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

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 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.

Survival in congenital heart disease has steadily improved since 1938, when Dr. Robert Gross successfully ligated for the first time a patent ductus arteriosus in a 7-year-old child. To continue the gains made over the past 80 years, transformative changes with broad impact are needed in management of congenital heart disease. Three-dimensional printing is an emerging technology that is fundamentally affecting patient care, research, trainee education, and interactions among medical teams, patients, and caregivers. This paper first reviews key clinical cases where the technology has affected patient care. It then discusses 3-dimensional printing in trainee education. Thereafter, the role of this technology in communication with multidisciplinary teams, patients, and caregivers is described. Finally, the paper reviews translational technologies on the horizon that promise to take this nascent field even further.

Closure of a ventricular septal defect (VSD) is the most commonly performed operation in congenital cardiac surgery, either as an isolated defect or as a component of a more complex repair such as tetralogy of Fallot or double outlet right ventricle. Most of these defects are classified as type 2 defects, synonymously referred to as perimembranous, paramembranous, or conoventricular [1Jacobs J.P. Burke R.P. Quintessenza J.A. Mavroudis C. Congenital Heart Surgery Nomenclature and Database Project: ventricular septal defect.Ann Thorac Surg. 2000; 69: S25-S35Abstract Full Text Full Text PDF PubMed Google Scholar], the group that will be the focus of this article. In the most recent report of The Society of Thoracic Surgeons Congenital Heart Surgery Database, type 2 VSD closure was a principal component of 15% of all operations. These defects are typically closed with the use of a patch by a right atrial approach. A running or interrupted suture technique may be used to affix the patch. I prefer the interrupted technique in general, but particularly when training residents because it affords better exposure of the critical anatomy one must understand to accomplish a successful repair. Success depends on accomplishing three goals: (1) completely closing the hole, (2) avoiding injury to adjacent structures (valves and conduction system), and (3) avoiding residual outflow tract obstruction. These goals can only be achieved if the surgeon has a solid understanding of the anatomy of the defect and its adjacent structures. Despite important individual anatomic variability (some related to the embryology of the conotruncal defects making up a large portion of these cases), I have found it helpful to simplify the basic approach to these defects under a single unifying concept: VSDs are triangles, not circles. The initial preparation of the trainee involves establishing an understanding of the triangle concept of VSD anatomy (Fig 1). Triangles have three sides and three angles, or transitions. The three sides of the VSD triangle are the tricuspid valve annulus on the right, the muscular septum on the left, and the conal septum superiorly. Quite often, though, the triangle does not exist in a simple plane, rather a distorted Escher-like version of a triangle. This is related to the developmental factors that result in the VSD; normally the muscular septum ascends toward the base of the heart from the apex, and the conal septum descends from between the great vessels with the final septation of the ventricles dependent on their fusion as the membranous septum. If this approximation is not complete, or if the two septae are not aligned (either in the anteroposterior plane, or off rotation from one another), a defect results. The cases of malalignment (typical in tetralogy and double outlet right ventricle) result in the transitions between sides of the triangle being distorted. In addition to reviewing the indications for timing of the operation with the trainee, the anatomy of the individual case should be reviewed on the preoperative echocardiograms. The features I encourage trainees to focus on are the location of adjacent structures to the VSD, particularly the tricuspid and aortic valves. In addition, any additional anatomy (right ventricular outflow tract stenosis, for example) that may need to be managed as part of the repair should be discussed, and a stepwise plan for addressing each defect must be articulated. As mentioned previously, I prefer using an interrupted suture technique when training residents because it allows more precise visualization and understanding of the relevant anatomy. It also allows tailoring the patch to be performed after the size and contour of the defect is well delineated by the initially placed sutures. After initiation of cardiopulmonary bypass with bicaval cannulation and cardioplegic arrest, the right atrium is opened and the intracardiac anatomy is carefully inspected. Specifically related to the VSD anatomy, the three sides of the triangle and the three transitional angles are identified. The sides of the triangle are often easily defined, and sutures along these lines are usually easily placed. The angles or transition zones pose the challenges in VSD repair. The transition from tricuspid annulus to muscular septum is where the His bundle lies, and the sutures in this area will cause heart block if carelessly placed. The transition from tricuspid annulus to conal septum superiorly is often the most difficult to see behind the tricuspid leaflets. This transition is near the aortic valve and is often a site for residual defects if not well visualized. Exposure of this angle can often be facilitated if the assistant presses down on the outside of the heart at the base of the aorta with the back end of a forceps to invert this part of the conal septum. Some advocate opening the base of the tricuspid valve at the annulus to improve exposure of this transition zone [2Gaynor J.W. O’Brien Jr., J.E. Rychik J. Sanchez G.R. DeCampli W.M. Spray T.L. Outcome following tricuspid valve detachment for ventricular septal defects closure.Eur J Cardiothorac Surg. 2001; 19: 279-282Crossref PubMed Scopus (53) Google Scholar]. The transition from muscular to conal septum is often the easiest to see but may be distorted when the conal septum and muscular septum are not aligned. Most of the interrupted sutures will be pledgeted mattress stitches, although a few simple sutures will often be used on the conal septum where exposure makes mattress sutures more difficult to place. The initial sutures are placed through the tricuspid valve annulus, from right atrial to the ventricular side, which facilitate exposure of the defect by retracting the tricuspid septal leaflet out of the way. The first transition suture is at the superior end of the tricuspid annular side. The first bite of the mattress stitch simply passes through the base of the annulus, but the second must course deeper and away from the atrium that exits on the conal septum. The external aortic root pressure described earlier may assist in exposure for this placement. Next, the transition stitch near the conduction system is placed. Because the His bundle crosses from the floor of the right atrium into the interventricular septum on the left side of the septal crest, the suture bites must stay well to the right side of the septal crest and be relatively superficial. This transition stitch begins in the right ventricle with the first bite exiting near the edge of the VSD, usually at the base of a consistently seen corda that supports the septal leaflet. The second bite of this mattress stitch runs superficially over to and through the tricuspid annulus, then is passed back through the tricuspid annulus next to the lowest annular stitch placed previously (Fig 2). The next sutures are typically safe sutures that can be relatively deeply placed, working along the muscular septum, following the sometimes distorted transition onto the conal septum. The final sutures are usually nonpledgeted, simple sutures that enter and exit the conal septum parallel to the annulus of the aortic valve. Both ends of these sutures will eventually be passed through the edge of the patch, making these function as a mattress suture as well. Exposure for these conal septal sutures may be facilitated by the external pressure maneuver, particularly in double outlet right ventricle cases. Next, with optimal visualization of the size and contour of the defect due to traction on each of the previously placed sutures (Fig 3), the patch can be cut to an appropriate size, usually circular and a bit larger than the defect to allow bulging to the right to avoid subaortic compromise. I prefer Dacron (Sauvage Filamentous Knitted Polyester, Bard, Tempe, AZ) for these patches, but pericardium and Gore-Tex (W.L. Gore & Assoc, Flagstaff, AZ) work equally well. Similar to the technique typically used in prosthetic valve placement, the circumferential sutures are placed through the edge of the patch, usually beginning at the highest tricuspid annular stitch and proceeding counterclockwise around the patch (Fig 3C). The final two or three sutures are best placed on the patch after releasing the patch from where it is clamped to the drapes and allowing it to hover over the middle of the right atrium. The patch can be seated against the defect with gentle traction on the sutures to assist this process. Trainees must be reminded to be gentle when tying down these sutures to avoid tearing through the often delicate ventricular musculature. Only enough force is needed to dent the patch, not enough to tear the tissue. I typically recommend beginning the tying sequence with the muscular septal sutures, because they are often easiest to see, proceeding clockwise to the top of the tricuspid annulus, then counterclockwise across the conal septum, leaving the nonpledgeted sutures as the last to be tied. Next, the tricuspid valve leaflets should be inspected and freed from any points that may have become trapped by the sutures in the process of tying. The edges of the patch should be visualized to ensure it is well seated circumferentially. After separation from bypass, the transesophageal echocardiogram should be reviewed with the cardiologist performing the study with particular focus on the three transition zones which are the most likely sites for residual defects. Tricuspid and aortic valve functions should be assessed as well as unobstructed flow from the left ventricle to the aorta, especially in cases of double outlet right ventricle. Although running suture techniques often require less time and may have some advantages, exposing portions of the defect while placing traction on the suture and patch while following, the need to precut the patch and the obstructed view that the patch itself sometimes imposes make this a technique that I feel is best reserved until after the trainee has a solid understanding of the relevant anatomy and concepts of VSD closure. The principals in the triangle approach apply equally well to the running suture method, helping the trainee understand where along the defect he or she will be at most risk of conduction system injury or residual defects.

