Superior Cavopulmonary Connection Research Articles

Reprint of: How He Did It – The James S. Tweddell Approach to Single Ventricle Palliation in Patients With Hypoplastic Left Heart Syndrome

This article will detail Dr. James Tweddell's operative technique and considerations for 26 all three stages of SVP in patients with HLHS. This will include the Norwood Procedure with 27 Right-Ventricle-to-Pulmonary-Artery Conduit (Sano Modification), Bidirectional Glenn 28 (Superior Cavopulmonary Connection), and Extracardiac, Fenestrated Fontan Completion (Total 29 Cavopulmonary Connection). These techniques are the culmination of over 20 years of experience 30 and represent the final techniques he employed at both Children's Hospital of Wisconsin and 31 Cincinnati Children's Hospital Medical Center.

Gabapentin as a novel adjunct for postoperative irritability after superior cavopulmonary connection operation in children.

Describing our institution's off-label use of gabapentin to treat irritability after superior cavopulmonary connection surgery and its impact on subsequent opiate and benzodiazepine requirements. This is a single-center retrospective cohort study including infants who underwent superior cavopulmonary connection operation between 2011 and 2019. Gabapentin was administered in 74 subjects (74/323, 22.9%) during the observation period, with a median (IQR) starting dose of 5.7 (3.3, 15.0) mg/kg/day and a maximum dose of 10.7 (5.5, 23.4) mg/kg/day. Infants who underwent surgery in 2015-19 were more likely to receive gabapentin compared with those who underwent surgery in 2011-14 (p < 0.0001). Infants prescribed gabapentin were younger at surgery (137 versus 146 days, p = 0.007) and had longer chest tube durations (1.8 versus 0.9 days, p < 0.001), as well as longer postoperative intensive care (5.8 versus 3.1 days, p < 0.0001) and hospital (11.5 versus 7.0 days, p < 0.0001) lengths of stays. The year of surgery was the only predisposing factor associated with gabapentin administration in multivariate analysis. In adjusted linear regression, infants prescribed gabapentin on postoperative day 0-4 (n = 64) had reduced benzodiazepine exposure in the following 3 days (-0.29 mg/kg, 95% CI -0.52 - -0.06, p = 0.01) compared with those not prescribed gabapentin, while no difference was seen in opioid exposure (p = 0.59). Gabapentin was used with increasing frequency during the study period. There was a modest reduction in benzodiazepine requirements associated with gabapentin administration and no reduction in opioid requirements. A randomised controlled trial could better assess gabapentin's benefits postoperatively in children with congenital heart disease.

Textbook Outcome for Superior Cavopulmonary Connection: A Metric for Single Ventricle Heart Surgery.

Background: To develop a more holistic measure of congenital heart center performance beyond mortality, we created a composite "textbook outcome" (TO) for the Glenn operation. We hypothesized that meeting TO would have a positive prognostic and financial impact. Methods: This was a single center retrospective study of patients undergoing superior cavopulmonary connection (bidirectional Glenn or Kawashima ± concomitant procedures) from 2005 to 2021. Textbook outcome was defined as freedom from operative mortality, reintervention, 30-day readmission, extracorporeal membrane oxygenation, major thrombotic complication, length of stay (LOS) >75th percentile (17d), and mechanical ventilation duration >75th percentile (2d). Multivariable logistic regression and Cox proportional hazards modeling were used. Results: Fifty-one percent (137/269) of patients met TO. Common reasons for TO failure were prolonged LOS (78/132, 59%) and ventilator duration (67/132, 51%). In multivariable analysis, higher weight [odds ratio, OR: 1.44 (95% confidence interval, CI: 1.15-1.84), P = .002] was a positive predictor of TO achievement while right ventricular dominance [OR 0.47 (0.27-0.81), P = .007] and higher preoperative pulmonary vascular resistance [OR 0.58 (0.40-0.82), P = .003] were negative predictors. After controlling for preoperative factors and excluding operative mortalities, TO achievement was independently associated with a decreased risk of death over long-term follow-up [hazard ratio: 0.50 (0.25-0.99), P = .049]. Textbook outcome achievement was also associated with lower direct cost of care [$137,626 (59,333-167,523) vs $262,299 (114,200-358,844), P < .0001]. Conclusion: Achievement of the Glenn TO is associated with long-term survival and lower costs and can be predicted by certain risk factors. As outcomes continue to improve within congenital heart surgery, operative mortality will become a less informative metric. Textbook outcome analysis may represent a more balanced measure of a successful outcome.

