood surgery, which has been associated with higher mortality.     

Outcomes of the Modified Norwood Procedure: Hypoplastic Left Heart Syndrome Versus Other Single-Ventricle Malformations

Recent studies have suggested that survival after the modified Norwood procedure is influenced by anatomy and is worse for patients with hypoplastic left heart syndrome (HLHS) than for patients with other forms of functional single ventricle. This study aimed to compare the outcomes of the modified Norwood procedure for these two groups at a single center. A retrospective chart review compared modified Norwood procedure outcomes between children with HLHS (n = 40) and children with other single-ventricle malformations (n = 19) from January 2002 to August 2012. The HLHS patients had a lower mean age (10.0 ± 13.2 days) at the time of operation than the non-HLHS patients (16.5 ± 18.5 days). The difference was not significant. The number of patients with a Sano modification was significantly higher in the HLHS group (HLHS 85 % vs non-HLHS 32 %; p < 0.001). The follow-up data did not differ between the groups (HLHS 3.6 ± 3.2 years vs non-HLHS 4.8 ± 3.3 years, nonsignificant difference). The overall operative survival was 85 %: 83 % for the patients with HLHS and 89 % for the patients with non-HLHS (nonsignificant difference). The Kaplan–Meier transplant-free survival estimate at 10 years was 73 % and did not differ between the HLHS patients (67 %) and the non-HLHS patients (84 %, nonsignificant difference). The timing of stages 2 and 3 palliation (bidirectional Glenn and external conduit Fontan) did not differ significantly (HLHS, 7.0 ± 2.8 months and 3.0 ± 0.5 years vs non-HLHS 6.1 ± 1.7 months and 2.8 ± 0.8 years). The two groups were similar with respect to the need for post-Norwood extracorporeal membrane oxygenation support, aortic arch reinterventions, atrioventricular valve repair, pulmonary artery reconstruction, pacemaker implantation, and progression to bidirectional Glenn and Fontan completion. The modified Norwood reconstruction provides good palliation for HLHS as well as other forms of functional single-ventricle anatomy. Survival, postoperative morbidity, and progression to Glenn and Fontan are not significantly different.     

Risk Factors for Interstage Death After the Norwood Procedure

Operative survival after the Norwood procedure has significantly improved during the past 10 years. However, there remains attrition among Norwood survivors before reaching planned second-stage palliation. The purpose of this study was to evaluate potential risk factors for interstage mortality among Norwood survivors. All patients undergoing the Norwood procedure at the Medical University of South Carolina from January 1996 through January 2001 were retrospectively reviewed. Patient and procedural variables were examined as potential risk factors for interstage mortality. Among 50 Norwood survivors, 8 (16%) died prior to second-stage palliation. The mean age at death was 102 ± 72 days (median, 61; range, 35–208). By multivariate analysis, the presence of an arrhythmia in the postoperative period (p = 0.02) and decreased ventricular function at hospital discharge (p = 0.05) were identified as risk factors for interstage mortality. There remains a significant risk for interstage mortality among Norwood survivors. Patients with postoperative arrhythmias and/or decreased ventricular function at discharge are at increased risk for interstage death after Norwood procedure. More frequent follow-up and aggressive medical management of arrhythmia or decreased function may be warranted for these high-risk patients.     

Clinical Outcomes and Resource Use for Infants With Hypoplastic Left Heart Syndrome During Bidirectional Glenn: Summary From the Joint Council for Congenital Heart Disease National Pediatric Cardiology Quality Improvement Collaborative Registry