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. (C) 2018 by The Society of Thoracic Surgeons

ObjectivesDevelop 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.MethodsStandard 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.ResultsThirty‐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.ConclusionThis 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 Evidence5 Laryngoscope, 129:244–255, 2019

Objectives/HypothesisOver 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 DesignRetrospective analysis at an academic children's hospital.MethodsPatients 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.ResultsTwenty‐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.ConclusionsDespite 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 Evidence4. Laryngoscope, 128:2181–2186, 2018

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.

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.

Background. Historically, the options for mechanical circulatory support in infants, particularly those with single-ventricle physiology, have been limited and outcomes have generally been poor. We report a new approach implemented for long-term support in a series of such patients.& para;& para;Methods. This study is a single-center case series of 7 patients with single-ventricle physiology after stage 1 palliation supported with mechanical circulatory support using a novel technique, between May 2014 and September 2015. Our technique included modification and implantation of commercially available pediatric cannulae into the common atrium and the ascending aorta or reconstructed neoaorta and utilization of a centrifugal extracorporeal pump.& para;& para;Results. Median circulatory support duration was 64 days (range, 35 to 99). One adverse neurologic event was observed in 1 patient, and bleeding requiring reoperation in 2 patients. Support to recovery, decision, or heart transplantation was accomplished in all cases. Of all patients, 43% were successfully discharged home.& para;& para;Conclusions. Our experience shows that long-term extracorporeal mechanical circulatory support of patients with underlying single-ventricle physiology after stage 1 palliation is feasible utilizing our technique. This approach overcomes several major challenges encountered in these patients, such as high flow requirement and stability of the cannulae, and allows extubation, rehabilitation, and at times, myocardial recovery. (C) 2017 by The Society of Thoracic Surgeons

We applied 3-dimensional (3D) printing in patients with congenital heart disease to precisely visualize complex anatomy, plan surgical procedures, and teach trainees and patients. Cases presented range from infants to adults with congenital heart disease. A variety of pathologies are shown,