Abstract 13267: Early Predictors of High-Risk Lymphatic Subtypes on T2-Weighted MRI in Patients After Superior Cavopulmonary Connection

Introduction: Lymphatic subtype as determined by T2-weighted MRI (T2-CMR) after superior cavopulmonary connection (SCPC) is known to predict clinical outcome after Fontan. Factors related to lymphatic subtype development are not well understood. This study aims to identify early predictors of high-risk lymphatic subtypes. Methods: Retrospective cohort study of consecutive patients with pre-Fontan T2-CMR from 2017 to 2021. Patients divided into two groups based on lymphatic subtype: Group 1 - subtypes 1, 2, and 3; Group 2 - subtypes 3+ (defined as type 3 with history of chylothorax) and 4. Pearson’s chi square, Fisher’s exact, Wilcoxon rank sum, univariate and multivariate logistic regression analysis were performed. Results: We identified 171 patients. The groups differed significantly in total hospital, ICU, and chest tube days, number of catheterizations and number of cardiac surgeries (Table 1). Moderate or greater atrioventricular valve regurgitation (Mod+ AVVR), increased catheterizations and increased chest tube days were associated with high-risk lymphatic subtype. Maintenance of antegrade pulmonary blood flow at the time of SCPC was not significant (Table 2). In multivariate analysis, Mod+ AVVR and number of chest tube days remained in the model after adjusting for other covariates. Overall model significance was 0.007 with an area under the curve of 0.777. Conclusions: Mod+ AVVR and increased chest tube days are predictive of high-risk lymphatic subtype. Further study is needed to determine if lymphatic subtype is acquired via clinical exposure or whether native lymphatic subtype may be predictive of clinical course even prior to cavopulmonary connection.

Combined ventricular dysfunction and atrioventricular valve regurgitation after the Norwood procedure are associated with attrition prior to superior cavopulmonary connection

BackgroundInfants with hypoplastic left heart syndrome (HLHS) or a variant are at risk of ventricular dysfunction (VD) and atrioventricular valve regurgitation (AVVR) prior to superior cavopulmonary connection (SCPC). Although the impact of these complications in isolation has been described, their effect in combination on attrition is poorly defined. MethodsA retrospective observational study of patients with HLHS or variants undergoing a Norwood procedure between 2008 and 2020 at a single center was performed. VD and AVVR were defined as moderate or severe when seen on 2 sequential echocardiograms outside the perioperative period. Attrition was defined as death, listing for heart transplant, or unsuitability for SCPC or transplant. Descriptive statistics and regression models were used for analysis. ResultsA total of 397 patients were included, of whom 75% had HLHS and 57% had received a Blalock-Thomas-Taussig shunt. Isolated VD occurred in 9% of patients, AVVR occurred in 13%, and both occurred in 6%. Attrition prior to SCPC occurred in 19% of the overall cohort, in 52% of patients with combined VD and AVVR (odds ratio [OR], 5.2; 95% confidence interval [CI], 2.3-12.0; P < .01), 26% of those with VD (OR, 1.5; 95% CI, 0.7-3.3; P = .32), 25% of those with AVVR (OR, 1.5; 95% CI, 0.7-2.9; P = .27), and 15% in those with neither (OR, 0.3; 95% CI, 0.2-0.6; P < .01). Other factors associated with attrition included prematurity, total bypass time at Norwood, and extracorporeal membrane oxygenation after Norwood, whereas later year of Norwood was protective (P < .01 for all). ConclusionsThe presence of combined VD and AVVR markedly increases the likelihood of attrition prior to SCPC, identifying a high-risk group.

Does leaving native antegrade pulmonary blood flow at the time of the superior cavopulmonary connection impact long-term outcomes after the Fontan?

ObjectivesAntegrade pulmonary blood flow (APBF) may be left or eliminated at the time of the superior cavopulmonary connection (SCPC). Our aim was to assess the impact of leaving native APBF at the SCPC on long-term Fontan outcomes. MethodsIn the Australia and New Zealand Fontan Registry (1985-2021), 587 patients had pre-existing native APBF at the SCPC. At the SCPC, 302 patients had APBF eliminated (APBF−) and 285 patients had APBF maintained (APBF+). The incidence of Fontan failure (composite end point of Fontan takedown, transplant, plastic bronchitis, protein losing enteropathy and death) and atrioventricular (AV) valve repair/replacement post SCPC was compared between the 2 groups. ResultsSex, predominant-ventricle morphology, isomerism, primary diagnosis, and age/type of Fontan were similar between groups. APBF− versus APBF+ had a higher incidence of arch obstruction/coarctation (17% vs 7%) and previous pulmonary artery band (54% vs 45%) and a lower rate of Fontan fenestration (27% vs 41%). The risk of Fontan failure was similar between the 2 groups (hazard ratio [HR], 1.01; 95% confidence interval [CI], 0.58-1.78; P = .96). The risk of AV-valve repair/replacement was greater in APBF+ versus APBF– (HR, 2.32; CI, 1.13-4.75; P = .022). The risk of AV-valve repair/replacement remained after adjustment for arch obstruction/coarctation, previous pulmonary artery band and Fontan fenestration (HR, 2.27; CI, 1.07-4.81; P = .033). ConclusionsMaintaining APBF at the time of the SCPC does not impact the risk of Fontan failure but may increase the incidence of AV-valve repair and/or replacement post-SCPC.

