Norwood Operation: Immediate vs Delayed Sternal Closure.

BACKGROUND: The Norwood operation is a complex neonatal surgery. There are limited data to inform the timing of sternal closure. After the Norwood operation, delayed sternal closure (DSC) is frequent. We aimed to examine the association of DSC with outcomes, with a particular interest in how sternal closure at the time of surgery compared with the timing of DSC. Our outcomes included mortality, length of ventilation, length of stay, and postoperative complications. METHODS: This retrospective study included neonates who underwent a Norwood operation reported in the Pediatric Cardiac Critical Care Consortium registry from February 2019 through April 2021. Outcomes of patients with closed sternum were compared to those with sternal closure prior to postoperative day 3 (early closure) and prior to postoperative day 6 (intermediate closure). RESULTS: The incidence of DSC was 74% (500 of 674). The median duration of open sternum was 4 days (interquartile range 3-5 days). Comparing patients with closed sternum to patients with early sternal closure, there was no statistical difference in mortality rate (1.1% vs 0%) and the median hospital postoperative stay (30 days vs 31 days). Compared with closed sternum, patients with intermediate sternal closure required longer mechanical ventilation (5.9 days vs 3.9 days) and fewer subsequent sternotomies (3% vs 7.5%). CONCLUSIONS: For important outcomes following the Norwood operation there is no advantage to chest closure at the time of surgery if the chest can be closed prior to postoperative day 3.

Interruption of the Ascending Aorta-Biventricular Surgical Repair.

Interruption of the ascending aorta is an extremely rare anomaly defined by a point of interruption between the intrapericardial and extrapericardial aorta and can be explained by developmental errors proximal to the embryologic right aortic sac. Herein, we present a case of interruption of the ascending aorta and describe a successful biventricular surgical repair of this unique anomaly.

Most High-Intensity Transient Signals Are Not Associated With Specific Surgical Maneuvers.

BACKGROUND: Mortality after congenital heart defect surgery has dropped dramatically in the last few decades. Current research on long-term outcomes has focused on preventing secondary neurological sequelae, for which embolic burden is suspected. In children, little is known of the correlation between specific surgical maneuvers and embolic burden. Transcranial Doppler ultrasound is highly useful for detecting emboli but has not been widely used with infants and children. METHODS: Bilateral middle cerebral artery blood flow was continuously monitored from sternal incision to chest closure in 20 infants undergoing congenital heart defect repair or palliative surgery. Embolus counts for specific maneuvers were recorded using widely accepted criteria for identifying emboli via high-intensity transient signals (HITS). RESULTS: An average of only 13% of all HITS detected during an operation were correlated with any of the surgical maneuvers of interest. The highest mean number of HITS associated with a specific maneuver occurred during cross-clamp removal. Cross-clamp placement also had elevated HITS counts that significantly differed from other maneuvers. CONCLUSIONS: In this study of infants undergoing cardiac surgery with cardiopulmonary bypass, the great majority of HITS detected are not definitively associated with a specific subset of surgical maneuvers. Among the measured maneuvers, removal of the aortic cross-clamp was associated with the greatest occurrence of HITS. Future recommended research efforts include identifying and confirming other sources for emboli and longitudinal outcome studies to determine if limiting embolic burden affects long-term neurological outcomes.

Williams Syndrome and Neonatal Cardiac Surgery for Congenital Single Ventricle.

Williams syndrome (WS) is an arteriopathic derangement associated with supravalvular aortic stenosis and branch pulmonary stenosis. We describe double-outlet right ventricle with mitral atresia and aortic arch hypoplasia in an infant with WS. This case demonstrates the difficulty in managing patients with WS with complex cardiac defects. To our knowledge, this is the first reported single-ventricle physiology in a patient with WS. (Level of Difficulty: Advanced.).

Supplemental Perfusion Techniques for Aortic Arch Reconstruction, With Emphasis on Direct Cannulation of the Descending Aorta.

The established techniques of deep hypothermia with circulatory arrest and regional cerebral perfusion expose infants and children to additional physiologic stress and deleterious effects which may adversely affect the outcome of operations involving reconstruction of the aortic arch. Alternative techniques to supplement perfusion support are an area of innovation today. The most effective adjunct for somatic perfusion during arch reconstruction is direct cannulation of the innominate artery and the descending aorta, with full flow at mild hypothermia distributed throughout the entire body just as it is during routine, single cannulation surgery with an intact aorta. Detailed facilitating techniques for descending aortic cannulation are discussed.

Evaluation of cellular ingrowth within porcine extracellular matrix scaffolding in congenital heart disease surgery.