The Video can be viewed in the online version of this article [http://dx.doi.org/10.1016/j.athoracsur.2017.06.059] on http://www.annalsthoracicsurgery.org.In the pediatric population, tracheal reconstruction is most frequently used in the management of congenital tracheal stenosis associated with complete rings of tracheal cartilage. Although congenital tracheal stenosis may be seen involving various lengths of the trachea and exhibiting variable caliber of narrowing, the majority of cases involve a long segment of the trachea (greater than 50% of the total tracheal length), and present in the first months of life with stridor and respiratory distress. Although “noisy breathing” may be described in these infants from the first days of life, many do not come to medical attention until they decompensate in association with an upper respiratory viral illness. Many children with congenital tracheal stenosis are also found to have associated cardiovascular anomalies; most frequently seen is a left pulmonary artery sling. Because tracheal reconstruction in this group is done utilizing cardiopulmonary bypass support, simultaneous repair of the cardiovascular anomalies should also be planned. The Video can be viewed in the online version of this article [http://dx.doi.org/10.1016/j.athoracsur.2017.06.059] on http://www.annalsthoracicsurgery.org. Historically many techniques for tracheal reconstruction in children have been described, many borrowed from adult experiences with acquired tracheal stenosis [1Backer C.L. Mavroudis C. Gerber M.E. Holinger L.D. Tracheal surgery in children: an 18-year review of four techniques.Eur J Cardiothorac Surg. 2001; 19: 777-784Crossref PubMed Scopus (110) Google Scholar]. In recent years, the slide tracheoplasty technique has emerged as the preferred method for pediatric tracheal reconstruction. It is an extremely versatile technique that may be applied to a wide variety of tracheal pathology from short segment stenosis to full-length tracheal hypoplasia [2Manning P.B. Rutter M.J. Lisec A. Gupta R. Marino B.S. One slide fits all: the versatility of slide tracheoplasty with cardiopulmonary bypass support for airway reconstruction in children.J Thorac Cardiovasc Surg. 2011; 141: 155-161Abstract Full Text Full Text PDF PubMed Scopus (118) Google Scholar, 3Butler C.R. Speggiorin S. Rijnberg F.M. et al.Outcomes of slide tracheoplasty in 101 children: a 17-year single-center experience.J Thorac Cardiovasc Surg. 2014; 147: 1783-1789Abstract Full Text Full Text PDF PubMed Scopus (118) Google Scholar]. This technique offers advantages particularly important in children over other reconstructive methods. The slide tracheoplasty results in a reconstruction utilizing rigid, vascularized tissue with a normal mucosa. That facilitates early extubation in many cases, results in less granulation tissue formation compared with other techniques, and results in growth of the reconstructed trachea [4Speggiorin S. Gilbert T.W. Broadhead M. Roebuck D.J. McLaren C.A. Elliott M.J. Do tracheas grow after slide tracheoplasty?.Ann Thorac Surg. 2012; 93: 1083-1086Abstract Full Text Full Text PDF PubMed Scopus (20) Google Scholar]. Optimal outcome from a surgical procedure such as the slide tracheoplasty relies on good planning as much as it relies on expert surgical technique. The trainee should understand the critical diagnostic studies that must be obtained and reviewed as part of the planning process, as well as important issues that impact decisions related to optimal timing of the procedure. In our practice, we rely primarily on rigid bronchoscopy to identify the complete rings of tracheal cartilage and the proximal extent of the stenosis. Careful bronchoscopy by an experienced otolaryngologist can often traverse the length of the stenosis without traumatizing the tracheal mucosa and allow for evaluation of the distal extent of the pathology as well. It is helpful to be able to review videos of the bronchoscopy with the trainee to identify these key details without having to prolong the actual examination of the patient and risk further compromise of a potentially unstable airway. Chest computed tomography angiography is also an essential study for diagnosis and planning, although we do not rely on this study to define the tracheal stenosis itself. This examination is most useful in defining any anomalous branching of the trachea, such as a bronchus suis, as well as vascular anomalies, such as a left pulmonary artery sling. I also find it useful to review with the trainee the relative position of the carina with respect to adjacent vascular structures to facilitate expedient dissection of the trachea at the time of the reconstruction. Decision making related to timing of the operation may be just as important to the outcome as anatomic features. Plastic is not a friend of the small airway, and early extubation postoperatively can be a very important strategy to avoid complications. Because the reconstructed airway is sturdy and much larger than it was preoperatively, early extubation should be feasible in most patients. As many patients present with respiratory compromise and are intubated as part of their initial management, a critical decision point during preoperative planning is whether to attempt to extubate the patient before surgery or simply proceed with reconstruction more expediently. In an analysis of our early experience with the slide tracheoplasty in children, we found that preoperative mechanical ventilation was a risk factor for failure of an early postoperative extubation strategy [5Manning P.B. Rutter M.J. Border W.L. Slide tracheoplasty in infants and children: risk factors for prolonged postoperative ventilatory support.Ann Thorac Surg. 2008; 85: 1187-1192Abstract Full Text Full Text PDF PubMed Scopus (61) Google Scholar]. Migrating to a strategy of attempting preoperative extubation resulted in greater success with postoperative early extubation. In addition, we observed that inflammatory changes in the stenotic trachea from endotracheal tube trauma would largely heal given a week or two without an artificial airway before operation. Success with this strategy often relied on having an expert team in managing the compromised airway in addition to adjuncts such as heliox. The operation itself relies on good collaboration in the operating room between the thoracic surgeon, the otolaryngologist, and the anesthesiologist. All should be present and engaged during key events during the procedure. First among these is the rigid bronchoscopic visualization of the proximal end of the stenosis (Fig 1), which will make identification of this level easier when it must be viewed using a small, flexible scope later in the procedure. Guided by this bronchoscopic information, placement of the endotracheal tube can be performed, ideally positioning the tip just above the stenosis, which may result in somewhat tenuous security of the tube during positioning and initial dissection. The patient should be positioned and draped in such a way to ensure the anesthesiologist will have adequate access to the airway to reposition and possibly replace the endotracheal tube during the conduct of the procedure. Standard monitoring for cardiopulmonary bypass should include placement of a cerebral near-infrared spectroscopy probe on the right side of the forehead, as the right innominate artery is often subject to periods of retraction during the tracheal reconstruction. Because the airway may be tenuous in the younger patients with long segment tracheal stenosis, it is important to be expedient with sternotomy and dissection to the point of being able to cannulate for bypass. That involves thymic resection to afford optimal upper mediastinal exposure, and usually only upper pericardial opening. Because most cases can be done using aortic and right atrial