Impact of Cardiac Risk Factors in the Postsurgical Outcomes of Patients With Cleft Lip.

ABSTRACT: Congenital cardiac comorbidities represent a potentially elevated risk for complications in patients undergoing cleft lip repair. National databases, such as the National Surgical Quality Improvement Program Pediatric (NSQIP-P) allow for analysis of large national datasets to assess these risks and potential complications. The aim of this study is to assess the risk of complications in patients undergoing cleft lip repair with congenital cardiac co-morbidities using the NSQIP-P.The 2012 to 2014 NSQIP-P databases were queried for patients undergoing cleft lip repair. Data abstracted for analysis included demographic, clinical, and outcomes data. Patients with cleft lip were stratified based on the presence or absence of congenital cardiac comorbidities. Univariate analysis and step-wise, forward logistic regression were performed to compare these groups.Nationally, between 2012 and 2014, 2126 patients underwent cleft lip repair, 227 with cardiac disease, and 1899 without cardiac disease. Weights were similar between the groups at the time of surgery, though patients with cardiac comorbidities were older. Postoperatively, cardiac disease patients were more likely to experience an adverse event. Specifically, they were more likely to experience reintubation, reoperation, longer length of stay, and death. Rates of surgical site infection and dehiscence were not different between the groups.This study demonstrates that cleft lip repair in patients with congenital heart defects is safe. However, patients undergoing cleft lip repair with comorbid congenital cardiac disease were more likely to experience adverse events. Cardiac patients require special preoperative evaluation before repair of their cleft lip, but do not appear to experience worse wound-related outcomes.

Multimodality lymphatic imaging of postoperative chylothorax in an infant with Noonan syndrome: a case report.

BACKGROUND: Chylothorax is a rare complication of pediatric cardiac operations that occurs more frequently in children with Noonan syndrome, a genetic disorder associated with cardiac defects and lymphatic anomalies. CASE PRESENTATION: We report a case of postoperative chylothorax in a 6-month-old infant with Noonan syndrome where multimodality lymphatic imaging guided management was followed. Drainage patterns of the lymphatic capillaries in the lower and upper extremities were visualized during near-infrared fluorescence lymphatic imaging (NIRFLI). Dynamic magnetic resonance lymphangiography (MRL) further identified the site of leakage in the thoracic duct and subsequently guided surgical intervention. CONCLUSIONS: Application of multimodality imaging allows for greater individualization of treatment and should be considered in patients with complex cases such as those with syndromes associated with a higher incidence of chylothorax. IRB Number: HSC-MS-13-0754, December 10, 2013.

Resuscitating the Chimney Graft to Innominate Artery for Straightforward Cannulation During Infancy.

Arterial cannulation with a chimney polytetrafluoroethylene graft to the innominate artery is commonly used for antegrade cerebral perfusion during neonatal aortic arch surgery. When properly retained and prepared before sternal closure, resuscitation of the polytetrafluoroethylene graft to innominate artery can be performed months later during sternal reentry. It is a safe and reproducible technique for expeditious arterial cannulation at stage II palliation in single-ventricle patients or complete intracardiac repair of biventricular lesions. We report our experience utilizing this technique successfully during reoperation in 90 of 92 patients, with no adverse thromboembolic events identified.

Impact of Cardiac Risk Factors in the Postsurgical Outcomes of Patients With Cleft Palate: Analysis of the 2012-2014 NSQIP Database.

OBJECTIVE: To assess the risk of complication in patients undergoing cleft palate repair with congenital cardiac comorbidities in a large, national cohort. DESIGN: Retrospective review. PATIENTS/SETTING: Using the 2012-2014 National Surgical Quality Improvement Program (NSQIP) Pediatric database, patients undergoing cleft palate repair were selected for analysis. Patients with cleft palate repairs were stratified based on the presence or absence congenital cardiac comorbidities. Univariate and stepwise forward logistic regression were conducted. MAIN OUTCOME MEASURES: It is hypothesized that risk of postoperative adverse events in patients with congenital cardiac comorbidities is higher than in patients without cardiac disease. RESULTS: Nationally, between 2012 and 2014, 3240 patients underwent cleft palate repair, 422 (13.0%) with cardiac disease, and 2818 (87.0%) without cardiac disease. Patients with cardiac disease were smaller (10.5 [6.6] kg vs 11.6 [8.6] kg, P < .01) and more likely to be premature (4.6% vs 13.0%, P < .01) compared to those without cardiac disease. Postoperatively, patients with cardiac conditions were more likely to experience an adverse event (8.8% vs 4.2%, P < .01). Specifically, they were more likely to experience reintubation (1.7% vs 0.4%, P < .01), reoperation (2.1% vs 0.6%, P < .01), and longer length of stay (2.7 [7.0] vs 1.6 [2.8] days, P < .01). Rates of surgical site infection and dehiscence were not different. CONCLUSIONS: Cleft palate repair in patients with concurrent congenital cardiac defects is a safe procedure but carries elevated risk in the postoperative period as demonstrated in this analysis of the NSQIP-Pediatric database. Technical risks are equivalent. Additional anesthesia and surgical awareness of these potential complications is essential to minimize perianesthesia risks.