The search for an ideal material for cardiac tissue repair has led to utilization of porcine small intestinal submucosa extracellular matrix (CorMatrix). Here, we examine the histologic features of CorMatrix and the associated cellular growth at a variety of time intervals. Tissues with CorMatrix from ten patients (4 male, 6 female) with ages ranging from 2 weeks to 2 years, and implant duration ranging from 1 week to 2 years were included in this study. Samples for analysis were collected at autopsy. Surgical repair sites included great vessel repair (n=9), atrial septum defect (n=1), coronary vessels (n=1), as well as aortic (n=1) and mitral valve (n=2) leaflets. In all specimens, CorMatrix was composed of dense laminated regions of collagen, without appreciable elastin staining. In most grafts, especially those implanted for extended periods of time, tissue with luminal CD31 positivity covered the intimal surface of the CorMatrix graft. This tissue (neo-intima) consisted of spindled myofibroblasts (SMA) and small CD31 positive vessels with occasional mononuclear cells in a matrix composed of collagen, glycosaminoglycans, and rarely elastin, after extended periods of implantation. These features were readily identified in patients as early as 1 month after CorMatrix implantation. The matrix comprising the CorMatrix itself remained largely acellular, despite implantation times up to 2 years, with degradation of the graft material. We provide a framework for histologic expectations when evaluating explanted CorMatrix grafts. In this regard, the CorMatrix matrix is likely to remain acellular without significant elastin deposition, whereas the intimal and adventitial surfaces become coated by proliferating cells in a novel matrix of collagen and glycosaminoglycans.

Outcomes related to immediate extubation after stage 1 Norwood palliation for hypoplastic left heart syndrome.

OBJECTIVE: Immediate extubation may have outcome benefits when judiciously instituted after neonatal congenital cardiac surgery. We sought to evaluate the outcomes of immediate extubation specifically in neonates undergoing stage 1 Norwood palliation of hypoplastic left heart syndrome. METHODS: Consecutive neonates undergoing stage 1 Norwood (January 2010 to December 2016) for hypoplastic left heart syndrome were retrospectively studied. Immediate extubation was defined as successful extubation before termination of anesthetic care. Preoperative and intraoperative variables were compared between immediate extubation and nonimmediate extubation groups, and bivariate analyses and descriptive methods were used to express the association of outcome variables with immediate extubation. Data were expressed as number and percent for categoric variables, and median and interquartile range for continuous variables. RESULTS: Of 23 patients who underwent stage 1 palliation, 5 had immediate extubation (22%). There were no differences in preoperative or intraoperative factors between patients who did and did not undergo immediate extubation. There were no deaths in the immediate extubation group. In the nonimmediate extubation group, 3 patients died before hospital discharge. One patient who had immediate extubation and 4 patients among those who did not have immediate extubation had to be reintubated in the 96 hours that followed extubation (P = 1). Intensive care unit length of stay was 8 (3-17) and 8 (5-18) (days) for the immediate extubation group and nonimmediate extubation groups, respectively (P = .71). CONCLUSIONS: Immediate extubation strategy was safely accomplished in one-fifth of this cohort of hypoplastic left heart syndrome. A larger cohort may delineate the determinants of immediate extubation and its benefits in infants undergoing stage 1 single ventricle palliation.

Infundibular sparing versus transinfundibular approach to the repair of tetralogy of Fallot.

INTRODUCTION: The right ventricular infundibular sparing approach (RVIS) to the repair of tetralogy of Fallot (TOF) avoids a full-thickness ventricular incision, typically utilized in the transinfundibular (TI) method. METHODS: We performed a retrospective, age-matched cohort study of patients who underwent RVIS at Texas Children's Hospital or TI at Children's Hospital Medical Center in Nebraska and subsequently underwent cardiac magnetic resonance imaging (CMR). We compared right ventricular end-diastolic and systolic volumes indexed to body surface area (RVEDVi and RVESVi) and right ventricular ejection fraction (RVEF) as primary endpoints. Secondary endpoints were indexed left ventricular diastolic and systolic volume (LVEDVi and LVESVi), left ventricular ejection fraction (LVEF), right ventricular (RV) sinus ejection fraction (EF) and RV outflow tract EF (RVOT EF). RESULTS: Seventy-nine patients were included in the analysis; 40 underwent RVIS and 39 underwent TI repair. None of the patients in the TI repair group had an initial palliation with a systemic to pulmonary arterial shunt compared to seven (18%) in the RVIS group (P < .01). There was no appreciable difference in RVEDVi (122 ± 29 cc/m2 vs 130 ± 29 cc/m2 , P = .59) or pulmonary regurgitant fraction (40 ± 13 vs 37 ± 18, P = .29) between the RVIS and TI groups. Compared to the TI group, the RVIS group had higher RVEF (54 ± 6% vs 44 ± 9%, P < .01), lower RVESV (57 ± 17 cc/m2 vs 67 ± 25 cc/m2 , P = .03), higher LVEF (61 ± 11% vs 54 ± 8%, P < .01), higher RVOT EF (47 ± 12% vs 41 ± 11%, P = .03), and higher RV sinus EF (56 ± 5% vs 49 ± 6%, P < .01) CONCLUSIONS: In this selected cohort, patients who underwent RVIS repair for TOF had higher right and left ventricular ejection fraction compared to those who underwent TI repair.