appendage cannulation, that can often be accomplished quite quickly. Assuming the patient is stable, exposure of the entire anterior surface of the trachea can be performed before heparin administration and initiation of cardiopulmonary bypass (Fig 2). The exception to this is the child with a left pulmonary artery sling who will not tolerate dissection of the distal trachea owing to the necessary manipulation of the pulmonary arteries needed to dissect at this level. Anterior tracheal exposure is done in two steps, one above the innominate vessels, the other below. The upper dissection simply involves division of the soft tissue above the innominate vessels in the midline. That should extend superiorly to the level of the cricoid cartilage, which includes division of the thyroid isthmus with the electrocautery. The lower dissection is facilitated by placing a traction suture on the right side of the ascending aorta to pull this structure leftward. The lower half of the trachea is exposed by excision of the packet of lymph nodes in the roughly rectangular area bordered by the ascending aorta and superior vena cava laterally, and the right pulmonary artery and innominate artery inferiorly and superiorly. The recurrent laryngeal nerves, particularly on the left, are at risk of injury at a few points of this operation. During this anterior tracheal exposure, dissecting this lymph node cluster medially behind the aorta may put this nerve in danger. After the anterior surface of the distal trachea is exposed, dissection behind the right pulmonary artery is often tolerated to extend the exposure down the left and right mainstem bronchi, and beneath the carina. Cannulation for bypass is now performed. I prefer a flexible aortic cannula that can be draped off the field inferiorly, and a single right-angle venous cannula in the right atrial appendage, assuming no intracardiac repair is necessary. Bypass support is initiated, and the patient may be maintained normothermic if no intracardiac procedure is to be performed. Careful identification of the proximal and distal extent of the intended tracheoplasty is essential before transecting the airway to ensure the superior and inferior segments that will be anastomosed to each other are an equal match in length. The proximal extent of the stenosis is visualized with the flexible bronchoscope while a fine gauge needle is passed through the anterior tracheal wall from the surgical field at what is judged to be the same level. The upper extent of the reconstruction should be marked a few millimeters higher than the first complete tracheal ring. The distal end of the reconstruction almost always extends to the carina, so this level need not be visualized bronchoscopically. The length of the proposed reconstruction is then measured and the midpoint identified as the level of planned tracheal transection (Video). Circumferential dissection of the trachea is performed over a limited distance at the level of the planned transection. Dissection must stay adjacent to the tracheal wall to avoid injury to the recurrent laryngeal nerve laterally and the esophagus posteriorly. Transection of the trachea with a slight bevel creates ends that are not so blunt, making the most superior and inferior ends of the anastomosis fit together better. Dissection of the posterior tracheal wall to the same extent proximally and distally as the earlier anterior dissection allows for satisfactory mobilization of the segments to allow for a tension-free anastomosis, even in the case of a full-length slide. Avoiding lateral dissection preserves blood supply and protects the recurrent laryngeal nerves from injury. Much of the posterior dissection can be done bluntly, and it is important to be extra careful at the extreme ends of the dissection where there are no longer complete cartilaginous rings and thus a risk of perforating the membranous trachea. Opening the back wall of the lower tracheal segment to the level of the carina (Fig 3) and the front wall of the superior segment through the first normal tracheal cartilage encountered (Fig 4) will result in a reconstruction sliding the superior segment behind the inferior segment. The opposite orientation has also been described, but I have found it far easier to visualize suture placement starting inferiorly and posteriorly.Fig 4Proximal tracheal segment opened.View Large Image Figure ViewerDownload Hi-res image Download (PPT) The slide tracheoplasty anastomosis is done using a running, monofilament, absorbable suture, usually 6-0 polydioxanone in infants. Placing the first few bites on each segment with the segments slightly separated, then pulling the suture line tight allows for precise suture placement on the part of the anastomosis that will be least accessible to deal with a leak. As the upper segment is under some traction from the beginning of the anastomosis and the lower segment is not, it is essential to stretch the lower segment superiorly as each bite is taken to ensure a match in the length of the segments. Bites are taken full thickness and spaced close enough to ensure an airtight anastomosis. It is not essential that each bite passes through cartilage, but it is wise to incorporate cartilage as frequently as possible. The inferior part of the suture line is completed from inside the trachea, but transitioning so the assistant can follow from outside the trachea should be performed as early as possible as that may help minimize some of the inevitable inversion of the suture line that leads to a “figure 8” deformity. When training someone to do this procedure, the surgeon and the assistant can each do a side of the anastomosis, allowing the attending surgeon to demonstrate and then the trainee to replicate the teaching on the opposite side. After completion of the anastomosis, the mediastinum is filled with saline and a leak test is performed, asking the anesthesiologist to gradually inflate the lungs to a pressure of 35 cm H2O. The most common site for leaks is at the superior aspect of the reconstruction owing to the contour mismatch of the tracheal segments. These can be easily repaired with simple or mattress sutures. To aid in identifying the position of the endotracheal tube relative to the anastomosis on postoperative radiographs, small hemostatic clips are placed at the extremes of the reconstruction: one on the tracheal adventitia just superior to the anastomosis, and another just below the carina. Fibrin glue may be used to coat the anastomosis to assist with sealing any small leaks. Before weaning from bypass, flexible bronchoscopy should be performed by the otolaryngology colleague to clear the airway of bloody secretions and aid in proper positioning of the endotracheal tube. For long-segment reconstructions, the tube is best positioned near the midpoint of the anastomosis. For a more distal reconstruction, it may be positioned completely above the anastomosis. Follow-up bronchoscopies are performed by our otolaryngology colleagues 1 week and 2 weeks after reconstruction, and that affords the trainee an excellent opportunity to see the evolution of the healing anastomosis and the contour changes that occur in the airway as the typical “figure 8” deformity relaxes. eyJraWQiOiI4ZjUxYWNhY2IzYjhiNjNlNzFlYmIzYWFmYTU5NmZmYyIsImFsZyI6IlJTMjU2In0.eyJzdWIiOiIyZjU1MDNiYjI3NTljMmRjNjAxYTQ0MTViNDY0ZmFjYyIsImtpZCI6IjhmNTFhY2FjYjNiOGI2M2U3MWViYjNhYWZhNTk2ZmZjIiwiZXhwIjoxNjc4NDI1MDY4fQ.paDK8DlhQr-EW30BE3hRMUcaNeD59a8MPhxvAdgq3R6dC80S8L8jdS1mxxmqdFFc8CAYD7mpsFrSzcz5fwj52SunqB6Didu7Al8mAQG-3azNPpQYSXCWT7rZKqnR87-3AqQvS1fjQNhh3nqiViGS61ZtRcVpuSW1TFXtcFG-COh21Vi5S1TorlNOQ7ZmItxJPT7NBWWYsnj2sbTLHLVRLjxauPvEp7nnJy2xHiaZoIQbooDLAqiugX5oc_azx86CkORwCRL9x3SiPSZZQfAEsWt67W_QAwxG2iuqzELhVofJidGRbhPhEcZI9DJE-UGbHZFoIkf29USXTTd09a5wzA Download .mp4 (6.93 MB) Help with .mp4 files Video

