Symptom persistence after vascular ring repair in children.

PURPOSE: Vascular rings are often diagnosed after evaluation for swallowing and breathing difficulties. Data regarding symptoms following vascular ring repair is sparse. We sought to determine whether symptoms persist using chart review and a survey. METHODS: Sixty-three patients underwent open vascular ring repair from July 2007 to May 2018. Data regarding vascular anatomy, demographics, pre- and postoperative symptoms, and chromosomal abnormalities were collected. Freedom from reoperation, 30-day mortality, and complications were assessed. Patient families were contacted for a symptom focused survey. RESULTS: The median age of surgical intervention was 14.4 months (IQR 5.8-34.7 months) for single aortic arches with an aberrant subclavian artery (SAA), and 5.3 months (IQR 1.3-10.1 months) for double aortic arches (DAA) (Table). Prior to surgery, all but two SAA were symptomatic. There was no operative mortality. Three patients required re-exploration for chylothorax, and three required late aortopexy. At last follow-up, 45% (18/40) SAA and 65% (15/23) DAA had post-operative symptoms. Fourteen patient families completed the symptom survey (10 SAA, 4 DAA). Five SAA had breathing and swallowing symptoms, and 3 SAA and 3 DAA had breathing difficulties. CONCLUSIONS: Open vascular ring repair remains a safe repair. However, further investigation of the persistent symptoms in these patients is merited. STUDY TYPE / LEVEL OF EVIDENCE: Retrospective Comparative Study, Level III.

Surgical Versus Percutaneous Closure of PDA in Preterm Infants: Procedural Charges and Outcomes.

BACKGROUND: Studies comparing percutaneous closure of patent ductus arteriosus (PDA) with surgical ligation tend to exclude premature infants and have not assessed procedural charges. We compared our contemporary outcomes and charges of device closure to surgical ligation of PDA in preterm infants. MATERIAL AND METHODS: Preterm infants who underwent isolated PDA closure during their newborn hospitalization (January 2014 to September 2017) were grouped based on intention to treat (surgery versus device closure). Patient demographics, procedural details, and immediate postprocedural outcomes were compared. Procedural charges for device closure versus surgical ligation were compared. RESULTS: Compared with the device group (n = 33), patients undergoing surgical ligation (n = 39) were younger, smaller, and required more preoperative support (P < 0.05). The procedure time was shorter for surgical ligation (P < 0.01). Although there was no procedural mortality in either group, the complication rate was higher for device closure than for surgical ligation (15.2% versus 0%; P = 0.02). The proportion of patients returning to preprocedural respiratory support by 48 h after procedure was similar. There was a higher proportion of surgical patients who required increased inotropic support in the first 24 h after procedure (P = 0.19). The procedural charges for transcatheter device closure were twice as expensive as those for surgical ligation. CONCLUSIONS: In our early experience with percutaneous PDA closure, we found a percutaneous approach in preterm infants feasible and well tolerated. Both surgical ligation and device closure were associated with perioperative or postoperative complications. Procedural charges were higher for percutaneous closure, driven by device charge and catheterization room utilization. Further investigation is needed to establish guidelines for first-line therapy for PDA closure in preterm infants, including cost-benefit analysis.

Anomalous Origin of Left Pulmonary Artery From the Aorta: A Rare Entity in Congenital Heart Disease.

Anomalous origin of the left pulmonary artery (ALPA) from the aorta is a rare form of congenital heart disease, leading to long-term morbidity if not treated early. There is a reported association of this anomaly with 22q11 microdeletion syndrome. Surgical repair is commonly performed by direct reimplantation to the main pulmonary artery with or without patch or flap techniques. This report presents two unique cases of ALPA, describing embryologic considerations for morphogenesis of ALPA with 22q11 microdeletion syndrome and a novel surgical technique involving unroofing of the unique anatomy of the common wall between the left and main pulmonary arteries.

Outcome measures for pediatric laryngotracheal reconstruction: International consensus statement.