Association of Interstage Monitoring Era and Likelihood of Hemodynamic Compromise at Intervention for Recoarctation Following the Norwood Operation.

Background Intensive monitoring has been associated with a lower death rate between the Norwood operation and superior cavopulmonary connection, possibly due to early identification and effective treatment of residual anatomic lesions like recoarctation before lasting harm occurs. Methods and Results Neonates undergoing a Norwood operation and receiving interstage care at a single center between January 1, 2005, and September 18, 2020, were studied. In those with recoarctation, we evaluated association of era ([1] preinterstage monitoring, [2] a transitional phase, [3] current era) and likelihood of hemodynamic compromise (progression to moderate or greater ventricular dysfunction/atrioventricular valve regurgitation, initiation/escalation of vasoactive/respiratory support, cardiac arrest preceding catheterization, or interstage death with recoarctation on autopsy). We also analyzed whether era was associated with technical success of transcatheter recoarctation interventions, major adverse events, and transplant-free survival. A total of 483 subjects were studied, with 22% (n=106) treated for recoarctation during the interstage period. Number of catheterizations per Norwood increased (P=0.005) over the interstage eras, with no significant change in the proportion of subjects with recoarctation (P=0.36). In parallel, there was a lower likelihood of hemodynamic compromise in subjects with recoarctation that was not statistically significant (P=0.06), with a significant difference in the proportion with ventricular dysfunction at intervention (P=0.002). Rates of technical success, procedural major adverse events, and transplant-free survival did not differ (P>0.05). Conclusions Periods with interstage monitoring were associated with increased referral for catheterization but also reduced likelihood of ventricular dysfunction (and a suggestion of lower likelihood of hemodynamic compromise) in subjects with recoarctation. Further study is needed to guide optimal interstage care of this vulnerable population.

Impact of Ventricular Dysfunction and Atrioventricular Valve Regurgitation on Pre-Fontan Attrition

BackgroundThere is a significant incidence of pre-Fontan attrition—defined as failure to undergo Fontan completion—after superior cavopulmonary connection. This study investigated the impact of at least moderate ventricular dysfunction (VD) and atrioventricular valve regurgitation (AVVR) on pre-Fontan attrition. MethodsThis single-center retrospective cohort study included all infants who underwent Norwood palliation from 2008 to 2020 and subsequently underwent superior cavopulmonary connection. Pre-Fontan attrition was defined as death, listing for heart transplantation before Fontan completion, or unsuitability for Fontan completion. The study’s secondary outcome was transplant-free survival. ResultsPre-Fontan attrition occurred in 34 of 267 patients (12.7%). Isolated VD was not associated with attrition. However, patients with isolated AVVR had 5 times the odds of attrition (odds ratio, 5.4; 95% CI 1.8-16.2), and patients with both VD and AVVR had 20 times the odds of attrition (odds ratio, 20.1; 95% CI 7.7-52.8) compared with patients without VD or AVVR. Only patients with both VD and AVVR had significantly worse transplant-free survival compared with patients without VD or AVVR (hazard ratio, 7.7; 95% CI 2.8-21.6). ConclusionsThe additive effect of VD and AVVR is a powerful contributor to pre-Fontan attrition. Future research investigating therapies that can mitigate the degree of AVVR may help improve Fontan completion rates and long-term outcomes.

The so-called "one-and-a-half" ventricular repair: where are we after 40 years?