Historically, mechanical circulatory support for the newborn, particularly those with a single-ventricle physiology, such as hypoplastic left heart syndrome, has been limited to extracorporeal membrane oxygenation. The results using extracorporeal membrane oxygenation have been less than optimal, and the application of the Berlin Heart, the only pediatric ventricular assist device the Food and Drug Administration has approved to date, has also been unsuccessful. We present a compilation of techniques that can be applied to these infants. The key principle revolves around the cannulation technique (and cannula used), which involves the placement of an "aortic" cannula flush with the surface of the common atrium (avoiding any extracorporeal surface exposure within the heart chambers). Further, the application of a centrifugal pump allows for a quite effective decompression of the heart in this setting. Lastly, the described approach allows the provision of support in a rather simple fashion without the use of cardiopulmonary bypass support and without the need for a ventriculotomy. Historically, mechanical circulatory support for the newborn, particularly those with a single-ventricle physiology, such as hypoplastic left heart syndrome, has been limited to extracorporeal membrane oxygenation. The results using extracorporeal membrane oxygenation have been less than optimal, and the application of the Berlin Heart, the only pediatric ventricular assist device the Food and Drug Administration has approved to date, has also been unsuccessful. We present a compilation of techniques that can be applied to these infants. The key principle revolves around the cannulation technique (and cannula used), which involves the placement of an "aortic" cannula flush with the surface of the common atrium (avoiding any extracorporeal surface exposure within the heart chambers). Further, the application of a centrifugal pump allows for a quite effective decompression of the heart in this setting. Lastly, the described approach allows the provision of support in a rather simple fashion without the use of cardiopulmonary bypass support and without the need for a ventriculotomy. Despite medical and surgical advances, two-thirds of neonates who have undergone Norwood stage I palliation for single-ventricle anomalies survive infancy without transplant. The 2014 report from the multi-institutional Single Ventricle Reconstruction (SVR) trial showed 64% 3-year transplant-free survival at 15 high-volume and experienced centers.1Newburger J.W. Sleeper L.A. Frommelt P.C. et al.Transplantation-free survival and interventions at 3 years in the single ventricle reconstruction trial.Circulation. 2014; 129: 2013-2020Crossref PubMed Scopus (145) Google Scholar For patients with failing physiology, extracorporeal membrane oxygenation (ECMO) has been the primary method of mechanical circulatory support as a bridge to buy time until transplantation. Attempts have also been made to "unload" the ventricle with salvage bidirectional Glenn palliation, but rarely has this approach been successful enough to avoid transplantation. Overall poor outcomes2Sherwin E.D. Gauvreau K. Scheurer M.A. et al.Extracorporeal membrane oxygenation after stage 1 palliation for hypoplastic left heart syndrome.J Thorac Cardiovasc Surg. 2012; 144: 1337-1343Abstract Full Text Full Text PDF PubMed Scopus (83) Google Scholar, 3Fernandez R.P. Joy B.F. Allen R. et al.Interstage survival for patients with hypoplastic left heart syndrome after ECMO.Pediatr Cardiol. 2017; 38: 50-55Crossref PubMed Scopus (9) Google Scholar following these approaches have led to a generally pessimistic view of ECMO and any other measures to rescue the failing single-ventricle patient with a shunt-dependent circulation. In a recent review of data from the investigational device exemption trial of the Berlin Heart EXCOR device (the only circulatory support device other than ECMO currently approved for use in children), only 1 out of 9 infants with single-ventricle physiology and shunt-dependent pulmonary blood flow survived to transplantation.4Weinstein S. Bello R. Pizarro C. et al.The use of the Berlin Heart EXCOR in patients with functional single ventricle.J Thorac Cardiovasc Surg. 2014; 147: 697-705Abstract Full Text Full Text PDF PubMed Scopus (166) Google Scholar Shunt-dependent single-ventricle patients are particularly challenging to support with these devices partly because both systemic and pulmonary blood flow must be supported with a single-support device, often exceeding the flow capabilities of systems designed to supply only systemic circulation. Beginning in 2011, our group began to implement alternative approaches for supporting these infants using centrifugal mechanical circulatory support systems. Specifically, we set goals to avoid the use of an oxygenator (to decrease resistance within the circuitry and hence to allow greater flows) and to cannulate in a way that would allow chest closure for an improved chronic rehabilitation of patients. The operative technique is described in detail as follows (Figure 1, Figure 2, Figure 3, Figure 4, Figure 5).Figure 1(A, B) Initially, we used standard bypass cannulas, recapitulating some adult experience for temporary support. This was obviously a simple procedure and easily implemented even during the index Norwood procedure. The cannulas could be externalized after securing them to the edges of the native pericardium inside the chest (in addition to the usual sutures on the outside) (A). We could achieve the high flows necessary for adequate support of these infants (up to cardiac index of ~6 to 7 L/min/m2). This approach, however, had a significant limitation mainly because of thromboembolic events likely from clot burden within and surrounding the cannulas. It was clear from these early cases, as well as from our experience with paracorporeal lung assist devices,5Hoganson D.M. Gazit A.Z. Sweet S.C. et al.Neonatal paracorporeal lung assist device for respiratory failure.Ann Thorac Surg. 2013; 95: 692-694Abstract Full Text Full Text PDF PubMed Scopus (14) Google Scholar that the cannula design was the rate-limiting step to successful support. Specifically, the large cannula tip within the relatively small atrium leads to a large thrombogenic surface area, and any "cul de sacs" devoid of moving blood (eg, between the cannula edge and the atrial wall or the residual septum, B) could also be a nidus for clot formation despite adequate anticoagulation. The alternative of introducing a smaller portion of the cannula into the atrium would likely create much angst with a possible dislodgement.Show full caption(C, D) For this reason, in the next evolution of this approach, 5-mm ringed GORE-TEX grafts were sewn to the neo-Ao or dunked into the common atrium; the grafts were then tunneled through the skin allowing chest closure (C). Femoral arterial cannulas (14 Fr) were then introduced into these inflow and outflow grafts to provide support. For the purposes of this report, the descriptions of "inflow" and "outflow" follow the same convention as that used in terminology related to assist devices: inflow cannula refers to that which draws blood into the pump from the heart, whereas outflow cannula refers to that which conveys blood from the pump to the ascending Ao. In cases where we anticipate a high likelihood of needing support post Norwood (eg, significant preoperative tricuspid regurgitation), then a 5-mm ringed GORE-TEX graft is sewn to the homograft patch before arch reconstruction. The graft is temporarily clipped and used only in case mechanical support is needed at the end of the case. If the intent is to leave the operating room with these grafts clipped (for possible use in the later postoperative period), it is critical that the grafts are clipped flush with the anastomotic surface to avoid the formation of a thrombus in the graft. It is critical to introduce the venous cannula in the inflow limb all the way up to the site of entry of the GORE-TEX graft into the atrium. Otherwise, the walls of the graft will get sucked down with the initiation of support. Lastly, at least 2 cm of the GORE-TEX graft should be protruding through the skin exit site to allow a reasonable handle for manipulation or removal of the cannulas from the graft. An alternative strategy that we have recently pursued is to attach the GORE-TEX outflow graft to the descending Ao because we routinely cannulate this structure during the Norwood procedure (D). This procedure would make it easier to remove the cannulas postoperatively even when the chest is closed. With these GORE-TEX graft extensions, we have not had thromboembolic events that we observed using the standard bypass cannula. The use of GORE-TEX extensions with bypass cannulas potentially allows the exchange of the cannulas in case of concerns with clot buildup and avoids the primary problem of a large plastic surface protruding in the middle of the common atrium. Further, these extensions facilitate the implementation of support for very small babies in whom larger-size ready-made or off-the-shelf cannulas become challenging. There are limitations, however, with these composite GORE-TEX bypass cannulations as well. After about a week, the cannulas become quite stuck within the grafts, and for that reason, we evolved to the use of Berlin Heart cannulas when anticipating prolonged support (months). The Berlin Heart cannulas are specifically designed for long-term use in a paracorporeal fashion. Ao = aorta.View Large Image Figure ViewerDownload Hi-res image Download (PPT)Figure 2In our experience, 50% of the single-ventricle infants referred for heart failure management are already on ECMO when they were sent to our institution. In nearly all these instances, the patients had been cannulated via a cervical approach. Although these peripheral ECMO cannulas are limited in how much support they can provide, the ECMO configuration does allow for oxygenation of the neonate, which would otherwise not be feasible with the right ventricle-to-pulmonary artery (RV-to-PA) shunt configuration, the case for all of our referrals thus far. For this reason, our first step involves the creation of a systemic to pulmonary artery shunt from the innominate artery to the right pulmonary artery. The RV-to-PA conduit is then divided so that as much of the native cardiac output is directed systemically (A). BT = blalock-taussig.Show full caption(B) Alternatively, the RV-to-PA conduit can be disconnected from its attachment to the right ventricle and sewn to the side of the polytetrafluoroethylene graft that is attached to the Berlin outflow cannula; this