Airway evaluation in children with single ventricle cardiac physiology.

OBJECTIVE: Children with single ventricle cardiac physiology (SVC) often require airway procedures as an adjunct to their care. Descriptive analysis with a focus on outcomes of airway procedures in SVC patients have not been fully described in the literature. METHODS: Retrospective, single-center cohort review of 270 patients born between Aug-2007 and Jan-2017. Patients were identified by cardiac database for single ventricle pathophysiology. A subset of these patients were identified to have been evaluated by otolaryngology with airway evaluations and/or interventions. RESULTS: 88/270 patients (32.6%) required investigation or intervention for airway pathology. The most frequent procedure was flexible fiberoptic laryngoscopy (58/88 patients); it was the only procedure performed in 40 patients. Seventeen patients required tracheostomies with an associated increased length of stay (p < 0.001). Patients with cardiac procedures involving dissection around the aortic arch were considered higher airway risk due to the threat of recurrent laryngeal nerve injury, and were more likely to have vocal cord paralysis (58%) compared to patients with lower risk procedures (21%; p < 0.001). However, on multivariate logistic regression, vocal cord paralysis did not statistically impact the odds for tracheostomy placement, although the presence of subglottic stenosis increased the odds ratio of tracheostomy by 14.7 (p = 0.02). CONCLUSIONS: Children with SVC often require airway evaluation and intervention. Patients with high risk cardiac procedures had a higher risk of recurrent laryngeal nerve injury but the presence of subglottic stenosis was the best predictor for a tracheostomy. This study represents one of the largest series of SVC children evaluated for airway pathology.

Cardiac Injury After All-Terrain Vehicle Accidents in 2 Children and a Review of the Literature.

All-terrain vehicle (ATV) accidents leading to severe morbidity and mortality are common. At our institution, 2 children presented within weeks of each other after ATV accidents. Both children required cardiac valve surgery. The surgical management of these 2 children is discussed, and the literature is reviewed. On initial patient presentation, the diagnosis of a ruptured cardiac valve or ventricular septal defect (VSD) associated with these types of accidents is often delayed. We propose that patients presenting with evidence of high-energy blunt thoracic trauma after an ATV accident should undergo an electrocardiogram, cardiac enzyme assessment, and cardiac echocardiogram as part of the initial work-up to rule out significant myocardial injury.

A minimally invasive, algorithm-based approach for anomalous aortic origin of a coronary artery.

OBJECTIVE: Operative repair for anomalous aortic origin of a coronary artery (AAOCA) has been described using various innovative techniques. Common to each series is the use of a full sternotomy. As demand for minimally invasive approaches to adult cardiac surgery has increased, the upper hemisternotomy has emerged as a safe and effective technique for aortic valve and root replacement. This report reviews our results and describes the application of an upper hemisternotomy to an algorithm-based surgical approach for AAOCA. METHODS: From January 2012 to March 2013, the aortic root was approached via a 7-cm skin incision and upper hemisternotomy for all patients undergoing repair of an AAOCA. The type of repair performed was in accordance with a predefined surgical algorithm. The anomalous vessel had a slit-like ostium and followed a supracommissural intramural course in three patients with symptomatic anomalous right coronary artery. These patients underwent coronary unroofing. In contrast, a patient with an anomalous left coronary artery presented without an intramural segment and underwent vessel translocation and reimplantation. RESULTS: All patients underwent AAOCA repair according to our surgical algorithm and via an upper hemisternotomy. The median length of stay was 4 days. All patients had resolution of symptoms, and there were no reported complications at a median follow-up of 16.5 months. CONCLUSIONS: This series describes a minimally invasive approach to AAOCA repair. When used in conjunction with a defined surgical algorithm, this technique enables a safe and effective repair in all forms of AAOCA without concomitant coronary artery disease.

Norwood reconstruction using continuous coronary perfusion: a safe and translatable technique.