The indications, timing, and results of the so-called "one-and-a-half ventricle repair", as a surgical alternative to the creation of the Fontan circulation, or high-risk biventricular repair, currently remain nebulous. We aimed to clarify these issues. We reviewed a total of 201 investigations, assessing selection of candidates, the need for atrial septal fenestration, the fate of an unligated azygos vein and free pulmonary regurgitation, the concerns regarding reverse pulsatile flow in the superior caval vein, the growth potential and function of the subpulmonary ventricle, and the role of the superior cavopulmonary connections as an interstage procedure prior to biventricular repair, or as a salvage procedure. We also assessed subsequent eligibility for conversion to biventricular repair and long-term functional results. Reported operative mortalities ranged from 3% to 20%, depending on the era of surgical repair with 7% risk of complications due to a pulsatile superior caval vein, up to one-third incidence of supraventricular arrhythmias, and a small risk of anastomotic takedown of the superior cavopulmonary connection. Actuarial survival was between 80% and 90% at 10 years, with two-thirds of patients in good shape after 20 years. We found no reported instances of plastic bronchitis, protein-losing enteropathy, or hepatic cirrhosis. The so-called "one-and-a-half ventricular repair", which is better described as production of one-and-a-half circulations can be performed as a definitive palliative procedure with an acceptable risk similar to that of conversion to the Fontan circulation. The operation reduces the surgical risk for biventricular repair and reverses the Fontan paradox.

Lymphatic Abnormalities on Magnetic Resonance Imaging in Single-Ventricle Congenital Heart Defects Before Glenn Operation.

Background In the palliative pathway of single-ventricle physiology, lymphatic abnormalities on T2-weighted magnetic resonance imaging have been shown after the Glenn operation. It is believed that postsurgical hemodynamic changes contribute to the lymphatic changes.However, little is known about how early these abnormalities occur. Our purpose was to determine if lymphatic abnormalities occur as early as before the Glenn operation. Methods and Results We retrospectively reviewed patients with single-ventricle physiology and a T2-weighted magnetic resonance imaging scan before their Glenn operation (superior cavopulmonary connection) at The Children's Hospital of Philadelphia from 2012 to 2022. Lymphatic perfusion patterns on T2-magnetic resonance imaging were categorized from type 1 (no supraclavicular T2-signal) to type 4 (supraclavicular, mediastinal, lung parenchymal T2-signal). Types 1 and 2 were considered normal variants. Distribution of lymphatic abnormalities were tabulated, as well as secondary outcomes including chylothorax and mortality. Comparison was done using analysis of variance, Kruskal-Wallis test, and Fisher's exact test. Seventy-one children were included: 30 with hypoplastic left heart syndrome and 41 with nonhypoplastic left heart syndrome. Lymphatic abnormalities were present before Glenn operation in 21% (type 3) and 20% (type 4), and normal lymphatic perfusion patterns (type1-2) were seen in 59% of patients. Chylothorax was present in 17% (only types 3 and 4). Pre-Glenn mortality and mortality at any time was significantly increased when having a type 4 lymphatic abnormality compared with types 1 and 2 (P=0.04). Conclusions Lymphatic abnormalities can be found on T2-weighted magnetic resonance imaging in children with single-ventricle physiology before their Glenn operation. Mortality and chylothorax were more prevalent with advancing grade of lymphatic abnormality.

Novel Utilization of Ultrasound Enhancing Agents in Complex Congenital Heart Disease Following Superior Cavopulmonary Connection

Children with single-ventricle congenital heart disease typically undergo a superior cavopulmonary connection (SCPC) as the second stage in their surgical palliation. Postoperatively, stenoses of the SCPC and branch pulmonary arteries can occur. If there are clinical concerns and echocardiography is insufficient for diagnosis, patients undergo invasive evaluation with exposure to radiation and anesthesia. The use of ultrasound enhancing agents (UEAs) to improve echocardiographic diagnostic capabilities has not previously been described in this population. A single-center, retrospective case review was conducted of children who underwent echocardiography with UEA, following SCPC, from March 1, 2020, to April 15, 2022, at the Children's Hospital of Philadelphia. Twenty-two patients with hypoxemia or concern for obstruction following SCPC underwent UEA echocardiography. Extracted clinical data included patient demographics, echocardiographic images, angiography, surgical and transcatheter interventions, as well as available follow-up data. Six of the 22 UEA echocardiograms demonstrated stenosis or occlusion of either the SCPC or a pulmonary artery. All six patients underwent cardiac catheterization and five required intervention. Angioplasty was performed in each case with one requiring subsequent surgical revision. Sixteen of 22 UEA echocardiograms demonstrated no evidence of stenosis. Ten of these 16 patients improved, while six experienced persistent hypoxemia prompting referral for cardiac catheterization. Angiography confirmed the UEA echocardiographic findings (absence of stenosis) in four of these six patients. There were no adverse reactions related to UEA administration. Echocardiography with UEAs is a valuable and safe adjunctive imaging modality following SCPC, particularly when two-dimensional and color imaging is limited. This novel application of UEAs in complex patients with congenital heart disease provides an "angiogram-like" image, better delineating vessel walls and improving assessment of postoperative obstruction. As experience with UEAs increases in the congenital heart disease population, there may be opportunities to decrease invasive and costly procedures, while expediting the care of patients in need of intervention.