Surgical Atrioventricular Valve Replacement With Melody Valve in Infants and Children.

Background Pediatric patients with atrioventricular valve disease have limited options for prosthetic valve replacement in sizes <15 mm. Based on successful experience with the stented bovine jugular vein graft (Melody valve) in the right ventricular outflow tract, the prosthesis has been modified for surgical valve replacement in pediatric patients with atrioventricular dysfunction with the intention of subsequent valve expansion in the catheterization laboratory as the child grows. Methods and Results A multicenter, retrospective cohort study was performed among patients who underwent atrioventricular valve replacement with Melody valve at 17 participating sites from North America and Europe, including 68 patients with either mitral (n=59) or tricuspid (n=9) replacement at a median age of 8 months (range, 3 days to 13 years). The median size at implantation was 14 mm (range, 9-24 mm). Immediately postoperatively, the valve was competent with low gradients in all patients. Fifteen patients died; 3 patients underwent transplantation. Nineteen patients required reoperation for adverse outcomes, including valve explantation (n=16), left ventricular outflow tract obstruction (n=1), permanent pacemaker implantation (n=1), and paravalvular leak repair (n=1). Twenty-five patients underwent 41 episodes of catheter-based balloon expansion, exhibiting a significant decrease in median gradient ( P<0.001) with no significant increase in grade of regurgitation. Twelve months after implantation, cumulative incidence analysis indicated that 55% of the patients would be expected to be free from death, heart transplantation, structural valve deterioration, or valve replacement. Conclusions The Melody valve is a feasible option for surgical atrioventricular valve replacement in patients with hypoplastic annuli. The prosthesis shows acceptable short-term function and is amenable to catheter-based enlargement as the child grows. However, patients remain at risk for mortality and structural valve deterioration, despite adequate early valvular function. Device design and implantation techniques must be refined to reduce complications and extend durability. Clinical Trial Registration URL: https://www.clinicaltrials.gov. Unique identifier: NCT02505074.

Eliciting Narratives to Inform Care for Infants With Trisomy 18.

: media-1vid110.1542/5804913218001PEDS-VA_2018-0321Video Abstract.

Right ventricular energetics and power in pulmonary regurgitation vs. stenosis using four dimensional phase contrast magnetic resonance.

OBJECTIVE: We investigated a full energetic profile of pressure and volume loaded right ventricle (RV) in porcine models by evaluating kinetic energy (KE), stroke power, power output and power loss across pulmonary valves with stenosis (PS) or with regurgitation (PR). METHODS: Fifteen pigs (6 PS and 6 PR, 3 unoperated controls) were studied. Phase-contrast 4D-flow MRI was performed in models of PS and PR at baseline and at 10-12 weeks, in conjunction with cardiac catheterization. Phase contrast velocities over 1 cardiac cycle were registered with a dynamic mask of the RV segmented from cine images. Mean KE and KE curve profiles were measured, normalized for RV volumes and compared between groups. Right heart catheterization pressures were used to calculate RV stroke power and power output, from which pulmonary valve power loss and RV power output ratio were calculated, and compared between groups. RESULTS: PS and PR groups had similar KE pre procedure but significant changes in KE post procedure. The PR group had higher RV power output ratio and KE (72.1% ±â€¯11.4%; 20.6 ±â€¯6.1) than PS group (25.6% ±â€¯4.7%; 13.8 ±â€¯5.0) post procedure. Volume loaded RV from PR had higher KE and power output ratio compared to pressure load from PS. CONCLUSIONS: In porcine models of PS and PR, the RV presents altered systolic and diastolic energetic profiles. Pulmonary valve efficiency appeared to decrease in the medium term with somatic growth, with increased power loss in all groups studied, and greatly within the PS group.