The National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) registry captures information on interstage management of infants with hypoplastic left heart syndrome (HLHS). The purpose of this study was to identify interstage risk factors for increased resource use and adverse outcomes during bidirectional Glenn (BDG) hospitalization. All infants in the NPC-QIC registry (31 United States hospitals) undergoing BDG surgery were included (December 2009 to August 2010). Patient demographics, interstage variables, operative procedures, and complications were recorded. Days of hospitalization, ventilation, inotrope use, and complications were surrogates of resource use. Logistic regression analysis determined the associations between predictor variables and resource use. Of 162 infants, 105 (65 %) were males. At BDG, the median age was 155 days (range 78–128), mean weight-for-age z-score was ?1.6 ± 1.1, mean length-for-age z-score was ?1.5 ± 1.7, and mean preoperative oxygen saturation was 78 % ± 7 %. Caloric recommendations were met in 60 % of patients, and 85 % of patients participated in a home-surveillance program. Median days of intubation, inotrope use, and hospitalization were 1, 2, and 7, respectively. There were 4 post-BDG deaths and 55 complications. In multivariate analysis, lower weight-for-age z-score, female sex, and aortic atresia with mitral stenosis were associated with a higher risk of BDG complications. Meeting caloric recommendations before BDG was associated with fewer hospitalization days. Lower weight-for-age z-score was an independent and potentially modifiable risk factor for BDG complications. HLHS infants who met caloric recommendations before BDG had a lower duration of hospitalization at BDG. These data justify targeting nutrition in interstage strategies to improve outcomes and decrease costs for patients with HLHS.     

Fate of Ventricular and Valve Performance Following Early Bidirectional Glenn Procedure After Norwood Operation Controlled for Hypoplastic Left Heart Syndome Anatomic Subtype

The Norwood operation (NO) with a right ventricle (RV)-to-pulmonary artery (PA) shunt (NRVPA) is reportedly associated with early hemodynamic advantage. Shunt strategy has been implicated in ventricular function. Outcomes after NRVPA compared with classic procedure as part of a strategy involving early bidirectional Glenn (BDG) procedure were analyzed with reference to RV, tricuspid, and neoaortic valve performance. Between January 2005 and December 2010, 128 neonates with hypoplastic left heart syndrome (HLHS) underwent NO. Controlled for aortic/mitral stenosis (AS–MS) subtype, 28 patients underwent NRVPA (group A), and 26 patients had classic procedure (group B). The patients with a non-HLHS single-ventricle anatomy and those who had undergone a hybrid approach for HLHS were excluded from the study. The mean age at NO was 6.8 ± 3.5 days in group A and 6.9 ± 3.6 days in group B. Transthoracic echocardiographic evaluation (TTE) after NO (TTE-1) at the midinterval between NO and BDG (TTE-2), before BDG (TTE-3), before Fontan (TTE-4), and at the last follow-up evaluation (TTE-5) was undertaken. Cardiac catheterization was used to assess hemodynamic parameters before the Glenn and Fontan procedures. The operative, interstage, and pre-Fontan survival rates for AS–MS after NO were respectively 88.1 % (90.3 % in group A vs. 84.7 % in group B; p = 0.08), 82.5 % (82.7 % in group A vs. 81.8 % in group B; p = 0.9), and 80.7 % (79.5 % in group A vs. 81.8 % in group B; p = 0.9). The median follow-up period was 39.6 months (interquartile range 2.7–4.9 months). The RV global function, mid- and longitudinal indexed dimensions, fractionated area change before BDG (TTE-1, TTE-2, TTE-3) and after BDG (TTE-4, TTE-5), and right ventricular end-diastolic pressure did not differ statistically between the groups (p > 0.05). No statistically significant difference in tricuspid or neoaortic intervention was found between the groups (p > 0.05). Controlled for the AS–MS HLHS subtype, shunt strategy showed no midterm survival or hemodynamic (ventricular or valve) impact. At midterm, the follow-up need for neoaortic or tricuspid valve surgical intervention was not affected by shunt selection. The structural ventricular adaptation after reversal of shunt physiology was irrespective of shunt strategy.     

Right-Ventricular Global Longitudinal Strain May Predict Neo-Aortic Arch Obstruction After Norwood/Sano Procedure in Children With Hypoplastic Left Heart Syndrome

Neo-aortic arch obstruction (NAAO) is a common complication following the Norwood/Sano procedure (NP) for hypoplastic left heart syndrome (HLHS) and is associated with increased morbidity and mortality. However, there is currently no objective method for predicting which patients will develop NAAO. This study was designed to test the hypothesis that hemodynamic changes from development of NAAO after NP in patients with HLHS will lead to changes in myocardial dynamics that could be detected before clinical symptoms develop with strain analysis using velocity vector imaging. Patients with HLHS who had at least one cardiac catheterization after NP were identified retrospectively. Strain analysis was performed on all echocardiograms preceding the first catheterization and any subsequent catheterization performed for intervention on NAAO. Twelve patients developed NAAO and 30 patients never developed NAAO. Right ventricular strain was worse in the group that developed NAAO (?6.2 vs. ?8.6 %, p = 0.040) at a median of 59 days prior to diagnosis of NAAO. Those patients that developed NAAO following NP were significantly younger at the time of first catheterization than those that did not develop NAAO (92 ± 50 vs. 140 ± 36 days, p = 0.001). This study demonstrates that right ventricular GLS is abnormal in HLHS patients following NP and worsening right ventricular strain may be predictive of the future development of NAAO.     