OBJECTIVES: Develop multidisciplinary and international consensus on patient, disease, procedural, and perioperative factors, as well as key outcome measures and complications, to be reported for pediatric airway reconstruction studies. METHODS: Standard Delphi methods were applied. Participants proposed items in three categories: 1) patient/disease characteristics, 2) procedural/intraoperative/perioperative factors, and 3) outcome measures and complications. Both general and anatomic site-specific measures were elicited. Participants also suggested specific operations to be encompassed by this project. We then used iterative ranking and review to develop consensus lists via a priori Delphi consensus criteria. RESULTS: Thirty-three pediatric airway experts from eight countries in North and South America, Europe, and Australia participated, representing otolaryngology (including International Pediatric Otolaryngology Group members), pulmonology, general surgery, and cardiothoracic surgery. Consensus led to inclusion of 19 operations comprising open expansion, resection, and slide procedures of the larynx, trachea, and bronchi as well as three endoscopic procedures. Consensus was achieved on multiple patient/comorbidity (10), disease/stenosis (7), perioperative-/intraoperative-/procedure-related (16) factors. Consensus was reached on multiple outcome and complication measures, both general and site-specific (8 general, 13 supraglottic, 15 glottic, 17 subglottic, 8 cervical tracheal, 12 thoracic tracheal). The group was able to clarify how each outcome should be measured, with specific instruments defined where applicable. CONCLUSION: This consensus statement provides a framework to communicate results consistently and reproducibly, facilitating meta-analyses, quality improvement, transfer of information, and surgeon self-assessment. It also clarifies expert opinion on which patient, disease, procedural, and outcome measures may be important to consider in any pediatric airway reconstruction patient. LEVEL OF EVIDENCE: 5 Laryngoscope, 129:244-255, 2019.

Examining variation in interstage mortality rates across the National Pediatric Cardiology Quality Improvement Collaborative: do lower-mortality centres have lower-risk patients?

BACKGROUND: Although interstage mortality for infants with hypoplastic left heart syndrome has declined within the National Pediatric Cardiology Quality Improvement Collaborative, variation across centres persists. It remains unclear whether centres with lower interstage mortality have lower-risk patients or whether differences in care may explain this variation. We examined previously established risk factors across National Pediatric Cardiology Quality Improvement Collaborative centres with lower and higher interstage mortality rates. METHODS: Lower-mortality centres were defined as those with >25 consecutive interstage survivors. Higher-mortality centres were defined as those with cumulative interstage mortality rates >10%, which is a collaborative historic baseline rate. Baseline risk factors and perioperative characteristics were compared. RESULTS: Seven lower-mortality centres were identified (n=331 patients) and had an interstage mortality rate of 2.7%, as compared with 13.3% in the four higher-mortality centres (n=173 patients, p<0.0001). Of all baseline risk factors examined, the only factor that differed between the lower- and higher-mortality centres was postnatal diagnosis (18.4 versus 31.8%, p=0.001). In multivariable analysis, there remained a significant mortality difference between the two groups of centres after adjusting for this variable: adjusted mortality rate was 2.8% in lower-mortality centres compared with 12.6% in higher-mortality centres, p=0.003. Secondary analyses identified multiple differences between groups in perioperative practices and other variables. CONCLUSIONS: Variation in interstage mortality rates between these two groups of centres does not appear to be explained by differences in baseline risk factors. Further study is necessary to evaluate variation in care practices to identify targets for improvement efforts.

Revision thoracic slide tracheoplasty: Outcomes following unsuccessful tracheal reconstruction.