approach, however, requires the composite graft attached to the Berlin cannula to be longer, otherwise can lead to a potential kinking of the graft at the anastomosis.View Large Image Figure ViewerDownload Hi-res image Download (PPT)Figure 3Unfortunately, the 5-mm EXCOR arterial cannula is no longer manufactured in United States, which would be ideal for a smaller baby (~2.5 kg). Therefore, in most instances, we attach the 6-mm EXCOR arterial cannula with a short (~1 cm) polytetrafluoroethylene extension (8-mm graft) to the neo-Ao (A). The 8-mm graft is stretched using serial Hegar dilators up to 10 mm, which allow for a snug fit of the GORE-TEX extension to the cannula tip. Although implantation onto the homograft patch along the inner curve near the lower aspect of the neo-Ao reconstruction may be easier, placement on the greater curve a centimeter or 2 below the innominate artery takeoff leads to a better flow directed down the arch. This approach is obviously restricted to those who are more remote from their Norwood procedure so that one is not dealing with proximity to the suture line between the patch and the diminutive ascending Ao. The preferred position of the cannula along the outer curve of neo-Ao ensures that the inflow jet is not directed at the innominate artery, which can give rise to an inadvertently high Qp-to-Qs ratio; we observed this unusual phenomenon in a couple of patients (in whom the cannula had been placed on the medial side of the neo-Ao) as an incidental finding during a subsequent cardiac catheterization. Similarly, when using the Berlin arterial cannula, it is important that the angulation of the cannula or composite graft is not directed toward the neoaortic valve to avoid neoaortic valve insufficiency. In patients who have undergone a hybrid approach, a modified approach can be implemented. Ao = aorta; Pa = pulmonary artery.Show full caption(B) After the placement of bands on the left and then the right branch pulmonary arteries, a 10-mm graft is sewn to the distal MPA just before the takeoff of the ductus. In this instance, we use a 10-mm graft (instead of 8 mm) because serial dilations of the graft are not feasible after the attachment to the pulmonary artery.(C) The ductal stent is then placed through this graft in standard fashion.(D) Finally, a clamp is applied to the base of the graft and the aortic cannula is sewn to the graft with 4 separate 5.0 Prolene sutures (at 4 quadrants: 3, 6, 9, and 12 o'clock). The graft is further secured with a 2.0 silk tie placed around the flange of the cannula over the GORE-TEX graft. This construction ends up being slightly longer than when the composite is created before sewing the graft to the pulmonary artery simply because there is less ability to manipulate the graft after it is sewn to the pulmonary artery. In this instance, it is critical to ensure the GORE-TEX graft does not rotate or move much; a suture can be applied to the sewing cuff of the cannula and then tack that down onto the MPA if the natural lie seems likely to lead to rotation and a possible kinking of the GORE-TEX portion. The cannula is deaired and then tunneled through the abdominal wall. MPA = main pulmonary artery.View Large Image Figure ViewerDownload Hi-res image Download (PPT)Figure 3Unfortunately, the 5-mm EXCOR arterial cannula is no longer manufactured in United States, which would be ideal for a smaller baby (~2.5 kg). Therefore, in most instances, we attach the 6-mm EXCOR arterial cannula with a short (~1 cm) polytetrafluoroethylene extension (8-mm graft) to the neo-Ao (A). The 8-mm graft is stretched using serial Hegar dilators up to 10 mm, which allow for a snug fit of the GORE-TEX extension to the cannula tip. Although implantation onto the homograft patch along the inner curve near the lower aspect of the neo-Ao reconstruction may be easier, placement on the greater curve a centimeter or 2 below the innominate artery takeoff leads to a better flow directed down the arch. This approach is obviously restricted to those who are more remote from their Norwood procedure so that one is not dealing with proximity to the suture line between the patch and the diminutive ascending Ao. The preferred position of the cannula along the outer curve of neo-Ao ensures that the inflow jet is not directed at the innominate artery, which can give rise to an inadvertently high Qp-to-Qs ratio; we observed this unusual phenomenon in a couple of patients (in whom the cannula had been placed on the medial side of the neo-Ao) as an incidental finding during a subsequent cardiac catheterization. Similarly, when using the Berlin arterial cannula, it is important that the angulation of the cannula or composite graft is not directed toward the neoaortic valve to avoid neoaortic valve insufficiency. In patients who have undergone a hybrid approach, a modified approach can be implemented. Ao = aorta; Pa = pulmonary artery.Show full caption(B) After the placement of bands on the left and then the right branch pulmonary arteries, a 10-mm graft is sewn to the distal MPA just before the takeoff of the ductus. In this instance, we use a 10-mm graft (instead of 8 mm) because serial dilations of the graft are not feasible after the attachment to the pulmonary artery.(C) The ductal stent is then placed through this graft in standard fashion.(D) Finally, a clamp is applied to the base of the graft and the aortic cannula is sewn to the graft with 4 separate 5.0 Prolene sutures (at 4 quadrants: 3, 6, 9, and 12 o'clock). The graft is further secured with a 2.0 silk tie placed around the flange of the cannula over the GORE-TEX graft. This construction ends up being slightly longer than when the composite is created before sewing the graft to the pulmonary artery simply because there is less ability to manipulate the graft after it is sewn to the pulmonary artery. In this instance, it is critical to ensure the GORE-TEX graft does not rotate or move much; a suture can be applied to the sewing cuff of the cannula and then tack that down onto the MPA if the natural lie seems likely to lead to rotation and a possible kinking of the GORE-TEX portion. The cannula is deaired and then tunneled through the abdominal wall. MPA = main pulmonary artery.View Large Image Figure ViewerDownload Hi-res image Download (PPT)Figure 4Placement of the common atrial cannula is perhaps the most critical step in this configuration because inflow or venous drainage is typically the major determinant of the flow capacity of a mechanical circulatory support system. We again use a 6-mm (luminal dimension) Berlin Heart arterial cannula for this purpose up to 3-4 months of age. For the larger or older infant, we will consider the use of a 9-mm cannula. A key step is to ensure none of the atrial wall tissue obstructs the lumen of the cannula, particularly given the relatively short lip of this cannula. Often the atrium is big enough that a side-biting clamp can be safely applied, allowing a controlled atriotomy of appropriate size (A).Show full caption(B) To accomplish this, an over-and-over sewing of the atrial edge with a 5.0 Prolene suture ensures the tissue fold into the lumen of the cannula. In the very young, this step is not necessary because typically the atrial tissue is quite thin. If the heart is arrested (for purposes of performing some intracardiac procedure), then the introduction of the cannula can easily be performed on the "way out." Alternatively, 1 of 2 different approaches can be used for insertion either off pump or with ECMO support. If there is adequate atrial tissue that can be occluded within the side-biting clamp, then one can easily proceed with the next step.(C) A series of pledgeted 5.0 Prolene sutures are placed circumferentially around the opening and passed through the sewing cuff of the cannula and tied down. A running technique can also be implemented and is faster, but in our experience, the interrupted technique appears to be more hemostatic. The cannula is then filled with blood in retrograde fashion and then passed through a tunnel created in the subxiphoidal area. This is particularly critical for the inflow cannula as deairing is not particularly effective or feasible once it has been tunneled through abdominal wall.(D) Alternatively, if there is not enough room for the application of a side-biting clamp, then the following approach can be implemented. The edges of the cannula are painted with a marker pen and then this cannula is applied onto the surface of the atrium to create a mark approximating the size of the opening necessary for allowing the introduction of the cannula. This method will guide the length of the incision to be made, which should be larger than the size of the marked spot to decrease the chances of the cannula falling out or bleeding during this maneuver. Four or 5 pledgeted sutures are placed around the circumference of the mark and brought up through the sewing cuff circumferentially. A few of these sutures are then passed through a snare, ready to be cinched down as needed. A stab incision is then made (while ventilation is being held) and the cannula is quickly slipped into the hole. While the cannula is being held against the atrial wall by the assistant, the previously placed sutures are tied down. Additional sutures can be applied either individually or in a running technique to further secure the cannula in place. A safety tourniquet suture line can be placed in case the cannula slips out to protect against significant bleeding. Once again, the cannula is filled with blood and passed in a retrograde fashion through the subxiphoid space.View Large Image Figure ViewerDownload Hi-res image Download (PPT)Figure 5Infants and newborns can be quite active particularly with the Berlin Heart and the composite GORE-TEX graft configurations described here. The exit sites do not appear to cause any discomfort for them as the babies can easily lay prone on these cannulas (and without any change in the flow patterns of the device). Early after insertion, the infants require quite high pump flows (confidence interval of 6-7 L/min/m2). Often the amount of support needed decreases with time perhaps in part because of some recovery of a native cardiac function that we have invariably seen in all infants despite the grave magnitude of dysfunction early on. Despite the relatively long segment of tubing outside the body (~3 to 4 ft), there is no thermoregulation problems in these infants. We follow a regimen of heparin (initially infusion followed by transition to Lovenox with an anti-Xa goal-directed titration) and aspirin anticoagulation. We aggressively pursue normal oral feeding in these