Midterm Survival of Infants Requiring Postoperative Extracorporeal Membrane Oxygenation After Norwood Palliation

This study reports the mid-term survival for neonates undergoing extracorporeal membrane oxygenation (ECMO) after Norwood palliation at a single center. Limited data exist on the mid-term survival of patients undergoing ECMO after Norwood palliation. We reviewed our ECMO experience from July 1994 to October 2008 and compared two groups: patients who required ECMO after Norwood palliation and patients who underwent Norwood palliation without ECMO. We analyzed 30-day survival, survival to hospital discharge, and survival to most recent follow-up. One hundred sixty patients underwent Norwood palliation for hypoplastic left heart syndrome (HLHS) and its variants. A total of 32 patients (20 %) required postoperative ECMO. Using Kaplan–Meier analysis, the predicted survival rates for Norwood/non-ECMO patients to 30 days, 1 year, and 3 years after the procedure are 87.6 % (CI 79.5–91.5 %), 62.5 % (CI 54.3–71.0 %), and 59.9 % (CI 50.8–67.8 %), respectively. Survival to 30 days, 1 year, and 3 years after Norwood was significantly decreased in Norwood/ ECMO patients, with predicted survival rates of 50.0 % (CI 31.9–65.7 %), 24.6 % (CI 11.4–40.4), and 13.2 % (CI 3.9–28.3 %), respectively (p < 0.0001). Risk factors for hospital mortality included nonelective or emergency placement onto ECMO, longer duration of ECMO support, and the development of acute renal failure while on ECMO. Of the original Norwood/ECMO hospital survivors, only half of these patients survived a mean of nearly 4 years. ECMO after Norwood palliation is associated with significant mortality. Our data suggest that neonates who require ECMO after Norwood palliation are prone to continued attrition once discharged from the hospital.     

Hypoplastic Left Heart Syndrome and Aortic Atresia-Mitral Stenosis Variant: Role of Myocardial Protection Strategy and Impact of Ventriculo-coronary Connections After Stage I Palliation

Aortic atresia-mitral stenosis (AA-MS) has been implicated as a determinant of outcome after Stage-1 palliation (S1P) in hypoplastic left heart syndrome (HLHS).Studies evaluating the association of AA-MS with ventriculo-coronary connections (VCC) and mortality report conflicting results. The significance of VCC, myocardial protection, and shunt strategy after S1P has yet to be determined. Between January 2005 and July 2009, 100 neonates with HLHS underwent S1P. Mitral and aortic valves and presence of VCC were assessed. Antegrade continuous cold blood cardioplegia was administered throughout the vast extent of the neo-aortic reconstruction. A right ventricle-to-pulmonary shunt was used for an ascending aortic diameter of 0.6 mm/kg or less. Survival analysis was performed to determine predictors and assess impact of AA-MS and VCC on hospital and interstage mortality. Twenty-seven (of 100) patients had AA-MS. The mean age and weight at S1P were 6.5 ± 2.8 days and 3.09 ± 0.47 kg, respectively. VCC were found in 56% of AA-MS. Twenty-two had Norwood-Sano, 3 had classic Norwood, and 2 had hybrid S1P. VCC were associated with AA-MS, endocardial fibroelastosis, and ascending aortic size <2 mm (P < 0.05) but not higher mortality (P = ns). Operative and interstage survival for AA-MS after S1P was 85.2 and 71%, respectively (not statistically different compared to all other subtypes; P = ns). Actuarial survival after S1P at 1, 3, 6, 12, and 36 months was 92.9 ± 4.9, 78.6 ± 7.8, 75 ± 8.2, 71.3 ± 8.3, and 71.3 ± 8.3%, respectively. Intact atrial septum and post-S1P renal dysfunction (P < 0.05) were independent predictors of hospital and interstage mortality. In patients with HLHS, AA-MS carries no survival disadvantage after S1P during the hospital and interstage period regardless of VCC. Intact atrial septum and post-S1P renal dysfunction predict early and interstage mortality. Myocardial protection and shunt strategy might influence the outcome in this HLHS variant.     