OBJECTIVES/HYPOTHESIS: Over the past decade, thoracic slide tracheoplasty (TST) has become the principal operation in the management of congenital tracheal stenosis. The purpose of this report was to describe our experience with revision TST following unsuccessful prior tracheal reconstruction. STUDY DESIGN: Retrospective analysis at an academic children's hospital. METHODS: Patients undergoing TST on cardiopulmonary bypass between January 2005 and May 2014 were reviewed. Patients with a history of prior airway surgery were extracted for further analysis. Preoperative patient variables and postoperative outcomes were evaluated and compared between patients undergoing revision slide tracheoplasty (RTST) and a control group of 26 matched patients undergoing primary surgery TST. RESULTS: Twenty-six revision patients (25 referrals, one primary patient) of 162 patients reviewed over the study period met inclusion criteria. Twenty-three patients had a history of complete tracheal rings, and three patients had cartilaginous deficiency. A total of 41 airway reconstruction procedures had been performed prior to RTST. When compared to primary TST, patients undergoing RTST required fewer cardiac procedures intraoperatively, and fewer mean ventilator hours (P = .01) postoperatively. There was no significant difference in the median length of stay, requirement of >48 hours ventilation, or postoperative complications between groups. There was one nonsurgical postoperative mortality following RTST. CONCLUSIONS: Despite some differences in the postoperative management when compared to nonrevision cases, revision TST can be successfully performed after prior tracheal reconstruction with good postoperative outcomes. LEVEL OF EVIDENCE: 4. Laryngoscope, 128:2181-2186, 2018.

Preoperative Aortic Arch Size and Late Outcome After Coarctation Repair by Lateral Thoracotomy.

BACKGROUND: The approach (lateral thoracotomy versus median sternotomy) to repair coarctation of the aorta is frequently based on arch dimensions from the preoperative echocardiogram. Few studies have assessed the relationship between preoperative arch dimensions and late postoperative outcome. This study aimed to define how preoperative arch dimensions relate to late outcomes and identify long-term predictors of a successful operation. METHODS: We performed a retrospective review of 102 neonates and infants undergoing isolated coarctation repair by lateral thoracotomy between 2003 and 2012. Long-term surgical success was defined based on the following five factors: corrected arch gradient below 20 mm Hg, blood pressure cuff gradient below 15 mm Hg, systolic blood pressure below the 95th percentile during the clinic visit, no antihypertensive medication use, and freedom from reintervention. Regression analysis was performed to identify factors that would predict the need for reintervention and long-term success. RESULTS: At a median of 6 years of follow-up, long-term success was achieved in 63% (56 of 89) of patients, and 94% (96 of 102) were free of reintervention. Bivariate analysis showed that patients requiring reintervention had smaller absolute isthmus dimension (p = 0.04). No significant predictors for reintervention or long-term success could be identified, although a larger distal transverse arch dimension may play a role in long-term success (hazard ratio, 0.7; 95% confidence interval, 0.05 to 1.0; p = 0.06). CONCLUSIONS: Aortic arches of various dimensions were successfully repaired by lateral thoracotomy. No significant predictors for reintervention or long-term success could be identified, although the distal transverse arch dimension may play a role in long-term success.

Anomalous Systemic Artery to the Left Lower Lobe: Literature Review and a New Surgical Technique.

Anomalous systemic arterial supply to the basal segments of the left lower lobe without coexisting pulmonary artery connection is a rare anomaly. Most feel treatment is necessary; however, the ideal strategy is unclear. Treatments described include embolization, pulmonary resection, or anastomosis to the native pulmonary artery. We recently encountered an infant with this anomaly and present a literature review summarizing all recent reports. Additionally, we describe a novel surgical technique to create a tension-free anastomosis utilizing segmental aortic translocation that we employed in our patient due to a large distance between the anomalous vessel and native left pulmonary artery.

Slide tracheoplasty outcomes in children with congenital pulmonary malformations.