Abstract 13006: Association of Changes in Interstage Monitoring Practices and Transcatheter Intervention for Recurrent Coarctation Following the Norwood Operation

Introduction: Intensive interstage monitoring has been associated with lower mortality between the Norwood operation and superior cavopulmonary connection. This may be due to early identification of residual anatomic lesions like recoarctation before lasting harm occurs. Methods: Neonates undergoing a Norwood and followed interstage at a single center from 1/1/2005-9/18/2020 were studied. The exposure was Norwood date divided into 3 eras: 1. pre-interstage monitoring; 2. early era of monitoring; 3. current era. We evaluated the association of era and likelihood of hemodynamic insult (≥moderate ventricular dysfunction/AV valve regurgitation, initiation/escalation of vasoactive/respiratory support, cardiac arrest preceding cath, or interstage death with coarctation on autopsy) in subjects with recoarctation. We also analyzed whether era was associated with technical success in transcatheter recoarctation interventions, procedural major adverse events, and transplant-free survival. Results: 483 subjects were studied, with 22% (n=106) treated for interstage recoarctation. Number of catheterizations per Norwood increased ( p =0.005) with no significant change in the proportion of subjects with recoarctation ( p =0.36). There was a lower likelihood of hemodynamic insult in subjects with recoarctation after institution of interstage monitoring (63% Era 1, 38% Era 3), but this was not statistically significant ( p =0.06). The proportion of subjects with ventricular dysfunction at intervention decreased significantly ( p =0.002). Rates of technical success, procedural major adverse events, and transplant-free survival did not differ ( p &gt;0.05). Conclusions: Interstage monitoring was associated with increased referral for catheterization but was balanced by a decreased likelihood of hemodynamic insult in subjects with recoarctation. Future study is needed to optimize interstage monitoring strategies for early detection of recoarctation.

Reintervention for Superior Vena Cava Obstruction After Heart Transplant

BackgroundChildren undergoing orthotopic heart transplant (OHT) may require complex reconstruction of superior vena cava (SVC) anomalies. SVC anatomy and mode of reconstruction are potential risk factors for SVC obstruction. MethodsA retrospective single-center review was conducted of patients undergoing initial OHT between January 1, 1990, and July 1, 2021. Simple SVC anatomy included a single right SVC to the right atrium or bilateral SVCs with a left SVC to an intact coronary sinus, without prior superior cavopulmonary connection. Presence of anomalous SVC anatomy, superior cavopulmonary connection, or previous atrial switch operation defined complex anatomy. Reconstructive strategies included atrial anastomosis; direct SVC-to-SVC anastomosis; and augmented SVC anastomosis using innominate vein, patch, cavopulmonary connection, or interposition graft. The primary outcome was reintervention for SVC obstruction. ResultsOf 288 patients, pretransplant diagnoses included congenital heart disease (n = 155 [54%]), cardiomyopathy (n = 125 [43%]), and other (n = 8 [3%]). Most (n = 208 [72%]) had simple SVC anatomy compared with complex SVC anatomy (80 [28%]). Reintervention for SVC obstruction occurred in 15 of 80 (19%) with complex anatomy and 1 of 208 (0.5%) with simple anatomy (P = .0001). Reintervention was more common when innominate vein or a patch was used (9/25 [36%]) compared with an interposition graft (1/7 [14%]) or direct anastomosis (6/82 [7%]; χ2 = 13.1; P = .001). Most reinterventions occurred within 30 days of OHT (14/16 [88%]). ConclusionsPatients with complex SVC anatomy have a higher rate of reintervention for SVC obstruction after OHT compared with those with simple SVC anatomy. In cases of complex SVC anatomy, interposition grafts may be associated with less reintervention compared with complex reconstructions using donor tissue.

Analysis of inflammatory cytokines in the chest tube drainage of post-operative superior cavopulmonary connection patients.