Center Variability in Timing of Stage 2 Palliation and Association with Interstage Mortality: A Report from the National Pediatric Cardiology Quality Improvement Collaborative

For infants with single-ventricle lesions with aortic arch hypoplasia, the interstage period from discharge following stage 1 palliation (S1P) until stage 2 palliation (S2P) remains high risk. Significant variability among institutions exists around the timing of S2P. We sought to describe institutional variation in timing of S2P, determine the association between timing of S2P and interstage mortality, and determine the impact of earlier S2P on hospital morbidity and mortality. The National Pediatric Cardiology Quality Improvement Collaborative registry was queried. Centers were divided based on median age at S2P into early (n = 15) and late (n = 16) centers using a cutoff of 153 days. Groups were compared using Chi-squared or Wilcoxon rank-sum test. Multivariable logistic regression was used to determine risk factors for interstage mortality. The final cohort included 789 patients from 31 centers. There was intra- and inter-center variability in timing of S2P, with the median age by center ranging from 109 to 214 days. Late centers had a higher mortality (9.9 vs. 5.7 %, p = 0.03) than early centers. However, the event rate (late: 8.2 vs. early: 5.8 deaths per 10,000 interstage days) was not different by group (p = 0.26). Survival to hospital discharge and hospital length of stay following S2P were similar between groups. In conclusion, in a large multi-institution collaborative, the median age at S2P varies among centers. Although optimal timing of S2P remains unclear, centers performing early S2P did not experience worse S2P outcomes and experienced less interstage mortality.     

Normal Interstage Growth After the Norwood Operation Associated With Interstage Home Monitoring

After stage 1 palliation (S1P) with a Norwood operation, infants commonly experience growth failure during the initial interstage period. Growth failure during this high-risk period is associated with worse outcomes. This study evaluated the growth patterns of patients enrolled in the authors?? interstage home-monitoring program (HMP), which uses a multidisciplinary team approach to nutrition management. From 2000 to 2009, 148 infants were enrolled in the HMP after S1P. Families recorded daily weights during the interstage period and alerted the interstage monitoring team about protocol violations of nutritional goals. Interstage monitoring and inpatient data from the S1P hospitalization were reviewed to identify risk factors for poor growth. Growth outcomes were compared with published norms from the Centers for Disease Control. Interstage survival for patients in the HMP was 98?% (145/148). Growth velocity during the interstage period was 26?±?8?g/day. The weight-for-age z-scores decreased from birth to discharge after S1P (?0.4?±?0.9 to ?1.3?±?0.9; p?<?0.001) but then increased during the interstage period to the time of S2P (?0.9?±?1; p?<?0.001). The factors associated with improved growth during the interstage period included male gender, greater birth weight, full oral feeding at S1P discharge, and a later birth era. After S1P, infants enrolled in an HMP experienced normal growth velocity during the interstage period. Daily observation of oxygen saturation, weight change, and enteral intake together with implementation of a multidisciplinary feeding protocol is associated with excellent interstage growth and survival.     