infants and have concerns when such progress is not made; we have a low threshold to perform cardiac catheterization as well as computed tomography angiography to assess the anatomy and hemodynamics in these circumstances. The infants should thrive and have a correction of their weight-for-age z-scores typically within a month. (Color version of figure is available online at www.optechtcs.com.)View Large Image Figure ViewerDownload Hi-res image Download (PPT) (C, D) For this reason, in the next evolution of this approach, 5-mm ringed GORE-TEX grafts were sewn to the neo-Ao or dunked into the common atrium; the grafts were then tunneled through the skin allowing chest closure (C). Femoral arterial cannulas (14 Fr) were then introduced into these inflow and outflow grafts to provide support. For the purposes of this report, the descriptions of "inflow" and "outflow" follow the same convention as that used in terminology related to assist devices: inflow cannula refers to that which draws blood into the pump from the heart, whereas outflow cannula refers to that which conveys blood from the pump to the ascending Ao. In cases where we anticipate a high likelihood of needing support post Norwood (eg, significant preoperative tricuspid regurgitation), then a 5-mm ringed GORE-TEX graft is sewn to the homograft patch before arch reconstruction. The graft is temporarily clipped and used only in case mechanical support is needed at the end of the case. If the intent is to leave the operating room with these grafts clipped (for possible use in the later postoperative period), it is critical that the grafts are clipped flush with the anastomotic surface to avoid the formation of a thrombus in the graft. It is critical to introduce the venous cannula in the inflow limb all the way up to the site of entry of the GORE-TEX graft into the atrium. Otherwise, the walls of the graft will get sucked down with the initiation of support. Lastly, at least 2 cm of the GORE-TEX graft should be protruding through the skin exit site to allow a reasonable handle for manipulation or removal of the cannulas from the graft. An alternative strategy that we have recently pursued is to attach the GORE-TEX outflow graft to the descending Ao because we routinely cannulate this structure during the Norwood procedure (D). This procedure would make it easier to remove the cannulas postoperatively even when the chest is closed. With these GORE-TEX graft extensions, we have not had thromboembolic events that we observed using the standard bypass cannula. The use of GORE-TEX extensions with bypass cannulas potentially allows the exchange of the cannulas in case of concerns with clot buildup and avoids the primary problem of a large plastic surface protruding in the middle of the common atrium. Further, these extensions facilitate the implementation of support for very small babies in whom larger-size ready-made or off-the-shelf cannulas become challenging. There are limitations, however, with these composite GORE-TEX bypass cannulations as well. After about a week, the cannulas become quite stuck within the grafts, and for that reason, we evolved to the use of Berlin Heart cannulas when anticipating prolonged support (months). The Berlin Heart cannulas are specifically designed for long-term use in a paracorporeal fashion. Ao = aorta. (B) Alternatively, the RV-to-PA conduit can be disconnected from its attachment to the right ventricle and sewn to the side of the polytetrafluoroethylene graft that is attached to the Berlin outflow cannula; this approach, however, requires the composite graft attached to the Berlin cannula to be longer, otherwise can lead to a potential kinking of the graft at the anastomosis. (B) After the placement of bands on the left and then the right branch pulmonary arteries, a 10-mm graft is sewn to the distal MPA just before the takeoff of the ductus. In this instance, we use a 10-mm graft (instead of 8 mm) because serial dilations of the graft are not feasible after the attachment to the pulmonary artery. (C) The ductal stent is then placed through this graft in standard fashion. (D) Finally, a clamp is applied to the base of the graft and the aortic cannula is sewn to the graft with 4 separate 5.0 Prolene sutures (at 4 quadrants: 3, 6, 9, and 12 o'clock). The graft is further secured with a 2.0 silk tie placed around the flange of the cannula over the GORE-TEX graft. This construction ends up being slightly longer than when the composite is created before sewing the graft to the pulmonary artery simply because there is less ability to manipulate the graft after it is sewn to the pulmonary artery. In this instance, it is critical to ensure the GORE-TEX graft does not rotate or move much; a suture can be applied to the sewing cuff of the cannula and then tack that down onto the MPA if the natural lie seems likely to lead to rotation and a possible kinking of the GORE-TEX portion. The cannula is deaired and then tunneled through the abdominal wall. MPA = main pulmonary artery. (B) After the placement of bands on the left and then the right branch pulmonary arteries, a 10-mm graft is sewn to the distal MPA just before the takeoff of the ductus. In this instance, we use a 10-mm graft (instead of 8 mm) because serial dilations of the graft are not feasible after the attachment to the pulmonary artery. (C) The ductal stent is then placed through this graft in standard fashion. (D) Finally, a clamp is applied to the base of the graft and the aortic cannula is sewn to the graft with 4 separate 5.0 Prolene sutures (at 4 quadrants: 3, 6, 9, and 12 o'clock). The graft is further secured with a 2.0 silk tie placed around the flange of the cannula over the GORE-TEX graft. This construction ends up being slightly longer than when the composite is created before sewing the graft to the pulmonary artery simply because there is less ability to manipulate the graft after it is sewn to the pulmonary artery. In this instance, it is critical to ensure the GORE-TEX graft does not rotate or move much; a suture can be applied to the sewing cuff of the cannula and then tack that down onto the MPA if the natural lie seems likely to lead to rotation and a possible kinking of the GORE-TEX portion. The cannula is deaired and then tunneled through the abdominal wall. MPA = main pulmonary artery. (B) To accomplish this, an over-and-over sewing of the atrial edge with a 5.0 Prolene suture ensures the tissue fold into the lumen of the cannula. In the very young, this step is not necessary because typically the atrial tissue is quite thin. If the heart is arrested (for purposes of performing some intracardiac procedure), then the introduction of the cannula can easily be performed on the "way out." Alternatively, 1 of 2 different approaches can be used for insertion either off pump or with ECMO support. If there is adequate atrial tissue that can be occluded within the side-biting clamp, then one can easily proceed with the next step. (C) A series of pledgeted 5.0 Prolene sutures are placed circumferentially around the opening and passed through the sewing cuff of the cannula and tied down. A running technique can also be implemented and is faster, but in our experience, the interrupted technique appears to be more hemostatic. The cannula is then filled with blood in retrograde fashion and then passed through a tunnel created in the subxiphoidal area. This is particularly critical for the inflow cannula as deairing is not particularly effective or feasible once it has been tunneled through abdominal wall. (D) Alternatively, if there is not enough room for the application of a side-biting clamp, then the following approach can be implemented. The edges of the cannula are painted with a marker pen and then this cannula is applied onto the surface of the atrium to create a mark approximating the size of the opening necessary for allowing the introduction of the cannula. This method will guide the length of the incision to be made, which should be larger than the size of the marked spot to decrease the chances of the cannula falling out or bleeding during this maneuver. Four or 5 pledgeted sutures are placed around the circumference of the mark and brought up through the sewing cuff circumferentially. A few of these sutures are then passed through a snare, ready to be cinched down as needed. A stab incision is then made (while ventilation is being held) and the cannula is quickly slipped into the hole. While the cannula is being held against the atrial wall by the assistant, the previously placed sutures are tied down. Additional sutures can be applied either individually or in a running technique to further secure the cannula in place. A safety tourniquet suture line can be placed in case the cannula slips out to protect against significant bleeding. Once again, the cannula is filled with blood and passed in a retrograde fashion through the subxiphoid space. We have managed 10 post-Norwood patients with the variety of the configurations described here (9 after stage I palliation and 1 following hybrid palliation); there have been 5 other non-hypoplastic left heart syndrome single-ventricle patients, suggesting the potential application to other lesion sets. As noted previously, 50% of the patients were on ECMO at the time of implant; 40% had renal failure. In all instances, full support could be provided without complications until the decision regarding the plan of care was final (recovery, transplant, or redirection of care) for a median duration of 52 days (range 3-96 days). In summary, the described approaches have a number of advantages not previously achievable in this subset of patients. Most importantly, with this approach, the patient is cannulated via the atrium, as opposed to the ventricle, which requires much less intraoperative dissection and eliminates the need for the excision of obstructive muscle bundles and chordae frequently encountered in the morphologic right ventricle. By avoiding cardiopulmonary bypass, we have had minimal postoperative bleeding and, hence, less blood product exposure, which likely is beneficial for an infant under consideration for transplantation. The modification (with GORE-TEX connections, W. L. Gore & Associates, Inc. Flagstaff, Arizona) also has the advantage of allowing chest closure immediately following the Norwood procedure. Lastly, achieving the high flows necessary to support both systemic and pulmonary blood flows overcomes the main limitation of peripheral ECMO cannulation in these infants. The ease with which the procedures can be carried out has led to our routine adoption of these techniques for our high-risk patients.