Prolonged pleural effusions are common post Fontan operation and are associated with morbidity. Fontan pleural effusions have elevated proinflammatory cytokines. Little is known about the chest tube drainage after a superior cavopulmonary connection. We examined the chest tube drainage and the inflammatory profiles in post-operative superior cavopulmonary connection patients. This prospective cohort study enrolled 25 patients undergoing superior cavopulmonary connection and 10 age-similar controls. Data are also compared to 25 previously published Fontan patients and their 15 age-similar controls. Chest tube samples were analysed with a 17-cytokine BioPlex Assay. Descriptive statistics and univariate comparisons were made between groups. Duration of chest tube drainage was significantly shorter in superior cavopulmonary connection patients (median 4 days, [interquartile range 3-5 days]) versus Fontan patients (10 days, [7-11 days], p < 0.0001). Cytokine concentrations were higher on post-operative day 1 in superior cavopulmonary connection patients versus Fontan patients (all p ≤ 0.01), however levels were comparable to age-similar controls. While proinflammatory IL 8, MIP-1β, and TNF-α concentrations increased in chest tube drainage of Fontan patients from post-operative day 1 to last chest tube day (all p < 0.0001), there was no change in these biomarkers in superior cavopulmonary connection patients, their controls, or Fontan controls. Our study demonstrates that after superior cavopulmonary connection, proinflammatory cytokines in the chest tube drainage remain similar to biventricular controls of both age groups, unlike the significant rise over time observed in Fontan patients. Inflammation within the chest tube drainage is likely not innate to single ventricle patients.

Early superior cavopulmonary connection with preoperative computer tomography shows comparable outcomes for infants following Norwood palliation.

There is increasing evidence that performing superior cavopulmonary connection (SCPC) at 3 months reduces mortality: reducing the risky 'interstage' period, volume off-loading the ventricle and reducing coronary steal, hopefully preserving ventricular function. Our objective was to describe our experience of early SCPC with preoperative computer tomography (CT) assessment compared to later connection at 6 months. Patients undergoing Norwood procedure from 2005 to 2020 were divided into 2 eras were described and compared. Era 1 from 2005 to 2016 when SCPC was undertaken at 6 months: and era 2 (2017-2020) when an earlier operation was performed. Demographics, mortality (interstage, early and late following SCPC) and data on postoperative course and complications were recorded. In era 1, 191 patients underwent Norwood (120 survivors to SCPC) and 28 patients (23 survivors) in era 2. There were no significant differences in the demographics. Interstage mortality was 17.8% in era 1 and 8.0% in era 2 but not significantly significant (P = 0.22). The median (interquartile range) age at pre-imaging and SCPC was significantly lower: 99 (81-120) vs 77 (47-102) days and 175 (117-208) vs 106 (102-122) days in era 1 vs era 2 (P < 0.005). Weight was lower at SCPC in era 2 [mean (standard deviation) 6.2 kg (1.2) vs 5.1 kg (0.8), P < 0.05]. Intubation time and total length of stay were not statistically different. Median intensive care unit stay was statistically significantly longer, but not clinically significant: 2.5 (2-4) vs 3 (3-5) days, respectively (P < 0.05). There was no significant difference in early or late mortality, rates of diagnostic or interventional catheter, postoperative magnetic resonance imaging/CT or stroke. Logistic regression analysis demonstrates increasing age at SCPC was associated with increased chance of stroke or early death (P = 0.043). Early SCPC with CT assessment is feasible and although intensive care unit length of stay was slightly longer there was no change in the overall length of stay and no change in postoperative mortality or complications.

Is More Better or Is Less More?