Results of a Feeding Protocol in Patients Undergoing the Hybrid Procedure

Neonates with single-ventricle physiology are at increased risk of developing gastrointestinal morbidities. Feeding protocols in this patient population have been shown to decrease feeding complications after the Norwood procedure, but no data exist to determine the effectiveness of a feeding protocol in patients undergoing the hybrid procedure. Goal of this study was to examine the impact of a standardized feeding protocol on the incidence of overall postoperative gastrointestinal morbidity after the hybrid procedure. Retrospective chart review was performed on neonates undergoing the hybrid procedure. Neonates were divided into two groups, pre-feeding protocol (pre-FP), which encompassed the years 2002–2008, and post-feeding protocol (post-FP), which encompassed the years 2011–2014. Preoperative, operative, and postoperative data were collected. T test or Fisher’s exact test was used for analysis. p < 0.05 was considered significant. Seventy-three neonates were in the pre-FP and 52 neonates were in the post-FP. There were no significant differences between the pre-FP and the post-FP in cardiac diagnosis (62 HLHS, 11 other vs. 39 HLHS, 13 other, respectively). Pre-FP underwent hybrid procedure later than the post-FP (9.1 ± 5.8 vs. 5.7 ± 3.4 days, respectively, p < 0.01) and achieved full enteral feeds earlier than the post-FP (3.2 + 2.9 vs. 7.8 + 3.9 days, respectively, p < 0.01). The incidence of necrotizing enterocolitis was higher in the pre-FP versus post-FP [11.0 % (8/65) vs. 5.8 % (3/49), respectively, p = 0.36]. Though not significant, the incidence of necrotizing enterocolitis decreased by almost 50 % after initiating a feeding protocol in patients undergoing the hybrid procedure. This is consistent with previous studies showing beneficial results of a feeding protocol in this complex patient population.     

Supplemental Tube Feeding Does Not Mitigate Weight Loss in Infants with Shunt-Dependent Single-Ventricle Physiology

Infants with shunt-dependent single-ventricle (SV) physiology are at risk for poor weight gain before superior cavopulmonary connection (SCPC). Lower weight-for-age z-score is a risk factor for prolonged length of stay (LOS) after SCPC. We sought to characterize infant growth and feeding and determine the effect of method of feeding on outcomes. Chart review of infants with shunt-dependent SV physiology born between October 2007 and September 2010 was performed. The cohort was divided into three groups based on feeding method at discharge after initial palliation; 53 in the oral feeding (PO) group, 56 in the nasogastric (NG) tube group, and 26 in the gastrostomy tube (GT) group. Birth weight z-score did not differ among groups (p = 0.39), but infants fed by NG or GT were smaller than PO-fed infants at hospital discharge (p = 0.0001), a difference that persisted through SCPC (p < 0.0001). Predictors of need for GT included Norwood procedure (p = 0.008) and longer LOS after initial palliation (p < 0.001). Interstage mortality and age at SCPC did not differ among groups. Risk factors for longer LOS at SCPC included longer LOS and need for supplemental feeds at discharge from initial palliation as well as lower weight at SCPC. Poor growth is common among infants with shunt-dependent SV physiology. Infants who require GT have lower weight-for-age z-scores at hospital discharge and remain smaller at SCPC than those fed PO. Although GT after initial palliation is associated with longer LOS after SCPC, it is not associated with an increase in interstage morbidity or mortality.     

Factors Associated with Serum B-Type Natriuretic Peptide in Infants with Single Ventricles

Data regarding the value of B-type natriuretic peptide (BNP) measurements in infants with a single-ventricle (SV) physiology are lacking. This analysis aimed to describe the BNP level changes in infants with an SV physiology before and after superior cavopulmonary connection (SCPC) surgery. Levels of BNP were measured by a core laboratory before SCPC (at 5.0 ± 1.6 months) and at the age of 14 months during a multicenter trial of angiotensin-converting enzyme inhibition therapy for infants with SV. Multivariable longitudinal analysis was used to model the associations between BNP levels and three sets of grouped variables (echocardiography, catheterization, growth). Multivariable analysis was performed to assess associations with patient characteristics at both visits. Associations between BNP levels and neurodevelopmental variables were investigated at the 14 month visit because neurodevelopmental assessment was performed only at this visit. The BNP level was significantly higher before SCPC (n = 173) than at the age of 14 months (n = 134). The respective median levels were 80.8 pg/ml (interquartile range [IQR], 35–187 pg/ml) and 34.5 pg/ml (IQR, 17–67 pg/ml) (p < 0.01). A BNP level higher than 100 pg/ml was present in 72 subjects (42 %) before SCPC and in 21 subjects (16 %) at the age of 14 months. In the 117 patients who had BNP measurements at both visits, the median BNP level decreased 32 pg/ml (IQR, 1–79 pg/ml) (p < 0.01). In the longitudinal multivariable analysis, higher BNP levels were associated with a higher end-systolic volume z-score (p = 0.01), a greater degree of atrioventricular (AV) valve regurgitation (p < 0.01), a lower weight z-score (p < 0.01), and a lower length z-score (p = 0.02). In multivariable analyses, a higher BNP level at the age of 14 months was associated with arrhythmia after SCPC surgery (p < 0.01), a prior Norwood procedure (p < 0.01), a longer hospital stay after SCPC surgery (p = 0.04), and a lower Bayley psychomotor developmental index (p = 0.02). The levels of BNP decreases in infants with SV from the pre-SCPC visit to the age of 14 months. A higher BNP level is associated with increased ventricular dilation in systole, increased AV valve regurgitation, impaired growth, and poorer neurodevelopmental outcomes. Therefore, BNP level may be a useful seromarker for identifying infants with SV at risk for worse outcomes.     