OBJECTIVES/HYPOTHESIS: Evaluate and compare surgical outcomes of slide tracheoplasty for the treatment of congenital tracheal stenosis in children with and without pulmonary malformations. STUDY DESIGN: Retrospective chart review at a tertiary care pediatric medical center. METHODS: We identified patients with tracheal stenosis who underwent slide tracheoplasty from 2001 to 2014, and a subset of these patients who were diagnosed with congenital pulmonary malformations. Hospital course and preoperative and postoperative complications were recorded. RESULTS: One hundred thirty patients (18 with pulmonary malformations, 112 with normal pulmonary anatomy) were included. Pulmonary malformations included unilateral pulmonary agenesis (61%) and hypoplasia (39%). Children with pulmonary malformations had a greater median age compared to their normal lung anatomy counterparts. Preoperatively, patients with pulmonary malformations more frequently required preoperative mechanical ventilation (55.6% vs. 21.3%, P = .007), extracorporeal membrane oxygenation (ECMO) (11% vs. 0.9%, P = .05), and tracheostomy (22.2% vs. 3.6%, P = .01). Postoperatively, patients with pulmonary malformations more frequently required mechanical ventilation >48 hours (78% vs. 37%, P =.005) and ECMO use (11% vs. 0.9%, P = .05). Pulmonary malformation patients and children with normal anatomy did not differ in terms of postoperative tracheostomy (16.7% vs. 4.4%, P > .05), dehiscence (6% vs. 0%, P > .05%), restenosis (11% vs. 6%, P > .05) or postoperative figure 8 deformity (6% vs. 3%, P > .05). Mortality, however, was significantly increased (22.2% vs. 3.6%, P = .01) in children with pulmonary malformations. CONCLUSIONS: Although slide tracheoplasty can be successfully performed in patients with abnormal pulmonary anatomy, surgeons and families should anticipate a more difficult postoperative course, with possible associated prolonged mechanical ventilation, ECMO use, and higher mortality than in children with tracheal stenosis alone. LEVEL OF EVIDENCE: 4. Laryngoscope, 127:1283-1287, 2017.

Improved outcomes with peritoneal dialysis catheter placement after cardiopulmonary bypass in infants.

BACKGROUND: Acute kidney injury (AKI) is common in infants after cardiopulmonary bypass and is associated with poor outcomes. Peritoneal dialysis improves outcomes in adults with AKI after bypass, but pediatric data are limited. This retrospective case-matched study was conducted to determine if the practice of peritoneal dialysis catheter (PDC) placement during congenital heart surgery is associated with improved clinical outcomes in infants at high risk for AKI. METHODS: Forty-two infants undergoing congenital heart surgery with planned PDC placement (PDC+) were age-matched to infants undergoing similar surgery without PDC placement (PDC-). Demographic, baseline and outcome data were compared. Our primary outcome was negative fluid balance on postoperative days 1 to 3. Secondary outcomes included time to negative fluid balance, time to extubation, frequency of electrolyte corrective medications, inotrope scores, and other clinical outcomes. RESULTS: Baseline data did not differ between groups. The PDC+ group had a higher percentage of negative fluid balance on postoperative days 1 and 2 (57% vs 33%, P = .04; 85% vs 61%, P = .01). The PDC+ group had shorter time to negative fluid balance (16 vs 32 hours, P < .0001), earlier extubation (80 vs 104 hours, P = .02), improved inotrope scores (P = .04), and fewer electrolyte imbalances requiring correction (P = .03). PDC-related complications were rare. CONCLUSIONS: PDC use is safe and associated with earlier negative fluid balance and improved clinical outcomes in infants at high risk for AKI. Routine PDC use should be considered for infants undergoing cardiopulmonary bypass. Further prospective studies are essential to prove causative effects of PDC placement in this population.

Combining functional and tubular damage biomarkers improves diagnostic precision for acute kidney injury after cardiac surgery.