In staged palliation for single-ventricle congenital heart disease, the debate over whether adding or maintaining an additional source of pulmonary blood flow (APBF) at the time of the superior cavopulmonary connection (SCPC) has waged for several decades. All studies addressing this topic had retrospective single-center designs with heterogeneous patient populations. These studies commonly use surgeon preference as the indication to add, maintain, or eliminate APBF at SCPC. In one metaanalysis, the methodology used for some of these analyses was judged to be poor. 1 Alghamdi A.A. Bidirectional Glenn with additional pulmonary blood flow: systematic review and evidence-based recommendations. J Card Surg. 2015; 30: 724-730 Crossref PubMed Scopus (5) Google Scholar Not surprisingly, the findings from this collective work have been inconsistent. For example, proponents of adding APBF to SCPC cite higher peripheral oxygen saturations (Spo2) after the SCPC, improved pulmonary artery (PA) growth during the interval between SCPC and Fontan completion, and intentionally delaying Fontan surgery to allow for somatic growth as benefits of this strategy. 2 Ferns S.J. El Zein C. Multani K. et al. Is Additional pulsatile pulmonary blood flow beneficial to patients with bidirectional Glenn?. J Thorac Cardiovasc Surg. 2013; 145: 451-454 Abstract Full Text Full Text PDF PubMed Scopus (35) Google Scholar ,3 Gray R.G. Altmann K. Mosca R.S. et al. Persistent antegrade pulmonary blood flow post-Glenn does not alter early post-Fontan outcomes in single-ventricle patients. Ann Thorac Surg. 2007; 84: 888-893 Abstract Full Text Full Text PDF PubMed Scopus (40) Google Scholar However, the oxygen saturation difference between patients with and without APBF at SCPC is typically clinically modest and may become indistinguishable before the Fontan procedure. Data to show that any measured increase in PA size with APBF is sustained after the Fontan procedure or improves Fontan outcomes in the long term are lacking. Moreover, any extended time for somatic growth between SCPC and the Fontan operation in the patients with APBF would a priori allow for PA growth as well. Therefore, the link between preserved APBF at SCPC and durable benefit or sustained improved outcomes is weak on the basis of available information. Influence of Antegrade Pulmonary Blood Flow on Outcomes of Superior Cavopulmonary ConnectionThe Annals of Thoracic SurgeryVol. 114Issue 5PreviewWe sought to characterize short- and long-term outcomes after superior cavopulmonary connection (SCPC) in children eligible for inclusion of antegrade pulmonary blood flow (APBF) in the SCPC circuit, exploring whether maintaining APBF was associated with outcomes. Full-Text PDF

Influence of Antegrade Pulmonary Blood Flow on Outcomes of Superior Cavopulmonary Connection

BackgroundWe sought to characterize short- and long-term outcomes after superior cavopulmonary connection (SCPC) in children eligible for inclusion of antegrade pulmonary blood flow (APBF) in the SCPC circuit, exploring whether maintaining APBF was associated with outcomes. MethodsThis was a retrospective cohort study of patients with single-ventricle heart disease and APBF who underwent SCPC at our center between January 1, 2000, and September 30, 2017. Patients were divided into 2 groups: APBF eliminated (APBF−), and APBF maintained (APBF+) at the time of SCPC. ResultsOf 149 patients, 108 (72.5%) were in APBF− and 41 (27.5%) were in APBF+. Of those in APBF+, 5 (12.2%) subsequently had APBF eliminated after SCPC. Patients in APBF+ had a higher prevalence of chest tube duration >10 days and underwent more interventions during the post-SCPC hospitalization (1.9% vs 12%; P = .008 for both) but had shorter surgical support times at SCPC (P < .0001). There were no differences in post-SCPC intensive care unit or hospital length of stay. During the study period, 82 patients (76%) in APBF− and 22 patients (54%) in APBF+ underwent Fontan completion. Patients in APBF+ had a greater weight gain from SCPC to Fontan (6.7 [1.8-22] kg vs 8.15 [4.4-20.6] kg; P = .012) and a shorter hospital length of stay after Fontan (9 [4-107] days vs 7.5 [4-14] days; P = .044). ConclusionsShort-term morbidity associated with maintaining APBF at the time of SCPC is modest, but longer term outcomes suggest potential benefits in those in whom APBF can be successfully maintained.

Relationship Between Serum Brain-Type Natriuretic Peptide and Biomarkers of Growth in Infants With Shunt-Dependent Single Cardiac Ventricle

For infants with shunt-dependent or ductal-dependent single ventricle heart disease, poor growth is common and associated with morbidity and impaired neurodevelopmental outcomes. Although attention has focused on nutrition to promote weight gain, little is known about the relation between heart failure and growth factors. A prospective observational pilot study was performed to assess the relation between heart failure, assessed by brain natriuretic peptide (BNP), and growth factors (insulin-like growth factor 1 [IGF-1] and insulin-like growth factor-binding protein 3) at 3 visits: (1) before discharge from neonatal intervention with the establishment of stable pulmonary blood flow, (2) immediately before superior cavopulmonary connection, and (3) before discharge after superior cavopulmonary connection operation. The relation between BNP and growth factors was analyzed using Spearman pairwise correlations at each visit and modeled over time with a linear mixed-effects model. Correlations were considered worthy of further exploration using a p <0.10, given the exploratory nature of the study. The study included 38 infants (66% male, 68% hypoplastic left heart syndrome). Median BNP was elevated at visit 1 and decreased over time (287pg/dl [interquartile range 147 to 794], 85pg/dl [52 to 183], and 90pg/dl [70 to 138]). Median IGF-1 Zscore was <0 at each visit but increased over time (-0.9 [interquartile range -1.1 to 0.1], -0.7 [-1.2 to 0.1], and -0.5 [-1.2 to 0]). Inverse correlations were found between BNP and IGF-1 at visit 1 (r=-0.40, p=0.097), BNP and IGF-1 and insulin-like growth factor-binding protein 3 at visit 2 (r=-0.33, p=0.080 and r=-0.33, p=0.085, respectively) and BNP and IGF-1 Zscore at visit 3 (r=-0.42, p=0.049). Significant relations were likewise found between the change in BNP and the change in IGF-1 between visits 1 and 3 (p=0.046) and between visits 2 and 3 (p=0.048). In conclusion, this pilot study demonstrates an inverse correlation between BNP and growth factors, suggesting that the heart failure state associated with this physiology may play a mechanistic role in impaired growth.