Pain Management After Comprehensive Stage 2 Repair for Hypoplastic Left Heart Syndrome

Achieving optimal pain control for children after complex cardiac surgery can be challenging. Recently, the hybrid approach to palliation of hypoplastic left heart syndrome (HLHS) was introduced as an alternative to the classic Norwood procedure. The second stage of the hybrid approach is a complex procedure known as comprehensive stage 2 (CS2). The authors have noted that pain control after the CS2 procedure is particularly difficult to manage. This report presents a review of the authors’ pain management strategy in this clinical scenario and evaluates its efficacy. The medical records of patients who underwent CS2 repair of the hybrid procedure for HLHS between June 2008 and August 2011 were retrospectively reviewed. As a comparative group with a similar physiology, patients undergoing an isolated Glenn procedure also were reviewed. In addition to demographic data, the intraoperative use of narcotics and other adjunct medications for analgesia and sedation was recorded. Postoperatively, the mode of analgesia, the total opioid administered during the first 48 h postoperatively, and the nursing-assessed patient pain scores were recorded. Any adverse drug effect or need to adjust the analgesic regimen was recorded, as well as the timing of tracheal extubation. During the study period, 36 patients ranging in age from 4 to 14 months underwent the CS2 procedure, and 21 patients underwent a Glenn procedure. After CS2 repair, fentanyl was the opioid initially prescribed for all but 2 of the 21 patients managed with the nurse-controlled analgesia (NCA) delivery method and 15 patients managed with continuous infusion. After the Glenn shunt, all patients were prescribed NCA, with 20 patients receiving fentanyl and 1 patient receiving hydromorphone. The use of intraoperative dexmedetomidine caused a decrease in the total narcotic requirements, although this did not reach a statistical significance for either the extubated or intubated patients after CS2. The extubated patients who underwent the Glenn procedure received a smaller total equivalent dose of fentanyl during the first 24 h (29.02 ± 10.6 μg/kg) than did the extubated patients after the CS2 procedure, who received an average of 37.92 ± 8.5 μg/kg (P = 0.02). During the second 24 h, the extubated Glenn patients continued to receive less fentanyl, at an average dose of 7.02 ± 11.5 μg/kg compared with 27.7 ± 23.1 μg/kg for the CS2 patients (P = 0.02). The extubated patients who underwent the Glenn procedure required less NCA time (33.68 ± 17.7) than the CS2 patients (57.9 ± 31.8 h) (P = 0.04). Dexmedetomidine use with the CS2 patients resulted in a trend toward lowering of the total fentanyl dose, but this did not reach statistical significance. The intubated patients who received dexmedetomidine after the CS2 procedure had less NCA time (61.7 ± 39.2 vs. 128.1 ± 100 h; P = 0.02). After the CS2 procedure for palliation of HLHS, patients experience a complex pain profile that differs from the pain associated with the traditional Glenn procedure. This group of patients generally can be managed with fentanyl NCA. Achieving a balance between a proper level of analgesia and sedation in the setting of early tracheal extubation to optimize postoperative physiology can be challenging. The preliminary data suggest that improvements in pain management should be investigated given that more than 30 % of the pain scores in the CS2 group were in the moderate to severe range compared with 18 % after the Glenn procedure.     