BACKGROUND: Continuous coronary perfusion during Norwood reconstruction offers the theoretic advantage of less postoperative cardiac dysfunction. The avoidance of a cardiac and circulatory arrest period allows time for a more deliberate aortic reconstruction while the heart remains beating. This single-center study was designed to compare patient results using this method vs standard cardiac arrest for Norwood reconstruction. METHODS: A retrospective review was done of 32 patients undergoing Norwood reconstruction from November 2004 to July 2011. The operations in the most recent 16 consecutive patients were performed under deep hypothermia with constant coronary and cerebral perfusion. Continuous coronary perfusion was provided by a cannula inserted into the proximal aorta. The operations in the prior 16 consecutive patients were performed using deep hypothermia, selective cerebral perfusion, and cardioplegic arrest during aortic reconstruction. RESULTS: Survival in the beating-heart group was 87.5% (14 of 16) vs 62.5% (10 of 16) in the standard group (p = 0.22). No patients in the beating-heart group required extracorporeal membrane oxygenation vs 3 in the standard group. Postoperative cardiac function was similar for both groups. The beating-heart cohort had lower peak lactate levels (8.2 mEq/L) than the standard group (10.7 mEq/L, p = 0.022). CONCLUSIONS: This study presents the largest series of Norwood operations in which the entire aorta is augmented while delivering continuous coronary perfusion. The technique is applicable to any size aorta and represents a safe alternative because outcomes for survival, freedom from extracorporeal membrane oxygenation, postoperative cardiac function, and lactate levels were all noninferior compared with the standard technique.

The ΔF508 mutation causes CFTR misprocessing and cystic fibrosis-like disease in pigs.

Cystic fibrosis (CF) is an autosomal recessive disease caused by mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR) anion channel. The most common CF-associated mutation is ΔF508, which deletes a phenylalanine in position 508. In vitro studies indicate that the resultant protein, CFTR-ΔF508, is misprocessed, although the in vivo consequences of this mutation remain uncertain. To better understand the effects of the ΔF508 mutation in vivo, we produced CFTR(ΔF508/ΔF508) pigs. Our biochemical, immunocytochemical, and electrophysiological data on CFTR-ΔF508 in newborn pigs paralleled in vitro predictions. They also indicated that CFTR(ΔF508/ΔF508) airway epithelia retain a small residual CFTR conductance, with maximal stimulation producing ~6% of wild-type function. Cyclic adenosine 3',5'-monophosphate (cAMP) agonists were less potent at stimulating current in CFTR(Δ)(F508/)(Δ)(F508) epithelia, suggesting that quantitative tests of maximal anion current may overestimate transport under physiological conditions. Despite residual CFTR function, four older CFTR(ΔF508/ΔF508) pigs developed lung disease similar to human CF. These results suggest that this limited CFTR activity is insufficient to prevent lung or gastrointestinal disease in CF pigs. These data also suggest that studies of recombinant CFTR-ΔF508 misprocessing predict in vivo behavior, which validates its use in biochemical and drug discovery experiments. These findings help elucidate the molecular pathogenesis of the common CF mutation and will guide strategies for developing new therapeutics.

Cystic fibrosis pigs develop lung disease and exhibit defective bacterial eradication at birth.

Lung disease causes most of the morbidity and mortality in cystic fibrosis (CF). Understanding the pathogenesis of this disease has been hindered, however, by the lack of an animal model with characteristic features of CF. To overcome this problem, we recently generated pigs with mutated CFTR genes. We now report that, within months of birth, CF pigs spontaneously developed hallmark features of CF lung disease, including airway inflammation, remodeling, mucus accumulation, and infection. Their lungs contained multiple bacterial species, suggesting that the lungs of CF pigs have a host defense defect against a wide spectrum of bacteria. In humans, the temporal and causal relations between inflammation and infection have remained uncertain. To investigate these processes, we studied newborn pigs. Their lungs showed no inflammation but were less often sterile than controls. Moreover, after introduction of bacteria into their lungs, pigs with CF failed to eradicate bacteria as effectively as wild-type pigs. These results suggest that impaired bacterial elimination is the pathogenic event that initiates a cascade of inflammation and pathology in CF lungs. Our finding that pigs with CF have a host defense defect against bacteria within hours of birth provides an opportunity to further investigate CF pathogenesis and to test therapeutic and preventive strategies that could be deployed before secondary consequences develop.

Disruption of the CFTR gene produces a model of cystic fibrosis in newborn pigs.

Almost two decades after CFTR was identified as the gene responsible for cystic fibrosis (CF), we still lack answers to many questions about the pathogenesis of the disease, and it remains incurable. Mice with a disrupted CFTR gene have greatly facilitated CF studies, but the mutant mice do not develop the characteristic manifestations of human CF, including abnormalities of the pancreas, lung, intestine, liver, and other organs. Because pigs share many anatomical and physiological features with humans, we generated pigs with a targeted disruption of both CFTR alleles. Newborn pigs lacking CFTR exhibited defective chloride transport and developed meconium ileus, exocrine pancreatic destruction, and focal biliary cirrhosis, replicating abnormalities seen in newborn humans with CF. The pig model may provide opportunities to address persistent questions about CF pathogenesis and accelerate discovery of strategies for prevention and treatment.