BACKGROUND: Increases in serum creatinine (ΔSCr) from baseline signify acute kidney injury (AKI) but offer little granular information regarding its characteristics. The 10th Consensus Conference of the Acute Dialysis Quality Initiative (ADQI) suggested that combining AKI biomarkers would provide better precision for AKI course prognostication. OBJECTIVES: This study investigated the value of combining a functional damage biomarker (plasma cystatin C [pCysC]) with a tubular damage biomarker (urine neutrophil gelatinase-associated lipocalin [uNGAL]), forming a composite biomarker for prediction of discrete characteristics of AKI. METHODS: Data from 345 children after cardiopulmonary bypass (CPB) were analyzed. Severe AKI was defined as Kidney Disease Global Outcomes Initiative stages 2 to 3 (≥100% ΔSCr) within 7 days of CPB. Persistent AKI lasted >2 days. SCr in reversible AKI returned to baseline ≤48 h after CPB. The composite of uNGAL (>200 ng/mg urine Cr = positive [+]) and pCysC (>0.8 mg/l = positive [+]), uNGAL+/pCysC+, measured 2 h after CPB initiation, was compared to ΔSCr increases of ≥50% for correlation with AKI characteristics by using predictive probabilities, likelihood ratios (LR), and area under the curve receiver operating curve (AUC-ROC) values [Corrected]. RESULTS: Severe AKI occurred in 18% of patients. The composite uNGAL+/pCysC+ demonstrated a greater likelihood than ΔSCr for severe AKI (+LR: 34.2 [13.0:94.0] vs. 3.8 [1.9:7.2]) and persistent AKI (+LR: 15.6 [8.8:27.5] versus 4.5 [2.3:8.8]). In AKI patients, the uNGAL-/pCysC+ composite was superior to ΔSCr for prediction of transient AKI. Biomarker composites carried greater probability for specific outcomes than ΔSCr strata. CONCLUSIONS: Composites of functional and tubular damage biomarkers are superior to ΔSCr for predicting discrete characteristics of AKI.

The surgical prebrief as part of a five-point comprehensive approach to improving pediatric cardiac surgical team communication.

Communication is essential to the safe conduct of any critical task including cardiac surgery. After inspiration by airline crew resource management training, a communication system for the care plans of pediatric cardiac patients was developed and refined over time that encompasses the entire heart center team. Five distinct communication points are used to ensure preoperative, intraoperative, and postoperative care, which is transitioned efficiently and maintained at the highest level.

A Rare Case of Pulmonary Artery Sling and Complete Atrioventricular Canal Defect in an Infant With Trisomy 21.

Pulmonary artery sling is a very rare congenital vascular anomaly. Patients usually present in infancy with symptoms of airway compression. Patients with trisomy 21 often have upper airway obstruction, most commonly related to pharyngeal causes or subglottic stenosis. Although the incidence of congenital heart defects in patients with trisomy 21 is very high, a review of the literature showed only one previously reported case of pulmonary artery sling in an infant with trisomy 21. We report a case of pulmonary artery sling and complete atrioventricular canal defect in a one-month-old female with trisomy 21. Echocardiography is an important diagnostic method for pulmonary artery sling, but this anomaly may be easily overlooked in the presence of more commonly anticipated defects in this population.

Slide tracheoplasty for the treatment of tracheoesophogeal fistulas.

PURPOSE: The purpose of this study is to determine the surgical outcome of slide tracheoplasty for the treatment of tracheoesophageal (TE) fistula in pediatric patients. METHODS: After internal review board approval, the charts of pediatric patients (0-18years old) who had undergone slide tracheoplasty for tracheoesophageal fistula were retrospectively reviewed. Patient information and surgical outcomes were reviewed. RESULTS: Nine patients underwent slide tracheoplasty for correction of TE fistula. In five patients the original TE fistula was congenital. Other causes included battery ingestion, tracheostomy tube complications, foreign body erosion, and an iatrogenic injury. The average age at repair was 48±64 months (range: 1-190). Seven patients had undergone previous TEF repair either open or endoscopically. There were no recurrences after repair. Two patients had sternal periosteum interposed between the esophagus and trachea. There were no TEF recurrences. A single patient had dehiscence of the tracheal anastomosis and underwent a second procedure. CONCLUSION: Slide tracheoplasty is an effective method to treat complex TE fistulas. The procedure was not associated with any recurrences. This is the first description of a novel, effective, and safe method to treat TE fistulas.

The extracellular stress response to pediatric cardiopulmonary bypass.