Interventions After Norwood Procedure: Comparison of Sano and Modified Blalock–Taussig Shunt

Improved results have evolved from the modified Norwood procedure (NP). This study compares the incidence of interventions after NP with the Sano (n = 37) and modified Blalock–Taussig (BT n = 70) shunt. Incidence, location, interval of interventions, and weight were retrospectively analysed for 107 neonates undergoing NP during the period from October 2002 to December 2009. Forty-six (43.0 %) patients underwent interventions, mostly for dilatation of the aortic arch ([DAA] n = 26 [24.3 %]; Sano n = 10, BT n = 16, p = 0.6), dilatation of the shunt ([DS] n = 15 [14.0 %]; Sano n = 11, BT n = 4; p = 0.002), or closure of aortopulmonary collaterals ([APC] n = 15 [14.0 %]; Sano n = 3, BT n = 12; p = 0.08). Mean interval after NP and body weight at DAA, DS, and APC were 72.4 ± 18.9, 108.5 ± 15.8, and 110.7 ± 17.8 days and 4.5 ± 1.3, 4.9 ± 1.9, 5.3 ± 1.2 kg, respectively. The interventions were not associated with mortality but with a greater rate of complications (9 of 46 [21.4 %]) compared with the rate after diagnostic catheterization (0 of 45, p = 0.03). Complications included closure of the femoral or subclavian artery (n = 5), cerebral embolic or bleeding events (n = 4), cardiopulmonary resuscitation (n = 3), and temporary heart block (n = 2). Actuarial survival was similar from the postoperative month 8 onward at 78.6 ± 4.9 % (95 % confidence interval [CI] 67.0–86.5 %) for Sano and 78.4 ± 6.8 % (95 % CI 61.4–88.6 %) for BT (p = 0.95). Interventions after NP were common irrespective of shunt type. However, a significantly greater rate of shunt interventions was noted in the Sano group. In particular, interventions addressing the aortic arch and the shunt were related with a significant rate of complications.     

Cardiac Tumors in Infants and Children: Study of 120 Operated Patients

Cardiac tumors in children are rare, and patient series are limited in size. We report 120 children who underwent surgery to treat a cardiac tumor; the tumor type was known in 108 instances. The patients represented <0.1 % of 130,000 cardiac surgeries performed in children from a multi-institutional data base. The most common tumors and the number of patients were rhabdomyoma (n = 42), myxoma (n = 28), and fibromas (n = 10). The remaining 18 tumors of various types occurred in smaller numbers of patients. Nine patients (7 %) died after surgery. The mean age of death was 14 days, and death was most frequent in patients with fibromas (n = 4). Considering the variety of tumors, their varied location in cardiac structures, the patient ages, and the infrequent occurrence in an individual surgeon’s experience, the operative mortality was low.     

Arrhythmias After Stage I Hybrid Palliation in Single-Ventricle Patients

The hybrid procedure is an alternative palliative strategy for patients with single-ventricle physiology. No data exist documenting the incidence of arrhythmias after the hybrid procedure. Goal of this study was to determine the incidence and type of arrhythmias in patients undergoing the hybrid procedure. A retrospective chart review was performed including all patients undergoing the hybrid procedure between January of 2010 through December of 2013. Sixty-five patients underwent the hybrid procedure during this time period (43 HLHS, 22 other). Average gestational age at admission was 37.7 weeks. Average age at time of procedure was 7.6 days. Five patients had documented arrhythmias (7.7 %). Four were supraventricular tachycardias, and 1 was a sinus bradycardia. One patient with arrhythmia died during hospitalization, and another patient with arrhythmia died during the interstage period. Hybrid palliation for patients with single-ventricle physiology has a low incidence of arrhythmias. In this cohort of patients, arrhythmias did not contribute to mortality. There was a trend toward association between arrhythmias and longer total length of hospital stay.     

Current Outcomes of Hypoplastic Left Heart Syndrome With Restrictive Atrial Septum: A Single-Center Experience