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.

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.

See related article on pages 1524-30. See related article on pages 1524-30. I recall from early in my training a mentor routinely referring to staged reconstruction for single ventricle cardiac defects as “permanent palliation,” implying that although complete cavopulmonary connection may afford a physiologic correction with separation of the systemic and pulmonary circulations, there remained long-term liability inherent with the lack of a pulmonary ventricle. In 1984, Kawashima and associates1Kawashima Y. Kitamura S. Matsuda H. Shimazaki Y. Nakano S. Hirose H. Total cavopulmonary shunt operation in complex cardiac anomalies. A new operation.J Thorac Cardiovasc Surg. 1984; 87: 74-81PubMed Google Scholar described an experience of 4 patients with “presently uncorrectable cyanotic cardiac anomalies.” Patients with heterotaxia and anomalies of systemic venous drainage were at the time thought to be impossible to manage in the manner that was being proposed for other single ventricle anomalies. They described a “total cavopulmonary shunt operation” for patients with azygos or hemiazygos continuation of the absent inferior vena cava that became eponymously named the “Kawashima” operation. Although they recognized this operation did not constitute a complete diversion of systemic venous return to the pulmonary arteries, leaving the “hepatocardiac venous and coronary sinus flow” to continue to bypass the lungs, they anticipated that this would result in a satisfactory end state, or permanent palliation, for this patient cohort. The predictable development of pulmonary arteriovenous malformations and the resultant cyanosis and decline in functional status due to the lack of the unidentifiable “hepatic factor” bathing the lungs have led to the recognition that in most patients the Kawashima operation is, in fact, not an equivalent permanent palliation to the more conventional Fontan operation and its modifications. If we agree that the answer to the question, “Is the Kawashima operation a permanent palliation for this group of complex patients with single ventricles?” is “No,” then this raises 2 additional important questions: “What is the best way to enable this hepatic factor to bathe the lungs?”, and “What should be the proper timing of such a maneuver?” The article by Arrigoni and colleagues2Arrigoni S.C. van den Heuvel F. Willems T.P. Hillege H. Lindberg H.L. Berger R.M.F. et al.Off-pump hepatic to azygos connection via thoracotomy for relief of fistulas after a Kawashima procedure: Ten-year results.J Thorac Cardiovasc Surg. 2015; 149: 1524-1530Abstract Full Text Full Text PDF Scopus (6) Google Scholar helps to address the first question well and shares the authors' bias regarding the second. There are many disadvantages in simply performing a “routine” completion Fontan operation in this patient population, including the anatomic challenges posed by the often multiple sites of connection of the hepatic veins to the atrium and the difficulty that accompanies routing blood flow from the level of the diaphragm to the pulmonary arteries in the presence of atrial and ventricular situs abnormalities. The lower volume of flow compared with a typical Fontan pathway, in addition to the challenging geometry, likely increases the risk of pathway thrombosis, a complication I have unfortunately seen in my personal experience. The concept of performing an extra-anatomic connection of the hepatic veins to the azygos or hemiazygos vein was first described by Steinberg and colleagues3Steinberg J. Alfieris G.M. Brandt B. Smith F. Byrum C.J. Fink G.W. et al.New approach to the surgical management of pulmonary arteriovenous malformations after cavopulmonary anastomosis.Ann Thorac Surg. 2003; 75: 1640-1642Abstract Full Text Full Text PDF PubMed Scopus (26) Google Scholar in 2003 using a sternotomy approach and cardiopulmonary bypass support. The Groningen team reported applying this concept via thoracotomy, avoiding cardiopulmonary bypass in 2008.4Lopez F.E. van den Heuvel F. Pieper P. Waterbolk T.W. Ebels T. Off-pump connection of the hepatic to the azygos vein through a lateral thoracotomy for relief of arterio-venous fistulas after a Kawashima procedure.Cardiol Young. 2008; 18: 311-315Crossref PubMed Scopus (12) Google Scholar They advocated multiple advantages of this technique, including avoidance of a resternotomy, avoidance of cardiopulmonary bypass support, and creation of a short prosthetic communication. Their current communication is important because with follow-up to 10 years, they demonstrate the durability of this technique in relieving cyanosis and improving cardiac functional status.2Arrigoni S.C. van den Heuvel F. Willems T.P. Hillege H. Lindberg H.L. Berger R.M.F. et al.Off-pump hepatic to azygos connection via thoracotomy for relief of fistulas after a Kawashima procedure: Ten-year results.J Thorac Cardiovasc Surg. 2015; 149: 1524-1530Abstract Full Text Full Text PDF Scopus (6) Google Scholar Although the technique they describe is technically simple, their experience underscores the importance of adequately defining the often unusual hepatic venous connection to the atria when planning for such an intervention via a thoracotomy approach. Despite their practice of routinely obtaining computed tomography angiography to examine this anatomy, 2 of 11 patients required reoperation to manage additional hepatic venous connections that had not been incorporated into their shunt, which thus served as venovenous collaterals leading to persistent cyanosis. As in a game of chess, when one must always be thinking of the next move when planning an attack, examination of the hepatic venous connections at the time of the original Kawashima operation may have supplemented the understanding of this critical anatomy and aided in planning a subsequent hepatic to azygos shunt. In regard to the proper timing of an intervention to incorporate hepatic venous flow into the cavopulmonary circulation, a number of authors have advocated the routine addition of this step, either as a component of the initial Kawashima operation or in a time frame similar to completion of a traditional Fontan operation, citing the near certainty of the development of pulmonary arteriovenous malformations over time. Arrigoni and colleagues2Arrigoni S.C. van den Heuvel F. Willems T.P. Hillege H. Lindberg H.L. Berger R.M.F. et al.Off-pump hepatic to azygos connection via thoracotomy for relief of fistulas after a Kawashima procedure: Ten-year results.J Thorac Cardiovasc Surg. 2015; 149: 1524-1530Abstract Full Text Full Text PDF Scopus (6) Google Scholar choose to wait for specific indications of cyanosis or declining functional status. In the absence of any longitudinal follow-up studies of this population examining the actual incidence and liability of pulmonary arteriovenous malformations, their approach is easily defended. Regardless of one's bias about the timing issue, their experience certainly endorses consideration of this technique for all cases with anatomy amendable to its application. Off-pump hepatic to azygos connection via thoracotomy for relief of fistulas after a Kawashima procedure: Ten-year resultsThe Journal of Thoracic and Cardiovascular SurgeryVol. 149Issue 6PreviewAn almost universal incidence of developing pulmonary arteriovenous fistulas after the Kawashima operation has been reported. Exclusion of the hepatic venous flow from the pulmonary circulation causes the development of these malformations. Redirection of hepatic venous flow to the pulmonary circulation mostly leads to the regression of the arteriovenous fistulas. Full-Text PDF Open Archive