The heat shock response, also frequently referred to as the stress response, is an ancient, highly conserved, endogenous cellular defense mechanism characterized by the rapid upregulation of a specific class of proteins known collectively as heat shock proteins, or stress proteins. The 70 kDa family of heat shock proteins are highly inducible and have been shown to possess important immunomodulatory effects in both the intracellular and extracellular compartments. In the current prospective translational study, we measured extracellular (i.e. plasma) levels of heat shock protein 72 (Hsp72) in 49 children undergoing cardiopulmonary bypass (CPB) for either palliation or repair of congenital heart disease. There was a significant and transient increase (less than 24 h) in extracellular Hsp72 levels following CPB. Extracellular Hsp72 levels significantly correlated with levels of the pro-inflammatory cytokines interleukin (IL)-6 and IL-8, as well as the anti-inflammatory cytokine, IL-10. In addition, plasma Hsp72 levels correlated with troponin-I levels, a marker of myocardial injury. Increased extracellular Hsp72 levels at 6 h following CPB were independently associated with increased length of stay in the cardiac intensive care unit. Importantly, the source of extracellular Hsp72 does not appear to be cardiomyocytes. However, the mechanism of release and clinical relevance of the increase in extracellular Hsp72 need to be further delineated.

Microcephaly is associated with early adverse neurologic outcomes in hypoplastic left heart syndrome.

BACKGROUND: Hypoplastic left heart syndrome (HLHS) is associated with significant mortality and morbidity. Fetal head growth abnormalities have been identified in a subset of HLHS fetuses, but it is unclear whether specific patterns of maladaptive growth affect clinical outcomes. We hypothesized that poor fetal head growth is associated with an increased frequency of adverse clinical outcomes. METHODS: We retrospectively examined a cohort of HLHS patients from midgestation to 1 y of age. Fetal and birth anthropometric measurements were analyzed using the Olsen standard, and clinical outcomes were obtained. RESULTS: A total of 104 HLHS patients were identified over a 12-y period; fetal data were available in 38 cases. HLHS neonates demonstrated a high incidence of microcephaly (12%), small head size (27%), and poor head growth (32%). All-cause mortality was 31% at 30 d and 43% at 1 y. Neurologic outcomes were observed in 12% of patients and were significantly increased with microcephaly (43 vs. 4%; P = 0.02). The average length of hospital stay following stage I palliation was 33.4 ± 33 d, correcting for early death. CONCLUSION: In term nonsyndromic HLHS, fetal and neonatal microcephaly are associated with early adverse neurologic outcomes but not mortality.

Surgical management of neonatal atrioventricular septal defect with aortic arch obstruction.

BACKGROUND: For neonates with atrioventricular septal defect and aortic arch obstruction including coarctation of the aorta, we sought to determine whether a difference in outcomes exists after a primary neonatal versus staged surgical repair (neonatal arch repair with delayed intracardiac repair). METHODS: This retrospective cohort study included consecutive neonates with atrioventricular septal defect and aortic arch obstruction who underwent cardiac surgery before 28 days of age at six centers from 1990 to 2009. Characteristics and outcomes between patients undergoing neonatal versus staged repair were compared. RESULTS: Of 66 study patients, 31 (47%) underwent primary neonatal repair and 35 (53%) underwent staged repair. At baseline echocardiogram, a greater percentage of neonatal repair patients had relative unbalanced ventricular size (56% versus 35%, p = 0.02). There were no other differences in demographic characteristics, cardiac anatomical or functional details, or surgical technique. Those undergoing neonatal repair tended to be more likely to have at least moderate left atrioventricular valve regurgitation early after repair (42% versus 19%, p = 0.05) and to have at least one major in-hospital complication (42% versus 20%, p = 0.06). After the initial cardiac operation, compared with the neonatal repair group, patients undergoing staged repair had greater survival (87% versus 57% at 6 years, log-rank p = 0.02) and freedom from the first unplanned cardiac reoperation (69% versus 45% at 6 years, log-rank p = 0.005). CONCLUSIONS: For neonates with atrioventricular septal defect and aortic arch obstruction, when compared with neonatal repair, a staged approach was associated with improved survival and lower morbidity.