Advances in the management of hypoplastic left heart syndrome (HLHS) have resulted in improved survival. However, short and long-term mortality in patients with a restrictive atrial septum remains high. All neonates diagnosed with HLHS from 2003 to 2010 at our institution were evaluated. Patients who underwent atrial septostomy within the first 72 h conformed the restrictive atrial septum group (HLHS-RS). Patients with a non-restrictive communication (HLHS-NRS) formed the control group. Outcomes and survival status were determined from review of medical records. Of the 141 newborns diagnosed with HLHS, 20 (14 %) required intervention for a restrictive atrial septum. Procedural success was achieved in 17/20 (85 %) patients. Complications occurred in ten procedures, two of which were life threatening. No procedural deaths occurred. Overall median follow up was 35.5 months (0.4–104). Initial hospitalization survival was 16/20 (80 %) for the HLHS-RS group and 114/121(94 %) for the HLHS-NRS (p = 0.028). Twenty (14 %) patients were lost to follow up and 9 (6 %) underwent heart transplant. Overall survival was 10/16 (62 %) for HLHSRS patients and 77/95 (81 %) for HLHS-NRS (p = 0.1). Survival after initial discharge was 10/12 (83 %) for the HLHS-RS group and 77/88 (87 %) for the HLHS-NRS (p = 0.67). No predictors for HLHS-RS outcome were identified. Mortality at first-stage palliation in HLHS neonates with a restrictive atrial septum remains higher than in those with an unrestrictive communication. However, survival after initial hospital discharge is similar.     

Exercise Performance Following Repair of Hypoplastic Left Heart Syndrome: A Comparison with Other Types of Fontan Patients

Reports of exercise performance after Fontan surgery for hypoplastic left heart syndrome (HLHS) are lacking. We compared the exercise performance of total cavopulmonary connection type (TCPC) of Fontan subjects with HLHS (group 1, n= 7) to those not requiring a Norwood procedure having a systemic right ventricle (group 2, n= 6) or a systemic left ventricle (group 3, n= 8). The subjects underwent assessment of resting pulmonary mechanics followed by maximal exercise testing with a bicycle or treadmill protocol. ECG, oxygen consumption, and carbon dioxide production were measured continuously. There was not a significant difference seen between HLHS and the comparison groups for the following parameters: maximum heart rate, maximum oxygen consumption, respiratory exchange ratio, breathing reserve, and arterial oxygen saturation at rest or exercise. Exercise performance in the TCPC type of Fontan patients was comparable regardless of ventricular morphology or surgical approach.     

An unexpected diagnosis in children with male phenotype and bilateral nonpalpable gonad: congenital adrenal hyperplasia with female genotype

Purpose

Female pseudohermaphroditism is the most frequent form of ambiguous genitalia in children with congenital adrenal hyperplasia (CAH). However, a small group of children with complete urethral development in contrast to 46XX karyotype can be encountered. We aimed to define the characteristics of patients with 46XX CAH but having fully developed male external genitalia.

Methods

The records of 11 children with CAH and 46XX karyotype but having male phenotype, encountered from 1990 to 2012 were reviewed retrospectively. The age, presenting signs and symptoms, diagnostic studies, surgical procedures, early results and outcome were noted. All patients were evaluated by gender assignment team and the decision of the family was also taken into consideration during gender assignment.

Results

Eleven children (mean age 3.64 ± 3 years) (range 5 days–10 years) were enrolled. The main presenting signs were nonpalpable gonads (n = 7), hyperpigmentation (n = 2), jaundice (n = 1) and electrolyte imbalance (n = 1). All patients had bilateral nonpalpable gonads and male phenotype with mean phallus length of 4.5 ± 1.7 cm. Urethral meatus is located at normal position (n = 6) or hypospadiac (n = 5). Labioscrotal fusion was complete in all cases and they were classified as 4th (n = 3) or 5th (n = 8) degree of virilization according to Prader’s classification. All children had CAH and 46XX genotype. Pelvic ultrasound (n = 8) and genitocystogram (n = 9) were used, and genitocystoscopy was performed (n = 6). Male gender was assigned in most (n = 10) and female gender in one. Corrective surgery could be performed in 10 patients. The surgical procedures were hysterectomy + bilateral salphingo-oophorectomy + vaginectomy (n = 9), chordee release (n = 3) and then second-stage (n = 2) or one-stage urethra repair (n = 1), and pure one-stage urethra repair (n = 1). One case underwent surrenalectomy before the diagnosis of CAH. Mastectomy (n = 1) and fistula repair (n = 3) were additional operations. Only one child could be undergone feminizing genitoplasty and another was lost to follow-up.

Conclusion

Unfortunately, most of the children underwent surgery in adverse to genotype because of constituted sexual identity. Children with male phenotype and bilateral nonpalpable gonads should undergo promptly biochemical analyses for CAH and early chromosomal analysis.