Characterization of Inhaled Nitric Oxide Use for Cardiac Indications in Pediatric Patients.

Characterize the use of inhaled nitric oxide (iNO) for pediatric cardiac patients and assess the relationship between patient characteristics before iNO initiation and outcomes following cardiac surgery. Observational cohort study. PICU and cardiac ICUs in seven Collaborative Pediatric Critical Care Research Network hospitals. Consecutive patients, less than 18 years old, mechanically ventilated before or within 24 hours of iNO initiation. iNO was started for a cardiac indication and excluded newborns with congenital diaphragmatic hernia, meconium aspiration syndrome, and persistent pulmonary hypertension, or when iNO started at an outside institution. None. Four-hundred seven patients with iNO initiation based on cardiac dysfunction. Cardiac dysfunction patients were administered iNO for a median of 4 days (2-7 d). There was significant morbidity with 51 of 407 (13%) requiring extracorporeal membrane oxygenation and 27 of 407 (7%) requiring renal replacement therapy after iNO initiation, and a 28-day mortality of 46 of 407 (11%). Of the 366 (90%) survivors, 64 of 366 patients (17%) had new morbidity as assessed by Functional Status Scale. Among the postoperative cardiac surgical group (n = 301), 37 of 301 (12%) had a superior cavopulmonary connection and nine of 301 (3%) had a Fontan procedure. Based on echocardiographic variables prior to iNO (n = 160) in the postoperative surgical group, right ventricle dysfunction was associated with 28-day and hospital mortalities (both, p < 0.001) and ventilator-free days (p = 0.003); tricuspid valve regurgitation was only associated with ventilator-free days (p < 0.001), whereas pulmonary hypertension was not associated with mortality or ventilator-free days. Pediatric patients in whom iNO was initiated for a cardiac indication had a high mortality rate and significant morbidity. Right ventricular dysfunction, but not the presence of pulmonary hypertension on echocardiogram, was associated with ventilator-free days and mortality.

Variation in Advanced Diagnostic Imaging Practice Patterns and Associated Risks Prior to Superior Cavopulmonary Connection: A Multicenter Analysis.

Single ventricle patients typically undergo some form of advanced diagnostic imaging prior to superior cavopulmonary connection (SCPC). We sought to evaluate variability of diagnostic practice and associated comprehensive risk. A retrospective evaluation across 4 institutions was performed (1/1/2010-9/30/2016) comparing the primary modalities of cardiac catheterization (CC), cardiac magnetic resonance (CMR), and cardiac computed tomography (CT). Associated risks included anesthesia/sedation, vascular access, total room time, contrast agent usage, radiation exposure, and adverse events (AEs). Of 617 patients undergoing SCPC, 409 (66%) underwent at least one advanced diagnostic imaging study in the 60days prior to surgery. Seventy-eight of these patients (13%) were analyzed separately because of a concomitant cardiac intervention during CC. Of 331 (54%) with advanced imaging and without catheterization intervention, diagnostic CC was most common (59%), followed by CT (27%) and CMR (14%). Primary modality varied significantly by institution (p < 0.001). Median time between imaging and SCPC was 13days (IQR 3-33). Anesthesia/sedation varied significantly (p < 0.001). Pre-procedural vascular access did not vary significantly across modalities (p = 0.111); procedural access varied between CMR/CT and CC, in which central access was used in all procedures. Effective radiation dose was significantly higher for CC than CT (p < 0.001). AE rate varied significantly, with 12% CC, 6% CMR, and 1% CT (p = 0.004). There is significant practice variability in the use of advanced diagnostic imaging prior to SCPC, with important differences in associated procedural risk. Future studies to identify differences in diagnostic accuracy and long-term outcomes are warranted to optimize diagnostic protocols.